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Essentials of Pediatric Oral Pathology

Q: How does the role of early environmental exposure influence the progression and risk of dental caries in children, according to the document?

Early environmental exposure can significantly influence the risk and progression of dental caries in children. This is largely due to dietary habits and the introduction of foods rich in fermentable carbohydrates like sucrose, which can increase caries risk by promoting bacterial growth that leads to tooth decay . Studies show that frequent sugar consumption between meals poses a greater caries risk compared to sugar consumed primarily at mealtime . Furthermore, early exposure to cariogenic foods such as infant milk formulas and fruit juices, which contain added sugars, can promote early childhood caries, a virulent form of caries affecting infants and young children . These dietary exposures during crucial developmental periods without adequate oral hygiene can lead to more rapid and severe progression of dental caries in children ."}

Q: What are the recommended treatments and monitoring procedures for managing odontogenic myxomas, and how do they differ for small versus large lesions?

Odontogenic myxomas, benign but potentially aggressive tumors, necessitate different treatment approaches based on size. Small lesions are typically managed through conservative curettage, whereas larger ones may require extensive surgical resection due to their potential for significant bone expansion and destruction. Monitoring includes regular radiographic evaluations to assess lesion boundaries and post-surgical recurrence, given their infiltrative nature and association with unerupted teeth .

Q: Analyze the relationship between anatomical changes and their functional implications in patients with Treacher Collins syndrome.

In Treacher Collins syndrome, anatomical changes include hypoplastic zygomas and supraorbital rims, malformed pinnae, and mandibular retrognathism. These lead to significant functional implications, such as airway obstruction due to hypoplasia of pharyngeal structures, causing respiratory issues requiring tracheostomy. Malformed ear structures often result in conductive hearing loss, necessitating hearing aids. Additionally, severe facial anomalies complicate feeding and speech due to mechanical obstructions from cleft palate and mandibular features .

Q: Discuss the epidemiological patterns and factors contributing to the dichotomy in dental caries prevalence among children in developed countries.

In developed countries, dental caries exhibits a dichotomous epidemiological pattern, where 50% of children are caries-free, while 20% account for 75% of the lesions. This suggests a significant disparity in caries risk, which is influenced by environmental characteristics, socio-economic factors, and access to preventive care. The decrease in overall prevalence can be attributed to improved dietary habits, fluoride use, and dental hygiene, whereas the persistently high-risk group might face challenges like poorer access to dental care and lower socio-economic conditions .

Q: What treatment protocols and management strategies are suggested for children with Pierre Robin sequence facing severe respiratory and feeding difficulties at birth?

For severe respiratory obstruction due to Pierre Robin sequence, management includes using a nasopharyngeal airway or intubation at birth . Feeding difficulties, exacerbated by the cleft palate, require nipples with large holes or specialized feeding techniques to ensure adequate nutrition . A multidisciplinary team, including pediatricians, surgeons, and therapists, addresses comprehensive care and surgical interventions, such as single-stage palate closure and possibly mandibular lengthening for obstructive apnea due to mandibular hypoplasia .

Q: What are the therapeutic objectives and indications of direct pulp capping using calcium hydroxide for maintaining pulp vitality?

The objectives of direct pulp capping are to seal the pulp against bacterial leakage and stimulate dentin bridge formation to maintain pulp vitality. Indications include scenarios of mechanical pulp exposure during caries excavation or traumatic injury, provided the site is dry and sterile. The exposure should be small without significant hemorrhage, and the surrounding dentin should show no signs of degenerative changes .

Q: Explain the diagnostic advantages and limitations of using electrical resistance in detecting dental caries, especially early pit and fissure lesions.

Electrical resistance measures tooth demineralization by assessing increased conductivity in demineralized areas, making it effective for early pit and fissure caries detection. Advantages include monitoring caries progress and detecting shallow dentinal lesions, potentially preventing operative treatment. However, it cannot distinguish between developmental hypomineralization and caries, and the presence of enamel cracks can result in false positives. Additionally, it is time-consuming as separate measurements for each site are needed .

Q: What are the clinical and pathophysiological distinctions between reversible and acute pulpitis, particularly in the context of their histopathologic features?

Reversible pulpitis is characterized by the presence of high vascularity and minimal inflammation. Histopathologically, it shows acute extravasation of RBCs, some diapedesis of WBCs, and engorged pulpal vessels with small hemorrhages. The dentinoblastic layer is partially disrupted, but significant necrosis isn't typically seen . Acute pulpitis, on the other hand, involves more extensive inflammation with vasodilatation, fluid exudation, and leukocyte infiltration. There can be localized pulp tissue destruction and abscess formation . Inflammation in acute pulpitis is often more severe and persists despite removal of stimuli, accompanied by more persistent and severe pain .

Q: What are the primary genetic factors and their roles associated with the development of Treacher Collins syndrome, and how do these factors manifest in the condition's symptoms?

Treacher Collins syndrome is primarily caused by mutations in the TCOF1 gene, which is located on chromosome 5q31.3-33.3 and encodes the treacle protein necessary for normal craniofacial development. These mutations lead to a premature termination of the treacle protein, affecting the growth of craniofacial structures derived from the first and second pharyngeal arches . As a consequence, individuals with Treacher Collins syndrome exhibit distinctive facial dysmorphisms, such as downward-sloping palpebral fissures, malformed ears, hypoplastic zygomas, and a receding chin . Additionally, some patients may have a cleft palate and conductive hearing loss due to these developmental anomalies . The inheritance pattern is autosomal dominant with variable expressivity and complete penetrance in most cases, although around 60% of incidents arise from fresh mutations . The craniofacial abnormalities may lead to difficulties with feeding and hearing, necessitating early medical and surgical interventions .

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Contents i

Essentials of
PEDIATRIC ORAL PATHOLOGY
Essentials of
PEDIATRIC ORAL PATHOLOGY

Mayur Chaudhary
MDS (Oral Pathology and Microbiology)
Senior Lecturer
Department of Oral Maxillofacial Pathology
SMBT Dental College and Hospital
Sangamner, Maharashtra, India

Shweta Dixit Chaudhary

MDS (Pedodontics and Preventive Dentistry)
Senior Lecturer
Department of Pedodontics and Preventive Dentistry
SMBT Dental College and Hospital
Sangamner, Maharashtra, India

Forewords
Asha Singh
Minal Chaudhary

JAYPEE BROTHERS MEDICAL PUBLISHERS (P) LTD

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Published by
Jaypee Brothers Medical Publishers (P) Ltd
Corporate Office
4838/24 Ansari Road, Daryaganj, New Delhi - 110002, India
Phone: +91-11-43574357, Fax: +91-11-43574314
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Essentials of Pediatric Oral Pathology

All rights reserved. No part of this publication should be reproduced, stored in a retrieval system, or transmitted in any form or by any
means: electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of the editors and the publisher.

This book has been published in good faith that the material provided by the contributors is original. Every effort is made to ensure
accuracy of material, but the publisher, printer and editors will not be held responsible for any inadvertent error (s). In case of any
dispute, all legal matters are to be settled under Delhi jurisdiction only.

First Edition: 2011

ISBN 978-93-5025-374-8
Typeset at JPBMP typesetting unit
Printed in India
Dedicated to
our beloved parents
Shri Bhasker Chaudhary and Smt Maya Chaudhary
Shri Shivendra Dutt Dixit and Smt Manju Dixit
and our dearest daughter
Tvisha Chaudhary
whose constant support and encouragement
have been a guiding light for us.
Contributors

Amol Gulhane MBBS Mayur Chaudhary MDS

General Practitioner Senior Lecturer
Government Medical College Department of Oral Maxillofacial Pathology
Nagpur, Maharashtra, India SMBT Dental College and Hospital
Sangamner, Maharashtra, India
Anuraag B Chaudhary MDS
Prashant Dixit MD (Pediatrics)
Postgraduate Student
Intensivist and Neonatologist
Department of Oral Medicine
Diagnosis and Radiology Mumbai, Maharashtra, India
Sharad Pawar Dental College and Hospital Ragini Gulhane MBBS
Sawangi, Wardha, Maharashtra, India General Practitioner
Government Medical College
Gauri R Thakre (Chaudhary) MDS Nagpur, Maharashtra, India
Senior Lecturer
Department of Oral Pathology and Microbiology Sanket Kunte MDS
Swargiya Dadasaheb Kalmegh Smruti Senior Lecturer
Dental College and Hospital Department of Pedodontics and Preventive Dentistry
Nagpur, Maharashtra, India Bharti Vidyapeeth
Pune, Maharashtra, India
Iqbal Musani MDS
Shweta Dixit Chaudhary MDS
Professor
Senior Lecturer
Department of Pedodontics and Preventive Dentistry
Department of Pedodontics and Preventive Dentistry
Bharti Vidyapeeth SMBT Dental College and Hospital
Pune, Maharashtra, India Sangamner, Maharashtra, India

Manasi Dixit MPT Syed Ahmed Taqi MDS

Postgraduate Student Senior Lecturer
Pediatric Physiotherapy Department of Oral Pathology and Microbiology
Dr DY Patil College of Physiotherapy Government Dental College
Pune, Maharashtra, India Mumbai, Maharashtra, India
Foreword

I would like to heartily congratulate Drs Mayur Chaudhary and Shweta Dixit Chaudhary for their timely
recognition of the need for a piece of literature dedicated to Essentials of Pediatric Oral Pathology.
This book is the first of its kind and I have felt paucity of a book of this nature while teaching my
postgraduate students. The inspiration to write such a book probably stems from one of the countless
discussions that I have had with my postgraduate student Shweta Dixit.
The book consists of fifteen chapters including all the common oral pathologies in children. The
chapter on Caries includes pathology, prevention and treatment aspects of caries in detail.
I am extremely proud to be a part of the pioneer book on the subject of pediatric oral pathology, and
I am sure that the hard work, dedication and sincerity of the authors will be all and transparent to any student reading through
this book. I wish the authors all success in this brilliant endeavor of theirs.

Asha Singh
MDS (Pedodontics and Preventive Dentistry)
Senior Professor
MGM Dental College
Nasik, Maharashtra, India
Foreword
It is only in the past couple of decades that pediatric dentistry has come of age. There is an increasing
awareness in the minds of parents regarding the importance of deciduous dentition and its associated disease
conditions. This has led to an increase in the number of patients in the pediatric age-group seeking treatment.
The distinct environments and requirements of the child patient create a necessity for a book exclusively
based on pediatric oral pathology. Thus, there is a definite need for a separate book dealing specifically
with the diagnosis and treatment of diseases of the young.
Essentials of Pediatric Oral Pathology by Drs Mayur Chaudhary and Shweta Dixit Chaudhary caters
to this particular need. It is an easy-to-refer book with elaborate illustrations, latest treatment modalities as
well as lucid elaboration of clinical features.
The authors have also included a separate chapter on Pediatric Forensic Odontology, which should be of great help to the
reader. I am sure that this well-written book will be of immense help to teachers and students alike.

Minal Chaudhary MDS

Professor and Head
Department of Oral Pathology and Microbiology
Sharad Pawar Dental College
Sawangi, Wardha, Maharashtra, India
Preface

Pediatric dentistry is amongst the youngest of the branches of dentistry, yet has grown by leaps and bounds to command one of
the highest summits in dentistry. This book is a minuscule contribution to this effort.
It has been our dedicated and sincere effort to work within the limits of this book and concentrate on the more relevant
topics as far as possible. The topics are covered on the basis of the frequency at which a particular lesion is seen in the pediatric
population.
Numerous pathological entities including the commonly occurring ones, e.g. dental caries, infectious diseases, etc. and
some rare entities, e.g. salivary glands, bone, skin, etc. have been explained in a simplified manner in context with children.
A chapter on Forensic Odontology in Children has been included which also deals with the currently relevant topics of
child abuse and neglect. Each chapter has been provided with adequate and to-the-point references for greater exploration of
the topic by young enthusiastic minds.
To distinguish between normal and pathology, one must be well-versed with the physiology. Keeping this fact in mind,
comprehensive appendices defining the normal values of various aspects in children have been included.
We hope that this piece of work occupies the vacant niche for a comprehensive book on Pediatric Oral Pathology.

To err is human, to forgive is divine

With these words, we wish to admit that no one is ever perfect just as we are not. There might be some shortcomings despite
our most sincere efforts. The reader’s suggestions for further improvements shall be greatly encouraged and acknowledged.

Mayur Chaudhary
Shweta Dixit Chaudhary
Acknowledgments

We stand with our heads bowed to the almighty God for giving us the opportunity to undertake the writing of this book and
carry to its successful completion.
The writing of a book is an arduous undertaking and is impossible without the timely and sequential involvement of an
entire team. We wish to express our sincere gratitude to all the contributors whose inputs have added character to the book.
Our respected teachers Dr Asha Singh, Dr Meena Kulkarni and Dr Rajeev Desai have provided us the basic understanding
and knowledge of the subject and have always stood us in good stead.
The skilled technicians, artists, reviewers and the entire panel of M/s Jaypee Brothers Medical Publishers (P) Ltd deserves
a standing ovation for their tireless efforts in designing and refining the vast host of minute details in the book.
A special thanks to Shri Vijay Gulhane and Shri Vipin Sharma who have been a reassuring buttress in all our times of doubt
and hesitation.
We sincerely wish to acknowledge the overwhelming support provided to us by the staff and students, especially
Dr Ashok Patil, Dean, SMBT Dental College and Hospital, Sangamner, Maharashtra, India and Dr Anil Ghom, Professor
and Head, Department of Oral Medicine and Radiology, Chhattisgarh Dental College and Research Institute, Rajnandgaon,
Chhattisgarh, India.
Thanks to all those who have directly or indirectly helped us in this venture and last but not least, we would like to thank
our parents and family members for their ever-extended arms of support.
Contents
1. Developmental Disturbances in Children ........ 1 • Chronic pulpalgia (subacute pulpitis) 136
Mayur Chaudhary, Shweta Dixit Chaudhary, • Acute pulpitis with apical periodontitis 137
Prashant Dixit • Nonpainful pulpitis 137
• Pulpal granuloma 137
• Developmental disturbances of jaws 2
• Chronic hyperplastic pulpitis 138
• Developmental disturbances of lips and palate 3
• Irreversible pulpitis 138
• Developmental disturbances of the oral mucosa 17
• Pulp degeneration 139
• Developmental disturbances of the gingiva 18
• Pulp calcification 139
• Developmental disturbances of the tongue 18
• Reticular atrophy of pulp 140
• Developmental disturbances of oral lymphoid
• Pulp necrosis 140
tissue 25
• Developmental disturbances of the salivary glands 25
4. Sequelae of Pulp Pathologies in Children ... 142
• Developmental disturbances affecting the teeth 25
Shweta Dixit Chaudhary, Mayur Chaudhary
• Developmental defects in size of teeth 25
• Developmental defects in shape of teeth 26 • Classification of diseases of periradicular
• Developmental defects in number of teeth 31 tissues 142
• Developmental defects in structure of teeth 36 • Acute alveolar abscess 142
• Defects of growth (eruption) of teeth 45 • Parulis 143
• Fissural cysts of the oral region 49 • Acute apical periodontitis 144
• Acute exacerbation of chronic lesion 144
2. Caries in Children ........................................... 55 • Chronic alveolar abscess 146
Shweta Dixit Chaudhary, Mayur Chaudhary, Iqbal Musani, • Granuloma 147
Sanket Kunte, Gauri R Thakre (Chaudhary) • Apical periodontal cyst 148
• External root resorption 150
• Definition 55
• Osteomyelitis 151
• History 56
• Cellulitis (Phlegmon) 154
• Trends in caries epidemiology 56
• Intracranial complication of dental infections 155
• Early theories of dental caries 56
• Cavernous sinus thrombosis/thrombophlebitis 155
• Current concepts in caries etiology 61
• Focal infection 156
• Classification of dental caries 70
• Histopathology of dental caries 71 5. Gingival and Periodontal Diseases in
• Early childhood caries 74
Children ......................................................... 158
• Caries susceptibility and caries activity 76
Shweta Dixit Chaudhary, Mayur Chaudhary
• Caries activity tests 76
• Diagnosis of dental caries 79 • Prevalence of gingivitis in children 158
• Caries risk assessment 88 • Classification of periodontal diseases and
• Prevention of caries 89 conditions 158
• Management of caries 89 • Gingiva 158
• Allergy and gingival inflammation 161
3. Pulp Pathologies in Children ....................... 124 • Acute gingival disease 161
Shweta Dixit Chaudhary, Mayur Chaudhary • Recurrent aphthous ulcer 163
• Need to learn pulp pathology 125 • Chronic nonspecific gingivitis 167
• Concept of pain 125 • Conditioned gingival enlargement 168
• Behavior of dental pain 128 • Periodontal diseases in children 171
• Classification of pulpal pathoses 130 • Gingival recession 176
• Causes of pulp pathology 130 • Abnormal frenum attachment 177
• Pulpal reaction to an insult 131
• Reversible pulpitis 134 6. Cysts in the Pediatric Population ................ 179
• Hypersensitivity 134 Mayur Chaudhary, Shweta Dixit Chaudhary
• Focal reversible pulpitis/pulp hyperemia 135 • Definition 179
• Acute pulpitis 136 • Classification 179
xviii Essentials of Pediatric Oral Pathology

• Odontogenic keratocyst 179 • Hemangioma 240

• Dentigerous cyst (follicular cyst) 183 • Nasopharyngeal angiofibroma 244
• Eruption cyst 184 • Lymphangioma 245
• Gingival cyst of infant (GCOI) and midpalatal • Rhabdomyoma 246
raphae cyst (MPRC) 184 • Fibrosarcoma 247
• Radicular cyst (periapical cyst) 185 • Osteosarcoma 247
• Residual cyst 188 • Rhabdomyosarcoma 249
• Solitary bone cyst (SBC) (traumatic simple • Synovial sarcoma 250
hemorrhagic bone cyst) 188 • Alveolar soft part sarcoma 251
• Aneurysmal bone cyst (ABC) 189
• Calcifying odontogenic cyst 190 10. Bone Pathology in Children ......................... 253
• Dermoid cyst 191 Mayur Chaudhary, Shweta Dixit Chaudhary,
• Epidermoid cyst (epidermal inclusion cyst) 191 Manasi Dixit
• Teratoid cyst 192 • Osteogenesis imperfecta 253
• Thyroglossal tract cyst 192 • Osteopetrosis 255
• Intraoral lymphoepithelial cyst 193 • Cleidocranial dysplasia 257
• Lingual cyst of foregut origin 194 • Central giant cell granuloma 258
• Cystic hygroma 194 • Cherubism 259
• Parasitic cysts 194 • Fibrous dysplasia 261
• Cysts of salivary glands 195 • Monostotic fibrous dysplasia 261
• Polyostotic fibrous dysplasia 261
7. Odontogenic Tumors in Children ................... 200 • Chondromyxoid fibroma 262
• Familial gigantiform cementoma 263
Mayur Chaudhary, Shweta Dixit Chaudhary, • Juvenile ossifying fibroma 263
Anuraag B Chaudhary • Marfan syndrome 263
• Difference between a tumor and a hamartoma 200 • Achondrogenesis 265
• Difference between WHO classification and the • Chondrodysplasia punctata 266
currently accepted classification 201 • Pycnodysostosis 267
• Benign 201 • Mucopolysaccharidosis 267
• Solid/multicystic 203 • Rickets 269
• Peripheral ameloblastoma 206 • Hyperparathyroidism 271
• Odontogenic epithelium with odontogenic ectome ... • Hypoparathyroidism 272
• Craniosynostosis syndromes 273
senchyme with or without dental hard
• Craniofacial dysostosis – Crouzon syndrome 275
tissue formation 212
• Mandibulofacial dysostosis – Treacher-Collins-
• Mesenchyme and/or odontogenic ectomesenchyme
Franceschetti syndrome 276
with or without included odontogenic • Pierre-Robbin syndrome 278
epithelium 216 • Apert syndrome 279
• Malignant 218 • Thanatophoric dysplasia 283
• Achondroplasia 284
8. Epithelial Pathology in Children .................. 224 • Robinow syndrome 285
Mayur Chaudhary, Shweta Dixit Chaudhary • Hyperostosis corticalis generalisata 286
• Squamous papilloma 224 • Chondroectodermal dysplasia 286
• Verruca vulgaris (common wart) 225 • Tricho-dento-osseous syndrome 288
• Condyloma acuminatum 226 • Down syndrome 288
• Focal epithelial hyperplasia (Heck’s disease) 226 • Infantile cortical hyperostosis 290
• Ephelis 227 • Massive osteolysis (Gorham’s disease) 291
• Lentigo simplex 228 • Cementoblastoma 291
• Pigmented nevi 228 • TMJ abnormalities 292
• Langerhans cell histiocytosis 293
• Oral submucous fibrosis 230
• Hand-Schüller-Christian disease 295
9. Connective Tissue Pathology in Children ... 235 • Eosinophilic granuloma 296
Mayur Chaudhary, Shweta Dixit Chaudhary 11. Salivary Gland Lesions in Children ............. 299
• Myofibroma 235 Mayur Chaudhary, Shweta Dixit Chaudhary
• Pyogenic granuloma 236 • Developmental disturbances of salivary glands in
• Peripheral ossifying fibroma 237 children 301
• Melanotic neuroectodermal tumor of infancy (MNTI) 238 • Aplasia 301
• Congenital epulis of newborn 239
Contents xix

• Xerostomia 301 • Recurrent HSV infection 353

• Hyperplasia of palatal salivary glands 303 • Herpes zoster 353
• Developmental lingual salivary gland • Smallpox 354
depression 304 • Measles (rubeola) 356
• Heterotropic salivary glands 305 • Rubella (German measles) 356
• Heterotropic salivary gland tissue of the • Herpangina 357
lower neck 305 • Cytomegalovirus infection 358
• Heterotropic salivary gland tissue of the middle ear 306 • Acquired immunodeficiency syndrome (AIDS) 361
• Intraosseous heterotropic salivary gland tissue 306 • Replication cycle of virus 363
• Heterotropic salivary gland tissue of other sites 306
• Accessory parotid glands 306 14. Hematological Disorders in Children ........... 373
• Adenomatoid hyperplasia of mucous glands 307 Shweta Dixit Chaudhary, Mayur Chaudhary,
• Polycystic (dysgenetic) disease of the Prashant Dixit
parotid glands 307 • Classification 373
• Sialadenitis 308 • Anemia 373
• Sarcoidosis 309 • Folic acid deficiency anemia 374
• Sialolithiasis 310 • Pernicious anemia and vitamin B12 deficiency 376
• Benign neoplasms 312 • Aplastic anemia 377
• Malignant neoplasms 313 • Sickle cell anemia 378
• Mucoepidermoid carcinoma 313 • Thalassemia 381
• Acinic cell adenocarcinoma 315 • Hemophilia 383
• Polycythemia 386
12. Skin Lesions in Children .............................. 318 • Leukocytosis 389
Mayur Chaudhary, Shweta Dixit Chaudhary, • Neutrophilia 389
Amol Gulhane • Eosinophilia 390
• Ectodermal dysplasia 318 • Monocytosis 390
• Dyskeratosis congenita 321 • Basophilia 390
• Incontinentia pigmenti 322 • Agranulocytosis 390
• Pachyonychia congenita 324 • Neutropenia 391
• Ehlers-Danlos syndrome 325 • Lymphocytosis 392
• Osler-Weber-Rendu syndrome 326 • Leukemia 392
• Peutz-Jeghers syndrome 328 • Acute leukemias 393
• White sponge nevus 329 • Acute myeloblastic leukemia 393
• Epidermolysis bullosa 330 • Acute lymphoblastic leukemia 393
• Pemphigus 332 • Disorders of hemostasis 398
• Pemphigus foliaceous 333 • Thrombocytopenia 398
• Pemphigus vegetans 334 • Lymphoma 400
• Pemphigus erythematosus (PE) 334 • Hodgkin’s disease 401
• Paraneoplastic pemphigus 335 • Non-Hodgkin’s lymphoma 403
• Immunoglobulin A (IgA) pemphigus 335 • Burkitt’s lymphoma 405

13. Infectious Diseases in Children ................... 338 15. Forensic Odontology in Children ................. 410
Mayur Chaudhary, Shweta Dixit Chaudhary, Shweta Dixit Chaudhary, Mayur Chaudhary,
Ragini Gulhane Syed Ahmed Taqi
• Bacterial infections of oral cavity 338 • History 410
• Diphtheria 339 • Terminologies 411
• Tuberculosis 340 • Forensic odontology 411
• Leprosy (Hansen’s disease) 341 • Role of the pedodontist in forensic odontology 412
• Syphilis 343 • Oral autopsy protocol 412
• Acquired syphilis 343 • Scientific methods of identification 413
• Actinomycosis 346 • Technologies for age determination 413
• Noma 348 • Saliva: An identification tool 414
• Tetanus 349 • Palatal rugae pattern 415
• Mycotic infections of the oral cavity 350 • Lip prints and their use for identification 415
• Viral infections of the oral cavity 352 • Reconstruction of the facial tissue 416
• Primary HSV infection 352 • DNA identification 416
xx Essentials of Pediatric Oral Pathology

• Importance of blood group determination 420 Appendices ......................................................................... 451

• Dental tissues and their role in forensic science 421 • Normal laboratory values for children 451
• Bitemark analysis 422 • Serum lipid concentrations by age and gender 452
• Child abuse 422 • Thyroid function tests 453
• Bitemarks 424 • Hematology values 453
• Forensic anthropology 430 • WBC and differential leukocyte counts 453
• Development of human dentition by Schour • Blood gases 453
and Massler 431 • Erythrocyte sedimentation rates and reticulocyte
• Sex determination of skeletal remains 433 counts 454
• The fetal skeleton 435 • Cerebrospinal fluid values 454
• Assessment of skeletal remains 435 • Immunization schedule in children 454
• Chronology of dental development and age • The recommended immunization schedules or
assessment 436 persons aged 0 through 18 years are approved
• Age estimation in prenatal neonatal and early by the advisory committee on immunization
postnatal child 438 practices (http://www.cdc.gov/vaccines/recs/acip),
• Review of the various development surveys 439 the american academy of pediatrics (http://
• Forensic photography 441 www.aap.org), and the american academy of
• Role of forensic dentistry in mass disasters 443 family physicians (http://www.aafp.org) 455
• Annexure-1 449
• Annexure-2 450 Index ............................................................................... 457
Contents xxi

Introduction

As Bruno Bettelheim once remarked, “Raising children is a creative endeavor, an art rather than a science”, all of us who know
about the advent of dentistry right from the ages of the tooth worm theory to the present scenario where dentistry is brimming
with new techniques and newer materials would agree with him. Dentistry started from a single department in various schools
and universities to branch into various specialties, with post graduate and diploma courses in individual specialties. At this
juncture in the steep development curve of dentistry, this book aims to explore yet another aspect of dentistry, i.e. pediatric oral
pathology.

DEFINITION OF PEDIATRIC DENTISTRY

According to the American Academy of Pediatric Dentistry (AAPD), Pediatric dentistry is an age-defined specialty that provides
primary and comprehensive, preventive and therapeutic oral health care for infants and children through adolescence, including
those with special health care needs.
This gives us answers for the following questions:
• Who are the objects of pediatric dentistry?
— Infants and children through adolescence.
— Including those with special health care needs.
• What does pediatric dentistry provide?
— Provides both primary and comprehensive preventive oral health care.
— Provides both primary and comprehensive therapeutic oral health care.
• What are the key elements of this definition that make it so unique?
— Age-defined: Most specialties are procedure-defined (endodontics, periodontics, etc.). Pediatric dentists provide
care for their specific age group of patients. There is no limitation to what type of treatment they provide.
— Primary and comprehensive care: Pediatric dentists are primary providers. There is no need for a referral of patients.
Parents can choose to have their children evaluated and treated by a pediatric dentist just like they can choose to
have their child treated by a pediatrician.
— Infants and children through adolescence: Pediatric dentists see patients at any age from birth up to their late teens.
— Special health care needs: Pediatric dentists have the training and experience to evaluate and treat patients, that are
medically compromised. This includes patients with hemophilia, leukemia, congenital syndromes, etc. No other
dental specialty, other than oral and maxillofacial surgery is more involved in hospital care of patients.
We have talked about the four key elements in the definition that make the pediatric dentists so unique, and most of all it
is treatment provided with tender, loving care.
The primary focus of most dental specialties is a particular area of dental, oral, or maxillofacial expertise. Pediatric dentistry
encompasses a variety of disciplines, techniques, procedures and skills that share a common basis with other specialties, but are
modified and adapted to the unique requirements of infants, children, adolescents and those with special health care needs. By
being an age specific specialty, pediatric dentistry encompasses disciplines such as behavior guidance, care of the medically
and developmentally compromised and disabled patient, supervision of orofacial growth and development, caries prevention,
pharmacological management and hospital dentistry as well as other traditional fields of dentistry. These skills are applied to
the needs of children throughout their ever changing stages of development and to treat conditions and diseases unique to
growing individuals.
The AAPD founded in 1947, is the membership organization representing the specialty of pediatric dentistry. The membership
provides care to millions of infants, children, adolescents and persons with oral health care needs. They are primary contributors
to professional education programs and publications on pediatric oral health.
xxii Essentials of Pediatric Oral Pathology

ORAL PATHOLOGY
HISTORY
Oral pathology appears to have had its origin during the first Golden Age of Dentistry, from 1835 through the organization of
the American Dental Association in 1860. This era saw the establishment of organized, education-based dentistry and was
integrally associated with an obvious fascination for pathologic processes and an inherent wish to share scientific and clinical
knowledge with others in the dental profession. It encompassed the creation of the professorship of “Dental Pathology.”
In the practice of medicine, three main questions are given utmost consideration:
1. What is wrong? (Diagnosis)
2. What is going to happen? (Prognosis)
3. What can be done? (Treatment)
To this, two more questions should be added:
1. How did this happen? (Etiology)
2. Why did this happen? (Pathogenesis)
• Most of the times, diagnosis of a specific lesion depends upon the histopathologic examination of the same under the
microscope.
• Secondly, the cause and effect of the disease can be better understood (extent of the lesion).

DEFINITION
Basically, oral pathology is a science of dentistry that deals with the etiology, pathogenesis, management and prognosis of
various diseases affecting oral and paraoral structures as well as local and systemic effects of various diseases as reflected in
the oral cavity, diagnosis of various diseases, disorders and lesions under the microscope.

PEDIATRIC ORAL PATHOLOGY

The Pediatric Oral Pathology (POP) seminars began in 1977 as a joint, interstate effort between the Department of Dentistry,
Pittsburgh Children’s Hospital, Pittsburgh, Pennsylvania (USA) and the Department of Oral Pathology, West Virginia University
School of Dentistry, Morgantown, West Virginia. They were intended to provide hands-on discussion opportunities for pediatric
dentistry residents interested in lesions of the orofacial region in children and young adults. Weekly afternoon seminars were
offered during the Fall Semester in Children’s Hospital of Pittsburgh, with Dr Jerry Bouquot, Chairman of the WVU Department
of Oral Pathology, presiding as monitor and discussion leader.
The seminars are participation discussions of clinical cases, with participants being provided “notebook cases” a week
ahead of time, with appropriate photos, historical and clinical information provided. Participants are expected to come to the
next seminar with a differential diagnosis, a diagnostic work-up plan and a management plan for each case. They are called
upon individually to treat the cases, as best as they can within the limits of the format, as their own patients. Each seminar
encompasses lesions which can be included in a similar differential diagnosis category. From time to time, special topics are
provided in a more traditional lecture format, and these may carry over several seminars. As of June, 2000, there had been
discussion of more than 960 clinical cases.

DEFINITION
Pediatric Oral Pathology is an art and science of dentistry that deals with the etiology, pathogenesis, management and prognosis
of various diseases and developmental anomalies affecting oral and paraoral structures of infants, children through adolescence
and encompass primary and comprehensive, preventive and therapeutic oral health care through variety of disciplines, techniques,
procedures, and skills that are modified and adapted to the unique requirements of infants, children, adolescents, and those
with special health care needs.
Introduction xxiii

These are one of the few milestones in the journey of the art and science of pediatric oral pathology, a subject which should
be read and comprehended by:
• Pediatric dentists
• Oral pathologists
• Students of dentistry
• General dental practitioners and other dental specialists
• Government agencies and health care policymakers
• Individuals interested in the health of children.
1
Developmental
Disturbances in
Children
Mayur Chaudhary, Shweta Dixit Chaudhary, Prashant Dixit

CHAPTER OVERVIEW
Introduction 6. Developmental disturbances of oral lymphoid tissue:
Can be broadly divided as: Reactive lymphoid aggregate
I. Those affecting the oral and paraoral structures excepting Lymphoid hamartoma
the teeth Angiolymphoid hyperplasia with eosinophilia
II. Those affecting the teeth Lymphoepithelial cyst
Those affecting the oral and paraoral structures excepting 7. Developmental disturbances of the salivary glands:
the teeth may be further divided as: Aplasia
1. Developmental disturbances of jaws: Xerostomia
Agnathia Hyperplasia of palatal glands
Micrognathia Atresia
Macrognathia Aberrancy
Facial hemihypertrophy Developmental lingual mandibular salivary gland depression
Facial hemiatrophy Anterior lingual depression
2. Developmental disturbances of lips and palate: Those affecting the teeth may be further divided as:
Congenital lip pits and fistula
Commissural lip pits 1. Developmental defects in size of teeth:
van der Woude syndrome Microdontia
Cleft lip and cleft palate Macrodontia
Cheilitis glandularis 2. Developmental defects in shape of teeth:
Cheilitis granulomatosa Gemination
Peutz-Jeghers syndrome Fusion
Labial and oral melanotic macule Concrescence
3. Developmental disturbances of the oral mucosa: Dilaceration
Fordyce's granules Talon's cusp
Focal epithelial hyperplasia Dens in dente
4. Developmental disturbances of the gingiva: Dens evaginatus
Fibromatosis gingivae Taurodontism
Retrocuspid papilla Supernumerary roots
5. Developmental disturbances of the tongue: 3. Developmental defects in number of teeth:
Aglossia and microglossia Anodontia
Macroglossia Supernumerary teeth
Ankyloglossia or tongue tie Predeciduous dentition
Cleft tongue 4. Developmental defects in structure of teeth:
Fissured tongue Amelogenesis imperfecta
Median rhomboid glossitis Environmental enamel hypoplasia
Benign migratory glossitis Dentinogenesis imperfecta
Hairy tongue Dentin dysplasia
Lingual varices Regional odontodysplasia
Lingual thyroid nodule Dentin hypocalcification
2 Essentials of Pediatric Oral Pathology

5. Defects of growth (eruption) of teeth: Median palatal cyst

Premature eruption Globulomaxillary cyst
Eruption sequestrum Median mandibular cyst
Delayed eruption Nasoalveolar cyst
Multiple unerupted teeth Palatal and alveolar cysts of newborns
Embedded and impacted teeth Thyroglossal tract cyst
Ankylosed deciduous teeth Epidermal inclusion cyst
6. Fissural cysts of the oral region: Dermoid cyst
Nasopalatine duct cyst Heterotopic oral gastrointestinal cyst

INTRODUCTION
Developmental disturbances comprise a group of disorders that
are manifested during the early months of gestation. They may
be genetically determined, environmentally determined or may
exhibit a role of both genetic and environmental factors. These
disorders may resolve after few months or may persist forever.
The disorders may be termed congenital when present at birth
and hereditary when transmitted from one generation to another.
Special attention is to be given to the term anomaly which
means irregularity or different from normal.
This chapter focuses on some of the developmental
disturbances and anomalies pertaining to children.
DEVELOPMENTAL DISTURBANCES OF JAWS
• Agnathia
• Micrognathia
• Macrognathia
• Facial hemihypertrophy
• Facial hemiatrophy.
FIGURE 1.1: Agnathia showing ears fused in the midline,
AGNATHIA complete absence of the mandible and clefting of both lips

Definition
MICROGNATHIA
A lethal anomaly characterized by hypoplasia or absence of
Micrognathia implies a small jaw and may affect either the
the mandible with abnormally positioned ears and any form of
maxilla or mandible. Lannelongue and Menard first described
holoprosencephaly (Fig. 1.1).
Pierre Robin syndrome in 1891 in a report on two patients with
micrognathia, cleft palate and retroglossoptosis.1 In 1926,
Etiology
Pierre Robin published the case of an infant with the complete
• May be due to autosomal recessive inheritance. syndrome.2 Until 1974, the triad was known as Pierre Robin
• Sporadic cases without inheritance have also been seen. syndrome; however, the term syndrome is now reserved for
those errors of morphogenesis with the simultaneous presence
Pathogenesis of multiple anomalies caused by a single etiology.
Agnathia probably results due to failure of migration of neural
Pathogenesis
crest mesenchyme into the maxillary prominence at the fourth
to fifth week of gestation (post-conception). When it is not • Autosomal recessive inheritance is possible. An X-linked
associated with central nervous system malformations it is variant has been reported involving cardiac malformations
referred to as “agnathia-microstomia-synotia.” and clubfeet.
Developmental Disturbances in Children 3

• Acquired type of micrognathia is of postnatal origin and

usually results from a disturbance in the area of the
temporomandibular joint. Trauma and infection may lead
to ankylosis of the joint which may result in varying degrees
of micrognathia.
• Three pathophysiological theories exist to explain the
occurrence of Pierre Robin sequence.
1. The mechanical theory: This theory is the most
accepted. The initial event, mandibular hypoplasia,
occurs between the 7th and 11th week of gestation. This
keeps the tongue high in the oral cavity, causing a cleft
in the palate by preventing the closure of the palatal
shelves. This theory explains the classic inverted U-
shaped cleft and the absence of an associated cleft lip. FIGURE 1.2: Micrognathia showing an underdeveloped mandible
Oligohydramnios could play a role in the etiology since
the lack of amniotic fluid could cause deformation of
the chin and subsequent impaction of the tongue
between the palatal shelves.
2. The neurological maturation theory: A delay in
neurological maturation has been noted on electro-
myography of the tongue musculature, the pharyngeal
pillars and the palate, as has a delay in hypoglossal
nerve conduction. The spontaneous correction of the
majority of cases with age supports this theory.
3. The rhombencephalic dysneurulation theory: In this
theory, the motor and regulatory organization of the
rhombencephalus is related to a major problem of
ontogenesis. FIGURE 1.3: Congenital lip pits on the lower lip

Clinical Features DEVELOPMENTAL DISTURBANCES OF

LIPS AND PALATE
• This heterogeneous birth defect has a prevalence of
approximately 1 per 8500 live births. • Congenital lip pits and fistula
• The male-to-female ratio is 1:1, except in the X-linked • Commissural lip pits
form. • van der Woude syndrome
• Micrognathia is reported in the majority of cases (91.7%). • Cleft lip and cleft palate
It is characterized by retraction of the inferior dental arch, • Cheilitis glandularis
• Cheilitis granulomatosa
10 to 12 mm behind the superior arch (Fig. 1.2).
• Peutz-Jeghers syndrome
• The mandible has a small body, obtuse gonial angle, and a
• Labial and oral melanotic macule
posteriorly located condyle. The growth of the mandible
catches up during the first year; however, mandibular CONGENITAL LIP PITS AND FISTULA
hypoplasia resolves and the child attains a normal profile
Congenital lip pits and fistula may occur due to notching of
approximately by the age 5 to 6 years.
the lip at an early stage of development with fixation of tissue
• However, due to posterior positioning of the mandible with
at the base of the notch.
regard to the skull, retrusion of the jaw may be apparent.
• Glossoptosis is noted in 70 to 85 percent of reported cases. Clinical Features
Macroglossia and ankyloglossia are relatively rare findings.
• They may be unilateral or bilateral.
Since the frequency of diagnosis of macrognathia, facial • Most commonly occur on the lower lip (Fig. 1.3).
hemihypertrophy and facial hemiatrophy is less in the pediatric • Sometimes, a mucous secretion may exude from the base
population and are generally diagnosed in adulthood, these of the pit which may be due to saliva from minor salivary
have not been explained in detail here. glands draining into the depth of the invagination.
4 Essentials of Pediatric Oral Pathology

Management
1. No specific treatment is required.
2. Surgical excision may be recommended if the pits get
secondarily infected.

VAN DER WOUDE SYNDROME

van der Woude syndrome (VWS) is an autosomal dominant
syndrome, single gene disorder showing high penetrance and
variable expressivity that typically consists of a cleft lip or cleft
palate and/or distinctive pits of the lower lips. It was
characterized in 1954.6

Etiopathogenesis
• This is an autosomal dominant syndrome with a penetrance
of 75 percent. But penetrance was recorded to be 100
percent when supposedly unaffected carriers were closely
examined for minor expressions of the syndrome.
FIGURE 1.4: Bilateral congenital lip pits at the • Viral infections may result in an interference with the IRF6
commissural area
gene which is involved in the immune response to viral
COMMISSURAL LIP PITS infections.
• De novo mutations may lead to single nucleotide
These are mucosal invaginations occurring at the corners of polymorphism resulting in a defect leading to the features
the mouth on the vermillion border. of van der Woude syndrome.
• The gene isolated for van der Woude syndrome is 1q32 to
Pathogenesis
q41.
• They follow a hereditary pattern and may occur alone or • A second modifying gene is 17p11.2-p11.1 with a second
in association with other developmental anomalies most chromosome locus being 1p34.
possibly following a Mendelian dominant inheritance. • The Interferon Regulatory Factor-6 (IRF-6) is the specific
• They mostly occur due to incomplete/failure of normal gene responsible for VWS. This gene has been found to
fusion of maxillary and mandibular processes. regulate fetal craniofacial development in mice.

Clinical Features Clinical Features

• Taylor and Lane, 1966,3 and McConell, 1970,4 reported • Lower lip pits
that 75-80 percent of the cases of lip pits were associated • Incomplete unilateral cleft lip
with cleft lip or cleft palate. • Cleft lip with or without cleft palate
• Commissural lip pits are same as congenital lip pits but • Bilateral cleft lip and palate (Fig. 1.5)
occur at the lateral commisures of lip (Fig. 1.4). • Isolated cleft palate
• Prevalence in children ranges from 0.2 to 0.7 percent.5 • Submucosal cleft palate
• They may be unilateral or bilateral. • Bifid uvula may occur as an isolated finding
• Most commonly occur at the corners of the mouth. • Cleft lip and palate
• Sometimes, a mucous secretion may exude from the base — Cleft lip and palate may be isolated, unilateral or
of the pit which may be due to saliva from minor salivary bilateral.
glands draining into the depth of the invagination. — Submucous cleft palate leads to hypernasal voice.
• Lip pits
Histopathologic Features — Lower lip pits are fairly distinctive and usually medial.
— May be associated with accessory salivary glands.
• Lesional area shows a narrow invagination lined by — Saliva, either visible or expressible, may be present.
stratified squamous epithelium. — Lip pits, at times, could be the only manifestation of
• Few areas show ducts of minor salivary glands. the syndrome.
Developmental Disturbances in Children 5

2. Secondary care
• Retraction of premaxilla – 7–8 weeks (Stage I)
• Obturator is given till the surgery for cleft is
planned and executed to help the patient in eating
(Stage I)
• Maxillary arch expansion is done at the
completion of deciduous dentition (Stage I)
• Buccal crossbite correction—4–6 years (Stage II)
• Anterior teeth are aligned—8–9 years (Stage III)
• Final orthodontics—11+ years (Stage III)
3. Tertiary care
Restorative procedures
4. Surgical care
• Surgical repair of cleft lip and palate or other
anomalies.
— Lip repair procedures (Cheiloplasty) –
Elaborated by Rose, Mirault, Le Mesurier,
Tennison, Millard.
— Palate repair procedures – von Langenback,
FIGURE 1.5: Bilateral cleft lip in van der Woude syndrome Veau-Wardill Kilner (V-Y pushback), Two flap
palatoplasty, Double opposing Z plasty,
• Teeth posterior pharyngeal flap for submucous cleft
Individuals may have hypodontia, most commonly palate.
manifested as missing maxillary lateral incisors or maxillary — Surgical excision of lip pits - either to alleviate
discomfort or for cosmetic reasons.
or mandibular second premolars. Again, this may be the
only manifestation of the syndrome. Sequelae to untreated deformity/anomaly:
• Other oral manifestations: 1. Feeding difficulties
— Syngnathia (congenital adhesion of the jaws). 2. Malocclusion
— Narrow, high arched palate. 3. Speech/Voice disorders
— Ankyloglossia (short glossal frenulum or tongue-tie). 4. Esthetics
5. Frequent otitis media
• Extraoral manifestations:
6. Hearing loss.
— Limb anomalies
— Popliteal webs
CLEFT LIP AND CLEFT PALATE
— Brain abnormalities
— Accessory nipples Millions of children and adults suffer from the social enigma
— Congenital heart defects of cleft lip and palate, battling to live a life of dignity. Failure
— Hirschsprung disease of fusion of palatal shelves, septum and primary palate, which
• Features of van der Woude syndrome have been seen in normally takes place between the 8th and 17th week of
individuals with popliteal pterygium syndrome, which has embryologic development leads to the formation of a cleft.
also been linked to mutations in the same gene.
Development of Palate
Management
The organization of the face requires tissues to proliferate,
1. Primary care fuse and differentiate. The polarizing signal candidates
• Enquiry about family history of genetic disorders. expressed in craniofacial primordia include sonic hedgehog
• Detailed ultrasonography scan at 10–14 weeks of (shh), its putative receptor patched, fibroblast growth factor
pregnancy. 8 (FGF-8) and bone morphogenetic protein 2 (BMP-2). 7
• Examination and genetic counseling by a pediatric
Evidence exists that the teratogen, retinoic acid exerts some
geneticist (dysmorphologist) is suggested.
• Regular antenatal check up.
of its effects on craniofacial development through the
• Restricted fragment length polymorphism (RFLP) disruption of the shh signaling pathway. Transforming growth
may be done factor -3 (TGF -3) also has a broad spectrum of biological
activities.
6 Essentials of Pediatric Oral Pathology

In humans, palate development begins towards the end of

the fifth week of intrauterine life and is complete at about
twelve weeks. The critical period is from the end of the sixth
week to the beginning of the ninth week. In normal palate
development, mesenchymal cells from the neural crest migrate
to the primitive oral cavity forming the maxillary processes in
association with the craniopharyngeal ectoderm.8
The primary palate arises from the fusion of two medial
nasal prominences that form the intermaxillary segment, which
develops towards the end of the fifth week of intrauterine life
in humans. It consists of two portions: a labial component that
forms the philtrum of the upper lip and a triangular palatal
component of bone that includes the four maxillary incisor
teeth. The primary palate extends posteriorly to the incisive
foramen. In humans, the secondary palate comprises at least
90 percent of the hard and soft palates. Development of the
intact secondary palate is a dynamic process which has been
arbitrarily split into three stages. Stages I to III namely Figs
1.6 and 1.7). Stage I of secondary palate development is
characterized by formation of the palatal shelves from the FIGURE 1.6: Schematic diagram of coronal section through a
maxillary processes. These shelves are orientated vertically, developing face showing three stages of secondary palate
either side of the developing tongue. It is not known why the development. At stage I, the palatal shelves are vertical, they elevate
shelves attain this vertical orientation. Ferguson, 1981, in stage II and fuse in stage III
proposed that the direction of shelf growth be related to the
amount of space available in the oronasal cavity during the
period of palatogenesis.9
At a precise developmental stage (Stage II), these vertical
palatal shelves elevate to a horizontal position above the
dorsum of the tongue.10 This event occurs rapidly, possibly in
a matter of hours.
Stage III of secondary palate development involves fusion of
the medial edge epithelium (MEE) of the approximating palatal
shelves with each other via numerous desmosome contacts to
form a midline palatal seam. This then separates the oral and
nasal cavities. Keratin fibrils and desmosomes are upregulated
in the medial edge epithelium seam at this point, presumably
to strengthen the bond between the newly adherent medial edge
epithelium cells. This seam rapidly degenerates, a process
characterized by loss of complex cytokeratins and basement
membrane components such as laminin and desmosomes and FIGURE 1.7: Hematoxylin and eosin stained section of a developing
by an increase of vimentin-rich connective tissue, tenascin, fetus where the palatal shelves have elevated with the tongue lying
proteoglycan and collagen expression. Medial edge epithelium inferiorly. Although there has been elevation, the shelves have still
to fuse
degeneration allows mesenchymal cells to flow across the now
intact horizontal palate. There have been several theories on postulate by Fitchett and Hay, 1989, offered the possibility that
the mechanism(s) of medial edge epithelium degeneration. the medial edge epithelium cells migrate into the body of the
Shapiro and Sweney, 1969, suggested that it occurred as a result mesenchyme and transform into mesenchymal cells.13 This
of programmed cell death.11 Gartner et al, 1978, disputed this process is known as epithelial-mesenchyme transformation
on the grounds that there was no evidence of any cellular debris (EMT). An alternative view is that medial edge epithelium cells
or phagocytic activity at any time during this process. 12 migrate nasally and orally out of the medial edge epithelium
Furthermore, some evidence of metabolic activity occurring seam and become incorporated into the oral and nasal epithelia
within so-called apoptotic cells was described. A radical on the palatal surface.
Developmental Disturbances in Children 7

On completion of stage III, the epithelia on the nasal aspect TABLE 1.1: Percentile familial distribution
of the palate are pseudostratified ciliated columnar cells whilst of cleft lip and palate
those on the oral aspect of the palate are stratified squamous, Relationship to index case Cleft lip/palate Cleft palate15
non-keratinizing cells.
Siblings (overall risk) 4.00% 1.80%
Cleft palate may result from disturbances at any stage of
Siblings (no other affected) 2.20% -
palate development like defective palatal shelf growth, delayed
Siblings (2 affected siblings) 10.00% 8.00%
or failed shelf elevation, defective shelf fusion, failure of medial
Siblings and affected parents 10.00% -
edge epithelium cell death, post-fusion rupture and failure of
Children 4.30% 6.20%
mesenchymal consolidation and differentiation. Sun et al,
Second degree relatives 0.60% -
1998a, suggested that lack of intimate palatal shelf contact after
Third degree relatives 0.30% -
elevation is one possible cause and recent research into palate
General population 0.10% 0.04%
development has concentrated on fusion of the shelves rather
than elevation.14 Ferguson, 1981, on the other hand, took the
UK 1: 1000
view that failure of palatal shelf elevation may be responsible
Blacks 0.41:1000 or 1:1200
for 90 percent of palatal clefting.
Clefts can occur as cleft lip alone, cleft lip/palate, or cleft
Palatal shelf elevation: In principle, an intrinsic force
palate alone (Table 1.1)
generated within the palatal shelves reaches a threshold level
Cleft lip alone: 21 percent of clefts
which exceeds the force of resistance culminating in shelf
Cleft lip/palate together: 46 percent of clefts
elevation. Elevation of the palatal shelves is rapid with a
Cleft palate alone: 33 percent of clefts
swinging ‘flip-up’ mechanism in the anterior one-third of the
Bilateral cleft lip is associated with a cleft palate in 86
palate and an oozing remodeling ‘flow’ mechanism in the percent of cases
posterior two-thirds of the palate. Clefts occur more commonly in boys than in girls
Etiopathogenesis Inheritance: Inheritance is variable depending on whether a
syndrome is associated and when it is, depending on the
• Isolated clefts are those that are associated with no other syndrome present. It could be autosomal recessive, autosomal
birth anomaly.
dominant, X-linked recessive, X-linked dominant, non-
• Syndromic clefts are those associated with other birth
Mendelian inheritance.
disorders.
• Clefts are a feature of over 660 syndromes and most are rare.
Classification
More common syndromes: Pierre-Robin sequence,
Crouzon, Apert, Pfeiffer, van der Woude, Treacher Collins, Veau classification of clefts of the lip: The severity of cleft
Velocardiofacial. Syndromal clefts makes up 15 percent of cleft lip can range from microform (very small defect) to complete
lip +/– palate. clefts and be unilateral or bilateral. Cleft lip will usually result
Isolated clefts are caused by an interaction between an in minor deformity of the nose characterized by a flattened
individual’s genes and certain environmental factors (often nostril on the affected side and flaring of the base on the
impossible to identify). Phenytoin, accutane, alcohol, tobacco, affected side. Cleft lip can involve the alveolus, in which case
folic acid and pyridoxine deficiencies have also been associated it has involved the primary palate.
with clefting.15 If there is a bilateral cleft of the lip, there may be extension
Increasing parental age, especially an older father, is also of the premaxillary segment.
associated. About 35 percent of clefts have a positive family Class I—A unilateral notching of the vermilion not
history. extending into the lip
Class II—A unilateral notching of the vermilion border,
Incidence of Clefting with the cleft extending into the lip but not including the floor
of the nose
There are significant ethnic differences in the prevalence of Class III—A unilateral clefting of the vermilion border of
cleft lip and palate, with the highest rates in Asian populations the lip extending into the floor of the nose
and Native Americans, intermediate rates in Caucasians and Class IV—Any bilateral clefting of the lip, whether it be
lowest rates in African Americans.16-18 incomplete notching or complete clefting.20
Clefting in US 1:750 births
Asians 2.1:1000 or 1:500 Veau classification of clefts of the palate: Cleft palate can
Caucasians 1:1000 to 1:75019 occur with cleft lip or less often by itself. Some patients will
8 Essentials of Pediatric Oral Pathology

present with submucous clefts where the mucosal lining of the

oral cavity roof is present without appropriate supportive bone
and muscle structure. Signs of submucous clefting include a
bifid uvula, diastasis of soft palate musculature (division of
muscles along midline) and notch in hard palate.
Veau divided palatal clefts into four classes as follows:
Class I—Involves only the soft palate.
Class II—Involves the hard and soft palates but not the
alveolar process.
Class III—Involves both the hard and soft palates and the
alveolar process on one side of the premaxillary area.
Class IV—Involves the soft palate and continues through
the alveolus on both sides of the premaxilla, leaving it free and
often mobile.21
Kernahan and Stark, 1958 and Spina, 1974, have classified
cleft lip and palate depending on embryological principles.
Kernahan and Stark classification21
Group I: Cleft of the primary palate only
FIGURE 1.8A: Diagrammatic representation of Kernahan
• Unilateral classification for clefts
• Bilateral 1, 5 : Nasal floor
• Total 2, 6 : Lip
• Subtotal 3, 7 : Alveolus
Group II: Cleft of the secondary palate only 4, 8 : Hard palate anterior to incisive foramen
• Total 9, 10 : Hard palate posterior to incisive foramen
11 : Soft palate
• Subtotal
12 : Congenital velopharyngeal incompetence without
• Submucous obvious clefts
Group III: Cleft of the both primary and secondary palate 13 : Protrusion of premaxilla
• Unilateral—Total, Subtotal
• Median —Total, Subtotal In the stripped Y classification, the involved area is shaded
• Bilateral—Total, Subtotal by pen to graphically represent the defect (Figs 1.8A and B).
Spina classification21
Group I: Pre-incisive foramen clefts Categories of Clefts
• Unilateral Depending on the elemental characteristics of the embryology,
• Bilateral anatomy and physiology of the cleft defect, the varieties of
• Median (cleft of the lip with or without an alveolar clefts of the lip and palate may be tabulated into four general
cleft)—Total, Partial categories:
Group II: Transincisive foramen clefts (cleft of the lip, alveolus 1. Those involving the lip and alveolus
and palate) 2. Those involving the lip and palate
• Unilateral 3. Those in which the palate alone is affected
• Bilateral 4. Congenital insufficiency of the palate
Group III: Post-incisive foramen clefts The term ‘palate’ will include both the hard palate and the
• Total velum or soft palate.
• Partial
Group IV: Rare facial clefts. Genetic Basis of Nonsyndromic Cleft Lip and/or Palate
Kernahan, 1971, has proposed stripped Y classification for Fogh-Anderson, 1942, provided the first population-based
rapid graphic presentation of the defect. This was subsequently evidence that CL +P has a strong genetic component.22
modified by Ehlsaky, 1972 and Millard, 1976.21 • Cleft lip and palate syndromes in humans are associated
The variation in clefts is considerable. A good way to record with polymorphisms in the gene (TGF ) encoding
a cleft lip is by photography. A better way to record a palatal transforming growth factor-a (TGF ), an epidermal growth
cleft is to fill in the following figure with stripes and dots. factor receptor (EGFR) ligand made by most epithelia.
Developmental Disturbances in Children 9

• Extracellular matrix: during the normal merging process, the

disappearance of the midline epithelial seam is accompanied
by an increase in both proteoglycans (PG) and collagen
expression. Control of ECM metabolism in the embryonic
oral facial region, therefore, appears to be essential for
normal palatal development. ECM molecules in turn promote
the activities of growth factors and cytokines present in the
epithelial cell and palatal mesenchyme.
• Although, the role of B-cell leukemia/lymphoma 3 (BCL3)
in the etiology of CLP is unknown, proto-oncogene BCL3
is related to genes involved in cell lineage determination
and cell cycle regulation.
• Retinoic acid receptors (RAR ): The region on chromo-
some 11 associated with CLP in this animal model is
homologous to 17q21 - q24 in humans.23 This region,
marked by retinoic acid receptor a (RAR ) has shown
association with CLP in some populations.24 This study has
strengthened the case for CLP locus linked to RAR in
humans.
• Chromosome 6: Chromosome 6 has been of interest to
investigators because of the association of alleles at the
H2 locus with corticosteroid induced clefting in the
mouse. HLA (on chromosome 6p) is the human
homologue of H2.
• Chromosome 2
• Chromosome 19 also implicated in formation of
• Chromosome 4 cleft lip and/or palate
• Chromosome 17
• Environmental factors: Naturally occurring folates are
found widely in foodstuffs, especially in liver, legumes and
fresh vegetables. Folic acid (pteroylmonoglutamic acid) is
a commercially available compound used for
supplementation. It does not occur naturally in living
tissues, but is readily converted in vivo into the biologi-
cally active folates. Folates are essential in the synthesis
of purines and pyrimidines, which are components of DNA
and RNA required in the regulation of gene expression and
cell differentiation. In humans, drugs that interfere with
folate metabolism, e.g. phenytoin, are known to have
teratogenic effects. Low blood folate levels were associated
with spontaneous abortion and developmental abnormalities
of the fetus.25 However, studies have found significant
protective effects of the role of folic acid in orofacial
clefting.
FIGURE 1.8B (A to D): Diagrammatic representation of • There also appears to be an association between maternal
Kernahan classification for clefts smoking and oral clefting.26
A: Cleft palate
• Deficiency of vitamins B1, B2 and B6 in the NMRI strain
B: Left-sided unilateral complete cleft lip and palate
C: Bilateral complete cleft lip and palate
led to embryolethality, teratogenicity and growth retardation
D: Bilateral right incomplete, left complete cleft lip and primary among the fetuses. The cleft palate rate was eight times
palate higher in the deficient group than in the control group.
10 Essentials of Pediatric Oral Pathology

TABLE 1.2: Some syndromes associated with cleft lip and/or TABLE 1.3: Other birth defects associated with
palate (+ = present, – = absent) cleft lip and/or cleft palate

Syndrome Cleft Lip Cleft Palate System Specific findings

Stickler syndrome – + Facial malformations • Hypertelorism

• Facial asymmetry
Treacher-Collins – +
• Hemangiomas
van der Woude syndrome + +
• Choanal atresia
Pierre Robin syndrome – + • Microstomia
Ectodermal dysplasia syndrome + + • Low-set ears
Saethre Chotzen syndrome – + • Auricular malformations
Pallister Hall syndrome – + • Preauricular skin tags
Waardenburg syndrome + + • Ear canal atresia
Basal cell nevus syndrome + +
Cardiac system • Ventricular septal defects
Pfeiffer syndrome – +
• Transposition of the great vessels
Holoprosencephaly + + • Patent ductus arteriosus
Retinoblastoma – + • Tetralogy of Fallot
Shprintzen Goldberg syndrome – + • Total anomalous pulmonary venous return
Marfan’s syndrome – + (or bifid uvula) • Single ventricle
Velocardiofacial – + • Peripheral pulmonary artery stenosis
DiGeorge syndrome + +
Gastrointestinal • Pyloric stenosis
Apert syndrome – +
system • Esophageal atresia
Crouzon craniofacial dysostosis – + • Anal fistula
Cleidocranial – + • Inguinal hernia
Langer Giedion syndrome – + • Diaphragmatic hernia
CHARGE syndrome + +
Central nervous • Visual impairment
system • Problems with balance
Genetic Basis of Syndromic Cleft Lip and/or Palate • Hydrocephalus
Syndromic cleft lip and/or palate is seen associated with variety • Holoprosencephaly
• Seizures
of syndromes which present myriad genetic pictures (Table 1.2).
Discussion of the genetic make-up of a few of the common Musculoskeletal • Lumbar spine kyphosis
craniofacial syndromes has been dealt with in the chapter on system • Vertebral and costal deformities
bone pathologies. Both syndromic and nonsyndromic cleft lip NOTE: These defects can also occur as isolated abnormalities and
and/or palate may be associated with other birth defects that do not always indicate that the infant has cleft lip and/or cleft palate.
are elaborated in Table 1.3. A physical assessment must always involve a thorough examination
of facial features, including the lip and palate, to rule out cleft lip
Prenatal Diagnosis and/or palate regardless of other findings on examination.

Ultrasound scanning: For cleft lip, with or without cleft palate, 1. Parents should be given accurate information by someone
fetal ultrasound studies are the only commonly used test with experience in clefts.
available for prenatal diagnosis (Figs 1.9 and 1.10). 2. The time from diagnosis to support should be as brief as
possible.
Prenatal Counseling 3. Parents should have early contact with members of the cleft
When the growing fetus in the womb has been identified as team.
having a cleft, the parents will have to go through the process 4. All members of the family should be given the opportunity
of adjustment in an atmosphere of uncertainty. They will not to express their concerns and their emotional responses.
know exactly what their baby will look like and struggle to 5. They should all be helped to prepare for the birth by having
cope with images of a fantasized imperfect child. There are a clear view of how the baby is likely to look.
often conflicting images of the anticipated child like the 6. Any discussion of termination should begin with the
concept of a chubby wholeness vies with the unknown. After parents on the basis of accurate information.27
the genetic evaluation is completed, it is time to sit with the
Perinatal Counseling
family for genetic counseling. This is usually the longest part
of the genetic evaluation, because it is necessary to educate It is important that parents receive experienced counseling
the family regarding issues of heredity and development. The when the baby is born. This helps parents feel less alone even
principles of good prenatal counseling are: though it is not a substitute for skilled professional care.
Developmental Disturbances in Children 11

FIGURES 1.9A and B: Cleft lip and palate at 38 weeks showing the two common ultrasound orientations to detect clefting. Left image is
axial through the lip and shows a cleft (arrow). Right image is coronal through the tip of the nose, lips and chin. Note the cleft (arrow)
which extends into and deforms the nostril (complete cleft lip)

Postnatal Counseling
Some parents do have great difficulty adjusting to the cleft
and find it becomes difficult to accept their child. This may
be expressed as emotional distancing from the baby, but it
may also be expressed as a defensive overprotectiveness as
the parent strives to cope with negative feelings and a sense
of personal incompetence. Problems with adjustment may be
masked by the physical needs of the new baby and whilst the
distraught mother is easy to identify and help, it is not so easy
to recognize the angry or depressed mother, nor the one who
withdraws into an introspective depressive state.
Principles of good postnatal counseling are:
1. Individual needs should be assessed in a non-
judgmental way.
2. Communication should be encouraged between family
FIGURE 1.10: Bilateral complete cleft lip/palate (small arrows) with members.
the premaxilla protruding anteriorly as a mass (open arrow)—Axial 3. Social and family support should be mobilized where
view through fetal lip at 25 weeks it is lacking.
4. If necessary, other agencies should be contacted to help
The principles of good perinatal counseling are: deal with particular problems.
1. All families should have access to experienced 5. Long-term dependency on the counselor should be
counseling. avoided and normalization of family life should be
2. Early assessment of problems is important. encouraged.
3. The needs of all family members should be recognized. Children can learn to live with a disability. But they cannot
4. Strengths should be emphasized rather than live well without the conviction that their parents find them
weaknesses. utterly lovable. If the parents, knowing his defect, love him
5. Parents should be empowered to gain mastery through now, he can believe that others will love him in the future.
interaction with professionals and access to relevant With this conviction he can live well now and have faith in
information.28 the years to come.29
12 Essentials of Pediatric Oral Pathology

Policy on Management of Patients with Cleft Lip/Palate 10. Evaluation of treatment outcomes must take into
and Other Craniofacial Anomalies account the satisfaction and psychosocial well-being
of the patient, as well as effects on growth, function
The American Academy of Pediatric Dentistry (AAPD), in
and appearance.
its efforts to promote optimal health for children with cleft
As members of the interdisciplinary team of physicians,
lip/palate and other craniofacial anomalies, endorses the
dentists, speech pathologists and other allied health
current statements of the American Cleft Palate-Craniofacial
professionals, pediatric dentists should provide dental services
Association (ACPA).
in close cooperation with their orthodontic, oral and maxillo-
Several fundamental principles were identified as critical
facial surgery and prosthodontic colleagues.
to optimal cleft/craniofacial care. These principles are:
1. Management of patients with craniofacial anomalies All dental specialists should ensure that:
is best provided by an interdisciplinary team of 1. Dental radiographs, cephalometric radiographs and
specialists. other imaging modalities as indicated should be utilized
2. Optimal care for patients with craniofacial anomalies to evaluate and monitor dental and facial growth and
is provided by teams that see sufficient numbers of development.
these patients each year to maintain clinical expertise 2. Diagnostic records, including properly occluded dental
in diagnosis and treatment. study models, should be collected at appropriate
3. Although referral for team evaluation and management intervals for patients at risk for developing
is appropriate for patients of any age, the optimal time malocclusion or maxillary-mandibular discrepancies.
for the first evaluation is within the first few weeks of 3. Presurgical maxillary orthopedics to improve the
life and whenever possible, within the first few days. position of the maxillary alveolar segments prior to
4. From the time of first contact with the child and family, surgical closure of the lip may be indicated for some
every effort must be made to assist the family in infants.
adjusting to the birth of a child with a craniofacial 4. As the primary dentition erupts, the team evaluation
anomaly and the consequent demands and stress placed should include a dental examination and if such
upon that family. services are not already being provided, referral to
5. Parents/caregivers must be given information about appropriate providers for caries control, preventive
recommended treatment procedures, options, risk measures and space management.
factors, benefits and costs to assist them in 5. Before the primary dentition has completed eruption,
a. Making informed decisions on the child’s behalf the skeletal and dental components should be evaluated
b. Preparing the child and themselves for all to determine if a malocclusion is present or developing.
recommended procedures 6. Depending upon the specific goals to be accomplished
The team should actively solicit family participation and also upon the age at which the patient is initially
and collaboration in treatment planning and, when the evaluated, orthodontic management of the
child is mature enough to do so, he or she should also malocclusion may be performed in the primary, mixed
participate in treatment decisions. or permanent dentition. In some cases, orthodontic
6. Treatment plans should be developed and implemented treatment may be necessary in all 3 stages.
on the basis of team recommendations. 7. While continuous active orthodontic treatment from
7. Care should be coordinated by the team, but should early mixed dentition to permanent dentition should be
be provided at the local level whenever possible; avoided, each stage of orthodontic therapy may be
however, complex diagnostic or surgical procedures followed by retention and regular observation.
should be restricted to major centers with appropriate Orthodontic retention for the permanent dentition may
treatment facilities and experienced care providers. extend into adulthood.
8. It is the responsibility of each team to be sensitive to 8. For some patients with craniofacial anomalies,
linguistic, cultural, ethnic, psychosocial, economic and functional orthodontic appliances may be indicated.
physical factors that affect the dynamic relationship 9. For patients with craniofacial anomalies, orthodontic
between the team, patient and family. treatment may be needed in conjunction with surgical
9. It is the responsibility of the team to monitor both short- correction of the facial deformity.
term and long-term outcomes. Thus, longitudinal 10. Congenitally missing teeth may be replaced with a
follow-up of patients, including appropriate removable appliance, fixed restorative bridgework, or
documentation and record keeping, is essential. osseointegrated implants.
Developmental Disturbances in Children 13

11. Patients should be closely monitored for dental and or divided laterally to one side of the cleft. In such cases, lip
periodontal disease. surgery becomes difficult if anteroposterior and vertical
12. Prosthetic obturation of palatal fistulae may be repositioning of the premaxilla is not carried out. After the
necessary in some patients. delivery of the obturator, one week later (period of
13. A prosthetic speech device may be used to treat adjustment), the infant is fitted with a premaxillary retraction
velopharyngeal inadequacy in some patients.30 appliance.
This could be:
Role of the Pedodontist in Management of — Premaxillary retraction strap with a baby bonnet made
Cleft Lip and Palate to provide “headgear anchorage”. Bonnet and strap
appliance to be worn 24 hours a day, removed only
The complete rehabilitation of this condition definitely
during feeding, for 6 to 8 weeks.
requires a multidisciplinary approach involving a pediatrician,
— In case of laterally deviated premaxilla with bilateral
oral surgeon, plastic surgeon, prosthodontist, dietician, speech
cleft lip and palate, an external acrylic bulb prosthesis
therapist, with the pedodontist providing an invaluable input.
is anchored to the infant’s head with a bonnet
Early dental management of cleft lip and palate includes:
appliance.
1. Intraoral maxillary obturator therapy
2. Appliance for premaxillary retractions Cheiloplasty (surgical lip closure): A general “rule of tens”
3. Management of dental problems is used in determining optimal timing of lip closure, i.e.
10 weeks of age, 10 pounds of body weight, 10 gms Hb. At
Intraoral maxillary obturator: Feeding problems are often the time of lip closure, when the infant is under general
associated with infants affected with cleft lip and palate, anesthesia, an impression is made for the new obturator.
making it difficult to maintain adequate nutrition. Maxillary orthopedics: Between the 3rd and 9th month of
These problems include: age, to prevent collapse of maxillary arches, the obturator is
— Insufficient suction to pull milk from the nipple used to provide cross arch stability and support. As pressure
— Excessive air intake during feeding is exerted on anterior segments of maxilla by the repaired lip,
— Choking orthopedic molding of the segments can be achieved. This is
— Nasal regurgitation facilitated by the obturator.
— Excessive time required for nourishment Closure of palatal cleft by bone grafting: Following are
Special nipples and bottles are available that create an easy the bone grafting procedures that are well accepted by the
flow. Feeding equipment includes bottles, teats, cups, spoons, practitioners:
NUK orthodontic nipple, Mead Johnson squeezable cleft • Primary bone grafting—Less than two years of age
palate feeder, the Haberman feeder. Feeding techniques (e.g. • Early secondary bone grafting—Two to four years of age
Richard’s 1991 Enlargement, Stimulate, Swallow, Rest [ESSR] • Secondary bone grafting—Six to fifteen years of age
method), breast-feeding, prostheses, and nutrition/lactation • Late secondary bone grafting—In adults (residual alveolar
advice also assist in the feeding routine of the child. A soft cleft reconstruction).
bottle can be squeezed gently during feeding to let the baby
Management of dental problems
get enough formula.
The intraoral maxillary obturator proves beneficial by This includes:
providing an artificial palate. • Establishment and maintainance of optimum oral health
Advantages of this are: • Prevention of decay in the teeth adjacent to the cleft (as
— It reduces feeding difficulties and helps to maintain these areas favor food lodgement)
• Correction of ectopically erupted teeth and crossbite
adequate nutrition.
• Interceptive correction of traumatic occlusions
— It provides maxillary cross arch stability; prevents arch
• Maxillary expansion - routine palatal expansion (especially
collapse after cheiloplasty.
in patients who have not undergone primary cleft bone
— It helps maxillary orthopedic moulding of the cleft
grafting)
segments into approximation before primary alveolar
• Orthodontic treatment for alignment and occlusion.31
cleft bone grafting.
The treatment protocol can be more conveniently
This appliance is most useful during 0-3 months period,
delineated according to the type of dentition as follows:
until the time of initial lip closure.
Primary dentition treatment: At this age, a proper
Appliances for premaxillary orthopedics (birth-4 to 5 months) alignment and/or expansion of the primary dentition can be
In cases of bilateral cleft lip and palate, the premaxillary done more easily. But, often the problems are not very severe
segment is either placed severely anterior to the maxillary arch at this stage and does not require a very active or enthusiastic
14 Essentials of Pediatric Oral Pathology

treatment. Simple forms of a fixed maxillary lingual appliance new life of dignity. Nothing short of a miracle for the hapless
are preferred over the removable split palatal type of appliance patient!!!
because of occasional cooperation problems and a high relapse Preventive measures, per se, are still a distant hope in the
rate with a removable appliance. In a few cases, speech legend of cleft lip and palate; yet with so many organizations
pathologists advise palatal expansion for improving speech. and research centers working towards unraveling the genetic
Mixed dentition treatment: Some problems requiring basis of cleft lip and palate, we can be hopeful of a day when
attention at this stage are: we would be able to prevent this condition altogether.
1. Minor crossbites: Crossbites may be corrected by
expansion by usual methods. Once correction is complete, CHEILITIS GLANDULARIS
full time retention is required. This is because there is no
midpalatal suture system to fill in bone and consolidate Von Volkman, 1870,37 coined the term cheilitis glandularis and
the expanded maxillary segments. Even if the crossbite is described it as a chronic inflammatory condition of the lower
corrected and a retention device given at this stage, the lip characterized by mucopurulent exudates from the ductal
possibility of a need to re-expand at the permanent orifices of the labial minor salivary glands. It is a chronic
dentition stage cannot be ruled out. This is because of progressive and uncommon inflammatory condition of the
aggravated maxillary hypoplasia with growth. minor salivary glands.
2. Retroclination of permanent incisors and anterior cross-
bite: May lead to esthetic, speech and psychological Etiology
problems. To correct this usually a partial banded approach • May be a manifestation of chronic irritation.
is needed. Once alignment is corrected, a full time retention • Factitial trauma, excessive wetting of lips by frequent licking.
device is needed. • May be associated with mouth breathing and asthma.
3. Crowded dentition: This may require serial extraction • Poor oral hygiene
whereby primary cuspids are removed to treat incisor • Syphilis
crowding and the primary molars may be removed to • Heredity
hasten the eruption of the first bicuspids.
4. After alveolar bone grafting: It is usually done just before Clinical Features
the canine erupts. Orthodontic movement of the canine may
be initiated 6 weeks following placement of the bone graft. • Usually found on the lower lip. May also involve upper
With orthodontic movement of the canine enough space lip and palate.
is created in the arch to allow the cuspids to erupt. • Cases have been reported in women and children, but occur
Permanent dentition treatment: The principles and most often in middle aged men.
techniques of permanent dentition treatment of cleft lip and • Eversion of lower lip, inflamed and dilated minor salivary
palate cases are similar to those in non cleft orthodontics, gland ducts, burning sensation at the vermillion border of
except the period of retention is invariably longer. The main the lip.
problems at this stage are posterior crossbites and malposed • Sometimes, a mucopurulent exudate is seen.
permanent incisors. • According to clinical features seen, it is classified into three
If orthognathic surgery is done to correct the underlying types:
skeletal imbalance, preoperative and postoperative orthodontic 1. Simple type: Multiple painless, papular surface lesions
treatment is a must to achieve proper alignment, position and with central umbilication.
inclination of the teeth on their respective arches. 2. Superficial suppurative type (Baelz Disease): Painless
The possibility of opening of the oronasal fistula due to indurated swelling of the lip with shallow ulceration
arch expansion resulting in increased hypernasality and nasal and crusting.
regurgitation must be discussed before starting orthodontic 3. Deep suppurative (cheilitis glandularis apostematosa):
treatment. Deep seated infection with formation of abscesses, sinus
Plastic surgery is one of those fascinating branches of tracts and fistulas.
medical science which challenges the very verdict of nature
by remodeling the shape of tissue/structure. In other words, Histopathologic Findings
plastic surgery can be termed as RECREATION. It gives an
• Lesional tissue shows inflamed, dilated minor salivary
opportunity to the individual to RELIVE. In the context of
gland ducts (Fig. 1.11).
our country, one can even say that the effects of plastic surgery
• In some cases, dysplastic changes may be seen in the
are akin to REINCARNATION and allow a person to live a
overlying surface epithelium.
Developmental Disturbances in Children 15

FIGURE 1.11: Histopathologic picture of cheilitis glandularis FIGURE 1.12: Cheilitis granulomatosa showing diffuse episodic
showing ductal ectasia, acinar atrophy, interstitial fibrosis and swelling on lower lip
inflammation of minor salivary glands

Management
1. Treatment is based on histopathologic findings.
2. Vermillionectomy, if the lesion is associated with
actinic damage.

CHEILITIS GRANULOMATOSA
It is a chronic swelling of lip due to granulomatous infla-
mmation.

Etiology
Etiology is unknown; a possibility of genetic predisposition
has been implicated. Sometimes contact antigens may play a
role. FIGURE 1.13: Histopathologic picture of cheilitis granulomatosa
showing a central zone of non-caseating granuloma
Clinical Features
• The lesion is seen most commonly in young adults. • Peri and paravascular aggegrates of chronic inflammatory
• There is no sex predilection. cell infiltrate mostly lymphocytes, plasma cells and
• Diffuse or nodular episodic swellings on lip or the face and histiocytes are seen.
mostly involving upper lip, lower lip, eyelids and one side
of the face (Fig. 1.12). Management
• Initially the lesion is soft but as time progress, it becomes 1. Avoidance of contact with known allergen.
rough, dry, painful and firm in consistency; especially those 2. Intralesional corticosteroid injections.
occurring on the lips. 3. Use of mast cell stabilizers and non-steroidal anti-
• Other features include fissured tongue, unilateral or bilateral inflammatory agents.
facial palsy and lymphadenopathy. 4. Surgery
5. Radiation
Histopathologic Features
PEUTZ-JEGHER'S SYNDROME
• Lesional areas show a central zone of noncaseating
granuloma consisting of epitheloid cells and Langhans giant Peutz-Jeghers syndrome is an autosomal dominant inherited
cells (Fig. 1.13). disorder characterized by intestinal hamartomatous polyps in
16 Essentials of Pediatric Oral Pathology

association with mucocutaneous melanocytic macules.32,33 Also

called as hereditary intestinal polyposis syndrome, intestinal
hamartomatous polyps in association with mucocutaneous
melanocytic macules.
A 15-fold elevated relative risk of developing cancer exists
in this syndrome over that of the general population; cancer
primarily is of the GI tract, including the pancreas and luminal
organs and of the female and male reproductive tracts and the
lung.
Jeghers, McKusick and Katz (1949) are credited with
rediscovery and establishment of clinical significance of
generalized intestinal polyposis. First described by Peutz in
1921,34 this obscure syndrome produced melanin spots on oral FIGURE 1.14: Peutz-Jegher’s syndrome showing cutaneous
pigmentation of the perioral region crossing the vermilion border
mucous membranes, extraoral labial tissues and fingers. Jeghers
et al presented ten cases in which they described syndromes
consisting of same signs and symptoms.35 • Mucous membrane pigmentation, primarily the buccal
mucosa (66 %) is seen.
Etiopathogenesis • Pigmentation may be present on the fingers and toes, on the
dorsal and velar aspects of the hands and feet, and around
Goldberg and Goldhaber (1954) believed that inheritance of the anus and genitalia and may fade after puberty.
Peutz-Jeghers syndrome was simple Mendelian dominant and • Gynecomastia and growth acceleration (due to Sertoli cell
single pleotrophic gene was believed to be responsible for both tumor) may be seen.
melanin spots and polyps. Melanin spots and patches varied • Testicular mass may also be present.
from 1 to 5 mm.36
The characteristic pathology of Peutz-Jeghers polyps Laboratory Findings
includes extensive smooth muscle arborization throughout the
polyp with the appearance of pseudoinvasion because some A CBC count should be obtained because the polyps may be a
of the epithelial cells, usually from benign glands, are source of blood loss.
surrounded by the smooth muscle.
The cause of Peutz-Jeghers syndrome appears to be a Histopathologic Findings
germline mutation of the STK11 (serine threonine kinase 11) Characteristic pathology of Peutz-Jeghers polyps includes
gene in most cases, located on band 19p13.3.
extensive smooth muscle arborization throughout the polyp,
with the appearance of pseudoinvasion because some of the
Clinical Features
epithelial cells, usually from benign glands, are surrounded by
• Peutz-Jeghers syndrome has been described in all races. the smooth muscle.
• The occurrence of cases in males and females is about
equal. Management
• The average age at diagnosis is 23 years in men and 26
1. Annual physical examination that includes evaluation
years in women.
of the breasts, abdomen, pelvis and testes
• Repeated bouts of abdominal pain in patients younger than
2. Annual complete blood count
25 years are reported.
3. Repeated removal of hemorrhagic or large polyps
• Unexplained intestinal bleeding in a young patient has also
(> 5 mm) by endoscopic polypectomy.
been reported.
• Prolapse of tissue from the rectum may be seen. Rectal mass
LABIAL AND ORAL MELANOTIC MACULE
or rectal polyp is seen.
• Menstrual irregularities in females (due to hyperestroge- Oral melanotic macule represents a focal area of melanin
nism from sex cord tumors with annular tubules). deposition, as a result of increase in number of melanocytes,
• Precocious puberty may also occur. mostly occurring on the buccal mucosa.
• Cutaneous pigmentation (1 to 5 mm macules) of the perioral Another term labial melanotic macule is used to represent
region crossing the vermilion border (94 %), perinasal and an entity similar to oral melanotic macule, occurring on the
perioral areas is seen (Fig. 1.14). vermillion border of the lip (Fig. 1.15).
Developmental Disturbances in Children 17

FIGURE 1.15: Labial melanotic macule presenting as a black, FIGURE 1.16: Fordyce’s granules appearing as small yellow,
round shaped lesion discretely separated spots

Clinical Features • They are most frequently found in a bilaterally symmetrical

pattern on the buccal mucosa opposite the molar teeth and
• The lesion may be seen occurring at any age.
also on the inner surfaces of lips, in the retromolar region
• Incidence of occurrence is more common in females as
lateral to the anterior faucial pillar and occasionally on
compared to males.
tongue, gingiva, frenum and palate.
• Lesions most commonly occur on lower lip, buccal mucosa,
• Ectopic sebaceous glands have been discussed in a review
gingiva and palate.
by Guiducci and Hyman and may occur in oesophagus,
• It occurs as a dark brown to black, round to oval shaped
uterine cervix, male genitalia, nipples, palms and soles,
lesion in the oral cavity.
parotid gland, larynx and orbit.38
• Studies by Halperin and coworkers, confirmed by Miles,
Histopathologic Features
have indicated that this oral condition is present in
1. Lesional area shows abundant melanin deposits within approximately 80 percent of the population.39
keratinocytes in the basal and parabasal layer. • Miles has reported that a large number of sebaceous glands
2. There is no underlying inflammatory cell infiltrate. in cheeks and lips may sometimes be found in children long
before the age of puberty.40
Management
Histopathologic Features
1. No specific treatment.
2. Lesion should be sometimes biopsied to rule out any • In a microscopic view of a Fordyce's granule, sebaceous
malignancy. glands are clearly seen (Fig. 1.17).
• Sebaceous glands are found normally in large numbers on
DEVELOPMENTAL DISTURBANCES the skin where they are associated with hair follicles.
OF THE ORAL MUCOSA • These sebaceous glands are similar histologically to those
seen in skin.
• Fordyce’s granules • A single hair follicle and hair shaft growing from gingiva
• Focal epithelial hyperplasia is a rare occurrence and has been reported by Baughman,
1980.41
FORDYCE'S GRANULES • Glands are superficial and may consist of few or many
This is not a disease of oral mucosa but a developmental lobules all grouped around one or more ducts which open
anomaly characterized by heterotropic collection of sebaceous on the surface of mucosa.
glands at various sites in the oral cavity. • Ducts may show keratin plugging.

Management
Clinical Features
No specific treatment required
• Fordyce’s granules appear as small yellow spots, either
discretely separated or forming relatively large plaques, often Focal Epithelial Hyperplasia
projecting slightly above the surface of tissue (Fig. 1.16). Refer to chapter on epithelial pathology.
18 Essentials of Pediatric Oral Pathology

Management
No specific treatment required

DEVELOPMENTAL DISTURBANCES
OF THE TONGUE
• Aglossia and microglossia
• Macroglossia
• Ankyloglossia or tongue tie
• Cleft tongue
• Fissured tongue
• Median rhomboid glossitis
• Benign migratory glossitis
• Hairy tongue
• Lingual varices
FIGURE 1.17: Histopathologic picture of Fordyce’s granules • Lingual thyroid nodule.
showing superficial sebaceous glands consisting of few or many
lobules
AGLOSSIA AND MICROGLOSSIA

DEVELOPMENTAL DISTURBANCES A very rare anomaly, only 35 cases have been reported in a
OF THE GINGIVA period spanning 258 years. It is mostly associated with cleft
palate and dental agenesia.
• Fibromatosis gingivae
• Retrocuspid papilla Pathogenesis

FIBROMATOSIS GINGIVAE Unknown, but may be due to lack of muscular stimulus between
alveolar arches, which results in retardation of growth of the
Refer to chapter on gingival and periodontal diseases in the mandible in an anterior direction.
pediatric population.
Clinical Features
RETROCUSPID PAPILLA
• Microglossia is characterized by an abnormally small tongue.
It is a small papule that most frequently appears bilaterally on • Even though aglossia indicates the absence of a tongue,
the lingual mucosa of mandibular bicuspid. It was first there is almost always the presence of a rudimentary small
described by Hirshfeld in 1933.42 tongue.
• May be associated with oromandibular-limb hypogenesis
Clinical Features syndrome. It is characterized by hypodactylia (absence of
one or more digits), hypomelia (hypoplasia of part or all
• It occurs as a small, well-circumscribed, soft, pink colored
of a limb) and microglossia.
papule, bilaterally on mandibular lingual mucosa between
• Microglossia is frequently associated with hypoplasia of
free gingival margin and mucogingival junction.
the mandible and lower incisors may be missing.
• Incidence of occurrence in children and young adults is
between 25 to 99 percent and gradually decreases with
Management
increase in age.
• According to Berman and Fay, 1976, the lesion occurs more 1. Depends upon nature and severity of the condition.
commonly in males as compared to females.43 2. Surgery may help in improving oral function.
3. Orthodontics may also help in improving esthetics and
Histopathologic Features oral function.
• The lesional area reveals vascularized fibrous connective
MACROGLOSSIA
tissue with presence of numerous large stellate fibroblasts
containing numerous nuclei. The term denotes an enlarged tongue. Macroglossia has been
• The overlying epithelium is atrophic and may show described as far back as the era of Egyptian Papyrus Ebers
hyperortho or parakeratosis. from around 1550 BC (Fig. 1.18).
Developmental Disturbances in Children 19

Traumatic:
• Surgery
• Hemorrhage
• Direct trauma
• Intubation injury.
Neoplastic
• Lingual thyroid
Infiltrative
• Amyloidosis
• Sarcoidosis.

Clinical Features
• Commonly occurs in children.
• Ranges from a mild to severe degree.
• In infants, it may be manifested by noisy breathing, drooling
and difficulty in eating
• Pressure on tongue against the teeth produce crenated
FIGURE 1.18: Macroglossia lateral borders to the tongue, open bite and mandibular
prognathism
Etiology • Tongue may ulcerate, get infected and finally necrosed
Etiology of macroglossia • Macroglossia is associated with Beckwith-Wiedemann
Congenital syndrome characterized by omphalocele (protrusion of part
• Hemangioma of the intestine through a defect in the abdominal wall at
• Lymphangioma the umbilicus), visceromegaly, gigantism and neonatal
• Down syndrome hypoglycemia. It follows an autosomal dominant mode of
• Beckwith-Wiedemann syndrome inheritance and carries a high risk of childhood visceral
• Lingual thyroid
tumors including Wilms tumor, adrenal carcinoma and
• Trisomy 22
• Laband syndrome
hepatoblastoma.
• Mucopolysaccharidosis • Appearance of the tongue varies with the cause:
• Multiple endocrine neoplasia Type 2B. — A diffuse, smooth, generalized enlargement is seen in
hypothyroidism
Acquired
— A multinodular appearance is seen in amyloidosis,
Metabolic/Endocrine:
• Hypothyroidism neurofibromatosis and multiple endocrine neoplasia
• Cretinism — A pebbly surface with multiple vesicle like blebs is seen
• Diabetes. in lymphangioma
— A papillary surface is seen in Down syndrome
Inflammatory/Infectious:
• Syphilis
— Unilateral enlargement is seen in hemifacial hyperplasia.
• Amebic dysentery
• Ludwig angina Pseudomacroglossia
• Pneumonia
• Pseudomacroglossia is the term applied to a condition
• Rheumatic fever
• Small pox
which forces the tongue to sit in an abnormal position.
• Typhoid These conditions could be:
• Tuberculosis — Habitual posturing of the tongue
• Actinomycosis — Enlarged tonsils or adenoids
• Candidiasis. — Low palate
Systemic/Medical conditions: — Transverse, vertical, anterior/posterior deficiency in the
• Hypertrophy maxillary or mandibular arches
• Neurofibromatosis — Severe mandibular retrognathism, neoplasm displacing
• Iatrogenic. the tongue, hypotonia of the tongue
20 Essentials of Pediatric Oral Pathology

Histopathologic Features • Inability to maintain oral hygiene in the mandibular

anteriors
• Depends on the specific cause
• May result in speech defects
— In case of Down syndrome, no histologic abnormality
• May be associated with dyspnea due to upward and forward
can be detected.
displacement of the epiglottis and larynx.
— In case of a tumor, neoplastic proliferation of a
particular tissue may be seen.
Management
— In case of Beckwith-Wiedemann syndrome, increased
muscle tissue is seen. 1. In mild cases, no specific treatment is required.
— In case of amyloidosis, an abnormal protein material 2. In severe cases, when there are functional or
may be seen deposited. periodontal difficulties, frenectomy is recommended.
Surgery is required only if:
Management • Speech problems are present
• Periodontal maintenance of the mandibular
1. In mild cases, no specific treatment is required.
anteriors is difficult.
2. In severe conditions, glossectomy is required to
3. In younger children, surgery is postponed until the age
improve functions.
of 4 or 5.

ANKYLOGLOSSIA (Fig. 1.19)

CLEFT TONGUE
It is commonly known as tongue tie. Ankyloglossia occurs when
It is a rare anomaly. Mostly occurs due to lack of emergence
a short lingual frenum attaches to the bottom of the tongue.
of lateral lingual swellings. More commonly seen is a partial
Mostly occurs in 2 to 3 of every 10,000 people.
cleft of the tongue or a manifestation of a cleft as only a
Clinical Features deep groove running along the midline of the dorsal surface.
• Occurs as a mild and severe form Etiology
• Mild form is of little clinical significance as compared to
the severe form in which the tongue is fused to the floor of Incomplete merging and failure of groove obliteration by
the mouth underlying mesenchymal proliferation.
• Occurs in 1.7 to 4.4 percent of neonates Clinical Features
• Follows a male predilection
• May contribute to the development of an anterior open bite. • Associated with oral-facial-digital syndrome with thick,
• Clefting of tongue may be occasionally associated with fibrous bands in the lower anterior mucobuccal fold
ankyloglossia eliminating the sulcus and with clefting of the hypoplastic
• High mucogingival attachment leads to periodontal problems mandibular alveolar process.
• Food debris and microorganisms may collect at the base
of the cleft and cause irritation.

Management
No specific treatment is required, except for keeping the
cleft free of debris and inflammation.

FISSURED TONGUE
It is also called as scrotal tongue and lingual plicata. Fissured
tongue is a condition frequently seen in the general population
that is characterized by grooves that vary in depth and are noted
along the dorsal and lateral aspects of the tongue (Fig. 1.20).

Etiology
Largely unknown, although Eidelman et al, 1976, suspected a
polygenic mode of inheritance because the condition is seen
FIGURE 1.19: Tongue-tie due to high lingual frenal attachment clustering in families who are affected.44
Developmental Disturbances in Children 21

Management
No specific treatment is required, except for keeping the
cleft free of debris and inflammation by brushing the
dorsum of the tongue to eliminate debris that may serve
as an irritant.

MEDIAN RHOMBOID GLOSSITIS

Median rhomboid glossitis is a condition characterized by a
shiny oval or diamond-shaped elevation, invariably situated on
the dorsum of the tongue in the midline immediately in front
of the circumvallate papillae (Fig. 1.21).46

Clinical Features
• Median rhomboid glossitis presents in the posterior midline
of the dorsum of the tongue, just anterior to the V-shaped
grouping of the circumvalate papillae.
• The long axis of the rhomboid or oval area of red
depapillation is in the anteroposterior direction.
• The erythematous clinical appearance is due primarily to
FIGURE 1.20: Fissured tongue the absence of filiform papillae, rather than to local
inflammatory changes, as first suggested in 1914 by Brocq
Clinical Features
and Pautrier.47
• Usually asymptomatic, and the condition is initially noted • Most cases are not diagnosed until middle age of the
on routine intraoral examination as an incidental finding. affected patient, but the entity is, of course, present in
• Slight male predilection has been seen. childhood.
• May be diagnosed initially during childhood, but it is • 3:1 male predilection is seen.
diagnosed more frequently in adulthood. The prominence • Frequently, irritation occurs by consumption of alcohol, hot
of the condition appears to increase with increasing age. drinks or spicy foods.
• Fissured tongue is also associated with Melkersson- • When it occurs in adults, it may be caused due to
Rosenthal syndrome consisting of a triad of persistent or candidiasis. This has prompted a recent shift towards the
recurring lip or facial swelling, intermittent seventh (facial) more appropriate diagnostic term of posterior midline
nerve paralysis (Bell palsy) and a fissured tongue. atrophic candidiasis.
• Lesions with atrophic candidiasis are usually more
• Also associated with Down syndrome and benign migratory
erythematous but some respond with excess keratin
glossitis (geographic tongue).
production and therefore, show a white surface change.
• Fissured tongue affects the dorsum and often extends to
the lateral borders of the tongue. The depth of the fissures
varies but has been noted to be up to 6 mm in diameter.
• When particularly prominent, the fissures or grooves may
be interconnected, separating the tongue dorsum into what
may appear to be several lobules.

Histologic Features
• A biopsy is rarely performed because of its characteristic
diagnostic clinical appearance and little clinical significance.
• However, histologic examination has shown an increase
in the thickness of the lamina propria, loss of filiform
papillae of the surface mucosa, hyperplasia of the rete
pegs, neutrophilic microabscesses within the epithelium
and a mixed inflammatory infiltrate in the lamina FIGURE 1.21: Median rhomboid glossitis showing
propria.45 erythematous appearance
22 Essentials of Pediatric Oral Pathology

• Infected cases may also demonstrate a midline soft palate difficulty, it is recommended that the patient be treated with
erythema in the area of routine contact with the underlying topical antifungals prior to biopsy of a suspected median
tongue involvement; this is euphemistically referred to as rhomboid glossitis.
a kissing lesion.
• Lesions are typically less than 2 cm. in greatest dimension Management
and most demonstrate a smooth, flat surface, although it is
1. No treatment is necessary.
not unusual for the surface to be lobulated. Occasional 2. Nodular cases may be removed and recurrence after
lesions have surface mamillations raised more than 5 mm. removal is generally not noted.
above the tongue surface, and occasional lesions are located 3. Antifungal therapy (topical troches or systemic
somewhat anterior to the usual location. None have been medication) will reduce clinical erythema and
reported posterior to the circumvallate papillae. inflammation due to candida infection.

Histopathologic Features BENIGN MIGRATORY GLOSSITIS

• Median rhomboid glossitis shows a smooth or nodular (GEOGRAPHIC TONGUE)
surface covered by atrophic stratified squamous epithelium An inflammatory disease of the tongue characterized by
overlying a moderately fibrosed stroma with somewhat multiple annular areas of desquamation of the filiform papillae,
dilated capillaries. presenting as reddish lesions outlined in yellow that shift from
• Fungiform and filiform papillae are not seen, although area to area every few days.
surface nodules may mimic or perhaps represent anlage of
these structures. Etiology
• A mild to moderately intense chronic inflammatory cell
infiltrate may be seen within subepithelial and deeper • It has been reported with increased frequency in patients
fibrovascular tissues. with psoriasis and in patients with fissured tongue.
• Chronic candida infection may result in excess surface • Although the etiology is unknown, associations with human
keratin or extreme elongation of rete processes and leukocyte antigen (HLA)-DR5, HLA-DRW6 and HLA-
premature keratin production with individual cells or as CW6 have been reported.48
epithelial pearls (dyskeratosis) deep in the processes (Fig.
1.22). Silver staining for fungus will often reveal candida Clinical Features
hyphae and spores in the superficial layers of the
• The lesion shows female predilection, which may be related
epithelium. This pseudoepitheliomatous hyperplasia may
to hormonal factors.
be quite pronounced and the tangential cutting of such a
• It is more predominant in adults than in children.
specimen may result in the artifactual appearance of cut
• The tongue exhibits a well-demarcated area of erythema,
rete processes as unconnected islands of squamous
primarily affecting the dorsum, and often extending to
epithelium, leading to a mistaken diagnosis of well
involve the lateral borders of the tongue (Fig. 1.23).
differentiated squamous cell carcinoma. Because of this
• Within the area of erythema, the normal tongue architecture
is effaced, with loss of the filiform papillae and atrophy of
the overlying mucosa.
• Surrounding this area of erythema is a well-defined, hyper-
keratotic, yellow-white border with an irregular serpiginous
outline.

Histopathologic Features
• Lesional area shows a lining of keratinized, stratified
squamous epithelium with thin and elongated rete ridges.
• Epithelial area also shows spongiosis and acanthosis.
Sometimes, there occurs presence of neutrophils within the
FIGURE 1.22: Candidal hyphae within the superficial epithelium leading to formation of multiple abscesses
layer of the epithelium (Munro abscesses).
Developmental Disturbances in Children 23

FIGURE 1.23: Benign migratory glossitis showing FIGURE 1.24: Black hairy tongue showing black appearance
depapillation in some areas of tongue

• Connective tissue shows presence of neutrophils and • Papillae may appear brown, black or yellow depending on
lymphocytes. growth of pigment producing bacteria and staining of food
• Presence of neutrophilic infiltrate may be responsible for (Fig. 1.24).
destruction of superficial portion of epithelium producing • Tongue appears thick and matted and may occasionally
atrophic and reddened mucosa. involve the entire dorsal surface.
• Asymptomatic, but occasionally patients may complain of
Management a gagging sensation due to irritation from the elongated
No medical intervention is required because the lesion is papillae or also of a foul taste in the mouth.
benign and most often asymptomatic. • Overgrowth of candida albicans may result in
glossopyrosis (burning tongue).
HAIRY TONGUE • Clinically and etiologically distinct from hairy leukoplakia.

Hairy tongue is a common condition of defective desquamation Histopathologic Features

of the filiform papillae, characterized by marked accumulation • Diagnosis is usually clinical and since the lesion is
of keratin on the filiform papillae of the dorsal tongue. It is asymptomatic, biopsy is rarely performed.
also called as lingua nigra, lingua villosa nigra and black hairy • However, on histopathologic examination, marked elonga-
tongue. It is uncommonly seen in children. tion and hyperparakeratosis of the filiform papillae is seen
Etiology (Fig. 1.25).

Uncertain, but the following associated factors are seen: Management

• Antibiotic therapy
1. No treatment is necessary as it is a benign, asympto-
• Poor oral hygiene
matic condition.
• General debilitation 2. Esthetic appearance of the tongue may be improved
• Radiation therapy with excellent oral hygiene measures.
• Use of oxidizing mouthwashes or antacids 3. Desquamation of the hyperkeratotic papillae may be
• Overgrowth of fungal or bacterial organisms. encouraged by periodic scraping or brushing with a
toothbrush or tongue scraper.
Clinical Features
4. Keratolytic agents like podophyllin have been used
• Usually appears in the midline, just anterior to the circum- with variable success, but are not widely recommen-
vallate papillae, sparing the lateral and anterior borders. ded in children.
24 Essentials of Pediatric Oral Pathology

FIGURE 1.25: Histopathologic picture of black hairy tongue FIGURE 1.26: Lingual thyroid nodule presenting as a large
showing elongation and hyperparakeratosis of the filiform papillae nodular mass on the posterior surface of the tongue

LINGUAL VARICES • Seventy five percent of patients with infantile hypo-

thyroidism have some ectopic thyroid tissue which may
Lingual varices usually involve the lingual ranine veins
enlarge as a secondary phenomenon, compensating for
appearing as red or purple shot like clusters of vessels on the
thyroid hypofunction.
ventral surface and lateral borders of the tongue as well as in
the floor of the mouth (A varix is a dilated, tortuous vein, most
Diagnosis
commonly subjected to increased hydrostatic pressure but
poorly supported by surrounding tissue). Usually appear on the • Thyroid scan using iodine isotopes or technetium 99 m.
ventral surface of the tongue and floor of the mouth, but may • Computed tomography and magnetic resonance imaging
also occur on the upper and lower lip, buccal mucosa and help in delineating the size and extent of the lesion.
buccal commissure. Generally considered an age related • Biopsy is avoided due to risk of hemorrhage and also as it
disease and rarely seen in the pediatric age group. may be the patient’s only thyroid tissue.
• Incisional biopsy may be occasionally required in the adult
LINGUAL THYROID NODULE (Fig. 1.26) age group, to rule out malignant changes.
Lingual thyroid nodule is nothing but ectopic thyroid tissue
usually found between foramen caecum and epiglottis. In fact, Histopathologic Features
90 percent of all ectopic thyroids are found in this region. • The lesion shows multiple large pieces of thyroid tissue
with thyroid follicles of varying size and shape, lined by
Etiopathogenesis uniform cuboidal cells and filled with colloid (Fig. 1.27).
• Some follicles show cystic macrophages in the lumen.
Thyroid gland begins as an epithelial proliferation in the floor
of the pharyngeal gut during the 4-5th week of intrauterine life.
Management
By the seventh embryonic week, this thyroid bud normally
descends into the neck to its final resting position anterior to 1. No treatment is necessary, except for periodic follow-
the trachea and larynx. If the primitive gland does not descend up in asymptomatic patients.
normally, ectopic thyroid tissue may be found between foramen 2. Suppressive therapy with supplemental thyroid
cecum and epiglottis. hormone is usually recommended in symptomatic
patients.
Clinical Features 3. If hormone therapy does not help, surgical removal
or ablation with radioactive iodine-131 can be
• More frequent in females, probably due to hormonal performed.
influences. 4. Autotransplantation of the surgically excised mass to
• Symptoms may develop during puberty, adolescence, another body site can be attempted to maintain
pregnancy or menopause resulting in large nodular masses functional thyroid tissue and to prevent hypo-
which may block the airway, causing dyspnea, dysphagia, thyroidism.
dysphonia.
Developmental Disturbances in Children 25

In hyaline-vascular type, there occur non cancerous growths

in lymph nodes and there are no associated symptoms.
Plasma cell type is characterized by fever, weight loss, skin
rash, early destruction of red blood cells and hypergamma-
globulinemia.
A third form termed as multicentric Castleman’s disease
has also been described which is characterized by hepato-
splenomegaly.

DEVELOPMENTAL DISTURBANCES OF
THE SALIVARY GLANDS
This section has been discussed in detail in the chapter on
salivary gland diseases in children.
• Aplasia
FIGURE 1.27: Histopathologic picture of a lingual thyroid nodule • Xerostomia
showing thyroid tissue with thyroid follicles • Hyperplasia of palatal glands
• Atresia
DEVELOPMENTAL DISTURBANCES OF • Aberrancy
ORAL LYMPHOID TISSUE • Developmental lingual mandibular salivary gland
depression
• Reactive lymphoid aggregate
• Anterior lingual depression.
• Lymphoid hamartoma
• Angiolymphoid hyperplasia with eosinophilia: This
DEVELOPMENTAL DISTURBANCES
condition is rare in pediatric population and hence does
AFFECTING THE TEETH
not warrant consideration here.
• Lymphoepithelial cyst: Refer to the chapter on cysts in They may be further divided as:
children.
DEVELOPMENTAL DEFECTS IN SIZE OF TEETH
REACTIVE LYMPHOID AGGREGATE
Tooth size varies among different races and the sexes. The sizes
The lingual tonsil located on the dorsolateral aspect of posterior are mostly influenced by hereditary, genetic and environmental
portion of the tongue frequently becomes inflamed and enlarged. factors.
Such an enlargement may be unilateral or bilateral and has been • Microdontia
often referred to as ‘foliate papillitis’. Similar reactive • Macrodontia
hyperplasia may be seen as a firm, nodular, tender mass on the
buccal mucosa. Sometimes, lymphoid polyps may be seen on MICRODONTIA
the gingival, buccal mucosa, tongue and floor of the mouth.
It is the term implied for unusually small teeth. They are of
LYMPHOID HAMARTOMA two types: True and Relative. True form is applied to the teeth
It is also called as ‘Castleman tumour’. It is a rare disorder that are physically smaller. Sometimes, normal dentition
occurring in chest, stomach, neck, armpit, pelvis and pancreas. appears small due to presence of large jaws and then the term
An abnormal enlargement of lymph nodes in the form of masses relative microdontia is applied.
is seen in the above locations.
Clinical Features
Etiology • It is most commonly associated with hypodontia.
The cause of Castleman’s disease is unknown, but some • It shows a female predilection.
researchers have implicated the role of increased production • Most commonly seen in maxillary lateral teeth, called as
of interleukin-6 (IL-6). ‘peg laterals’. This appears as a reduction in mesiodistal
dimension and convergence towards the incisal edges.
Types • This condition occurs as an autosomal dominant trait with
There are two types of Castleman’s disease: prevalence in 0.8 to 8.4 percent individuals.
1. Hyaline-vascular type • Generalized microdontia occurs in Down syndrome and in
2. Plasma cell type pituitary dwarfism (Fig. 1.28).
26 Essentials of Pediatric Oral Pathology

Management
No specific treatment required.

DEVELOPMENTAL DEFECTS IN SHAPE OF TEETH

• Gemination
• Fusion
• Concrescence
• Dilaceration
• Talon’s cusp
• Dens in dente
• Dens evaginatus
FIGURE 1.28: Generalized microdontia showing
• Taurodontism
interdental spacing • Supernumerary roots

GEMINATION (Fig. 1.30)

Initially the term gemination was used for a tooth with a bifid
crown and a common root and root canal as a result of a single
tooth bud dividing into two. There was a lot of controversy
over this definition, as most of the investigators objected over
a joining of two teeth in case of maxillary central incisor or
joining of maxillary central incisor with a mesiodens to be
counted as a single tooth as for then, the tooth number would
be correct.
As far as current concepts are concerned, gemination is
defined as a single enlarged tooth or joined tooth in which the
tooth count is normal, when the anomalous tooth is counted as
one.
FIGURE 1.29: Macrodontia
Clinical Features
Management
• Occurs in both primary and permanent dentitions.
1. Cosmetic recontouring or veneering of a localized • Prevalence is 0.5 percent in deciduous teeth and 0.1 percent
hypodont tooth may be done to improve esthetics. in permanent dentition.
2. In case of peg laterals, full porcelain crowns are • Most commonly seen in maxillary anterior region.
recommended.
• Geminated teeth show the presence of a single root canal.

MACRODONTIA (FIG. 1.29)

Opposite of microdontia, it is the term applied to unusual large-
sized teeth. They are of two types: True and Relative. True form
is applied to the teeth that are physically larger. Sometimes,
normal dentition appears large due to crowding or due to
presence of smaller jaws and then the term relative macrodontia
is applied.

Clinical Features
• It is most often seen associated with hyperdontia.
• It occurs more commonly in males as compared to females.
• Diffuse macrodontia is seen in pituitary gigantism and
pineal hyperplasia with hyperinsulinalism. FIGURE 1.30: Gemination seen in maxillary central incisors
Developmental Disturbances in Children 27

CONCRESCENCE
Concrescence was defined as the union of two teeth by
cementum without confluence of dentin. The same definition
holds true in recent concepts.

Etiopathogenesis
It may be developmental or inflammatory. Developmental union
occurs when two teeth in close proximity fuse at the cementum.
Inflammatory union occurs when inflammatory changes at the
apical portion of the root of either of the teeth may resolve by
fusion of the apices of the two teeth at the cementum.

Clinical Features
FIGURE 1.31: Fusion of left maxillary central and lateral incisors • Most commonly seen in the maxillary posterior region.
• Developmental pattern mostly involves second molar tooth
Management in which its roots are closely placed with the adjacent
impacted third molar.
1. In deciduous dentition, if there is presence of
crowding, spacing and delayed or ectopic eruption of
• Post inflammatory pattern is seen in carious molars in which
underlying permanent teeth, extraction of the involved apices of the roots lie closely to the horizontal or distally
teeth is recommended. angulated third molars.
2. Surgical division with endodontic therapy of a • Post inflammatory concrescence must be kept in mind
permanent tooth may be performed to improve whenever extraction is planned for nonvital teeth with
esthetics. apices that overlie the roots of an adjacent tooth.

FUSION (FIG. 1.31) Management

Initially fusion was considered as the union of two normally 1. If union interferes with eruption, surgical removal is
separated tooth buds with the resultant formation of a joint tooth recommended.
with confluence of dentin. The current concept defines fusion 2. Extraction difficulties may be experienced on
as a single enlarged tooth or joined tooth in which the tooth attempted removal of a tooth that is unexpectedly
joined to its neighbor.
count reveals a missing tooth when the anomalous tooth is
counted as one.
DILACERATION
Clinical Features Dilaceration is an abnormal angulation or bend in the root or
• Occurs in both primary and permanent dentitions. crown of a tooth.
• Most commonly seen in maxillary anterior region.
• It is difficult to differentiate between gemination and fusion, Etiopathogenesis
but mostly separate root canals are seen in case of fused
It may arise from an injury that displaces the calcified portion
teeth.
of the tooth germ and remainder of the tooth is formed at an
• Most common occurrence is unilateral, but also occa-
abnormal angle. The bend may also develop due to presence
sionally bilaterally seen with the prevalence of 0.02 percent.
of an adjacent cyst, tumor or odontogenic hamartoma.
Management
Clinical Features
1. In deciduous dentition, if there is presence of
crowding, spacing and delayed or ectopic eruption of • It occurs most commonly in permanent maxillary incisors
underlying permanent teeth, extraction of the involved followed by mandibular anterior teeth.
teeth is recommended. • Deciduous teeth may be involved due to injury during
2. Surgical division with endodontic therapy of a neonatal laryngoscopy and endotracheal intubation.
permanent tooth may be performed to improve • Abnormal angulation may be present anywhere along the
esthetics. length of the tooth (Fig. 1.32).
28 Essentials of Pediatric Oral Pathology

FIGURE 1.32: Dilaceration of the root of maxillary central incisor FIGURE 1.33: Talon’s cusp in left maxillary central incisor

• Age of the patient and the direction and degree of force • It may be associated with other dental anomalies such as
appear to determine the extent of the tooth’s malformation. supernumerary teeth, odontomas, impacted teeth, peg
• Affected anterior maxillary teeth fail to erupt in the oral shaped lateral incisors, dens invaginatus.
cavity as compared to mandibular anterior teeth. • Syndromes associated with this anomaly are Rubinstein-
• Most of the mandibular anterior teeth erupt in the labial or Taybi syndrome and Sturge-Weber syndrome.
lingual direction and may be non-vital.
Radiographic Features
Management
On radiographs, the cusp appears overlying the central portion
1. Extraction is necessary when the involved tooth of the crown and includes enamel, dentin and occasionally a
prevents eruption of its successor.
pulp horn.
2. Root dilaceration affects the tooth if it is used as an
abutment due to concentration of the stresses
Management
abnormally. It can be prevented by splinting the
dilacerated tooth to the adjacent tooth. 1. Talon’s cusp may result in malocclusion, caries,
periodontal problems and irritation to the adjacent soft
TALON’S CUSP (FIG. 1.33) tissue. So to prevent this, one mode of treatment is
to remove the cusp. Intentional root canal therapy may
Talon's cusp is an extra cusp present on the lingual surface of be required for the tooth.
the anterior teeth, extending from the cementoenamel junction 2. Periodic grinding of the cusp with application of
to the incisal edge. It appears as a three pronged pattern desensitizing agent such as fluoride varnish is also
resembling an Eagle’s Talon, hence the name. recommended.
3. If excessive dentin is removed, the cusp is completely
Clinical Features ground and then application of calcium hydroxide and
placement of composite resin is done.
• Incidence of occurrence ranges from 1 to 8 percent. 4. If a developmental groove is left after grinding the
• It shows no sex predilection. cusp, it is restored with restorative materials, or sealed
• It may occur unilaterally or bilaterally. with pit and fissure sealants in order to prevent further
• Mostly affects permanent maxillary lateral incisors, complications of caries, lateral periodontitis and
followed by maxillary central incisors, mandibular incisors various other lesions.
and maxillary canines.
• It is rarely seen in children and mostly occurs on maxillary DENS IN DENTE
central incisors.
• A developmental groove is seen in the area where the cusp It is also known as dens invaginatus. The term dens in dente is
fuses with the involved tooth. used for the large invagination of dental tissue within a tooth
Developmental Disturbances in Children 29

• Occurrence is most common in maxillary dentition in

decreasing order of lateral incisors, central incisors,
premolars, canines and molars.
• It may be seen as an enlargement of the cingulum pit to a
deep infolding extending towards the apex.
• Coronal dens invaginatus is divided into three types (Fig.
1.35):
— Type I, where the invagination is seen in the crown.
— Type II, where the invagination extends below
cementoenamel junction that may or may not
communicate with the dental pulp.
— Type III, where the invagination extends through the
FIGURE 1.34: Coronal dens invaginatus seen on left maxillary root and perforates in the apical or lateral radicular area
lateral incisor without any communication with the pulp.
• Sometimes, the invagination may be so dilated that it does
not permit the eruption of the tooth, in such cases it is
termed as dilated odontome.
• Radicular dens invaginatus though rare, may be seen to
arise secondary to proliferation of the Hertwig's epithelial
root sheath. Altered enamel forms an invagination into the
dental papilla.

Radiographic Features
• In coronal dens invaginatus, the radiograph shows the
invagination into the crown involving enamel, dentin, with
or without involvement of pulp with extension only into
crown or up to the radicular area (Fig. 1.36).
• In radicular dens invaginatus, the radiograph shows
Type I Type II Type III enlarged roots.
FIGURE 1.35: Various forms of dens invaginatus

giving a resemblance of a tooth within the tooth. It is an

invagination of crown or root, which is lined by enamel. Ohlers
described this condition for the first time in 1957.49

Types
It is of two types:
1. Coronal: Invagination of crown portion (Fig. 1.34)
2. Radicular: Invagination of root portion.

Etiopathogenesis
It is hypothesized that before eruption, the lumen of
invagination is filled with soft tissue similar to the dental
follicle. This soft tissue later on loses its vascular supply and
becomes necrotic.

Clinical Features
• Coronal dens invaginatus is most commonly seen with a FIGURE 1.36: Radiographic picture of coronal dens
prevalence of 0.04 to 10 percent. invaginatus affecting the maxillary lateral incisor
30 Essentials of Pediatric Oral Pathology

• This anomaly is often associated with shovel shaped

incisors in which the incisors show prominent lateral
margins with hollowing in the center on lingual surface
resembling scoop of shovel.

Management
1. Dens evaginatus may result in fracture of the tooth
causing exposure of the pulp. In such cases, root
canal therapy or apexification with calcium hydroxide
is often recommended.
2. Grinding the tooth followed by direct or indirect pulp
capping with calcium hydroxide cement has been
suggested.
3. Placement of composite restoration to reinforce the
FIGURE 1.37: Mandibular premolar showing dens evaginatus
tooth before it reaches occlusal level is also
recommended.

TAURODONTISM
(Tauro= bull, dont= tooth). Taurodontism implies an anomaly
where there is an enlargement of the body of the pulp chamber
with apical displacement of the pulpal floor. It mostly occurs
in multi-rooted teeth. It is also called ‘bull tooth’ as it resembles
the molar of cud chewing animals.

Classification (Fig. 1.38)

It is divided into three types depending upon the degree of
Mild form Moderate form Severe form apical displacement of the pulpal floor as:
FIGURE 1.38: Various forms of taurodontism 1. Mild (hypotaurodontism).
2. Moderate (mesotaurodontism).
Management 3. Severe (hypertaurodontism).
1. Small Type I invaginations should be sealed upon
eruption to prevent caries and pulpal inflammation. Clinical Features
2. In case of large invaginations with caries, the carious • Tooth generally appears rectangular in shape with the pulp
lesion should be removed, calcium hydroxide base chambers showing increased apico-occlusal height with
placed and the lesion restored.
bifurcation close to the apex of the root.
3. When the invagination involves the pulp, endodontic
treatment is recommended.
• The anomaly usually affects permanent teeth and rarely
4. In case where the teeth have an open apex, apexifica- deciduous teeth.
tion with calcium hydroxide is successful. • It may be unilateral or bilateral.
5. Larger invaginations require extraction of the tooth, if • It may occur isolated or may be associated with some
endodontic therapy is unsuccessful. syndromes.
• It has also been reported in association with cleft lip and
DENS EVAGINATUS (FIG. 1.37) palate.
Dens evaginatus is the exact opposite of dens in dente. It is an
Syndromes Associated with Taurodontism
elevation of enamel in the central groove or lingual ridge of
the buccal cusp of premolar or permanent molar teeth. • Amelogenesis imperfecta, hypoplastic type, type IE
• Amelogenesis imperfecta taurodontism, type IV
Clinical Features • Ectodermal dysplasia
• It is usually bilateral and shows mandibular predominance. • Klinefelter syndrome
• The elevation in the form of cusp consists of normal • Oral-facial-digital syndrome, type II
enamel, dentin and pulp. • Down syndrome
Developmental Disturbances in Children 31

Management
1. No specific treatment required.
2. During endodontic therapy, it is difficult to locate,
instrument and obturate the pulp canals.
3. Removal of pulp tissue from the chamber also
requires meticulous removal.

SUPERNUMERARY ROOTS
It refers to the development of an increased number of roots
on a tooth compared with that classically described in dental
anatomy.

Clinical Features
• Seen in both primary and permanent dentitions.
• Most commonly seen in third molars due to a develop-
mental malformation. Other teeth affected are molars,
mandibular cuspids and premolars.
• At times, the additional root is small and superimposed over FIGURE 1.39: Hypodontia showing absence of teeth
other roots, hence difficult to diagnose.
Oligodontia is genetic as well and is the term most commonly
Management used to describe conditions in which more than six teeth are
1. No specific treatment required. missing.
2. Diagnosis is critical, as during endodontic procedures,
failure to discover these additional canals often results Etiology
in a lack of resolution of the associated inflammatory
process. • Absence of the entire dental lamina which results in absence
3. During extractions, it is important to remove all the of an entire dentition.
roots associated with the tooth, hence diagnosis of a • Absence of one or more tooth follicles resulting in partial
supernumerary root becomes critical as it is not anodontia.
always evident radiographically. • Absence of tooth follicles of the third molars, mandibular
second premolars and maxillary lateral incisors as evolution
DEVELOPMENTAL DEFECTS IN occurs.
NUMBER OF TEETH
Syndromes Associated with Anodontia
• Anodontia
• Cherubism
• Supernumerary teeth
• Mulibrey nanism syndrome
• Predeciduous dentition
• Gorlin-Chaudhry-Moss syndrome
• Acro-dermato-ungual-lacrimal tooth syndrome
ANODONTIA
• Ectodermal dysplasia (Margarita Island)
It is also called as agomphosis, agomphiasis. In dentistry, • Rieger syndrome
anodontia, also called anodontia vera, is a rare genetic disorder • Rothmund-Thomson syndrome
characterized by the congenital absence of all primary or • Hay-Wells syndrome
permanent teeth. Complete anodontia is usually part of a • Schopf-Schulz-Passarge syndrome
syndrome, usually hereditary hypohydrotic ectodermal • Rosselli-Gulienetti syndrome
dysplasia and seldom occurs as an isolated entity. • Rapp-Hodgkin ectodermal dysplasia syndrome
Partial anodontia, known as hypodontia or oligodontia, is • Witkop’s syndrome
the congenital absence of one or more teeth, which is relatively • Ellis-van Creveld syndrome
common. Congenital absence of all wisdom teeth, or third • Sener syndrome
molars, is relatively common. Hypodontia is genetic in origin • Incontinentia pigmenti
and usually involves the absence of 1 to 6 teeth (Fig. 1.39). • Focal dermal hypoplasia
32 Essentials of Pediatric Oral Pathology

FIGURE 1.40: Schematic representation of classification of supernumerary teeth

• van der Woude syndrome SUPERNUMERARY TEETH

• Ehlers-Danlos syndrome, classic type
• Focal dermal hypoplasia Definition
• Hutchinson Gilford syndrome. A supernumerary tooth is one that is additional to the normal
series and can be found in almost any region of the dental
Clinical Features arch.
• One or more teeth missing in the dental arch, with no
previous history of extraction or exfoliation. Classification
• Hypodontia has a prevalence of 3.5 to 8 percent (excluding See Fig. 1.40.
third molars) in the permanent dentition and less than
1 percent in the deciduous dentition (usually involving the Etiology
mandibular incisors).
• 20 to 23 percent of the population shows missing third • The etiology of supernumerary teeth is not completely
molars. understood.
• Female predominance of 1.5:1 is seen. • Various theories exist for the different types of super-
• After the molars, second premolars and lateral incisors are numerary teeth. One theory suggests that the supernumerary
most frequently absent. tooth is created as a result of a dichotomy of the tooth bud.50
• Hypodontia is also associated with microdontia, reduced Another theory is the hyperactivity theory, which suggests
alveolar development, increased freeway space and retained that supernumeraries are formed as a result of local,
primary teeth. independent, conditioned hyperactivity of the dental
lamina.51,52
Management • Heredity may also play a role in the occurrence of this
1. Management depends on the severity of the case. anomaly, as supernumeraries are more common in the
2. Endodontic therapy followed by obturation with gutta- relatives of affected children than in the general population.
percha may be done in order to retain a deciduous However, the anomaly does not follow a simple Mendelian
tooth whose succedaneous tooth is missing. A full pattern.
coverage crown may be placed to simulate the size
of its succedaneous tooth. Clinical Features
3. Prosthetic treatment of the missing teeth may be
required, which may include resin-bonded bridges or • Brook, 1974, found that supernumerary teeth were present
osseointegrated implants with associated prosthetic in 0.8 percent of primary dentitions and in 2.1 percent of
crowns. permanent dentitions.53
4. In children, usually an implant is not recommended • Occurrence may be single or multiple, unilateral or
until full dental maturation. bilateral, erupted or impacted and in one or both jaws.
Developmental Disturbances in Children 33

• While there is no significant sex distribution in primary

supernumerary teeth, males are affected approximately
twice as frequently as females in the permanent dentition.
• In the primary dentition, morphology is usually normal or
conical. There is a greater variety of forms presenting in
the permanent dentition. However, it is comparatively
uncommon in the deciduous dentition.
• Usually may be asymptomatic and may be discovered on
routine radiographic examination.
• Multiple supernumerary teeth are rare in individuals with
no other associated diseases or syndromes.
• The conditions commonly associated with an increased
prevalence of supernumerary teeth include:
— Cleft lip and palate: Supernumerary teeth associated
with cleft lip and palate result from fragmentation of
the dental lamina during cleft formation. The frequency
of supernumerary permanent teeth in the cleft area in
children with unilateral cleft lip or palate or both was
found to be 22.2 percent.54 FIGURE 1.41: Peg lateral with over-retained
— Cleidocranial dysplasia: The frequency of super- deciduous lateral incisor
numeraries in patients with cleidocranial dysplasia
ranged from 22 percent in the maxillary incisor region primary dentition is of the supplemental type and
to 5 percent in the molar region.55 seldom remain impacted.
— Gardner syndrome — Odontoma: Howard lists odontoma as the fourth
• Supernumerary teeth in permanent dentition may be of the category of supernumerary teeth. However, this
following types: category is not universally accepted. The term
— Conical: This small peg-shaped conical tooth is the “odontoma” refers to any tumor of odontogenic origin.
most commonly found supernumerary tooth in the Most authorities, however, accept the view that the
permanent dentition (Fig. 1.41). It develops with root odontoma represents a hamartomatous malformation
formation ahead of or at an equivalent stage to that of rather than a neoplasm. The lesion is composed of more
permanent incisors and usually presents as a mesiodens. than one type of tissue and consequently has been called
It may occasionally be found high and inverted into the a composite odontoma. Two separate types have been
palate or in a horizontal position. In most cases, described: the diffuse mass of dental tissue which is
however, the long axis of the tooth is normally inclined. totally disorganized is known as a complex composite
The conical supernumerary can result in rotation or odontoma (Fig. 1.42), whereas the malformation which
displacement of the permanent incisor, but rarely delays bears some superficial anatomical similarity to a normal
eruption. tooth is referred to as a compound composite odontoma.
— Tuberculate: The tuberculate type of supernumerary • Another rare type of supernumerary teeth is a “third set of
possesses more than one cusp or tubercle. It is frequently teeth” that forms underneath and pushes out the second set
described as barrel-shaped and may be invaginated. Root of teeth, much like the second set formed underneath which
formation is delayed compared to that of the permanent pushes out the first set of teeth.
incisors. Tuberculate supernumeraries are often paired
Clinical Significance of Supernumerary Teeth
and are commonly located on the palatal aspect of the
central incisors. They rarely erupt and are frequently • Failure of eruption: The presence of a supernumerary
associated with delayed eruption of the incisors. tooth is the most common cause of failure of eruption of a
— Supplemental: The supplemental supernumerary refers maxillary central incisor. It may also cause retention of the
to a duplication of teeth in the normal series and is primary incisor. The problem is usually noticed with the
found at the end of a tooth series. The most common eruption of the maxillary lateral incisors together with
supplemental tooth is the permanent maxillary lateral failure of eruption of one or both central incisors.
incisor, but supplemental premolars and molars also Supernumerary teeth in other locations may also cause
occur. The majority of supernumeraries found in the failure of eruption of adjacent teeth.
34 Essentials of Pediatric Oral Pathology

Management
1. Usually may be asymptomatic and may not require
any treatment.
2. Supernumerary teeth may compromise secondary
alveolar bone grafting in patients with cleft lip and
palate. Erupted supernumeraries are usually removed
and the socket site allowed healing prior to bone
grafting.
3. The presence of an unerupted supernumerary in a
potential implant site may compromise implant
placement. The supernumerary may require removal
prior to implant placement.
4. Extraction of the supernumerary tooth is recommended
when:
• Central incisor eruption has been delayed or
FIGURE 1.42: Odontome in addition to inhibited
the normal complement of teeth • Altered eruption or displacement of central
incisors is evident
• Displacement: The presence of a supernumerary tooth may • There is associated pathology
cause displacement of a permanent tooth. The degree of • Active orthodontic alignment of an incisor in close
displacement may vary from a mild rotation to complete proximity to the supernumerary is envisaged
displacement. Displacement of the crowns of the incisor • Its presence would compromise secondary
teeth is a common feature in the majority of cases alveolar bone grafting in cleft lip and palate
patients
associated with delayed eruption.
• The tooth is present in bone designated for
• Crowding: Erupted supplemental teeth most often cause implant placement
crowding. A supplemental lateral incisor may cause • Spontaneous eruption of the supernumerary has
crowding in the upper anterior region. The problem may occurred.
be resolved by extracting the most displaced or deformed 5. Di Biase, 1971, found that 75 percent of incisors
tooth. erupted spontaneously after removal of the
• Dentigerous cyst formation may be associated with supernumerary.56 Eruption occurred on average within
supernumerary teeth. Primosch, 1981, reported an enlarged 18 months, provided that the incisor was not too far
follicular sac in 30 percent of cases, but histological displaced and that sufficient space was available.
evidence of cyst formation was found in only 4 to 9 percent 6. If there is adequate space in the arch for the
of cases.55 Resorption of roots adjacent to a supernumerary unerupted incisor following supernumerary removal,
space maintenance can be ensured by fitting a simple
may occur but it is extremely rare.
removable appliance. If the space is inadequate, the
Radiographic Features adjacent teeth will need to be moved distally to create
space for incisor eruption. In that case, the primary
• The buccolingual position of unerupted supernumeraries canines may need to be extracted at the same time
can be determined using the parallax radiographic principle. as the supernumerary tooth.
The horizontal tube shift method utilizes two periapical 7. When there is adequate space and the incisor tooth
radiographs taken with different horizontal tube positions, fails to erupt, surgical exposure of the incisor and
whereas an occlusal film together with a panorex view is orthodontic traction is usually required.
routinely used for vertical parallax. If the supernumerary
PREDECIDUOUS DENTITION
moves in the same direction as the tube shift it lies in a
palatal position, but if it moves in the opposite direction Predeciduous teeth have been described as hornified epithelial
then it lies buccally. structures without roots, occurring on the gingiva over the crest
• Intraoral views may give a misleading impression of the of the ridge, which may be easily removed. They are thought
depth of the tooth. A true lateral radiograph of the incisor to arise from an accessory bud of the dental lamina ahead of
region assists in locating the supernumeraries that are lying the deciduous bud or from the bud of an accessory dental
deeply in the palate and enables the practitioner to decide lamina.
whether a buccal rather than a palatal approach should be Spouge and Feasby, 1966, believe that predeciduous teeth
used to remove them. as an entity is a misinterpretation and such structures present
Developmental Disturbances in Children 35

FIGURE 1.43: Extracted natal tooth FIGURE 1.44: Natal tooth with Riga Fede disease

at birth undoubtedly represent only the dental lamina cyst of being found in the study that relied on personal examination
the newborn. This cyst commonly projects above the crest of of patients.
the ridge, is white in color and is packed with keratin, so that • Kates et al, 1984, stated that 85 percent of the natal teeth
it appears “hornified” and can be easily removed.57 are usually mandibular incisors, 11 percent are maxillary
Natal teeth were first described by Massler, 1950.58 They incisors and 4 percent are posterior teeth. The vast majority
are also called as accessory teeth which may be present at or (90-99%) is primary teeth; only 1 to 10 percent are reported
shortly after birth (Fig. 1.43). to be supernumerary teeth.60
For the purpose of nomenclature, natal teeth are considered • Riga Fede disease: Sublingual ulceration may occur in
as those teeth present in newborns and neonatal teeth are those infants as a result of chronic mucosal trauma from adjacent
which appear in the oral cavity within the first 30 days of life.
anterior primary teeth, often associated with nursing. These
However, Neville states that this is an artificial distinction
distinctive ulcerations of infancy have been termed Riga
and all teeth should be called as natal teeth.
Fede disease and should be considered a variation of the
Spouge and Feasby, 1966, stated that natal teeth rarely
represent predeciduous supernumerary teeth; rather most are pre- traumatic eosinophilic ulceration (Fig. 1.44).
maturely erupted deciduous teeth and not supernumerary teeth. Histopathologic Features
Etiology Histologically the enamel in natal and neonatal teeth is normal
Several sources suggest a possible hereditary component. The for the age of the child, but when the teeth erupt prematurely
Tlinget Indians in Alaska show a prevalence of 9 percent of the uncalcified enamel matrix wears off because mineralization
their newborns having natal or neonatal teeth, 62 percent of is not complete. The teeth turn yellow-brown and the enamel
them had affected relatives.59 continuously breaks down. The usually increased mobility causes
Environmental factors, especially polychlorinated biphenyls histologic changes in the cervical dentin and cementum.
(PCBs) seem to increase the incidence of natal teeth. These Hertwig’s sheath may degenerate and root formation may be
children usually show other associated symptoms, such as prevented.
dystrophic finger nails, hyperpigmentation, etc.
Natal teeth are sometimes associated with various Management
syndromes like Jadassohn-Lewandowsky syndrome, Ellis-van 1. Natal teeth must be approached individually with
Creveld syndrome, Hallermann-Streiff syndrome, etc. sound clinical judgment guiding appropriate therapy.
2. According to Massler and Savara, if they represent
Clinical Features the deciduous dentition, they should not be extracted
hastily, if they do not pose a problem to the mother
• Prevalence of natal teeth is around 1:700 to 1:30,000
or the infant.
depending on the type of study; the highest prevalence
36 Essentials of Pediatric Oral Pathology

3. If the teeth are mobile, there is a high risk of aspiration TABLE 1.4: Classification of amelogenesis imperfecta
and extraction of the teeth is recommended. (Witkop and Sauk)
4. If the teeth are stable, they should be retained. Type I Hypoplastic
5. Riga Fede disease can be resolved with appropriate IA Hypoplastic, pitted autosomal dominant
measures. If teeth are conical, they can be ground IB Hypoplastic, local autosomal dominant
off to prevent trauma. Some authors recommend IC Hypoplastic, local autosomal recessive
splinting or disking of the affected teeth to prevent ID Hypoplastic, smooth autosomal dominant
traumatic injuries. If trauma does not resolve, IE Hypoplastic, smooth X-linked dominant
IF Hypoplastic, rough autosomal dominant
extraction of the teeth is recommended.
IG Enamel agenesis, autosomal recessive
6. Extraction of natal teeth should usually be followed
by administration of vit. K injections because, in Type II Hypomaturation
newborns, liver enzymes are functionally not well IIA Hypomaturation, pigmented autosomal recessive
developed and hence deficiency of vit. K derived IIB Hypomaturation
factors like Factors VII, IX and X are likely. However, IIC Snow capped teeth, X-linked
IID Autosomal dominant?
hypothrombinemia should no longer be a concern,
since newborns are routinely given vitamin K to Type III Hypocalcification
prevent this problem. IIA Autosomal dominant
7. Teeth that are stable beyond 4 months have a good IIB Autosomal recessive
prognosis. Esthetically they are not pleasing due to Type IV Hypomaturation—Hypoplastic with taurodontism
their discoloration. IVA Hypomaturation—Hypoplastic with taurodontism, autosomal
dominant
DEVELOPMENTAL DEFECTS IN IVB Hypoplastic—Hypomaturation with taurodontism, autosomal
STRUCTURE OF TEETH dominant

• Amelogenesis imperfecta molecular and cellular activities that take place during its
• Environmental enamel hypoplasia genesis. These activities are controlled by a regulated expres-
• Dentinogenesis imperfecta sion of multiple genes.64 Deviation from this pattern leads to
• Dentin dysplasia
amelogenesis imperfecta. Witkop and Sauk listed the varieties
• Regional odontodysplasia
of AI, divided according to whether the abnormality lay in a
• Dentin hypocalcification
reduced amount of enamel (hypoplasia), deficient calcification
(hypocalcification), or imperfect maturation of the enamel
AMELOGENESIS IMPERFECTA
(hypomaturation) and also recognized combined defects
Amelogenesis imperfecta (AI) is a diverse collection of inherited (Table 1.4).65
diseases that exhibit quantitative or qualitative tooth enamel Aldred et al, 2003 has given a classification based on:66
defects in the absence of systemic manifestations. Also known • Mode of inheritance
by varied names such as hereditary enamel dysplasia, hereditary • Phenotype—Clinical and Radiographic
brown enamel, hereditary brown opalescent teeth, this defect is • Molecular defect (when known)
entirely ectodermal, since mesodermal components of the teeth • Biochemical result (when known)
are basically normal. The AI trait can be transmitted by either Amelogenesis imperfecta (AI) is a developmental, often
autosomal dominant, autosomal recessive or X-linked modes of inherited disorder affecting dental enamel. It usually occurs in
inheritance. Genes implicated in autosomal forms are genes the absence of systemic features and comprises diverse
encoding enamel matrix proteins: enamelin and ameloblastin, phenotypic entities.
tuftelin, MMP-20 and kallikrein-4.
Tooth enamel is the most highly mineralized structure in Etiopathogenesis
the human body, with 85 percent of its volume occupied by
unusually large, highly organized hydroxyapatite crystals.61,62 • The trait of AI can be transmitted by an autosomal-
The physical properties and physiological function of enamel dominant, autosomal-recessive, or X-linked mode of
are directly related to the composition, orientation, disposition, inheritance.
and morphology of the mineral components within the tissue.63 • Some of the genes encoding specific enamel proteins have
During organogenesis, the enamel transitions from a soft and been indicated as candidate genes for amelogenesis
pliable tissue to its final form, almost entirely devoid of protein. imperfecta. Mutational analyses within studied families
The final composition of enamel is a reflection of the unique have supported this hypothesis.
Developmental Disturbances in Children 37

• The amelogenin gene is a tooth-specific gene expressed in amelogenesis imperfecta (hypoplastic-hypomaturation

pre-ameloblasts, ameloblasts and in the epithelial root type), with taurodontism (AIHHT).
sheath remnants;67 while a low expression of amelogenin • However, AMELX, AMBN, ENAM, KLK4 and MMP-20
mRNAs has been recently shown in odontoblasts.68,69 To were excluded from a causative role in two families with
date, there are 14 AMELX-associated AI mutations.70,71 autosomal-dominant hypocalcified AI, suggesting that this
• The enamelin (ENAM) gene is a tooth-specific gene type of AI is caused by mutation of a gene that is either
expressed predominantly by the enamel organ and at a low not known or not considered to be a major contributor to
level, in odontoblasts. The human ENAM gene is localized enamel formation.81
on chromosome 4 (4q13.3). One autosomal-inherited form • Mutations in the amelogenin gene (AMELX) cause X-
of AI, namely, autosomal-dominant amelogenesis linked amelogenesis imperfecta, while mutations in the
imperfecta (ADAI), was linked to a 4Mb region on 4q21. enamelin gene (ENAM) cause autosomal-inherited forms
The ENAM gene has been mapped within this locus by of amelogenesis imperfecta. Recent reports involve
radiation hybrid analysis (RHA) and fluorescent in situ kallikrein-4 (KLK4), MMP-20 and DLX3 genes in the
hybridization (FISH) and was therefore considered a etiologies of some cases.82
candidate gene for this type of AI.72,73
• The ameloblastin (AMBN) gene is expressed at high levels Clinical Features
by ameloblasts and at low levels by odontoblasts and pre-
• The predominant clinical manifestations of affected
odontoblasts, while moderate expression is also observed
individuals are enamel hypoplasia (enamel is seemingly
in Hertwig’s epithelial root sheath, and in odontogenic
correctly mineralized but thin), hypomineralization
tumors, such as in ameloblastomas.74 The human AMBN
(subdivided into hypomaturation and hypocalcification), or
is localized on chromosome 4 at locus 4q21 near other
a combined phenotype, which is seen in most cases.
genes associated with mineralized tissues: osteopontin,
• The prevalence of this condition has been estimated to
bone sialoprotein and bone morphogenetic protein 3.
range from 1 in 78 to 1 in 14,000, depending on the
AMBN maps within the critical region for autosomal-
population studied. Hypoplastic (AI) represents 60 to 73
dominant AI and therefore is considered as a candidate
percent of all cases, hypomaturation (AI) represents 20 to
gene. However, it was excluded from a causative role by
40 percent and hypocalcification (AI) represents 7
mutational analyses within the families studied by Màrdh-
percent.83
Kärrman C et al.75
• The distribution of AI types is known to vary among
• The KLK4 gene is located near the telomere of
different populations. In a study in Sweden, 63 percent of
chromosome 19 (19q13.3–19q13.4) downstream of the
the cases were inherited as autosomal-dominant. In contrast,
KLK2 gene and is considered a member of the human tissue
in a study in the Middle East, the most common prevalent
kallikrein gene family.76,77 KLK4 is expressed by both
type of AI was found to be autosomal-recessive.
ameloblasts and odontoblasts.
• Due to abnormal enzymic activity, the crystallites of the
Radiographic Features
enamel grew to the normal length but incompletely in
thickness. • The enamel may appear totally absent on the roentgeno-
• MMP-20 gene codes for a calcium-dependent proteinase gram, or when present, may appear as a very thin layer,
that is a member of the matrix metallopeptidases family chiefly over the tips of the cusps and on the interproximal
(MMPs). Additionally, no other intact, physiologically surfaces.
normal, tissue has been demonstrated to express MMP-20, • In other cases the calcification of enamel may be so affected
apart from ameloblasts, pre-ameloblasts and odontoblasts, that it appears to have the same approximate radiodensity
whereas the expression of MMP-20 in human odontogenic as the dentin, making differentiation between the two
tumors and carcinoma cell lines originating from the tongue difficult.
has recently been described.78,79 The four major clinical variants have typical clinical and
• The DLX3 gene is a member of the family of homeobox radiographic features.
genes that are homologous to the distalless (Dll) gene of 1. Hypoplasia: Enamel thickness is reduced such that the
Drosophila, known to be expressed during development of dentin shows through and imparts a yellowish-brown color
the chondrocranium, dermatocranium, sensory organs, to the tooth. The enamel may be pitted, rough, smooth or
brain, limbs and appendages and in the processes of glossy. Radiographs demonstrate a square shaped crown,
osteogenesis and hematopoiesis.80 Mutation within the a thin layer of enamel of normal density and low or absent
human DLX3 gene homeodomain is associated with cusps (Figs 1.45 and 1.46).
38 Essentials of Pediatric Oral Pathology

3. Hypocalcification: Enamel tends to fracture away shortly

after it comes into function. An explorer point under
pressure can penetrate the soft enamel. Yet, caries is
unusual. Radiographically, enamel thickness appears to be
normal but the density is even less as compared to dentin.
4. Hypomaturation/hypocalcification: The term is referred to
depending on the dominant defect. The enamel is usually
mottled and discolored. Both the deciduous and permanent
dentitions are involved diffusely. In the hypomaturation
hypoplastic pattern, the predominant defect is one of
enamel hypomaturation in which the enamel appears as
mottled yellowish-white to yellow-brown. Pits are seen
frequently on the buccal surfaces of the teeth.
FIGURE 1.45: Clinical appearance of teeth affected by Radiographically, the enamel appears similar to dentin in
amelogenesis imperfecta density, and large pulp chambers may be seen in single
rooted teeth in addition to varying degrees of taurodontism.
In the hypoplastic- hypomaturation pattern, the predominant
defect is one of enamel hypoplasia in which the enamel is
thin; the enamel that is present demonstrates
hypomaturation. Except for the decrease in the thickness
of enamel, this pattern is similar to the hypomaturation-
hypoplastic variant.84

Histopathologic Features
• Histologically, a ground section of teeth involved shows
FIGURE 1.46: Radiographic appearance of teeth affected by very thin enamel composed of laminations of irregularly
amelogenesis imperfecta arranged enamel prisms (Fig. 1.47).85
• SEM studies of the extracted deciduous teeth in a case of
autosomal recessive rough hypoplastic amelogenesis
imperfecta showed the exposed outer enamel surface with
irregularly shaped globular protrusions. At the cervical
region of the crown, a series of wavy, parallel ridges was
seen in the enamel regions. The cementum area was clearly
distinguishable from the more coronal region by its mottled
and fibrillar pattern and the tendency for the cementum to
overlap the ridged coronal structure along the cervical line.
Enamel had a high organic content with some abnormal
prism formation. The dentinoenamel junction was sharply
defined and easily identifiable because of the more
homogenous appearance of the enamel matrix, as compared
with that of the dentin with its array of collagen fibrils.86
• The histology of autosomal dominant hypomaturation—
FIGURE 1.47: Ground section of amelogenesis imperfecta showing hypoplasia type of AI with taurodontism, definitively
a reduced enamel thickness and composed of laminations of described by Winter et al, comprises areas of severe
irregularly arranged enamel prisms hypomineralization with a pore volume of between 1 percent
and 25 percent. They described a normal prismatic structure
2. Hypomaturation: The enamel is softer with a normal to the enamel, but with considerable post-calcification
thickness but mottled appearance. The teeth may appear organic content and occasional bands of globular defects.
to be snow - capped. Radiographs reveal enamel whose The dentine was also reported as being defective, with a
density is the same as that of dentin. decreased number of tubules, an increased amount of
Developmental Disturbances in Children 39

intertubular dentin, dilatations and cellular inclusions. All • Inherited diseases: Amelo-cerebro-hypohydrotic syndrome,
these findings were more marked in the radicular dentin. The epidermolysis bullosa, mucoploysaccharidosis IV,
pulp was normal but enlarged in size.87 phenylketonuria, pseudohypoparathyroidism, tuberous
sclerosis, vitamin D dependent rickets
Management • Malnutrition: Generalized malnutrition, vitamin D
1. Management usually involves complete oral
deficiency, vitamin A deficiency
rehabilitation by way of full coverage crowns, direct • Metabolic disorders: Cardiac disease, celiac disease,
and indirect veneers and bonded esthetic hepatobiliary disease, hypocalcemia, hypothyroidism,
restorations, depending on the condition of the hypoparathyroidism, maternal diabetes, toxemia of
individual tooth and the age of the patient. pregnancy
2. Types ID, IE, IG, IIA, IIIA, IIIB and IVB demonstrate • Neurologic disorders: Cerebral palsy, mental retardation,
very thin enamel or highly defective enamel, which sensorineural hearing defects.
leads to rapid attrition. These variants require full Local
coverage as soon as is practical. If the treatment is • Local acute mechanical trauma: falls, neonatal mechanical
delayed, a loss of usable crown length occurs. In
ventilation, surgery
those patients without sufficient crown lengths, full
• Electric burn
dentures (overdentures in some cases), often become
the only satisfactory approach.
• Irradiation
3. The other types of AI demonstrate less rapid tooth • Local infection: acute neonatal maxillitis, periapical
loss and the esthetic appearance is the prime inflammatory disease
consideration. In some cases, a lack of good enamel The enamel develops in three major stages:
bonding of veneers occurs and does not result in a 1. Matrix formation
durable restoration. The use of glass ionomer 2. Mineralization
cements with dentinal adhesives often overcomes this 3. Maturation
weakness.
The timing of the ameloblastic damage has a great impact
4. Thus, the dentist has to balance the decision for early
intervention and the long-term survival of the
on the location and appearance of defect in the enamel. The
restorations. Dental practitioners should consider the cause of the damage is not particularly important as many local
social implications for these patients and intervene and systemic stimuli can result in defects having similar clinical
to relieve their suffering. appearances. The final enamel presents all significant insults
received during tooth developments as various defects in it.
ENVIRONMENTAL ENAMEL HYPOPLASIA Deciduous enamel contains a neonatal ring and the rate of
enamel apposition is estimated to be 0.023 mm per day. This
This term implies the defective formation of organic enamel helps the clinician to accurately time an insult to the deciduous
matrix of teeth. It is of two basic types: teeth within a week.
1. Environmental, i.e. influenced by environmental forces.
2. Hereditary Clinical Features
Ameloblasts in the developing tooth germ are extremely
• When examining enamel defects, it is imperative to clean
sensitive to external stimuli and many factors can result in
the dentition thoroughly and dry it with gauze. A good
abnormalities in enamel. source of light from a dental operatory is ideal. Plaque
Etiology disclosing agents may be used for minor defects.
• Depending on the extent and stage at which enamel
The etiological causes may be considered as: formation has been disrupted, all enamel defects can be
Systemic clinically classified into one of three patterns:
• Birth related trauma: Breech presentations, hypoxia, — Hypoplasia: Appears as pits, grooves or larger areas
multiple births, premature birth, prolonged labor of missing enamel.
• Chemicals: Antineoplastic chemotherapy, fluoride, lead, — Diffuse opacities: Appear as variations in translucency
tetracycline, thalidomide, vitamin D of enamel. The thickness of enamel is normal, but with
• Chromosomal abnormalities: Trisomy 21 increased white opacity which has no clear demarcation
• Infections: Chickenpox, cytomegalovirus (CMV), from the adjacent normal enamel.
gastrointestinal infections, measles, pneumonia, respiratory — Demarcated opacities: Appear as areas of decreased
infections, rubella, syphilis, tetanus translucence, increased opacity and a sharp boundary
40 Essentials of Pediatric Oral Pathology

with the adjacent enamel. The thickness of enamel is with mild developmental defects in both enamel and dentin.
normal and the affected opacity may be white, cream, Apart from dental defects, hypodontia, microdontia
yellow or brown. mandibular hypoplasia, reduction of the vertical
• Environmental enamel abnormalities are extremely development of the face and reduced alveolar bone growth
common. Small and Murray, 1979, reported that between may occur secondary to radiation.
the ages of 12 to15 years, the prevalence of enamel defects • In 1901, Dr Frederick McKay, first reported Colorado
in the permanent dentition was 68.4 percent with 67.2 brown stains which were later found by HV Churchill in
percent showing opacities, 14.6 percent showing hypoplasia 1930 to have been caused by high concentrations of fluoride
and 13.4 percent showing both hypoplasia and opacities.88 (13.7 ppm).89 Dr Trendley H Dean carried out a shoe
• Crowns of deciduous teeth start developing from 14th week leather survey in 1931 and recommended the presence of
of gestation till approximately when the child is 12 months an optimum level of 1ppm F in drinking water to minimize
of age. Crowns of permanent dentition start developing caries, strengthen teeth at the same time avoiding the risk
from 6 months to approximately 15 years of age. The site of dental fluorosis.90 Dental fluorosis is the presence of
of coronal damage correlates with the area of ameloblastic significant enamel defects resulting from ingestion of
activity at the time of injury. excessive amounts of fluorides during stages of tooth
• Systemic influences like exanthematous fevers that often formation. The appearance of these defects depends upon
occur during the first-two years of life commonly present the level of fluoride in water as well as the time and duration
as horizontal rows of pits or diminished enamel on the for which the developing tooth was exposed to the
anterior teeth and first molars. excessive levels of fluorides. Dental fluorosis may occur
• A similar pattern of enamel defects appears on the cuspids, due to excessive levels of fluoride in water, but apart from
bicuspids and second molars when the event occurs at the this a significant ingestion of fluoride may occur from
age of 4 to 5 years. fluoride toothpastes, fluoride supplements, infant formulae,
• Another frequent appearance is the Turner’s tooth caused soft drinks, fruit juices and industrial environmental
due to Turner’s hypoplasia of the affected tooth. Turner was emissions. These may create significant enamel defects
the dental clinician who widely publicized the presence of through retention of amelogenin proteins in the enamel
such a pattern of hypoplasia. Turner’s hypoplasia can be structure, leading to the formation of hypomineralized
described as enamel defects seen in the permanent teeth enamel. These alterations create a permanent hypomatura-
due to disruption of permanent teeth caused by periapical tion of the enamel in which there is an increased surface
inflammatory disease of the overlying deciduous tooth. and subsurface porosity of the enamel. This enamel
Quite a variation of defects occurs as white, yellow or structure alters the light reflection and creates the
brown discoloration to extensive hypoplasia depending appearance of white, chalky areas. The teeth affected by
upon the time and severity of the insult. Usually seen in fluorosis are caries resistant and the altered tooth structure
the permanent premolars because of their relationship to appears as areas of lustureless white opaque enamel that
overlying deciduous molars. may have zones of yellow to dark brown discoloration. True
• Turner’s teeth may also occur due to traumatic injury to enamel hypoplasia is uncommon but can occur as deep
deciduous teeth commonly seen in permanent maxillary irregular brownish pits. Clinical diagnosis is usually based
central incisors. As the permanent incisors develop lingual on history of excessive fluoride intake.
to the primary teeth, the facial surface of the maxillary • Congenital syphilis results in a distinct pattern of hypoplasia
incisors is the location frequently affected appearing as a that is extremely rare currently. Anterior teeth are called
zone of white or yellowish brown discoloration with or as Hutchinson’s incisors and exhibit crowns that are shaped
without an area of horizontal enamel hypoplasia. Trauma like straight edged screw-drivers with the greatest
leading to displacement of the already formed hard tooth circumference present in the middle one-third of the crown
substance may result in dilaceration and severe trauma early and a constricted incisal edge. The middle portion of the
in the development of tooth may result in such incisal edge often demonstrates a central hypoplastic notch.
disorganization of the bud that the resultant product may Altered posterior teeth are called as mulberry molars
result in complex odontoma. (Moon’s molars, Fournier’s molars) and demonstrate a
• Hypoplasia may also arise secondary to the use of constricted occlusal table with a disorganized surface
therapeutic radiation or chemotherapy provided against anatomy that resembles the bumpy surface of a mulberry.
childhood cancer, most commonly in patients under the age • Syndromes associated with hypoplasia:
of twelve, with extensive defects occurring in those under — Down syndrome
five years of age. Doses as low as 0.72 Gy are associated — Tuberous sclerosis
Developmental Disturbances in Children 41

— Epidermolysis bullosa
— Hurler syndrome
— Hunter syndrome
— Treacher Collins syndrome
— Phenylketonuria
— Pseudohypoparathyroidism
— Trichodento-osseous syndrome
— Vitamin D dependent rickets
— Lesch-Nyhan syndrome
— Fanconi’s syndrome
— Sturge-Weber syndrome
— Turner’s syndrome

Management
FIGURE 1.48: Clinical picture of dentinogenesis imperfecta
1. Management usually involves restoration of the showing opalescent dentin
esthetic defect, depending on the condition of the
individual tooth and the age of the patient. A revised classification mentions only dentinogenesis
2. Sometimes surface microabrasion is sufficient to imperfecta 1 which corresponds to Shields type II and dentino-
eliminate a minor defect in the enamel. genesis imperfecta 2 which corresponds to Shields type III.
3. Further cosmetic restoration may be done with the Another classification was given by Witkop in 1975 that
help of acid-etched composite resin restorations,
divides dentin defects into three types:92
veneers and full crowns.
1. Dentinogenesis imperfecta
2. Hereditary opalescent teeth
DENTINOGENESIS IMPERFECTA (FIG. 1.48) 3. Brandywine isolate
Dentinogenesis imperfecta is a hereditary developmental
disturbance of dentin in the absence of any systemic disorder. Etiology
This condition causes the teeth to be discolored (most often a • Mutations in the DSPP gene (gene map locus 4q21.3) cause
blue-gray or yellow-brown color) and translucent. Teeth are dentinogenesis imperfecta. They have been identified in
also weaker than normal, making them prone to rapid wear, people with type II and type III dentinogenesis imperfecta.
breakage and loss. These problems can affect both primary Mutations in this gene are also responsible for dentin
teeth and permanent teeth. dysplasia type II. Dentinogenesis imperfecta type I occurs
as part of osteogenesis imperfecta, which is caused by
Classification mutations in one of several other genes.
• The DSPP gene provides instructions for making proteins
Shields, 1973, have described three types of dentinogenesis like dentin phosphoprotein, dentin sialoprotein and dentin
imperfecta:91 sialophosphoprotein that are essential for normal tooth
1. Type I occurs in people who have osteogenesis imperfecta, development. These proteins are involved in the formation
a genetic condition in which bones are brittle and easily of dentin. DSPP mutations alter the proteins made from the
broken. gene, leading to the production of abnormally soft dentin.
2. Dentinogenesis imperfecta type II occurs in people without It is unclear how DSPP mutations are related to hearing
other inherited disorders and without any association loss in some families with dentinogenesis imperfecta
genetically with osteogenesis imperfecta. A few families type II.
with type II have progressive hearing loss in addition to • This condition is inherited in an autosomal dominant
dental abnormalities. pattern. In most cases, an affected person has one parent
3. Type III was first identified in a population in Brandywine, with the condition.
Maryland. Some researchers believe that dentinogenesis • Sauk et al, 1976, found an increase in glycosaminoglycans
imperfecta type II and type III, along with a similar in EDTA soluble dentin in the teeth from patients of
condition called dentin dysplasia type II, are actually forms dentinogenesis imperfecta and less glycosaminoglycans in
of a single disorder. EDTA insoluble residue.93
42 Essentials of Pediatric Oral Pathology

Clinical and Radiographic Features ued deposition of the dentin. Odontoblast may degenerate
readily and become entrapped in disorganized dentin
• Dentinogenesis imperfecta affects an estimated 1 in 6,000
matrix.
to 8,000 children.
• Dentinogenesis imperfecta 2: Histopathology has not been
• The teeth usually involved and more severely affected are
adequately documented.
deciduous teeth in type 1, whereas in type 2 both the
dentitions are equally affected. Management
• Common clinical features are bluish-gray teeth, amber-
1. Early and accurate diagnosis of dentinogenesis
colored teeth, bulbous teeth crowns, absent tooth roots,
imperfecta is of utmost importance to enable proper
absent root canals, absent pulp chambers, too small tooth
preventative interventions and optimum dental
roots, too small root canals, too small pulp chambers, handling.
enamel separation from the dentin, malaligned teeth, 2. Providing optimal oral health treatment for
recurring dental abscess, brittle bones and blue sclera. dentinogenesis imperfecta frequently includes
• Dentinogenesis imperfecta 1: Blue-gray or amber brown preventing severe attrition associated with enamel
and opalescent teeth with bulbous crowns. Radiograph loss and rapid wear of the poorly mineralized dentin,
reveals narrow roots, small pulp chambers and root canals rehabilitating dentitions that have undergone severe
which may be completely obliterated. Studies of dentin wear, optimizing esthetics and preventing the
have revealed an increase in as much as 60 percent of water common dental problems associated with caries and
content above the normal level, whereas the inorganic periodontal disease.
3. Placement of restorations may be compromised due
content is less than that of normal dentin. Likewise, the
to inadequate adhesion between dentin and the
density, X-ray absorption and hardness of dentin are low.
restorative material.
Microhardness of dentin is similar to cementum which 4. Prefabricated stainless steel crowns may be placed
explains the rapid attrition of the affected teeth. on deciduous molars to prevent attrition and collapse
• Dentinogenesis imperfecta 2: The crowns of deciduous and of the bite, as also to improve function.
permanent teeth wear rapidly after eruption and multiple 5. Full coverage cast metal crowns are preferred on
pulp exposures may occur. The dentin is amber and smooth. permanent posterior teeth; and porcelain fused to
Radiographs of deciduous dentition reveal very large pulp metal crowns may be used for the anterior teeth.
chambers and root canals. The permanent teeth have pulpal 6. Endodontic treatment is occasionally necessary and
spaces that are small or completely obliterated. Non- has a guarded prognosis if initiated after pulp canal
mineralized pulp chambers and canals are seen giving a obliteration has occurred.
general appearance of shell teeth (Fig. 1.49).
DENTIN DYSPLASIA
Histopathologic Features Dentin dysplasia (DD) is a genetic disorder of teeth,
• Dentinogenesis imperfecta 1: Shows the appearance of characterized by presence of normal enamel but atypical dentin
normal enamel except for a change in shade which is a with abnormal pulpal morphology. In 1920 Balchsmiede first
manifestation of the dentinal disturbance. Dentin shows reported 8 cases as “root less teeth”.94 Later, Rushton, in 1939,
irregular tubules with large areas of uncalcified matrix. The described it as “Dentinal Dysplasia” (DD).95
tubules are larger in diameter and less numerous than
normal dentin. They may be completely absent in few areas. Classification
Cellular inclusions probably odontoblasts are also seen and Carrol et al, published an extensive review of literature and
the pulp chamber is usually almost obliterated by the contin proposed a subclassification based on the radiographic findings.
They proposed 2 basic types: Type 1 was classified into 4
subtypes; 1a, 1b, 1c and 1d.96
• In type 1a, there is a complete obliteration of the pulp and
usually little or no root development.
• The Type 1b variant has a horizontal, crescent shaped,
radiolucent line, which separates normal coronal dentin
from abnormal radicular dentin. The roots are short, conical
and rudimentary.
• Teeth affected by type 1c variant show 2 crescent-shaped
FIGURE 1.49: Radiographic picture of dentinogenesis imperfecta horizontal radiolucent lines with their concavities toward
Developmental Disturbances in Children 43

each other at the cemento-enamel junction and the roots

one half the normal length.
• Type 1d is characterized by normal root formation, which
sometimes may be bulbous in the coronal third. Within the
pulp canal “a stone” may be found. In this type of DD, the
pulp chamber is usually not obliterated and normal root
formation occurs. This is the least severe form of DD. In
these cases, the pulp around the stones is healthy. In other
cases, the denticle is continuous with dentinal walls.
• The second type of DD, DD2 is characterized radio-
graphically by extension of the pulp chamber into the root,
pulp stones and sudden constriction of the chamber, which
forms a thin radiolucent radicular structure. FIGURE 1.50: Clinical features of dentin dysplasia
• Dean et al 97 1997, suggested that an updating of the
nomenclature for the developmental disturbances of dentin
is indicated, comparable to that presented by Aldred and
Crawford, 1995, for developmental disturbances in
amelogenesis imperfecta.

Etiology
• The similarity of the primary dentition phenotype between
dentinogenesis imperfecta 1 (DGI1) and type II dentin
dysplasia suggested that the gene for dentin dysplasia,
type II is allelic with the gene for dentinogenesis imperfecta,
Shields type II (DGI1), the isolated form of dentinogenesis
imperfecta that has been shown by linkage studies to be
FIGURE 1.51: Radiographic picture of dentin dysplasia showing
encoded by a gene on 4q13–q21. Microsatellite markers almost complete obliteration of the pulp chamber
specific for the area of 4q linked to DGI1 were used. Dean
et al, concluded that any candidate gene for DGI1 should chamber and periapical radiolucent areas or cysts. The pulp
also be considered a candidate gene for dentin dysplasia, chamber is sometimes described as having a “crescent
type II. shaped” appearance.
• On the basis of the phenotypic overlap between, and shared • Dentin dysplasia type II (coronal type) is characterized by
chromosomal location with dentinogenesis imperfecta primary teeth with complete pulpal obliteration and brown
type II, it has been proposed that dentin dysplasia type II or amber bluish coloration similar to that seen in hereditary
and dentinogenesis imperfecta type II are allelic. The opalescent dentin. The permanent teeth have a normal
substitution in the hydrophobic signal peptide domain appearance or a slight amber-coloration, the roots are
caused a failure of translocation of the encoded proteins normal in size and shape with a “thistle-tube” shaped pulp
into the endoplasmic reticulum. The authors hypothesized chamber with pulp stones (Figs 1.50 and 1.51). In the
that this would most likely lead to a loss of function of both deciduous dentition, coronal dentin dysplasia bears a
dentin sialoprotein and dentin phosphoprotein. resemblance to Dentinogenesis Imperfecta type II.
• Dentin dysplasia usually demonstrates an autosomal • Generally no signs of gingivitis or periodontitis are present.
dominant inheritance. Mobility of teeth may be present due to short, malformed
• The abnormal root morphology is postulated secondary to or apparently absent roots.
the abnormal differentiation and/or function of the
ectomesenchymally derived odontoblasts. Radiographic Features
Clinical Features Type 1: Roots are short, blunt and conical. In deciduous teeth,
pulp chambers and root canals are completely obliterated; in
• Dentin dysplasia type I (radicular type) is characterized by permanent teeth they may be crescent shaped.
the presence of primary and permanent teeth with normal
appearance of the crown but no or only rudimentary root Type 2: The pulp chamber of the deciduous teeth become
development, incomplete or total obliteration of the pulp obliterated in deciduous teeth, while in permanent teeth, large
44 Essentials of Pediatric Oral Pathology

pulp chamber is seen in coronal portion of the tooth-referred

to as “thistle tube” appearance. Pulp stones may also be found.

Histopathologic Features
Type I: Normal dentinal tubule formation is blocked and new
dentin forms around obstacles. This appearance is typically
known as “lava flowing around boulders”.
Type II: In deciduous teeth, the pattern is similar to that in
dentinogenesis imperfecta. In permanent teeth, enamel and
coronal dentin are normal. Radicular dentin is atubular,
amorphous and hypertrophic. Adjacent to the pulp, numerous
areas of interglobular dentin are seen. Pulp stones develop in
FIGURE 1.52: Clinical picture of regional odontodysplasia
any portion of the chamber.
showing hypoplastic teeth

Management
1. The sequelae of dentin dysplasia are difficult to
manage and provide a challenge for the dentist
concerning not only restorative and endodontic
treatment but also prosthetic treatment after loss of
teeth.
2. Successful oral rehabilitation with complete dentures
after extraction of all teeth and curettage of cysts has
been described.
3. Cast partial dentures may be provided if the basal
bone does not provide adequate support for acrylic
partial dentures.
4. Although various treatment strategies including
conventional endodontic therapy, periapical curettage
or preventive regimen have been proposed to
maintain the teeth for as long as possible, early
exfoliation of the teeth and maxillomandibular atrophy
as a consequence of abnormal root development,
periapical abscesses or cystic formations are FIGURE 1.53: Radiographic picture of regional
characteristics of dentin dysplasia type I. odontodysplasia showing ghost like teeth
5. When implant supported prostheses are planned in
patients affected by dentin dysplasia type I bone
regenerative therapy is required. Munoz-Guerra et al. of teeth are affected, and on radiographs the teeth are described
reported successful treatment of a 24-year-old girl as “ghost teeth” (Fig. 1.53).
after onlay bone grafting and sinus augmentation. The The first report of this condition was published by McCall
authors used cortico-cancellous bone blocks from the
and Wald99 in 1947, but the term ‘odontodysplasia’ was
iliac crest for onlay grafting and a mixture of
introduced by Zegarelli et al100 in 1963. Since that time, a
autologous bone graft and an autologous platelet
concentrate obtained from platelet-rich plasma for the number of cases have been described under a variety of names;
sinus lift procedure. The teeth were extracted 4 such as localized arrested tooth development, regional
months after bone augmentation was performed. No odontodysplasia, ghost teeth, odontogenesis imperfecta,
increased and accelerated bone resorption was unilateral dental malformation, amelogenesis imperfecta non-
observed.98 hereditary segmentalis and familial amelodentinal dysplasia.

REGIONAL ODONTODYSPLASIA (FIG. 1.52) Etiology

Regional odontodysplasia (RO) is a rare, nonhereditary • Etiology is uncertain but numerous factors have been
developmental anomaly affecting dental tissues derived from suggested and considered such as local trauma, irradiation,
both the mesoderm and ectoderm. The enamel, dentin, and pulp hypophosphatasia, hypocalcemia, hyperpyrexia.
Developmental Disturbances in Children 45

• Somatic mutation and activation of latent viruses in the Management

odontogenic epithelium have also been linked to the
etiology of regional odontodysplasia. 1. A continuous and multidisciplinary approach is
required for management of the varying clinical
• Presence of nevus, hemangiomas and hydrocephaly have
problems in regional odontodysplasia.
also been associated with regional odontodysplasia but with
2. Appropriate restorative procedures, if possible, should
no definite evidence. be employed to protect the affected erupted teeth.
3. Most clinicians advocate extracting the affected teeth
Clinical Features
as soon as possible and inserting a prosthetic
• Both the primary and permanent dentitions may be affected replacement.
in the maxilla, mandible or both together. It appears to have 4. Retention of teeth should be an endeavour, parti-
a marked preference for the maxilla. cularly in younger children, but if that is not possible,
• Generally has a higher prevalence in females. partial dentures may be provided until final
• Though the condition most often affects only one quadrant, rehabilitation with implants and/or fixed prothesis.
cases with bilateral or multiquadrant involvement have also 5. Growth of maxillary and mandibular arches should be
been reported. The maxillary teeth are affected more monitored.
frequently than the mandibular, the maxillary central and
lateral incisors and canines being more affected than the DENTIN HYPOCALCIFICATION
posterior teeth. Normal dentin is calcified by deposition of inorganic material
• In the primary dentition, teeth erupted may be hypoplastic, in the form of calcium salts. These calcium salts are deposited
hypocalcified, with changes in color and form. Affected
in the organic matrix in the form of small globules which fuse
teeth are likely to be small, brown, grooved and
with each other. But sometimes these globules do not fuse with
hypoplastic. Gingival tissue can be hyperemic and usually
each other leaving behind interglobular areas of uncalcified
presents a fistula.
• In the permanent dentition, teeth usually are not erupted matrix. These areas are visible under the microscope. Dentin
or can be partially erupted with fibrous gingival tissue and hypocalcification is caused by similar factors responsible for
swelling. Root formation may be immature and roots may environmental enamel hypoplasia, e.g. hypoparathyroidism,
be aplastic. rickets, etc. Management of this condition is same as that for
dentin dysplasia.
Radiographic Features
• There is a lack of contrast between the enamel and dentin, DEFECTS OF GROWTH (ERUPTION) OF TEETH
both of which are less radiopaque than their unaffected • Premature eruption
counterparts. • Eruption sequestrum
• Additionally, enamel and dentin layers are thin, giving the • Delayed eruption
teeth a ‘ghost-like’ appearance.
• Multiple unerupted teeth
• The pulp chambers are noticeably enlarged with open
• Embedded and impacted teeth
apices and enlarged follicles.
• Ankylosed deciduous teeth
Histopathologic Features
PREMATURE ERUPTION
• Areas of hypocalcified enamel are visible and enamel
prisms appear irregular in direction. This has already been discussed under the heading of natal
• Coronal dentin is fibrous, consisting of clefts and a reduced teeth.
number of dentinal tubules; radicular dentin is generally
more normal in structure and calcification. ERUPTION SEQUESTRUM
• Irregular dentin with areas of interglobular dentin and
presence of immature odontogenic epithelium in the Eruption sequestrum is a small spicule of calcified tissue that
connective tissue. is extruded through the alveolar mucosa that overlies an
• Pulpal calcification of various degrees is also commonly seen. erupting molar or any other tooth in children. This entity was
• The mineral content of the affected enamel has been found first described by Starkey and Shafer in 1963.101
to be higher than that of dentin in microradiographic
studies. The greater density of the enamel is not evident in Etiology
conventional radiographs, probably because of the thinness • As the molar teeth erupt through the bone, they will
of the enamel layer in affected teeth. occasionally separate a small osseous fragment from the
46 Essentials of Pediatric Oral Pathology

surrounding contiguous bone, much in the fashion of a Etiology

corkscrew.
• Delayed eruption of both deciduous and permanent teeth
• In most cases, this fragment probably undergoes total
may be due to systemic or local causes.
resorption prior to eruption. If the bony spicule is larger or
• The following medical conditions are some of the possible
eruption is fast, complete resorption cannot occur and the
systemic causes of delayed eruption of teeth:
eruption sequestrum is observed.
— Hypothyroidism
Clinical Features — Hypoparathyroidism
— Cleidocranial dysostosis
• A small, irregular hard tissue fragment, white in color, with — Gardner syndrome
bone-like hardness on the occlusal surface of molars is — Vitamin D deficiency
seen.102 It is seen just prior to or immediately following the — Ectodermal dysplasia
emergence of the tips of the cusps through the oral mucosa. — Pyknodysostosis
• The spicule directly overlies the central occlusal fossa but — Deficiency of membrane-type 1 matrix metallopro-
is contained within the soft tissue. teinase has been found to be associated with delayed
• Chronic inflammatory alterations may also be observed in eruption and incomplete root formation.103
the gingiva in the area of contact with the osseous tissue. — Primary tooth eruption occurs significantly later in
• As the tooth continues to erupt and the cusps emerge, the
children with a birth weight less than 1000 g.104
fragment of bone completely sequestrates through the
— Hauk et al 2001, found a correlation between the
mucosa and is lost.
progression of HIV infection to pediatric AIDS and
• For a few days, the fragment of bone may be seen lying
delayed dental eruption and this delay is most closely
the crest of the ridge in a tiny depression from which it
linked to severity of symptoms and not CD4 depletion.105
may easily be removed.
— The following local causes are also seen to delay
• The child may complain of a slight soreness in the area,
eruption of teeth:
probably produced by compression of the soft tissue over
i. Fibromatosis gingivae
the spicule during eating and just prior to its breaking
ii. Supernumerary teeth
through the mucosa.
iii. Retention of deciduous teeth
Radiographic Features iv. Crowding of the jaw
• Since the permanent teeth have a wider range of variation
• May be diagnosed before eruption of teeth. It appears as a in the time of eruption, it is frequently difficult to pinpoint
tiny irregular opacity overlying the central occlusal fossa a retardation of eruption.
but separated from the tooth itself.
Management
Histopathologic Features
1. Treatment of the underlying systemic cause may
• Histopathologically, the fragments consist of necrosed
facilitate eruption.
cortical bone.
2. Removal of the local factors like fibromatosis or
• X-ray microanalyzer findings have revealed the percentages supernumerary teeth helps in alleviating the condition.
of calcium and phosphorous (by weight) as 78.41 percent 3. Recent researches have shown that tooth eruption
and 21.59 percent, respectively, for a calcium to phosphorous depends on the presence of osteoclasts to create an
ratio of 3.63, which is higher than that seen in normal eruption pathway through the alveolar bone. Hua F
osseous tissue. et al, 2007, postulated a new approach that targets
osteoclast formation and activation to accelerate the
Management eruption of the affected tooth. These strategies include
stimulating osteoclastogenesis by applying the
1. No treatment is necessary, since the condition cytokines or small molecules, such as TNF-alpha,
corrects itself. IL-1 alpha and MCP-1; triggering osteoclast
2. Removal of the spicule may be required. differentiation by applying molecule associated
RANKL signaling, enhancing the function of
DELAYED ERUPTION osteoclasts by applying proteinases, such as CTSK.
These molecules can be injected into the oral mucosa
Delayed tooth eruption is the emergence of a tooth into the
of the affected tooth to induce bone resorption, then
oral cavity at a time that deviates significantly from norms
to rebuild the pathway of tooth eruption.106
established for different races, ethnicities, and sexes.
Developmental Disturbances in Children 47

MULTIPLE UNERUPTED TEETH

Multiple unerupted teeth are uncommon without associated
local or systemic causes.

Etiology
• Sometimes may be due to a lack of eruptive force, where
appearance of teeth and jaws may be normal.
• May be due to local and systemic causes elaborated in the
section on delayed eruption.

Clinical Features
• Multiple retained deciduous teeth with delayed eruption of FIGURE 1.54: Radiographic picture of an impacted maxillary
permanent teeth. right central incisor and mandibular left first premolar
• Deciduous teeth may have been shed but the permanent
teeth may have failed to erupt. This is often called as • Not uncommonly, impaction may result due to placement
“pseudoanodontia”. of the tooth in the jaw in a wrong direction. This results in
• Jaw and appearance of teeth may be normal. a direction of eruption where the long axis of the tooth is
not parallel to a normal eruption path.
Radiographic Features
Clinical Features
Jaw and appearance of teeth may be normal.
• Impacted and embedded teeth need to be differentiated
Management from missing teeth.
• It is also important to determine whether a tooth is
1. Treatment of the underlying systemic cause may completely or partially impacted. A completely impacted
facilitate eruption. tooth is one which lies completely within the bone and has
2. Removal of local factors like fibromatosis or
no communication with the oral cavity. A partially impacted
supernumerary teeth help in alleviating the condition.
tooth is not completely encased in bone.
• Most commonly impacted teeth are maxillary third molars
EMBEDDED AND IMPACTED TEETH (22 %), followed by mandibular third molars (18 percent)
and maxillary canines (0.9 %) (Fig. 1.54).
Embedded teeth are those teeth that are unerupted due to a lack
• Primary tooth impaction is usually associated with a defect
of eruptive force.
in the development and eruption of the permanent
An impacted tooth results from failure of the tooth to erupt
successors.
into its normal position because of some physical barrier in
• Impaction may be mesioangular, distoangular, vertical,
the path of eruption.
horizontal or inverted; all with buccal or lingual deflection.
Secondary retention refers to the cessation of eruption of a
• At times, the mandibular third molar may be situated
tooth after emergence neither because of a physical barrier in
completely within the ramus of the mandible.
the path of eruption nor as a result of an unusual position.
• Dental caries of an impacted tooth may occur due to a
discrete communication of the tooth with the oral cavity.
Etiology
• A completely embedded or impacted tooth cannot become
• Generally this is an acquired condition but it may be genetic. carious.
• Impaction can be caused by trauma or simply because of • Impaction of permanent maxillary cuspids occurs mainly
the tooth’s position in the alveolus so that it is not capable due to:
of erupting into its normal position. — A long eruption pathway from the place of development
• Lack of space due to crowding of the dental arches may to that of eruption.
result in partial or complete impaction of a tooth. — Sequence of eruption in the maxillary arch being first
• Loss of space due to premature loss of a primary tooth and premolar followed by canine followed by second
subsequent partial closure of the space may also result in premolar. Hence most of the impactions occur simply
impaction. due to a lack of space.
48 Essentials of Pediatric Oral Pathology

• A maxillary cuspid usually points anteriorly and may the successor is always involved. The terms submerged teeth
impinge on roots of the lateral incisors or premolars. and infraocclusion applied to this condition are inaccurate.
• Periodic pain and trismus may also result due to an Henderson, 1979, pointed that ankylosis should be considered
impacted tooth. an interruption in the rhythm of eruption.107
• A dentigerous cyst may also develop around the coronal
portion of an impacted tooth. Etiology
• Impacted teeth allowed to remain in situ may occasionally
Etiology of this entity is unknown but three hypotheses have
undergo resorption. However, the cause of resorption is not
been put forth:
known.
1. Familial pattern, probably non-sex linked trait.
• Cases of ameloblastoma have also been reported to form
2. Intermittent resorption and repair during the routine
in the wall of such a cyst.
exfoliation process of the deciduous tooth.
3. A relationship between congenital absence of permanent
Management
teeth and ankylosed primary teeth has been suggested.
1. Impacted teeth should be surgically removed or at
least monitored on a regular basis. Clinical Features
2. In most cases of the impacted cuspids, a suitable
orthodontic appliance may be used to bring the tooth • The lower second deciduous molars are affected most
into normal occlusion. frequently; in the permanent dentition a single or all first
3. Impaction may be avoided by providing a suitable molars may show signs of ankylosis. If more permanent
space maintainer in case of premature loss of primary teeth of the buccal segment are affected, this is defined as
teeth. a general disturbance of the periodontal tissue.
• Ankylosis of primary anterior teeth is a rare phenomenon
ANKYLOSED DECIDUOUS TEETH (FIG. 1.55) and occurs following trauma. Alexender et al 1980, reported
an unusual case of ankylosis of multiple primary molars
Ankylosis in this context means the fusion of dental hard tissue,
where all the primary molars were ankylosed to the alveolar
cement and dentine with alveolar bone accompanied by loss
bone.108
of soft tissue. In most cases, however, it does not involve the • Usually, ankylosis of the primary molar occurs only after
whole periodontal ligament; single bone bridges in the its root resorption begins.
periodontal ligament suffice to disturb the normal vertical • If ankylosis occurs early, eruption of adjacent teeth may
development of the tooth in comparison to its unaffected progress enough that the ankylosed tooth is far below the
neighbouring teeth. As long as the vertical discrepancy of single normal plane of occlusion and may even be partially
or even all deciduous teeth does not exceed 2 to 3 mm, there covered with soft tissue.
is no clinical relevance. But if a deciduous tooth reoccludates • However, an epithelium lined tract will extend from the oral
into bony structure up to the level or even below the gingiva, cavity to the tooth.
• Ankylosis may occasionally occur even before the eruption
and complete root formation of the primary tooth.
• The ankylosed tooth will have a solid sound when tapped
upon with a blunt instrument whereas the normal tooth will
have a cushioned sound due to an intact periodontal
membrane which absorbs some of the shock of the blow.
This test aids in clinical diagnosis of the ankylosed tooth.

Radiographic Features
A break in the continuity of the periodontal membrane indicates
an area of ankylosis.

Management
1. Early recognition and diagnosis are extremely
important.
2. Treatment may involve surgical removal, but if the
FIGURE 1.55: Clinical picture of an ankylosed tooth submerged ankylosed tooth poses no space problems in the arch
below the occlusal plane
Developmental Disturbances in Children 49

and is not involved by caries, it may be kept under channels (the incisive canals) persist. The palatine processes
observation. It is quite likely that the ankylosed tooth probably partly overgrow the primary palate on either side of
would undergo root resorption and normal exfoliation the nasal septum. Thus, the incisive canals represent
sometime later. passageways in the hard palate, which extend downward and
3. The prognosis for active elongation of ankylosed teeth forward from the nasal cavity. Just before exiting the bony
is uncertain or poor. surface of the hard palate (incisive foramen or incisive fossa),
the paired incisive canals usually fuse to form a common canal
FISSURAL CYSTS OF THE ORAL REGION in a Y shape.
• Nasopalatine duct cyst The fusion of facial processes in the embryologic
• Median palatal cyst development of the maxilla results in the formation of a pair
• Globulomaxillary cyst of epithelial strands (the nasopalatine ducts) that traverse the
• Median mandibular cyst incisive canals downward and forward, connecting the nasal
• Nasoalveolar cyst and oral cavities. The nasopalatine duct leads from the incisive
• Palatal and alveolar cysts of newborns fossa in the oral cavity to the nasal floor, in which it ends in
• Thyroglossal tract cyst the nasopalatine infundibulum.
• Epidermal inclusion cyst The types of epithelia that line the nasopalatine duct are
• Dermoid cyst highly variable, depending on the relative proximity of the nasal
• Heterotopic oral gastrointestinal cyst and oral cavities. The most superior part of the duct is
characterized by a respiratory-type epithelial lining. Moving
NASOPALATINE DUCT CYST downward, the lining changes to cuboidal epithelium. In the
most inferior portion closest to the oral cavity, squamous
The nasopalatine duct cyst (NPDC) is a developmental, non- epithelium is usually present. In addition to the nasopalatine
neoplastic cyst that is considered to be the most common of ducts, branches of the descending palatine and sphenopalatine
the nonodontogenic cysts. NPDC is one of many pathologic arteries, the nasopalatine nerve, and mucus-secreting glands are
processes that may occur within the jawbones, but it is unique present within the incisive canals.
in that it develops in only a single location, which is the midline The nasopalatine ducts ordinarily undergo progressive
anterior maxilla (Fig. 1.56). degeneration; however, the persistence of epithelial remnants
may later become the source of epithelia that gives rise to
Etiopathogenesis NPDC, from either spontaneous proliferation or proliferation
The development of the face and the oral cavity takes place following trauma (e.g. removable dentures), bacterial infection,
between the fourth and eighth weeks of intrauterine life. The or mucous retention. Genetic factors have also been suggested.
secondary palate is formed during the eighth and twelfth weeks. The mucous glands present among the proliferating epithelium
In the midline between the primary and secondary palates, two can contribute to secondary cyst formation by secreting mucin
within the enclosed structure. NPDC can form within the
incisive canal, which is located in the palatine bone and behind
the alveolar process of the maxillary central incisors, or in the
soft tissue of the palate that overlies the foramen, called the
cyst of the incisive papilla.

Clinical Features
• Males are affected 1.8 to 20 times more often than
females.109
• NPDCs occur over a wide age range (7to 88 years) and
they also occur in fetuses.110 Most patients who are affected
are aged 30 to 60 years.
• Large and more destructive cysts that have perforated the
labial and palatal bony plates may present as expansile,
fluctuant swellings of the anterior palate and the posterior
palate.
FIGURE 1.56: Radiographic picture of nasopalatine duct cyst • Extrabony cysts that develop within the soft tissues of the
showing radiolucent area in the palate incisive papilla area of the anterior hard palate (called the
50 Essentials of Pediatric Oral Pathology

cyst of the incisive papilla) may present as a translucent or Management

bluish colored, dome-shaped swelling. The clinically
Treatment includes surgical excision and thorough
apparent discoloration is due to accumulation of fluid
curettage.
contents within the cyst.
• NPDCs clinically demonstrate slow and progressive
GLOBULOMAXILLARY CYST
growth, sometimes exceeding 60 mm in diameter.
• Tooth displacement is a common finding, having been The globulomaxillary cyst is a cyst that appears between a
reported to occur in 78 percent of patients, whereas bony maxillary lateral incisor and the adjacent canine. It was first
expansion is noted in only 1.4 percent of patients.111 described by Thoma as a developmental (fissural) cyst.
It is present within the maxilla at the junction of the globular
Management
portion of the medial nasal process and the maxillary process.

1. NPDCs are treated by enucleation via a palatine or Etiology

buccal approach.
2. Recurrence is uncommon, having been reported in 0 It is thought to be a developmental fissural cyst arising in the
to 11 percent of patients.112 If components of the long area between the nasal process and maxillary process. It is now
sphenopalatine nerve are removed during surgery, it believed that all these lesions are actually other odontogenic
may cause paresthesia to the anterior palate. cysts, such as odontogenic keratocysts or lateral periodontal
3. Complete bone regeneration within the bony defect cysts. In fact, it is now recommended that this entity should be
is expected postoperatively. used only as a clinically descriptive term.

MEDIAN PALATAL CYST Clinical Features

Median palatal cyst is an epithelium lined sac containing fluid, • Found between the maxillary lateral incisor and the adjacent
present in the midline of the hard palate, between the lateral canine.
palatal processes. • All regional teeth are found to be vital.
• It may often cause the roots of adjacent teeth to diverge.
Etiology • This cyst should not be confused with a nasopalatine cyst.
• The lesion is usually discovered during routine dental
It is of developmental origin. examination.
• It is asymptomatic, but becomes slightly tender if infected.
Clinical Features • Bilateral lesions are reported, but rarely.
• It is asymptomatic and discovered incidentally during Radiographic Features
routine dental or radiological examination.
• Its occurrence is rare. • It appears as an oval, round or “inverted pear-shaped
• A swelling on the oral surface of the hard palate is generally radiolucency” on radiographs.
seen. • According to Wysocki,113 the majority of the lesions (over
• Rarely, it may cause elevation of the nasal floor and nasal 80 percent) presenting with the radiographic features of a
obstruction. globulomaxillary cyst are of periapical origin.

Histopathologic Features
Radiographic Features
• Histologically, it can be a variety of odontogenic lesions
It appears as a radiolucent area in the midline of the palate.
predominantly of periapical origin, i.e. periapical cyst and
granuloma, odontogenic keratocyst, or more rarely
Histopathologic Features
odontogenic myxoma, squamous odontogenic tumor,
• The cyst consists of a dense fibrous connective tissue lined adenomatoid odontogenic tumor or central giant cell
by stratified squamous epithelium. granuloma (the latter is not of tooth origin). However,
• The connective tissue consists of chronic inflammatory cell evidence in literature is in favor of this lesion being
infiltrate. predominantly of tooth origin.
• Some of the cystic lining shows pseudostratified ciliated • Histologic features will usually not be supportive of a
columnar epithelium. periapical lesion.
Developmental Disturbances in Children 51

NASOALVEOLAR CYST
Nasoalveolar cyst is a fissural cyst arising outside the bones at
the junction of the globular portion of the medial nasal process,
lateral nasal process, and maxillary process.

Etiology
• It is formed as a result of proliferation of entrapped
epithelium along the fusion line of the globular portion of
the medial nasal process, lateral nasal process, and
maxillary process.
• Roed-Peterson,114 1969, and Christ,115 1970, suggested that
FIGURE 1.57: Radiographic picture of median mandibular cyst the cyst originates from the lower anterior part of the naso-
lacrimal duct, rather than the entrapment of epithelium.
Management
Treatment is by enucleation, or surgical removal.
Clinical Features
• Most commonly seen in females.
• Incidence of occurrence ranges from 12 to 75 years of age.
MEDIAN MANDIBULAR CYST (FIG. 1.57)
• Causes a swelling in the mucolabial fold and floor of the
It is a soft tissue sac which develops in the mouth near the nose.
middle of the lower jaw in conjunction with normal growth or
as the result of an odontogenic cyst. Histopathologic Features
The cyst is lined by pseudostratified or stratified ciliated
Etiology
columnar epithelium containing goblet cells (Fig. 1.58).
Two schools of thoughts have been put forth for the origin of
this cyst Management
1. Some researchers suggest the origin of this cyst from Treatment is by surgical excision.
proliferation of epithelial remnants entrapped in the
median mandibular fissure during the fusion of The following have been discussed in the section on cysts in
mandibular arches. the pediatric population:
2. Cyst may originate from supernumerary enamel organ • Palatal and alveolar cysts of newborns
in the anterior mandibular segment. • Thyroglossal tract cyst
• Epidermal inclusion cyst
Clinical Features • Dermoid cyst
• They are often asymptomatic and diagnosed during routine
radiographic examination.
• They produce expansion of cortical plates.

Radiographic Features
It appears either as a unilocular or multilocular well-
circumscribed radiolucent lesion.

Histopathologic Features
The cyst is lined by stratified squamous epithelium showing
numerous papillary projections.

Management
FIGURE 1.58: Histopathologic picture of nasoalveolar cyst showing
Treatment is by conservative surgical excision.
stratified ciliated columnar epithelium containing goblet cells
52 Essentials of Pediatric Oral Pathology

Heterotopic oral gastrointestinal cyst is generally not seen in 18. Murray JC. Gene/environment causes of cleft lip and / or palate.
the pediatric population, hence does not warrant explanation Clinical Genet; 2002;61:248-56.
in this textbook. 19. Bonaiti-Pellie C, et al. An epidemiological and genetic study
of facial clefting in France. I. Epidemiological and frequency
in relatives. J Med Genet 1982;11:374-7.
REFERENCES
20. Ralph E McDonald, David R Avery. Dentistry for the child
1. Lannelongue V, Menard M. Affections congenitales, 1891 vol and adolescent; seventh edition; Harcourt India Pvt Ltd;
1 Paris: Hasselin and Houzeau, 423. 2001;742-4.
2. Robin P. La chute de la base de la langue considérée comme 21. Tandon S, Kumar P. Chapter 11: Dental care for the special
une nouvelle cause de gans la respiration naso-pharyngienne. child. In Tandon Shobha's Textbook of Pedodontics, Paras
Bull Acad natl Med (Paris), 1923;89:37-41. Publishing: 2003;576-9.
3. Taylor WB, Lane DJ. Congenital fistulas of the lower lip. Arch 22. Murray C Jeffrey. Face facts: Genes, environment and clefts.
Dermatol, 1966;94:421. Am J Hum Genet 1995;57:227-32.
4. McConell FMS, Zellweger H, Lawrence RA. Labial pits: cleft 23. Jurilof DM, Mah DG. The major locus for multifactorial
lip and/or palate syndrome. Arch Otolaryngol, 1970;91:407. nonsyndromic cleft lip maps to mouse chromosome 11. Mamm
5. Neville BW, Damm DD, Allen CM, Bouquot JE. Chpter 1: Genome 1995;6:63-9.
Developmental defects of the oral and maxillofacial region. In: 24. Chenevix-Trench G, Jones K, Green AC, Duffy DL, Martin NG.
Oral and Maxillofacial Pathology, Saunders, 2nd edition, 2005; Cleft lip with or without cleft palate: associations with
45. transforming growth factor alpha and retinoic acid receptor loci.
6. Van der Woude A. “Fistula labii inferioris congenita and its Am J Hum Genet 1992;51:1377-85.
association with cleft lip and palate”. Am J Hum Genet, 1954; 25. Hartridge T, Illing HM, Sandy JR. The role of folic acid in
6(2):244-56. orofacial clefting. Brit J Orthod 1999;26:115-20.
7. Helms JA, et al. Sonic hedgehog participates in craniofacial 26. Kallen K. Maternal smoking and orofacial cleft. Cleft Palate
morphogenesis and is down-regulated by teratogenic doses of Craniofac J 1997;34:11-6.
retinoic acid. Dev Biol 1997;187:25-35. 27. Watson ACH, Sel DA, Grunwell P. Management of cleft lip and
8. Kerrigan JJ, Mansell JP, Sengupta A, Brown N, Sandy JR. palate. Whurr Publishers, London and Philadelphia 2005;117-9
Palatogenesis and potential mechanisms for clefting. JR Coll 28. Bradbury E, Bannister P. Chapter 8: prenatal, perinatal and
Surg Edinb, 45, December 2000;351-8. postnatal counseling. Whurr Publishers, London and
Philadelphia 2005;119-20.
9. Ferguson MWJ. Developmental mechanisms in normal and
abnormal palate formation with particular reference to the 29. Bradbury E, Bannister P. Chapter 8: prenatal, perinatal and
etiology, pathogenesis and prevention of cleft palate. Br J Orthod postnatal counseling.
1981;8:115-37. 30. Policy on management of patients with cleft lip/palate and other
10. Ferguson MWJ. Palate development. Development 1988;103: craniofacial anomalies. American Academy of Pediatric
Dentistry: Reference Manual 2004-2005;26:7;154-5.
41-60.
31. Damle SG. Chapter 48: Dental management of patients with cleft
11. Shapiro BL, Sweney L. Electron microscopic and histochemical
lip and cleft palate. In Pediatric Dentistry. Arya Medi Publishing
examination of oral epithelial-mesenchymal interaction
House 2001;293-5.
(programmed cell death). J Dent Res 1969;48:652-60.
32. Neville BW, Damm DD, Allen CM, Bouquot JE. Abnormalities
12. Gartner LP, Hiatt JL, Provenza DV. Palatal shelf epithelium: A
of the teeth. In: Neville BW, Damm DD, Allen CM, Bouquot
morphologic and histochemical study in X-irradiated and normal
JE, (Eds): Oral and Maxillofacial Pathology. 1st ed.
mice. Histochem J 1978;10:45-52.
Philadelphia, Pa: WB Saunders; 1995;653-4.
13. Fitchett JE, Hay ED. Medial edge epithelium transforms to
33. Rajendran R, Shivapathasundharam B. Developmental
mesenchyme after embryonic palatal shelves fuse. Develop Biol
disturbances of Oral and Paraoral structures. In: Shafer's
1989;131:455-74. textbook of Oral Pathology. Elsevier, 5th edition, 2006;29-30.
14. Sun D, Vanderburg CR, Odierna GS, Hay ED. TGFb3 promotes 34. Peutz JLA. Very remarkable case of familial polyposis of
transformation of chicken palate medial edge epithelium to mucous membrane of intestinal tract and nasopharynx
mesenchyme in vitro. Development 1998a;125:95-105. accompanied by peculiar pigmentations of skin and mucous
15. cirujanosplástikosmundi.com. WS-India-Manual.pdf. membrane. Nederl Maandischr v Geneesk 1921;10:134-46.
16. Marazita ML, Spence MA, Melnick M. Major gene determi- 35. Jeghers H, Mc Kusick FA, Katz KH. Generalized intestinal
nation of liability to cleft lip with or without cleft palate: a polyposis and melanin spots of oral mucosa, lips and digits: a
multiracial view. J Craniofac Genet Dev Biol 1986;2(Suppl): syndrome of diagnostic significance. New England J Med,
89-97. 1949;241-25:993-1005.
17. Melnick M, Marazita ML, Hu DN. Genetic analysis of cleft lip 36. Goldberg, Hyman, Goldhaber Paul. Hereditary intestinal
with or without cleft palate in Chinese kindreds. American J polyposis with oral pigmentation. Oral Surg, Oral Med, Oral
Med Genet; 1986;21:183-90. Pathol, 1954;7:378-82.
Developmental Disturbances in Children 53

37. Von Volkmann R, Einige Falle von Cheilitis Glandularis, 60. Kates GA, Needleman HL, Holmes LB. Natal and neonatal
Apostematosa (Myxadenitis Labialis). Virchows Arch Pathol teeth: a clinical study. J Am Dent Assoc 1984;109:441-3.
Anat 1870;50:142-4. 61. Robinson C, Briggs HD, Atkinson PJ, Weatherell JA. Matrix
38. Guiducci A, Hyman AB. Ectopic sebaceous glands. and mineral changes in developing enamel. J Dent Res
Dermatologica, 1962;125:44. 1979;58:871-82.
39. Halperin V, Kolas S, Jefferis KR, Huddleston SD, Robinson 62. Simmer JP, Fincham AG. Molecular mechanisms of dental
HBG. The occurence of Fordyce spots, benign migratory enamel formation. Crit Rev Oral Biol Med 1995;6:84-108.
glossitis and fissured tongue in 2,478 dental patients. Oral 63. Mahoney EK, Rohanizadeh R, Smail FSM, Kilpatrick NM,
Surgery, 1953;6:1072. Swain MV. Mechanical properties and microstructure of
40. Miles AEW. Sebaceous glands in the lip and cheek mucosa of hypomineralized enamel of permanent teeth. Biomaterials
man. Br Dent J, 1958;105:235. 2003;25:5091-100.
41. Baughman RA, Heidrich PD, Jr. The oral hair: an extremely 64. Paine ML, White SN, Luo W, Fong H, Sarikaya M, Snead ML.
rare phenomenon. Oral Surg 1980;49:530. Regulated expression dictates enamel structure and tooth
42. Hirshfeld I. The retrocuspid papilla. Am J Orthod, 1947;33:447. function (review). Matrix Biol 2001;20:273-92.
43. Berman FR, Fay JT. The retrocuspid papilla. A clinical survey. 65. Witkop CJJ. Amelogenesis imperfecta, dentinogenesis
Oral Surg, 1976;42:80. imperfecta and dentin dysplasia revisited, problems in
44. Eidelman E, Chosack A, Cohen T. Scrotal tongue and classification. J Oral Pathol 1989;17:547-53.
geographic tongue: polygenic and associated traits. Oral Surg 66. Aldred MJ, Savarirayan R, Crawford PJM. Amelogenesis
Oral Med Oral Pathol 1976;42(5):591-6. imperfecta: A classification and catalogue for the 21st century.
45. Rogers RS. Melkersson-Rosenthal syndrome and orofacial Oral Disease 2003;9:19-23.
granulomatosis. Dermatol Clin 1996;14(2):371-9. 67. Cook DC. Hereditary enamel hypoplasia in a prehistoric Indian
46. James William D, Berger Timothy G, et al. Andrews' Diseases child. J Dent Res 1980;59:1522.
of the Skin: Clinical Dermatology. Saunders Elsevier, 2006. 68. Schulze C. Beitrag zur Frage der “angeborenen Schmel-
47. Brocq L, Pautrier LM. Glossite losangue mediane de la face zhypoplasie.” Dtsche Zahn Mund Kieferheilkd 1952;16:108-
36.
dorsale de la langue. Ann Derm Syph (Paris) 1914;5:1-18.
69. Schulze C. Erbbedingte Strukturanomalien menschlicher Zahne.
48. Gonzaga HF, Torres EA, Alchorne MM, Gerbase-Delima M.
Miinchen: Urban and Schwarzenburg, 1956.
Both psoriasis and benign migratory glossitis are associated with
HLA-Cw6. Br J Dermatol Sep 1996;135(3):368-70. 70. Winter GB, Brook AH. Enamel hypoplasia and anomalies of
the enamel. Dent Clin North Am 1975;19:3-24.
49. Oehlers FA. Dens invaginatus: Variation of the invagination
process and associated posterior crown forms. Oral Surg 71. Haug RH, Ferguson FS. X-linked recessive hypomaturation
1957;10:1302-16. amelogenesis imperfecta: report of a case. J Am Dent Assoc
1981;102:865-7.
50. Liu JF. Characteristics of premaxillary supernumerary teeth: a
survey of 112 cases. ASDC J Dent Child 1995;62:262-5. 72. Witkop CJ, Stewart RE. Amelogenesis imperfecta. In: Stewart
RE, Barber TK, Troutman KC, Wei SHY (Eds). Pediatric
51. Levine N. The clinical management of supernumerary teeth. J
dentistry: scientifc foundations and clinical practice. St Louis:
Can Dent Assoc 1961;28:297-303.
CV Mosby; 1982. p. 110.
52. Brook AH. Dental anomalies of number, form and size: their
73. Witkop CJ. Genetic disease of the oral cavity. In: Tiecke RJ,
prevalence in British schoolchildren. J Int Assoc Dent Child
(Ed): Oral Pathology, New York: McGraw-Hill, 1965;805.
1974;5:37-53.
74. Escobar VH, Goldblatt LI, Bixler D. A clinical, genetic, and
53. Vichi M, Franchi L. Abnormalities of the maxillary incisors in
ultrastructural study of snow-capped teeth: X-linked
children with cleft lip and palate. ADSC J Dent Child 1995;
amelogenesis imperfecta, hypomaturation type. Oral Surg Oral
62:412-7.
Med Oral Pathol 1981;52:607-14.
54. Jensen BL, Kreiborg S. Development of the dentition in
75. Crawford PJ, Aldred MJ. X-linked amelogenesis imperfecta.
cleidocranial dysplasia. J Oral Pathol Med 1990;19:89-93.
Presentation of two kindreds and a review of the literature. Oral
55. Primosch RE. Anterior supernumerary teeth - assessment and Surg Oral Med Oral Pathol 1992;73:449-55.
surgical intervention in children. Pediatr Dent 1981;3:204-15.
76. Schulze C. Erbbedingte Strukturanomalien menschlicher Zahne.
56. Di Biase DD. The effects of variations in tooth morphology and Acta Genet Med Gemellol 1957;7:231-5.
position on eruption. Dent Pract Dent Rec 1971;22:95-108. 77. Witkop CJ, Sauk JJ. Heritable defects of enamel. In: Stewart
57. Spouge JD, Feasby WH. Erupted teeth in the newborn. Oral RE, Prescott GH (Eds). Oral facial genetics. St Louis: CV
Surg 1966;22:198. Mosby; 1976. p. 151-226.
58. Massler M, Savara BS. Natal and Neonatal teeth. J Pediatr 1950; 78. Sauk JJ, Lyon HW, Witkop CJ. Electron optic microanalysis of
36:349. two gene products in enamel of females heterozygous for X-
59. Mayhall JT. Natal and neonatal teeth among the Tlinget Indians. linked hypomaturation amelogenesis imperfecta. Am J Hum
J Dent Res 1967;46:748-9. Genet 1972;24:267-76.
54 Essentials of Pediatric Oral Pathology

79. Darling AI. Some observations on amelogenesis imperfecta and 97. Dean JA, Hartsfield JK Jr, Wright JT, Hart TC. Dentin dysplasia,
calcifcation of the dental enamel. Proc R Sot Med 1956;49:759- type II linkage to chromosome 4q J Craniofac Genet Dev Biol
65. 1997;17:172-7.
80. Backman B, Anneroth G. Microradiographic study of 98. Munoz-Guerra MF, Naval-Gias L, Escorial V, Sastre-Perez J.
amelogenesis imperfecta. Scand J Dent Res 1989;97:316-29. Dentin dysplasia type I treated with onlay bone grafting, sinus
81. Backman B, Anneroth G, Horstedt P. Amelogenesis imperfecta- augmentation, and osseointegrated implants. Implant Dent,
a scanning electron microscopic and microradiographic study. 2006;15(3):248-53.
J Oral Pathol Med 1989;18:140-5. 99. McCall JO, Wald SS. Clinical dental roentgenology. 3rd ed.
82. McLarty EL, Giansanti JS, Hibbard ED. X-linked hypomatura- Philadelphia: WB Saunders; 1952. p. 170.
tion type of amelogenesis imperfecta exhibiting lionization in 100. Zegarelli EV, Kutscher AH, Applebaum E, Archard HO.
females. Oral Surg Oral Med Oral Pathol 1973;36:678-85. Odontodysplasia. Oral Surg Oral Med Oral Pathol 1963;16:187-
83. Witkop CJ. Hereditary defects in enamel and dentin. Acta Genet 93.
Med Gemollol (Roma) 1957;7:236-9. 101. Starkey PE, Shafer WG. Eruption sequestra in children. J Dent
84. Schulze C. Developmental abnormalities of teeth and jaws. In: Child 1963;30:84.
Gorlin RJ, Goldman HM (Eds). Thoma's oral pathology. 6th 102. Maki K, Ansai T, Nishida I, Zhang M, Kojima Y, Takehara T,
ed. St Louis: CV Mosby; 1970. p. 130-6. Kimura M. Eruption sequestrum: x-ray microanalysis and
85. Ooya K, Nalbandian J, Noikura T. Autosomal recessive rough microscopic findings. J Clin Pediatr Dent 2005;29(3):245-7.
hypoplastic amelogenesis imperfecta. A case report with clinical, 103. Bartlett JD, Zhou Z, Skobe Z, Dobeck JM, Tryggvason K.
light microscopic, radiographic and electron microscopic Delayed tooth eruption in membrane type-1 matrix
observations. Oral Surg Oral Med Oral Pathol 1988;65:449- metalloproteinase deficient mice. Connect Tissue Res 2003;44
58. (Suppl 1):300-4.
86. Backman B, Holmgren G. Amelogenesis imperfecta: a genetic 104. Viscardi RM, Romberg E, Abrams RG. Delayed primary tooth
study. Hum Hered 1988;38:189-206. eruption in premature infants: relationship to neonatal factors.
87. Aldred MJ, Crawford PJ. Amelogenesis imperfecta-towards a Pediatr-Dent 1994;16(1):23-8.
new classifcation. Oral Dis 1995;1:2-5. 105. Hauk MJ, Moss ME, Weinberg GA, Berkowitz RJ. Delayed
88. Murray JJ, Shaw L. Classification and prevalence of enamel tooth eruption: association with severity of HIV infection.
opacities in the human deciduous and permanent dentitions. Pediatr Dent 2001;23(3):260-2.
Arch Oral Biol 1979;24:7-13. 106. Hua F, Zhang L, Chen Z. Trigger osteoclast formation and
89. Churchill HV. Occurrence of fluorides in some water of the activation: molecular treatment strategy of delayed tooth
United States. Ind Eng Chem 1931;23:996-8. eruption. Med Hypotheses 2007;69(6):1222-4.
90. Dean HT. Classification of mottled enamel diagnosis. J Am Dent 107. Henderson HZ. Ankylosis of primary molars: a clinical,
Assoc 1934;21:1421-6. radiographic and histologic study. J Dent Child, 1979;46:117-
91. Shields ED, Bixter D, El-Kafrawy AM. Proposal classification 22.
for heritable human dentin defects with a description of new 108. Alexender SA and others: Multiple ankylosed teeth. J Pedod
entity. Arch Oral Biol 1973;18:543-53. 1980;4:354-9.
92. Witkop CJ, Jr. Hereditary defects of dentin. Dent Clin N Am, 109. Cabrini RL, Barros RE, Albano H. Cysts of the jaws: a statistical
1975;19:25-45. analysis. J Oral Surg 1970;28(7):485-9.
93. Sauk JJ Jr, Witkop CJ Jr, Brown DM, Corbin KW Glyco- 110. Abrams AM, Howell FV, Bullock WK. Nasopalatine cysts. Oral
saminoglycans of EDTA soluble and insoluble dentin in Surg Oral Med Oral Pathol 1963;16:306-32.
dentinogenesis imperfecta type I.Oral Surg Oral Med Oral 111. Swanson KS, Kaugars GE, Gunsolley JC. Nasopalatine duct
Pathol 1976;41(6):753-7. cyst: an analysis of 334 cases. J Oral Maxillofac Surg
94. Balchsmiede G. Disseration In Steidler NE, Radden BG, Reade 1991;49(3):268-71.
PC. Dentinal dysplasia; a clinicopathological study of eight 112. Bodin I, Isacsson G, Julin P. Cysts of the nasopalatine duct. Int
cases and review of literature: Br JOral Maxillofac Surg 1984; J Oral Maxillofac Surg 1986;15(6):696-706.
22:274-86. 113. Wysocki GP. The differential diagnosis of globulomaxillary
95. Rushton MA. A case of dentinal dysplasia: Guys Hosp. Rep. radiolucencies. Oral Surg 1981;51:281.
1939;89:369-73. 114. Roed-Peterson B. NAsolabial cysts: a presentation of five
96. Carrol MKO, Duncan WK, Perkins TM. Dentin dysplasia: patients with a review of literature. Br J Oral Surg 1969;7:84.
Review of literature and a proposed sub classification based 115. Christ TF. The globulomaxillary cyst: an embryologic
on radiographic findings. Oral Surg 1991;72:119-25. misconception. Oral Surg 1970;30:515.
2

Caries in Children

Shweta Dixit Chaudhary, Mayur Chaudhary, Iqbal Musani,

Sanket Kunte, Gauri R Thakre (Chaudhary)

CHAPTER OVERVIEW
Introduction Caries activity tests
Definition Diagnosis of dental caries
History Caries assessment tool
Trends in caries epidemiology Caries risk assessment
Early theories of dental caries Prevention of caries
Current concepts in caries etiology Management of caries:
Saliva Remineralization of early lesions
Caries vaccine Restoration
Tooth Pulp treatment:
Plaque and plaque microflora Indirect pulp capping
Diet Direct pulp capping
Time Partial pulpotomy in permanent teeth
Classification of dental caries Vital pulpotomy
Histopathology of dental caries Non-vital pulpotomy
Early childhood caries Partial pulpectomy in permanent teeth
Caries susceptibility and caries activity Pulpectomy in primary teeth

INTRODUCTION DEFINITION
Epidemiologically, caries has become a dichotomous disease • A histopathologist may define caries in terms of the stages
in children in most developed countries: 50 percent of the of the lesion viewed microscopically
children are caries free whereas 20 percent of them account • A chemist may describe the process in terms of the inter-
for 75 percent of the lesions. This indicates a dramatic change relationship between pH, mineral flux and solubility at the
in the pattern of disease. Bowen, 1997, stated that while there tooth-saliva interface
is a decline in caries prevalence, it is clear that dental caries • A microbiologist on the other hand may define caries in
still remains the most prevalent disease afflicting humans.1 terms of interactions involving oral bacteria and the dental
Hence, a continued updating on the etiologic, preventive tissues
and management aspects of caries is required to develop an • Thus dental caries is a multifactorial disease, which is the
appropriate response to the current changes in patterns of result of interaction between the tooth (host) and the
disease. environment around it including the bacteria, saliva and diet
Dental caries is known to be a multifactorial disease. It of an individual with the time factor playing a significant
should be learned, not as small parts of individual subjects, role
but as a subject of cariology including the biochemistry, • Derived from Latin word for rot or decay
nutrition, oral pathology, oral microbiology, preventive • Greek word ‘Ker’ meaning death
dentistry, public health dentistry, pedodontics and operative • Shafer, 2007, defines dental caries as an irreversible
dentistry aspects. microbial disease of the calcified tissues of the teeth,
56 Essentials of Pediatric Oral Pathology

characterized by demineralization of the inorganic portion • Fairly extensive decay was seen in one skull of a Rhodesian
and destruction of the organic substance of the tooth, which man from Neanderthal age
often leads to cavitation.2 • About one half of 24 skulls of prehistoric race from central
• Newbrun, 1989, defines dental caries as a pathological Europe 15,000 years ago showed dental caries
process of localized destruction of tooth tissues by • Lots of studies demonstrate that populations which have
microorganisms.3 not acquired dietary habits of modern, industrialized man
• Ostrom, 1980, defines dental caries as a process of enamel have relative freedom from dental caries
or dentin destruction that is caused by microbial action at • Prevalence of dental caries was low in Australian
the tooth surface and is mediated by physiochemical flow aborigines, New Zealand Maoris, Eskimos, Ghanaians,
of water dissolved ions. tristan da Cunhans and Bantu tribes of South Africa prior
• Hume, 1993, defines dental caries as essentially a prog- to exposure to European diet.
ressive loss by acid dissolution of the apatite mineral
component of the enamel then the dentin, or of the
TRENDS IN CARIES EPIDEMIOLOGY
cementum then dentin.
• Pinkham, 2005, defines dental caries as a dietary See Table 2.1.
carbohydrate-modified infectious disease in which saliva
functions as a critical regulator.4 EARLY THEORIES OF DENTAL CARIES5

HISTORY WORM THEORY

• First evident in Homosapiens since paleolithic times • According to ancient Sumerian texts, toothache was caused
• von Leeuwenhoek observed that dolichocephalic skulls of by worms that drank the blood of the teeth and fed on the
men from preneolithic periods showed no caries roots of the jaws
• Skulls from brachycephalic men showed carious teeth • Clay tablets have been discovered from the Mesopotamian
• No carious lesions were seen in the Pithecanthropus men area dated 5000 BC engraved with the legend of the worm.

TABLE 2.1: Epidemiology of dental caries

Sr. No. Investigators Year Place Age group No. of Prevalence DMFT/deft
(years) Children

1. Kokila 1951 Gujarat 3-15 12.60

2. Chaudhary and Chawla 1957 5-16 2991 1.9/11.1
3. Miglani and Sharma 1963 15-25 1125 5.0
4. Tiwari and Chawla 1977 Chandigarh 6-16 1511 3.93
5. Brunelle and Carlos 1987 Bethesda, Maryland 5-17 50%
6. Holt 2001 Greater Manchester 3-4 762 1.4
7. Pankaj et al 2002-03 Delhi (urban) 5-6 1.3
(rural) 1.1
8. Rawalani et al 2002-03 Wardha 12 3.8
9. Pankaj et al 2002-03 Delhi (urban) 15 1.7
(rural) 1.5
10. Jose and King 2003 Kerala 8-48 months 44% 1.84
(mean=2.5)
11. Mahesh 2005 Chennai 5-6 3.51
12. Hegde et al 2005 Belgaum 12 2.41
13. Goyal A et al 2007 Chandigarh 6 4.0
9 4.61
12 3.03
15 3.82
14. Livny et al 2007 Jerusalem 1-3 102 17.6%
Caries in Children 57

• Oracle bones from the Shang dynasty dated before 1000 BC • Ficinus, 1847, noticed filamentous organisms called
bear the Chinese character for caries depicting the worm denticolae in carious material, which he thought were
invading the mouth responsible for decomposition of teeth
• This theory was earlier universally believed including • Orland and Fitzgerald, Jordan and Achard emphasized that
China, India, Finland, Scotland, etc caries cannot occur without microorganisms
• Guy de Cahuliac (1300-1368), surgeon, advocated • Dubois, 1954, stated that microorganisms had a toxic effect
fumigation with seeds of leek, onion and hyoscyamus on tooth tissue which led to its breakdown
• Fumigation was also used by the Chinese, Egyptians and • Antony von Leeuwenhoek, from early observations of
British in the 19th century scrapings, suggested that caries is a microbial process.
• Antony von Leeuwenhoek (1700), ‘father of modern
microscopy’, described little worms “taken out of a corrupt CHEMOPARASITIC THEORY
tooth” and said that they caused the pain in toothache
• This is a blend of two theories and finds the maximum favor
• Shakespeare has also mentioned worms in a tooth in his
among the researchers
famous play “Much Ado about Nothing”
• Willoughby D Miller, 1890, has been credited with this
• Japanese: Mush-ha=hollow teeth, carious teeth. The
theory, but tremendous work was done by Pasteur and Emil
Chinese also follow similar terminology.
Magitot before him
• Pasteur discovered that microorganisms transform sugars
HUMORS
to lactic acid in the process of fermentation
• According to the ancient Greeks, there are four elemental • Emil Magitot, 1867, demonstrated that fermentation of
fluids—blood, phlegm, black bile, yellow bile. Thus the sugars caused dissolution of tooth in vitro
four humors—sanguine, phlegmatic, melancholic, choleric. • Berlin, Leber and Rottenstein, 1867, implicated bacteria
Any imbalance in these causes disease as the causative agent, as they observed Leptothrix buccalis
• Galen, a Greek physician, suggested that it was this internal in dentinal tubules of carious teeth
action of acid and corroding humors that caused caries • Underwood and Miles, 1881, observed micrococci, oval
• Hippocrates accepted the prevailing Greek philosophy but and round bacteria in histological sections of carious teeth.
also drew attention to stagnation of food resulting in caries • Miller learned methods of isolating and staining in Koch's
• Aristotle mentioned that soft sweet figs putrefied and laboratory and demonstrated the following facts in a series
produced damage to the teeth. of experiments:
— Acid was present within the deeper carious lesion as
VITAL THEORY shown by reaction to litmus paper.
• This theory regarded caries as originating within the tooth
itself analogous to bone gangrene
• This theory was proposed at the end of the 18th century
and remained dominant till the middle of the 19th century
• Caries is known to have extensive penetration into the
dentin with a barely detectable catch in the fissure; hence
the support for this theory.

CHEMICAL THEORY
• Parmly, 1819, rebelled against the vital theory. He suggested
that a ‘chymical agent’ is responsible for caries.
• Robertson, 1835 and Regnart, 1938, carried out experiments
and showed that different dilutions of inorganic acids
corroded enamel and dentin.

PARASITIC OR SEPTIC THEORY

• Erdl, 1843, described filamentous parasites in the ‘surface FIGURE 2.1: Stephen's curve. A plot of the pH of plaque before
membrane’ of teeth and after a glucose mouth rinse. (After Stephan and Miller, 1943)
58 Essentials of Pediatric Oral Pathology

— Different kinds of foods (bread, sugar, but not meat) • Rapid growth of S. mutans lactate major end product
mixed with saliva and incubated at 37°C could • Slow growth of S. mutans volatile fatty acids predomi-
decalcify the entire crown of a tooth nate
— Several types of mouth bacteria (at least 30 species • Other metabolic processes within plaque (Fig. 2.5):
were isolated) could produce enough acid to produce decarboxylase
dental caries — L-amino acid ––––––––––––> amine CO2
— Lactic acid was an identifiable product in carbo- directly
hydrate-saliva incubation mixtures — RCHNH2COOH + 0.5 O2 ––––––> RCOCOOH + NH3
— Different microorganisms (filamentous, long and short L-amino acid -keto acid
bacilli and micrococci) invade carious dentin.
• Williams, 1897, observed that plaque on enamel surfaces
localizes organic acids and prevents their dilution and
neutralization by saliva.

Evaluation
Drop in pH
• Amount and duration of the drop in pH is influenced by:
— Amount of interdental plaque
— Predominant flora
— Rate of salivary flow FIGURE 2.3: Schematic representation of acid formation by
— Type of concentration of substrate micro-organisms (lactobacilli) responsible for dental caries
— Location of the plaque
• Lactic, acetic, propionic, formic and butyric acids have
been identified in plaque (Figs 2.3 and 2.4)
• Plaque consists of both homo and heterofermentative
organisms
• S. mutans is homofermentative, i.e. 90 percent lactic acid
is formed alongwith other acids, CO2, ethyl alcohol
• Actinomyces can be both homo- and heterofermentative
depending on the environment (Fig. 2.2)
• Veillonella, peptostreptococci, arachnia and propioni-
bacteria on fermentation produce propionic acid.
• Neisseria produces acetate by utilizing glucose, pyruvate
and lactate. FIGURE 2.4: Schematic representation of acid formation by micro-
organisms (S. mutans and S. sanguis) responsible for dental caries

FIGURE 2.2: Schematic representation of acid formation by FIGURE 2.5: Schematic representation of formation of -keto
micro-organisms (actinomy as responsible for dental caries) acid as one of the metabolic processes within plaque
Caries in Children 59

In anaerobic bacteria, the Strickland reaction forms ammonia include hemoglobin (containing iron), chlorophyll (con-
as follows: taining magnesium), vitamin B12 (containing cobalt), etc
RCHNH2COOH + 2R’CHNH2COOH + H2O • Chelation has been proposed as an explanation for tooth
COOH + CO2 + 2R’CH2COOH + 3NH3 decay, whereby the inorganic components of enamel can
be removed at a neutral or alkaline pH
Stephen's Curve (Fig. 2.1), outlining the drop in pH after a
• The initial attack is on the organic components of enamel,
glucose rinse in persons with varied caries activity has formed
whose breakdown products have chelating properties and
the basis of understanding the role of glucose in caries since
thereby dissolve the minerals in the enamel
decades.
• March et al, 1971, proposed the hypothesis that deminera-
Thus, the chemoparasitic theory remains the most widely
lization is initiated at acid pH and continued by complex-
accepted, with newer experiments throwing more light on the
forming agents when the pH of plaque is neutral.
intricate biomechanisms of caries.
Evaluation
PROTEOLYTIC THEORY
• Although chelators are present in plaque and saliva, it is
• The human tooth consists of only 1.5 to 2 percent organic not clear whether they are present in sufficient quantity.
material • Amount of calcium removed as an ionic salt v/s calcium
• According to Gottlieb, 1944, the initial action on the tooth chelate complex is also not clear.
was due to proteolytic enzymes attacking the lamellae, rod • Zipkin, 1953 and Larson and her associates, 1959,
sheaths, tufts and walls of the dentinal tubules performed animal studies with EDTA in the cariogenic diet
• He based these observations on histological specimens and and showed that it resulted in an increase in the severity of
the similarity between carious enamel and enamel whose dental caries as well as a difference in the distribution
organic components were stained with silver nitrate pattern of lesions.
• Frisbie, 1944, described caries as proteolytic involving
depolymerization and liquefaction of the organic matrix of SUCROSE—CHELATION THEORY
enamel
• Pincus, 1949, contended that the proteolytic organisms • Also called as the phosphate sequestration theory.
attacked the protein elements initially, then prism sheaths • Eggers-Lura, 1967, suggested that sucrose itself and not
were destroyed and the loosened prisms fell out. He also the acid derived from it can cause dissolution of the tooth
suggested that sulfatases of Gram negative bacilli by forming an ionized calcium saccharate. Calcium
hydrolyzed “mucoitin sulfate” of enamel or chondroitin saccharates and calcium complexing products require
sulfate of dentin and produced sulfuric acid. inorganic phosphate, which is subsequently removed from
the enamel by phosphorylating enzymes.
Evaluation • Luoma, 1964, suggested that since bacteria require
phosphate, inorganic phosphate from the tooth is taken up
• Gottlieb, Frisbie and Pincus were all histologists, who by plaque bacteria.
observed slides and assumed a mechanism of action of the
organisms, which are biochemical events. However, in early Evaluation
enamel lesions, there is an actual increase in organic matter • Saliva itself is an abundant source of inorganic phosphates,
• Also, nonproteolytic bacteria have also been known to hence it is highly unlikely that microorganisms would
cause extensive cavitation remove complex phosphates from the tooth, rather than the
• No experimental support was obtained for this contention. easily available ones.
• Reinvestigation failed to confirm the theory.
PROTEOLYSIS—CHELATION THEORY • Soluble complexes between sucrose and calcium oxide
• Schatz and Martin, 1955, challenged the chemoparasitic and between sucrose and calcium hydroxide can be
theory and advocated the proteolysis—chelation theory formed even at an alkaline pH, although not with calcium
stating that acid may actually prevent tooth decay by phosphate.
interfering with growth and activity of proteolytic bacteria
thus protecting the enamel organic matter AUTOIMMUNE THEORY
• Greek chele = claw. A chelating agent is a molecule capable • Burch and Jackson, 1966, based on their epidemiologic
of seizing and holding a metal ion in a claw like grip and studies, suggested that genes determine whether a site on a
forming a heterocyclic ring. Well known chelates in biology tooth is at risk or not.
60 Essentials of Pediatric Oral Pathology

Evaluation • In EB-junctionalis, enamel has greater porosity and thus

increased surface area for the effects of acids generated by
• Jenkins, 1961, argued that the data is purely epidemio-
cariogenic bacteria
logic.6
• Enamel contains large amounts of serum albumin that
• Four studies state that there is a statistically significant
inhibits crystal formation and thus remineralization of
genetic component in susceptibility to caries. altered sites
• Evidence of a genetic contribution to caries was based on • Genetic origin for EB-junctionalis has been linked to one
four questions as a part of a questionnaire: of three different genes: laminin 5, b 4-integrin and Type
— Altered dental hard tissue XVII collagen
— Immune response • All have the potential to alter the relationship of the
— Dietary consumption of sugar ameloblast to the developing enamel extracellular matrix
— Saliva and thus lead to a primary defect in the enamel hard tissue.
• Bachrach and Young, 1927, examined 301 pairs of twins
of which 130 were monozygotic and 171 dizygotic.7 Genetic modification of immune response
• 93 were same sex dizygotic and 78 were different sex • Inherited or acquired immune deficiency subjects an individual
dizygotic. to increased risks for and incidence of dental caries.
• They proved that heredity plays a subsidiary part in • Specific immune complex molecules for association with
inheritance of caries. increased risk for caries have been identified.
• Lehner et al, 1981, examined 24 individuals and found that
• Goldberg, 1930, reported that identical twins showed decay
HLA DRw6-1, 2, 3 had a significant relationship to the
in corresponding teeth.8
DMFS index and to low dose response to Streptococci
• Few investigators state that heredity affects only the shape
mutans antigens.10
of a tooth, its pits and fissures, its position in the dental
• HLA-DR4 was found to have no association.
arch.
• de Vries, 1985, reported no association between HLA-DR
• Caries experience of monozygotic twins had greater
types and caries incidence.11
concordance.
• Studies by Senpuku,199812 and Acton, 1999,13 correlated
• Mansbridge, 1959, observed 224 pairs of twins and specific HLA DR types with binding S. mutans antigens
suggested that environmental factors had a greater influence and S. mutans colonization.
on development of caries.9 • Acton concluded that “genes within MHC modulate the
• Comparison between pit and fissure and smooth surface level of oral cariogenic organisms”.
caries was also done, but equal genetic weight cannot be
ascribed to both. Inherited alterations in sugar metabolism
• DMF difference between twins was most dramatic for • There is a paradoxical relationship between sensitivity to
smooth surface caries on anterior teeth. sweet taste and caries incidence
• Hereditary fructose intolerance is a condition which results
Genetic modification of dental enamel was also examined: in low caries incidence
• Highly defined clinical phenotype to identify the altered • Inherited defects in sugar metabolism would most likely alter
biomineralized matrix protein and to begin to search the substrate availability in a manner identical to any other
genome for linkage to a precise change in DNA sequence dietary restriction and not by a genetically unique
was delineated mechanism.
• Epidermolysis bullosa (EB) has been shown to have both Genetic regulation of salivary gland function
an alteration in the enamel and an increased caries incidence Xerostomia is associated with dramatically increased rates of
• The mutations in EB result in four different forms of the dental caries.
disease: Recessive dystrophic, Dominant dystrophic,
Junctionalis and the Simplex types NUTRITIONAL DEFICIENCY THEORY
• Wright examined 252 patients of EB-junctionalis and EB-
recessive dystrophic. Both were associated with an increased Insufficient phosphate intake and improper dietary calcium-to-
incidence of dental caries phosphate ratio may contribute to the development of caries.
• Junctionalis form showed altered chemical composition of
the enamel, whereas recessive dystrophic form does not Evaluation
exhibit altered enamel No experimental support.
Caries in Children 61

BACTERIAL ALKALINE PHOSPHATASE THEORY

It was suggested that bacterial alkaline phosphatase was
found to release phosphate from enamel in vitro, thus
participating in caries destruction by acting on phos-
phoproteins of enamel.

Evaluation
• Commercial enzyme preparation was utilized and it was
observed that ammonium sulfate itself can release
phosphate from teeth in the absence of bacteria
• Alkaline phosphatase is an intracellular enzyme. Hence
lysis of the cells would have to occur to free the enzyme.

CURRENT CONCEPTS IN CARIES ETIOLOGY

Keyes’ triad given in 1960 (Fig. 2.6):14 FIGURE 2.7: Schematic representation of factors of tetrad
• Interplay of three principle factors: (Newbrun, 1982)14
1. The host (saliva and teeth)
2. The microflora Etiology
3. The substrate or diet • Sjögren’s syndrome
• In addition, a fourth factor time must be considered. Hence, • Therapeutic radiation
it is now considered to be a tetrad (Newbrun, 1982) • Surgical removal of salivary glands
(Fig. 2.7).14 • Chronic administration of anticholinergic drugs
• Congenital absence or malformation
HOST FACTORS: SALIVA • Anxiety, mental stress and depression
• It is a mixture of secretions in the oral cavity, mostly from
the major and minor salivary glands Alterations in the Oral Cavity Due to Xerostomia
• Xerostomia: (xeros = dry; stoma = mouth) • Longer eating (modification of eating habits to compensate
• First described by Bartley in 1868 decreased salivary flow)
• Xerostomia has been explained in detail in the section on • Greater food retention
Salivary gland lesions in children. • Possible alterations in bacterial flora of the mouth
• Decreased mineralization of enamel

Management
Management of patients with xerostomia is based on
symptomatic treatment:
• Fluoride therapy
• Dietary control
• Oral hygiene
• Avoidance of xerostomic drugs
• Use of artificial saliva (Orex saliva substitutes, xero-
lube) (Table 2.2)

Salivary Composition and its Relation to Caries

Salivary buffers
• Buffer is a solution that tends to maintain a constant pH
• In saliva, the chief buffer systems are bicarbonate-carbonic
acid and phosphate, of which bicarbonate is the most
important salivary buffer due to the following factors:
FIGURE 2.6: Schematic representation of Keye’s triad — It can buffer rapidly by losing carbon dioxide
62 Essentials of Pediatric Oral Pathology

TABLE 2.2: Components of various saliva substitutes — Animal studies

i. Local immunity (direct stimulation of sIgA)
Ingredients Category
ii. Oral immunization (indirect stimulation of sIgA)
Salt NaCl, KCl, CaCl2, MgCl2, etc. iii. Stimulation of serum antibodies
Lubricant Carboxymethyl cellulose, animal mucins, — Human studies
glycerin i. Secretory IgA
Sweetener Sorbitol, xylitol ii. Serum antibodies
Flavor Mint, wintergreen, coriander-spice, lemon — Passive immunity
Preservative Methyl p-hydroxybenzoate, paraben — Outlook towards caries immunization
Color FDC Red Dye No. 40 — Modern techniques of genetic bioengineering
Therapeutic NaF
Animal Studies
— Its pK is close to that encountered in plaque, and
therefore, it is more effective in that range • Animal studies involve active immunization against
— As the flow rate increases, the bicarbonate concentra- streptococcus mutans done with the help of antigens
tion increases rapidly, whereas phosphate falls slightly derived from killed S. mutans, cell extracts, cell free culture
with increased flow rate fluid and glucosyltransferase (GTF)
— After removal of bicarbonate, the buffering capacity of • Complete Freund's Adjuvant (CFA) may also be used.
saliva is markedly reduced.
Local immunity
• Urea is continuously secreted in saliva. Plaque micro-
• Rodents were immunized by repeated injections near the
organisms can convert urea to other nitrogenous products
and ammonia. This ammonia, thus formed can also act as parotid and submandibular glands with the vaccines
a buffer prepared from S. mutans (either killed cells or GTF)
• Longitudinal studies have proved that patients having • Salivary IgA was formed that agglutinated S. mutans or
higher buffer capacity tend to have less caries inhibited glucans synthesis
• Critical pH: it is not a particular value, but a concept wherein • Greater reduction of caries on smooth surfaces than occlusal
it can be considered as the pH at which any particular saliva surfaces was observed.
ceases to be saturated with calcium and phosphate. Hence, Oral immunization (Fig. 2.8)
below this value, the inorganic material of a tooth may
• Peyer's patches in the gut associated lymphoid tissues
dissolve
contain B cells that populate the lamina propria of the
• It varies according to the calcium and phosphate
concentration but is usually about 5.5 for hydroxyapatite gastrointestinal tract and become IgA producing plasma
and 4.5 for fluorhydroxyapatite. cells. These cells migrate to local sites and the antigen
sensitized T and B cells pass through the mesenteric lymph
Antibacterial factors in saliva nodes and enter the blood stream via the thoracic duct
• Lysozyme: lymph. From the blood stream, these cells settle in the
— Found in saliva, tears, egg white, tissues and body fluids lamina propria of the gut, upper respiratory tract and
— Hydrolytic enzyme which acts on cell wall peptido- glandular tissues (mammary, lacrimal, salivary, etc)
glycans of bacteria and lyses many cariogenic and • Studies suggest that salivary IgA responses can be induced
noncariogenic streptococci
by oral administration of S. mutans antigens of various
• Salivary peroxidase and thiocyanate ion:
forms, and that the antibody-mediated protection may result
— Acts on hydrogen peroxide generated by certain
bacteria from inhibition of GTF activity or from reaction with cell
— The oxidation reaction can inactivate various enzymes surface receptors important in adherence.
of glycolytic pathway and thereby temporarily inhibit Stimulation of serum antibodies
the growth, respiration and metabolism of most species • Animals are repeatedly injected using killed cariogenic
of oral bacteria. bacteria
• Thus, these animals develop high serum levels of specific
HOST FACTORS: IMMUNIZATION antibodies (IgG) to these bacteria
(CARIES VACCINE) • However, the effect on caries was not found to be uniform
• Humans are blessed with humoral and cellular immunity. • Intraoral submucosal injection is apparently more effective
• Studies on immunity against caries include the following: than intravenous, based on experiments on monkeys done
Caries in Children 63

FIGURE 2.8: Schematic representation of pathway for induction of sIgA responses in distant mucosal tissues

with intravenously and submucosally administered avirulent • Oral administration of GTF from S. sobrinus raised the
strains of S. mutans and GTF level of anti-GTF salivary IgA and interfered with
• McGhee and Michalek, 1981, stated that since sIgA is the repopulation of the oral cavity by S. mutans; however, these
principal immunoglobulin in external secretions, including effects were short lived.
saliva, local induction of antibodies of these types should
Serum antibodies
be of greater importance in caries immunity.15
• Presence and titer of serum antibodies to S. mutans and
S. sanguis increases with age from early infancy to around
Human Studies
16 years of age
Secretory IgA • It is not a very successful mode of immunization because
• Volunteers swallowed capsules containing formalin killed S. mutans possesses antigenic components that elicit
S. mutans daily for several weeks. antibodies that cross react with human heart muscle
• Salivary and lacrimal IgA antibodies were detected within (sarcolemmal sheaths)
one week; however no change in serum antibody titers was • Any vaccine that may induce myocarditis represents an
noted unacceptable risk with no justifiable gain
64 Essentials of Pediatric Oral Pathology

Passive Immunity of decay in descending order is occlusal, buccal, mesial,

distal and lingual.
• Studies on rats have shown that the offspring of vaccinated
• There is a variation in susceptibility to caries between
rats were protected from colonization by S. mutans by the
different tooth types. Most susceptible permanent teeth are
passive transfer of antibody via the colostrum and milk
the mandibular first molars then maxillary first molars, then
• In humans, infection by S. mutans does not occur till the
mandibular and maxillary second molars.
deciduous teeth erupt, and by that time, most of the infants
• Irregularities in arch form, crowding and overlapping of
are no longer nursing
the teeth also favor the development of carious lesions.
• Hence, tests were done where weanling rats were fed
mil k either from cows that had been previously
Tooth Composition
immunized with S. mutans vaccine or from nonimmunized
cows. When the rats were subsequently challenged with • Enamel surface is more caries resistant than the subsurface,
an infecting dose of S. mutans, those rats which had as it is continuously being regenerated by precipitation of solid
passively received the antibody in cow's milk were phases like dicalcium phosphate dihydrate and fluorapatite.
protected against the infection. • Surface enamel has more minerals and more organic matter
but relatively less water.
Outlook Towards Caries Immunization • Small microdefects at the enamel surface reaching the
deeper enamel layer are also seen, which may contribute
• The agglutinating activity of standardized sIgA against
to the development of caries.
strains of oral streptococci changes over time because of
detectable alterations in bacterial antigens
PLAQUE (MICROFLORA)
• Serum antibodies may be an effective approach to
vaccination, but the application of the vaccine requires • Dental plaque is a tenacious microbial deposit which forms
elimination of possible antibody cross reactions with human on the hard tissue surfaces of mouth, comprising living,
heart muscle. dead and dying bacteria and their products, together with
the host compounds derived from saliva
Modern Techniques of Genetic Bioengineering • Plaque contains the microorganisms involved in initiation
and propagation of caries
• Gene from S. mutans (GTF) is inserted in DNA of E. coli
• Plaque is generally found in anatomical areas protected
and specific proteins are synthesized. Thus, antibodies
from host defenses, e.g. occlusal fissures, interproximal
against S. mutans are produced which can be administered
areas, etc. (Fig. 2.9)
to humans.
• Specific and nonspecific plaque hypothesis:
• Spleen cells of mice are immunized with cell wall antigen
— Although streptococcus mutans is recognized as the
to myeloma cells thereby generating a murine hybridoma
major organism for formation of caries, there is still
cell line, which secretes antibodies against cell wall
controversy whether a specific group of organisms are
antigens. These antibodies are purified and subjected to
responsible for formation of caries, i.e. the specific
affinity chromatography. Culture fluid of S. mutans is
plaque hypothesis or disease is caused by a hetero-
passed through the column and only the specific antigen is
geneous mixture of nonspecific bacteria, i.e. the
absorbed. Thus, protein antigens that are suitable as a
nonspecific plaque hypothesis.
vaccine but do not elicit antibodies cross reacting with
cardiac tissue can be prepared in unlimited quantities.
• While a vaccine against caries is not imminent, the future
partial prevention of caries by this means has been predicted.

HOST FACTORS: TOOTH

Tooth Morphology and Arch Form

• Pit and fissure areas of posterior teeth are highly susceptible
to caries as food debris and microorganisms readily impact
in the fissures.
• Certain surfaces of tooth are more prone to decay whereas FIGURE 2.9: Schematic representation of type of plaques and
others are not, e.g. in mandibular first molar, likelihood areas in which they occur
Caries in Children 65

FIGURE 2.10: Intermolecular interactions involved in plaque formation

— There is a lot of controversy about the specific plaque receptors (cryptitopes) for the other species. This is an
hypothesis because some authors say that streptococcus important factor in regulation of colonization (Gibbons
mutans may not be found at the same site in the same et al 1989)16
mouth at different times and also although — S. mutans is less efficient than S. sanguis in adhering
streptococcus mutans is responsible for formation of to the tooth surface.
caries, there are some nonspecific microorganisms • Microbial succession:
having potential for formation of caries. — As microbiota ages there is a shift from streptococcus
• Microbial colonization in plaque occurs in the following dominated plaque to plaque dominated by actinomyces
manner (Fig. 2.10): (Loesche and Syed, 1978).17 Such population shifts are
— Initially, microorganisms attach to the tooth surface by known as microbial succession
van der Waal’s forces. Firm attachment is achieved by • Pioneer bacteria:
a specific mechanism between molecules of microbial — The first bacteria to be established in plaque are the
cells and pellicle pioneer bacteria
— Initial colonizers are S. sanguis, S. oralis and S. mitis • Climax community:
alongwith Actinomyces and gram negative bacteria, e.g. — The plaque environment is attractive to secondary
Hemophilus and Neisseria invaders or unfavorable for the preceding microbial
— Attachment by recognition system on bacterial surface colonies because of the lack of nutrients. Decrease in
enables components of bacterial surface adhesions to oxygen results in shift of flora from aerobes to obligate
bind to the complementary molecules in the pellicle anerobes. The dying bacteria are constantly replaced
— Microbial enzymatic activity results in destruction of by new ones. As a result of this dynamic process, the
receptors for some species and also creates new hidden plaque mass reaches a critical size at which a balance
66 Essentials of Pediatric Oral Pathology

between the deposition and loss of plaque bacteria is • Anaerobic Eubacterium Saburreum also formed a consider-
established; this community is termed as the climax able proportion of bacteria in advanced cavitated dentinal
community lesions.
• Microbiology of mature smooth surface/approximal plaque:
— Prominent microflora belong to genus actinomyces Pathogenic Properties of Cariogenic Bacteria
especially A. naeslundii (Bowen et al 1975).18 Mean • Ability to transport fermentable sugars rapidly when in
percentage of streptococci is lower. Veillonella species competition with other plaque bacteria and conversion of
are found in high numbers. sugars to acid
• Presence of intracellular and extracellular polysaccharides.
Dental Plaque and Caries Extracellular polysaccharides include glucans and fructans
• Streptococcus mutans is considered to be the main culprit both of which contribute to plaque matrix. Intracellular
behind causing smooth surface caries polysaccharides are glycogen like storage compounds that
• In a cross-sectional study, 71 percent of fissure caries had can be used for energy production and can be converted to
viable counts of S. mutans, i.e. greater than 10 percent of acid when free sugars are not available
total cultivable plaque microflora whereas 70 percent of • Ability to maintain sugar metabolism under extreme
caries free fissures had no detectable S. mutans (Loesche environmental conditions at low pH. S. mutans and
et al 1975)19 lactobacilli are acidogenic. This is because of:
• In a longitudinal study of fissures, proportions of S. mutans — Ability to maintain free intracellular environment and
increased significantly at the time of diagnosis of the lesion pump out protons even under acidic conditions.
or when a patient was prompted to visit a dentist because — Possession of enzymes with more acidic pH optimum.
of symptoms in the tooth (Loesche and Straffon 1979)20 — Production of specific acid-stress response proteins.
• In a study on Dutch army recruits aged 18 to 20 years where
S. mutans were subdivided, it was found that S. mutans MICROFLORA RESPONSIBLE FOR CARIES
serotype c was isolated from caries active as well as caries
• Gram positive cocci: Streptococci
free individuals while S. sobrinus was isolated from caries
— S. sanguis
active recruits
— S. mitior
• A longitudinal study on patients undergoing radiation
— S. mutans
treatment and showing rampant caries showed that there
— S. salivarius
was increased number of lactobacillus and S. mutans in
— S. milleri
saliva and plaque
• Gram negative cocci:
• Similar organisms were found in early childhood caries
— Neisseria and Branhamella
(nursing bottle caries) suffered by young infants.
— Veillonella
Microbiology of Root Surface Caries • Gram positive rods and filaments:
— Lactobacillus
• Early studies have indicated the role of actinomyces. — Actinomyces
• Billings et al, 1985,21 Brown et al, 1986,22 suggested that — Arachnia
S. mutans alone or in combination with lactobacilli were — Eubacterium
found in higher numbers in root surface carious lesions. — Propionibacterium
• Studies have shown that microflora of root surface caries, — Bacterionema
in addition to S. mutans and lactobacilli, commonly — Rothia
includes species belonging to actinomyces, non-mutans — Bifidobacterium
streptococci, bifidobacterium, rothia, viellonella, candida, • Gram negative rods and filaments:
enterococci and other gram negative species (Bowden, — Bacteroides
1990,23 Nyvad and Kilian, 1987).24 — Fusobacterium
• In advanced lesions, significantly high levels of S. mutans — Capnocytophaga
were reported at the expense of actinomyces while lacto- — Selenomonas
bacilli were common from sites of soft and necrotic dentin
(Shupbach et al, 1996).25 Streptococcus
• Other studies have reported a high proportion of lactobacilli
and gram positive rods from infected dentin like A. israelli Streptococcus is a genus of spherical Gram-positive bacteria
and A. gerencseriae. belonging to the phylum Firmicutes and the lactic acid bacteria
Caries in Children 67

— S. salivarius: Present in saliva, epithelial surfaces of

tongue, throat and establishes itself in dental plaque
— S. sanguis: Present in dental plaque and is capable of
producing adhesive extracellular polymers which aid
in colonization of cariogenic organisms
— S. mitior: Commonly isolated from plaque, present in
smooth surface and pit and fissure caries alongwith
other strains
— S. milleri: Also present in dental plaque and produces
enzymes which help in sucrose degradation.
• Other streptococcal strains involved are S. oralis, S. lactis,
S. fecalis and S. bovis.

Lactobacillus (Fig. 2.12)

It is a gram positive bacillus, facultative anaerobe and found
in a small percentage in normal flora. Mostly lactobacillus
acidophilus and lactobacillus casei are mainly responsible for
FIGURE 2.11: Streptococci occurring in chains smooth surface caries.
group. Cellular division occurs along a single axis in these Role of lactobacilli:
bacteria, and thus they grow in chains or pairs, hence the name They are considered to be candidate organisms in caries
from Greek streptos, meaning easily bent or twisted, like a because:
chain (twisted chain) (Fig. 2.11). • Their high numbers in most carious lesions affecting enamel
have been reported
Role of streptococcus mutans:
• The positive correlation between their numbers in plaque
• It comes under the category of -hemolytic streptococci.
and saliva and caries activity has been established
• Evidence of the role of streptococcus mutans in dental
• Their ability to grow in low pH environments below pH 5
caries includes the following:
and to produce lactic acid has been established
— Correlations of mutans streptococci in saliva and plaque
• Their ability to synthesize both extracellular and
with the prevalence and incidence of caries. (Mattee
intracellular polysaccharides from sucrose also aids in the
MIN et al, 1992;26 Krishnakumar R et al, 2002;27
production of caries
Barsamian-Wunsch P et al, 2004;28 Ersin et al, 2006)29
• They have two major properties necessary for progression
— It can be isolated from the tooth surface immediately
of a carious lesion:
before development of caries
— Acidogenic: produce acids and cause demineralization
— Positive correlation has been found between the
of enamel surface
progression of carious lesions and S. mutans count
— Aciduric: particularly grow well in acid environment.
— S. mutans has the property of production of
extracellular polysaccharides from sucrose (which help
to cement the plaque organisms together and to the
tooth surface)
— It has the ability to initiate and maintain microbial
growth and to continue acid production at low pH
values
— It can rapidly metabolize sugars to lactic acid and other
organic acids
— Ability to attain critical pH for enamel demineralization
more rapidly than other common plaque bacteria
— Ability to produce intracellular polysaccharides as
glycogen which may act as a food store for use when
dietary carbohydrates are low.
• Other cariogenic streptococci species responsible for
induction of caries: FIGURE 2.12: Lactobacilli occurring as rods
68 Essentials of Pediatric Oral Pathology

Although the role of lactobacilli in caries is not as well

defined as streptococci, it is believed by most investigators that:
• They are involved more in the progression of deep enamel
lesions
• They are pioneer organisms in the advancing front of the
carious process especially in dentin.

Actinomyces
Actinomyces is a genus of the actinobacteria class of bacteria.
They are all Gram-positive and can be either anaerobic or
facultatively anaerobic. Actinomyces species do not form
endospores and while individual bacteria are rod-shaped
morphologically, Actinomyces colonies form fungus-like
branched networks of hyphae.
• Actinomyces naeslundii and actinomyces viscosus are
mostly found in dental plaque
FIGURE 2.14: Veillonella
• Trauma, foreign bodies or poor oral hygiene may favor
tissue invasion by actinomyces
They can be crushed between slides, gram stained and
• In human beings, they occur in four clinical forms:
then examined.
— Cervicofacial: with indurated lesion on cheek and
Under the microscope, gram positive filaments are
submaxillary region. Caused mainly due to dental
observed surrounded by radiating club shaped structures.
infection.
— Thoracic: with lesions in lung that may spread outwards Role of actinomyces:
through the chest wall. • These are associated with development of root surface
— Abdominal: where lesions are found around cecum with caries, in that the calcified tissues are softened without
involvement of neighboring tissues and abdominal wall. obvious cavitation.
— Pelvic • Some studies have reported both mutans streptococci and
• It has been incriminated in inflammatory diseases of the lactobacilli in these lesions.
oral cavity such as gingivitis and periodontitis and is also
found in subgingival plaque leading to root surface caries. Veillonella (Fig. 2.14)
Diagnosis is made by presence of sulfur granules which
• It is a coccus, an obligate anaerobe, isolated from most
are white or yellowish and range in size from minute specs
surfaces in oral cavity
to about 5 mm. They are examined microscopically under
• High numbers are found on tongue and also in most of the
a cover slip (Fig. 2.13).
supragingival plaque.
Role of veillonella:
• It is present in more numbers in supragingival plaque
samples
• They require lactate for their growth but are unable to
metabolize normal dietary carbohydrates; they therefore
utilize lactate and other intermediate metabolites formed
by plaque bacteria as energy sources and convert it into a
range of other less cariogenic organic acids, e.g. propionic
acid
• Hence, these organisms may have a beneficial effect on
dental caries.

DIETARY FACTORS
• Dental caries is widely accepted as being caused by the
FIGURE 2.13: Actinomyces showing yellow sulfur granules ingestion of fermentable carbohydrates particularly sucrose.
Caries in Children 69

• Fermentable carbohydrates are generally eaten as Hopewood house study, 1958, was a longitudinal study of
components of food that contain other ingredients and have ten years on 3 to 14 year children residing at Hopewood house,
different textures. Bowral, New South Wales.31
• The cariogenic potential of food depends upon: • These institutionalized children were genetically
— Its ability to be retained by teeth heterogeneous
— Its ability to form acids • They were on a strict lactovegeterian diet with absence of
— Its ability to dissolve enamel meat and restriction of refined carbohydrate, except for
— Its ability to neutralize buffer acids weekends. Between-meal snacks were limited to milk, fruit
• The frequency and time of ingestion of food are also important and raw vegetables
• Sucrose containing food becomes more cariogenic if it is • Caries prevalence was almost negligible in the primary
eaten frequently dentition in these children and approximately one tenth that
• Food eaten at meals produces less caries than eating the seen in the permanent teeth of the average Australian child
same food between meals. • Surprisingly, their oral hygiene was extremely poor (75%
were found to have gingivitis)
Important Studies Relating Diet with Dental Caries • As these children grew older and left Hopewood House, a
Vipeholm study, 1954, was a five year investigation of 436 adult sharp rise in caries rate occurred at about the age range 13
inmates at a mental institution at the Vipeholm hospital, Sweden.30 to 18 years
• According to the experimental design, the inmates were • This increase shows the deleterious effects to dental health
divided into seven groups with varying sugar intake: caused by a strongly cariogenic diet and also the fact that
— A control group the caries-free teeth did not acquire any permanent immunity
— A sucrose group (300 gm of sucrose given in solution to caries.
but reduced to 75 gm during the last two years)
— A bread group (345 gm of sweet bread containing Seventh day adventist children, 1958, complied to
50 gm of sugar daily) principles depending on religious motivation advocated by the
— A chocolate group (65 gm of milk chocolate daily Seventh Day Adventist dietary counsels.32
between meals during the last two years). • This included abstinence from certain animal proteins, and
— A caramel group (22 caramels [70 gm of sugar] in four limited use of sugar, sticky desserts, highly refined starches
portions between meals) and between meal snacking
— An eight-toffee group (eight sticky toffees [60 gm of • They showed a lower caries prevalence than the non-
sugar] daily for three years) adventist children in the same geographic location and
— A 24-toffee group (24 sticky toffees [120 gm of sugar] socioeconomic stratum.
for 18 months) Turku sugar studies, 1975, were carried out in Turku,
• This study gave the following conclusions: Finland by Scheinin, Makinen et al to test the effects of chronic
— An increase in carbohydrate (mostly sugar) definitely
consumption of sucrose, fructose and xylitol on dental and
increases the caries activity.
general health.33
— The risk of caries is greater if the sugar is consumed in
• In the two year feeding study, 125 young adults divided
a form that will be retained on the surfaces of teeth
into three experimental groups: sucrose group (N = 35),
— The risk of sugar increasing caries activity is greatest
if the sugar is consumed between meals and in a form fructose group (N = 38), xylitol group (N = 52), consumed
that tends to be retained on the surfaces of the teeth the entire dietary intake using these sugars exclusively
— The increase in caries activity varies widely between • They were investigated by a comprehensive program
individuals including clinical, radiographic, biochemical and
— Upon withdrawal of the sugar-rich foods, the increased microbiochemical determinants of health
caries activity rapidly disappears • Conclusions were:
— Caries lesions may continue to appear despite the — Sucrose chewing gum was found to be definitely
avoidance of refined sugar and maximum restrictions cariogenic
of natural sugars and dietary carbohydrate — Between meals chewing of xylitol gum produced an
— A high concentration of sugar in solution and its anticariogenic effect
prolonged retention on tooth surfaces leads to increased — Fructose was as cariogenic as sucrose for the first year,
caries activity but showed lower rates in the second year
— The clearance time of the sugar correlates closely with — Xylitol consumption resulted in a reduction in dental
caries activity. caries.
70 Essentials of Pediatric Oral Pathology

TIME — Acute caries

— Chronic caries
• Time factor is certainly not the least important of the factors
• Related to degree and rate of progression of caries:
involved in the production of caries
— Incipient caries
• Development of caries is a slow process that may extend
— Arrested caries
for months before the development of a perceptible
— Recurrent caries
cavitation but when the challenge is overwhelming, lesions
• Classification based on chronology:
may start to form within three weeks
• Hence, the time duration for which the tooth is subjected — Nursing bottle caries
to cariogenic food substrates is extremely important in — Adolescent caries
regulating the phases of demineralization and reminerali- • Classification based on location:
zation. — Primary caries
— Backward caries
— Forward caries
CLASSIFICATION OF DENTAL CARIES — Residual caries
• Based on tooth morphology: — Root surface caries
— Pit and fissure caries — Secondary caries
— Smooth surface caries • Classification based on extent of caries:
— Root cementum caries — Incipient (reversible)
— Linear enamel caries — Cavitated (irreversible)
• GV Black’s classification34 (Fig. 2.15): • A recent classification based on site and size proposed by
Class I: Caries involving occlusal surfaces of posterior Graham J. Mount and Hume, 199735 (Table 2.3).
teeth, caries involving occlusal 2/3rd of buccal and lingual • Site:
surfaces of posterior teeth, caries involving the lingual pits — Site 1: All lesions originating in pits and fissure
of maxillary incisors. — Site 2: All lesions associated with contact areas
Class II: Caries involving proximal surfaces of posterior — Site 3: Lesions originating close to gingival margin
teeth. • Size:
Class III: Caries involving proximal surfaces of anterior — Size 1: Lesion that has progressed to the point where it
teeth, not involving the incisal angle. is first beyond remineralization
Class IV: Proximal caries involving incisal angle of anterior — Size 2: Larger lesions, but there is still sufficient sound
teeth. tooth structure remaining to support the restoration
Class V: Caries involving labial or buccal enamel near the without further modification of the cavity beyond caries
dentino-enamel or cemento-enamel junction. removal
Class VI: Caries involving cusp tips of posterior teeth and — Size 3: More extensive lesion that leaves remaining
incisal edges of anterior teeth. tooth structure at a risk of further bulk failure
• Based on severity and rate of caries progression: — Size 4: Extensive lesion in which there has already been
— Rampant caries serious loss of tooth structure.

FIGURE 2.15: GV Black’s classification of dental caries

Caries in Children 71

TABLE 2.3: Classification of caries:Graham J. Mount and Hume, 1997

Sr. Size
No. Site 1 = minimal 2 = moderate 3 = enlarged 4 = extensive

1. Pits and fissures 1.1 1.2 1.3 1.4

2. Proximal surfaces 2.1 2.2 2.3 2.4
3. Cervical areas 3.1 3.2 3.3 3.4

HISTOPATHOLOGY OF DENTAL CARIES

• Familiarity with the shape of the lesion in different locations
is of fundamental importance in understanding the design
of cavity preparations.
• Caries is commonly considered a chronic disease in man
because lesions develop over a period of months or years.
• The average time from the stage of incipient caries to
clinical caries is 18 ± 6 months.
• In some patients with xerostomia, following radiation
therapy, caries can be detected clinically within 3 months.

CARIES OF ENAMEL
FIGURE 2.16: Schematic representation of various types of
Macroscopic Changes in Enamel fissures on teeth
On smooth surfaces: Loss of transparency is seen resulting in
opaque chalky region (white spot); this indicates subsurface
demineralization, where the critical pH of the subsurface
hydroxyapatite is reached and there demineralization begins;
however, there is no break in the surface of enamel yet.
• In lesions where caries has progressed more slowly or
become arrested, brown or yellow pigmentation of enamel
may be seen.
• When sectioned longitudinally, cavitated carious lesions are
cone shaped with the apex directed towards dentin.
• What determines the shape of the lesion is still not definitely
proved, but many investigators suggest that the carious
lesion follows the direction of the enamel rods and
hypocalcified structures of enamel.
On occlusal surfaces: There are deep invaginations called
fissures. They can be classified as (Fig. 2.16):
• V type: Wide at top and gradually narrowing towards the
bottom (34%)
• U type: Almost same width from top to bottom (14%)
• I type: An extremely narrow slit (19%)
• IK type: Extremely narrow slit associated with larger space FIGURE 2.17: Incipient caries showing chalky
white appearance on the tooth surface
at the bottom (26%)
• Other types (7%)
• On the occlusal surface, a carious lesion starts at both sides • In newly erupted teeth, a brown stain is indicative of
of the fissure wall rather than at the base, penetrating nearly underlying decay, while in teeth of older individuals, it may
perpendicularly towards the dentinoenamel junction be due to arrested or remineralized lesions
• Visual changes are chalkiness or yellow, brown or black • Lesions are cone shaped with base directed towards dentin
discoloration (Figs 2.17 and 2.18) and apex towards the enamel.
72 Essentials of Pediatric Oral Pathology

Translucent zone
• Only seen when longitudinal ground sections are examined.
• Formation of this zone appears to be the earliest change in
the advancing front of the lesion
• Appears structureless and characterized by approximately
1.2 percent loss of mineral
• Shows negative birefringence in polarized light
• Lesion appears radiopaque on radiographs
• Pore volume is one percent (intact enamel pore volume is
0.1%).
Dark zone
• Common feature of a carious lesion
• Shows positive birefringence in polarized light (normal
enamel has negative birefringence)
• Mineral loss is six percent
• Appears radiopaque on radiographs
• Pore volume two to four percent.
Body of lesion
FIGURE 2.18: Caries in deciduous teeth
• Largest zone
• Shows positive birefringence
• Mineral loss is 24 percent
• Corresponding increase in unbound water and organic
content due to ingress of bacteria and saliva
• Appears radiolucent on radiographs
• Pore volume 5 to 25 percent.
Surface layer
• Ranges between 20 and 100 µm thickness
• Appears unaffected in initial lesion as compared with
subsurface zones
• Negative birefringence in polarized light
• Radiopaque on radiographs
• Mineral loss 10 percent
• Pore volume <1 percent
FIGURE 2.19: Four histopathological zones of caries
Ultrastructural Changes in Enamel (Fig. 2.20)

Microscopic Changes in Enamel • Scattered destruction of individual apatite crystals

• Dissolution results in broadening of intercrystalline space,
• In early stages, caries causes minimal damage to the outer which becomes filled with amorphous material, some of
smooth surface but considerable demineralization occurs which gives a positive histochemical reaction of carbo-
below the surface. hydrates
• Histopathologically, 3-7 zones can be seen of which 4 zones • More advanced dissolution has been seen of the crystals that
are clearly distinguishable. are directed vertically into the demineralizing zone in the
• Starting from inner advancing front of the lesion (from center of heads of the prisms. There is a lesser degree of
bottom to top) (Fig. 2.19) damage to the crystals in the tails of the prisms that are
— Translucent zone distributed at an angle or lengthwise to the demineralizing
— Dark zone zone
— Body of the lesion • Dissolution starts in the center of one end of the crystal
— Surface layer and proceeds anisotropically along the C-axis. The central
Caries in Children 73

Zone of dentinal sclerosis

• Altered tubules acquire a refractive index similar to the
adjacent matrix presenting a homogeneous transparent
appearance in transmitted light. That is the translucent zone
of dentin identical to the sclerosed dentin.
• This dentin is impermeable to stains like methylene blue.
• It represents an attempt to block the advancing carious
lesion.
Zone of demineralization
• Next to sclerosed dentin, it is a narrow zone.
FIGURE 2.20: Ultrastructure of enamel caries • Affects the intertubular matrix.
spreading along C-axis anisotropically • Occlusion of tubules observed in this zone is due to
reprecipitation of crystalline material that had dissolved
hole thus formed extends along the entire crystal length. during the carious process.
The central dissolution parallels the lateral external crystal
Zone of bacterial decomposition
surface, resulting in hollow hexagons or rectangles. After
• Most noticeable change observed in carious dentin.
formation of the central hole, the dissolution extends as
• Lumen of tubule is distended giving a ballooned or dilated
lateral localized destruction towards the external surface.
• Bacterial colonies observed by scanning electron micro- appearance.
scope consist primarily of rod shaped and occasionally • These dilated appearances are referred to as “Liquefaction
filamentous micro-organisms. foci of Miller”.
• Misnomer because distentions are filled with bacteria and
CARIES OF DENTIN debris and not liquid.

Macroscopic Changes in Dentin Zone of decomposed dentin

• These dilatations eventually coalesce forming the outermost
• On reaching dentin, the carious lesion spreads laterally zone of decomposed dentin.
along the dentinoenamel junction such that a cone shaped • Cracks or clefts containing bacteria and necrotic tissue
lesion is formed, where the base of the lesion lies towards appear at right angles to the course of dentin tubules.
the DEJ and the apex points towards the pulp. • This zone may follow the course of incremental lines or
• The lesion proceeds along a saucer-shaped front and arise by coalescence of liquefaction foci or by extension
follows the direction of the dentinal tubules. of proteolytic activity along interconnecting lateral branches
• Affected dentin displays different degrees of discoloration of odontoblast tubules.
ranging from brown to dark brown to almost black.

Microscopic Changes in Dentin

• Pathological changes have been divided into five zones
from pulpwards to carious lesion (Fig. 2.21):
1. Zone of fatty degeneration
2. Zone of dentinal sclerosis
3. Zone of demineralization
4. Zone of bacterial invasion
5. Zone of decomposed dentin
Zone of fatty degeneration
• After specific staining, lipid has been seen in active lesions.
• Two types of lipid staining have been seen:
1. One that is superficial due to bacterial origin
2. Other is due to unmasking of lipids present in FIGURE 2.21: Histopathologic picture of dentinal caries showing
peritubular dentin various zones
74 Essentials of Pediatric Oral Pathology

STAINS TO DETECT CARIES The caries prevalence in the primary dentition of preschool
children in urban Pondicherry, India was found to be 44.4
• Enamel lesions: Permeable to methyl green and contain free
percent with 26.7 percent in the maxillary arch and 36.9 percent
calcium ions detected by Alizarin red S.
in the mandibular arch;39 the caries prevalence in preschool
• Normal enamel remains uncolored by these dyes.
children in Dharwad city, India, was found to be 54.1 percent,38
• Dentinal lesions: Decomposed and bacterial zones stain
a very good indicator of the widespread hold of caries over
red with basic fuchsin.
our society.
• Acid red dyes react with collagen that has been treated with
ECC is a result of an interaction of the factors involved in
lactic acid but not with intact dentinal collagens.
other types of dental caries, viz cariogenic bacteria, refined
carbohydrates, host factors and time. The dietary factors at work
EARLY CHILDHOOD CARIES in the causation of ECC include frequent consumption of
Way back in 1962, Dr. Elias Fass published the first liquids containing fermentable carbohydrates, particularly
comprehensive description of caries in infants. He can be through a nursing bottle at sleep times. Juices, sodas, infant
quoted as: “Nothing is as shocking to a dentist as the formula and sweetened beverages have been implicated in
examination of a child patient suffering from rampant caries.” ECC. When taken via a nursing bottle while the infant sleeps,
The foundations established by this paper have essentially these liquids pool around the maxillary incisors and can cause
remained unchanged over the past 45 years. Early childhood rapidly progressive, severe destruction of tooth structure
caries (ECC) is the new term for an infectious disease that beginning typically on smooth surfaces around the cervical
affects the teeth of infants and toddlers. Its prevalence rate in margins of teeth or labial, lingual and proximal surfaces of
developing countries has been shown to be as high as 70 primary maxillary incisors, areas otherwise considered to be
percent. The cost of treating ECC is very high and affected to a minor extent. This indicates a particularly virulent
unfortunately, the risk factors for ECC point to various form of caries, which starts soon after teeth erupt, earlier than
parenting practices such as the use of baby bottles containing 12 months to two years of age, and it proceeds rapidly, to
sweetened beverages.36 involve primary molars and canines.
During the first year of life, the infant undergoes a rapid Fass, 1962, emphasized the possible cariogenic effects of
development that is accompanied by drastic changes within the milk fed from a bottle to young children in order to soothe
oral environment. Some of these changes may be related to them and encourage sleep. Under these conditions, milk may
the emergence of the first teeth during this time period. The remain in the mouth and possibly in contact with teeth while
presence of teeth facilitates a shift in nutrition from fluid to saliva flow ceases and swallowing becomes infrequent.40
solid food, and concomitantly, the oral microbial community Breast milk is a complete nutritious diet for neonates.
changes, because the teeth provide new ecological niches for However, some children have to settle for infant milk formulae
bacterial colonization.37 which contain lactose as the main ingredient.41 These alternate
Children suffer from many infectious diseases during the sources of nutrition such as baby bottles containing infant milk
first three years of life around the time of eruption of the formulae or fruit juices are known to be more cariogenic than
deciduous teeth. Early childhood caries, which is a combination breast milk. Both soy-based and protein hydrolysate formula
of a child being infected with cariogenic bacteria and the contain non-milk extrinsic sugars such as sucrose and glucose
frequent ingestion of sugar, is one of such diseases. Despite syrup as carbohydrate resources. According to European
improvement over several decades, oral disease amongst the Society for Pediatric Gastroenterology, Hepatology and
children remains a serious problem.38 Nutrition (ESPGHAN), 1990, the manufacturers were allowed
Although the etiology of ECC is similar to other types of to add other sugars of no more than 20 percent of the total
coronal and smooth surface caries, the biology may differ in carbohydrate content in follow-on formula.42 Breast feeding
some respects. when continued for a prolonged period of time has also been
The bacterial flora and host defense systems in the young found to support the development of early childhood caries.
infant are in the process of being established. In addition, the Early childhood caries is a specific form of severe dental
tooth surfaces are newly erupted and immature, and may show caries that affects infants and young children. It is defined as
hypoplastic defects. the presence of more than one decayed (non-cavitated or
Although dental caries has been declining globally in the cavitated lesions), missing (due to decay), or filled tooth
general population, more so among older children, caries surface in any primary tooth in a child seventy one months
prevalence in younger ones has not shown a significant or younger. Severe early childhood caries has been described
decline.38 as in Table 2.4. This presentation of dental caries was formerly
Caries in Children 75

TABLE 2.4: Age and surfaces involved in High risk group children with primary teeth decay should
severe early childhood caries be identified and categorized, which in turn is useful to
Sr. ECC Age Presence of caries determine needs for restorations and to implement primary
No. preventive procedures in the targeted group.
1. Severe form <3 years of age Smooth surface caries
in > 1 tooth
RECOMMENDATIONS TO PARENTS BY
PEDODONTISTS
2. Severe form 3-5 years of age > 1 cavitated, missing,
or filled smooth surface • Parents should be educated from time to time about the
in primary maxillary
importance of diet in the development of caries (with
anterior teeth or
decayed, missing, or regards to the type of carbohydrate).
filled surface (dmfs) • Parents should be made aware of the fact that bovine milk
score of is lower in sugar than formula milk and is higher in the
Age-3 years Greater than 4 protective factors, calcium and phosphorus.47 Hence, they
Age-4 years Greater than 5
should be encouraged to provide bovine milk for their
Age-5 years Greater than 6
children when an alternative or an adjunct to breast milk is
termed “baby bottle tooth decay” by Mim Kelly et al, 1987,43 sought.
“nursing bottle caries” by Tsamtsouris, 1986, “rampant • Parents should be discouraged from the frequent use of
caries”, “primary tooth caries”, “milk bottle syndrome” by bottles for feeding, especially for prolonged periods at
Ripa, 1988,44 “early childhood caries” by Davies, 199845 and night.
“maternally derived Streptococcus mutans disease.” Nursing • Parents should be educated about the need for commence-
caries and rampant caries are both considered forms of early ment of tooth brushing or cleaning the teeth with clean
childhood caries and the differences between the two have gauze or a clean muslin cloth, as soon as the first teeth
been outlined as in Table 2.5. erupt.48
Since 1986, the American Academy of Pediatric Dentistry • Other primary preventive measures that can be applied
has adopted a position on infant oral health recommending that during this period include water fluoridation or, in its
the first visit occur within six months of eruption of the first absence, the administration of fluoride supplements and
primary tooth.46 topical applications of fluoride solutions or varnishes soon

TABLE 2.5: Differences between nursing caries and rampant caries

Sr. Nursing caries Rampant caries

No.

1. It is a specific form of rampant caries Defined by Massler as a suddenly appearing, widespread, rapidly
burrowing type of caries, resulting in early involvement of the
pulp and affecting those teeth usually regarded as immune to
ordinary decay
2. Usually seen in infants and toddlers Seen at all ages, including adolescence
3. Maxillary incisors affected first followed by molars. All surfaces considered immune to decay are involved including
Mandibular incisors characteristically remain unaffected mandibular incisors.
Rapid appearance and spread of decay is seen
4. Etiology primarily based upon improper feeding practices such Etiology more so based upon the caries tetrad modified by
as prolonged bottle feeding containing sweetened milk, fruit Newbrun.
juices, etc. at will breast feeding, pacifiers dipped in honey or
other sweeteners
5. Management is based upon: Management is based upon:
Education (oral hygiene and dietary counseling) Removal of carious lesions and reduction of Streptococcus
mutans count (caries risk)
Prevention by topical fluoride applications Long-term treatment for restoration of the teeth may be required
Removal and restoration of carious lesions Adequate follow up to keep the caries risk at a low level
6. Prenatal counseling of pregnant women with regards to Dental health education amongst all age groups
diet and oral hygiene
76 Essentials of Pediatric Oral Pathology

after the teeth erupt to increase the resistance of enamel to wax or gum or use a drop of citric acid on the tongue.
demineralization. Collect the saliva in a small graduated vial over a two
• Parents and caregivers should be trained to recognize the minute period. Stimulated flow rate of less than 0.7 ml/min
early signs of the condition, using the "lift the lip" should be followed by further assessment of other risk
technique. factors.
• Ismail, 1998 49 and Weintraub, 1998 50 supported the • Buffering capacity of saliva: It is possible to measure
development of a special “Fall-Asleep-Pacifier”, as buffering capacity using commercially available kits.
suggested by Suhonen et al, 1994,51 that includes a slow- However, salivary buffering capacity is usually proportional
releasing lozenge containing sodium fluoride, xylitol and to the flow rate, so the latter is generally considered to be
sorbitol. It is recommended specially for children who have a reasonable indicator.
any white spot lesions or incipient caries. Originally Dreizen test was used followed by the
As a preventive measure, the dental professionals must modified Dreizen test. Both of these are titration tests with
thrive hard to make appropriate feeding of infants and children, the endpoint being determined by a dye color change.
a national movement supported by all health professionals. The In 1980, Frostell introduced the Dentobuff system.52
parents and the pedodontist should work as partners in Paraffin is chewed for two mins and 5 ml of saliva is
providing oral health care for children with ECC. collected. 1 ml of saliva is taken in a vial of Dentobuff and
shaken for 10 secs. Then let CO2 evaporate for 2 mins. The
CARIES SUSCEPTIBILITY AND CARIES ACTIVITY color of the indicator in the vial is matched with the
standard chart.
An advance in health research has resulted in a major paradigm Results (Table 2.6): A simpler modification of the Dentobuff
shift in the diagnosis, treatment and management of dental system using a pH indicator strip has been introduced by
caries. Hence, identifying the risk groups followed by providing Ericson and Bratthall, 1989.54
preventive care is the key to primary prevention. It is a cost • Viscosity of saliva: A special Ostwald pipette with a
beneficial method which is holistic and suitable even among calibrated bore may be used to measure the viscosity of
the developing countries. saliva. 5 ml of water is taken in the pipette and allowed to
Caries susceptibility and caries activity tests have been flow under gravity. The time taken for this flow is measured
developed with a goal of understanding and identifying oral in seconds. This is followed by a 5 ml saliva specimen.
conditions and have been used to predict future caries activity
as well as measure the effectiveness of therapy. Time required for flow of saliva
_______________________________________
Relative viscosity =
DEFINITIONS Time required for flow of water
Normal value is around 1.5.
• Caries activity—Increment of active lesions (new and
recurrent lesions) over a stated period of time.
CARIES ACTIVITY TESTS
• Caries susceptibility—Inherent tendency of the host and
target tissue, the tooth, to be afflicted by the carious process. Uses of caries activity tests:
• Caries activity tests—Tests to measure the degree to which • To identify high risk groups and individuals.
the local environment challenge favors the probability of • To determine the need and extent of personalized
carious lesions. preventive measures.
• Sensitivity—The ability of a test to identify correctly which • To serve as an index of the success of therapeutic measures.
sound teeth will become carious is termed as sensitivity. • To motivate and monitor the effectiveness of education
• Specificity—The ability of a test to predict no change in progress relating to dietary and oral hygiene procedures.
the status is termed as specificity. • To evaluate the progress of restorative procedures.

CARIES SUSCEPTIBILITY TESTING TABLE 2.6: Results interpreted in buffer

capacity test in saliva
• Quantity and quality of saliva: This is initially determined
Sr. Final pH Color Buffering capacity
by visual inspection and questioning. The extremely dry
No.
mouth may be identifiable by oral examination. Sometimes,
the statement that they frequently drink water or other fluids 1. 3-4 Yellow Poor
is an indicator of prolonged dryness. Carry out a stimulated 2. 4.5-5 Green Intermediate
3. 5.5-6.5 Purple Good
salivary flow rate by asking the patient to chew the paraffin
Caries in Children 77

Ideal Requisites of a Caries Activity Test Results following color observations in Snyder's test
(Table 2.8)
• Maximum correlation between predicted and actual caries.
• Reliability and validity. TABLE 2.8: Results interpreted in
Colorimetric Snyder test
• Simplicity with regard to technical procedures and skills
required. Color 24 hours 48 hours 72 hours
• Rapid results within few hours or days. Yellow Marked caries Definite caries Limited caries
• Measurement of mechanisms involved in caries process. susceptibility susceptibility susceptibility
• Inexpensive, noninvasive, easy to evaluate and applicable Green Continue to incubate Continue to Caries inactive
in clinical settings. and observe at incubate and
48 hours observe at
Lactobacillus colony count test 72 hours
• Introduced by Hadley, 1933.55
• This is done using the conventional plate count method. Swab test
• Principle: Estimates number of lactobacilli in the patients’ • Introduced by Grainger et al 1965.57
saliva by counting number of colonies on tomato peptone • Principle is same as Snyder test
agar plates. • Procedure:
— Oral flora sampled by swabbing the buccal surfaces of
• Procedure:
teeth with cotton applicator. Since no collection of saliva
— Patient chews paraffin wax vigorously for 1 to 5 mins,
is required, this test may be used in very young children.
moving the paraffin alternately from side to side. — The cotton applicator is incubated in the medium at 37°C.
— Saliva secreted in three min is collected. — Change in pH following a 48 hour incubation is read
— Saliva is diluted 1:10 with sterile saline. on a pH meter or color change is read by the use of a
— 1:100 dilution is also prepared. color comparator.
— Each dilution is inoculated on Rogosa's SL agar plate
Results:
for 3 to 4 days at 37°C. pH 4.1 = Marked caries activity
— Number of colonies are counted using a colony counter. pH 4.2-4.4 = Active
Results pH 4.5-4.6 = Slightly active
Refer Table 2.7. pH > 4.6 = Caries inactive
Colorimetric Snyder test Streptococcus mutans level in saliva
• Introduced by Snyder, 1951.56 • Principle: Measures colony forming units of S. mutans per
• Principle: Measures the ability of salivary microorganisms unit volume of saliva and from plaque samples of discrete
to form organic acids from a carbohydrate medium sites such as occlusal/proximal to detect and quantify S.
containing Bromocresol green as indicator dye. mutans on teeth.
• Procedure: • Procedure:
— 0.2 ml stimulated saliva collected by chewing paraffin — Sample is obtained by use of tongue blades. The tongue
is mixed with 10 ml melted agar containing medium in blade is pressed against S. mutans selective MSB (mitis
a test tube. salivarius bacitracin) agar.
— Incubated at 37°C. — Agar plates are incubated at 37°C for 48 hrs at 5 percent
carbon dioxide gas.
— Amount of acid produced is detected by changes in pH
— Counting colonies is done with characteristic
indicator.
morphologies of S. mutans on the MSB agar plates.
— Rate of color change checked at 24, 48 and 72 hrs of Colonies are counted using Quebec colony counter or
incubation. This indicates the degree of caries activity. with the help of microscope.
TABLE 2.7: Results interpreted in Results (Table 2.9):
Lactobacillus colony count test
TABLE 2.9: Results interpreted in Streptococcus mutans
Sr. No. of organisms Symbolic Degrees of caries levels in saliva test
No. per cc designation activity suggested Sr. No. CFU/ml saliva Grade

1. 0-1000 ± Little or none 1. <104 Light caries activity

2. 1000-5000 + Slight 2. 104-105 Slight caries activity
3. 5000-10,000 ++ Moderate 3. 105-106 Moderate caries activity
4. More than 10,000 +++ or ++++ Marked 4. >106 Marked caries activity
78 Essentials of Pediatric Oral Pathology

Dip slide method for S. mutans count Salivary reductase test

• Principle: Estimation of S. mutans levels in saliva • Principle: Measures the activity of the reductase enzyme
• Procedure: present in salivary bacteria. Measures the susceptibility of
— Paraffin stimulated saliva is poured on a special plastic the patient to caries.
slide coated with MSA (Mitis salivarius agar) • Procedure:
containing 20 percent sucrose. — Treatex kit is available for this test.
— Two disks containing 5 mg bacitracin are placed on the — Saliva is collected in a plastic container and the sample
agar 20 mm apart. is mixed with the dye Diazo-resorcinol.
— Slide is tightly screwed into a cover tube and incubated — Color change is read after 15 mins.
at 37°C for 48 hours in a sealed candle jar. Results (Table 2.10):
— Dentocult SM and Cariescreen SM kits are
TABLE 2.10: Results interpreted in
commercially available.
salivary reductase test
Results:
Color Time Score Caries activity
Score 1 = Low. The colonies are discrete and could be readily
counted at 15x magnification with the total count Blue 15 minutes 1 Non conducive
of CFU inside the inhibition zones less than 200. Orchid 15 minutes 2 Slightly conducive
Red 15 minutes 3 Moderately conducive
Score 2 = Medium. The colonies are discrete and the number
Red Immediately 4 Highly conducive
in the zone of inhibition is more than 200 Pink or white Immediately 5 Extremely conducive
visualized under 32x magnification.
Score 3 = High. The colonies are tiny and almost completely Alban test 58
or totally cover the inhibition zone with the number • Principle: Simplified substitute of Snyder test
of colonies uncontrollable even with 32x • Procedure:
magnification. — 60 g of Snyder test agar is placed in 1 liter of water.
Salivary buffer capacity test — When thoroughly melted, agar is distributed using 5 ml
• Principle: This test measures no. of ml of acid required to per tube.
lower the pH of saliva through an arbitrary pH interval — Tubes are autoclaved for 15 minutes and cooled.
• Procedure: — Patient expectorates directly in the tube.
— 10 ml of stimulated saliva is collected under oil at least — Tubes are incubated at 37°C for 4 days.
1 hour after eating. — Results are recorded on the patient's charts
— pH of 5 ml of saliva is adjusted to seven by addition of Scale for scoring:
lactic acid or base. • No change = 3/4
— Level of lactic acid is then added till a pH of six is reached. • Beginning color change = +
— Number of ml of lactic acid needed to reduce pH from (From top of medium down)
7 to 6 is a measure of the buffer capacity of saliva. • One half color change = ++
Results: (From top down)
• A high acid buffering capacity indicates a low caries • Three fourths color change = +++
activity. (From top down)
• A lower acid buffering capacity indicates a higher caries • Total color change to yellow = ++++
activity. The following method is used for final recordings, after
Enamel solubility test 72 or 96 hours of incubation:
• Principle: When glucose is added to saliva containing • Readings negative for entire incubation period are labeled
powdered enamel, organic acids are formed. These will “negative”
decalcify the enamel resulting in an increase in the amount • All other readings are labeled “positive” whether +, ++,
of soluble calcium in the mixture. The extent of increased +++ or ++++.
calcium is supposedly a direct measure of the degree of • Slower change or less color change (compared to previous
the caries susceptibility. test) is labeled “improved”.
• Since the equipment required is complex, this test is • Faster change or more pronounced color change (compared
generally not suited for in-office procedures. to previous test) is labeled “worse”.
• Trained personnel are also required which makes this test • When consecutive readings are nearly identical, they are
uneconomical. labeled “no change”.
Caries in Children 79

Streptococcus mutans screening test • Quite a few tests require lab support and may take days
A. Plaque/tooth pick method: for the result.
• Principle: Dilute plaque samples are screened by • Increased chair-side time is required.
culturing on a selective culture medium. • Trained personnel are required.
• Procedure: Plaque samples are collected from the
gingival thirds of buccal tooth surfaces, one from each CONCLUSION
quadrant and placed in Ringer's solution.
The best predictor of expected caries activity results from the
• Sample is homogenized and streaked across MSA
combined use of several selected tests, as one single test may
plates.
assess only one of the contributory factors of caries, and hence
• Incubated at 37°C for 72 hrs.
may not correlate with the clinical appearance of caries.
• Total colonies in 10 fields are recorded.
• Thus this test is a semi quantitative screening test for
S. mutans. DIAGNOSIS OF DENTAL CARIES
B. Saliva/tongue blade method: • Diagnosis of dental caries is fundamental to the practice
• Principle: Main difference from the plaque method is of dentistry and one of the basic principles of minimally
that a saliva sample is used. invasive dentistry.
• Procedure: • Numerous methods have been used to diagnose carious
— Paraffin wax is chewed for one min to displace lesions and the past few decades have shown a tremendous
plaque microorganisms. revolution in this field.
— Sterile tongue blade is rotated 10 times in the
mouth. VISUAL OBSERVATION (FIG. 2.22)
— This tongue blade is pressed into MSB agar in a
• Requires good lighting and dry, clean teeth.
petri dish and incubated at 37°C for 72 hours.
• Any deposit of calcium or plaque present should be cleaned
— Number of S. mutans is estimated.
before attempting an accurate diagnosis.
Fosdick calcium dissolution test59 • Each quadrant of the mouth is isolated with cotton rolls
• Principle: Measures milligrams of powdered enamel and observation is carried out with the help of a gentle blast
dissolved in 4 hrs by acid formed when the patient's saliva of air from a three way syringe.
is mixed with glucose and powdered enamel. • Any changes in tooth surface texture or color are noted
• Procedure: apart from frank cavitations.
— Twenty five milliliter stimulated saliva is collected and • But this method may not be sufficient to assess the extent
aliquot is analyzed for calcium content. of decay.
— Remaining saliva is placed in a test tube with 0.1 g of
powdered human enamel. TACTILE OBSERVATION (FIG. 2.23)
— Tube is sealed and shaken for 4 hrs at body temperature
after which it is analyzed again for calcium content. • Traditionally sharp probes were used to detect the tacky
— Amount of dissolution increases as the caries activity feel of early cavitation.
increases. • A sharp straight, pigtail (cowhorn), curved shank or the
11/12 type explorer have been used for tactile examination
Dewar test 60 of caries.
• Principle: Similar to Fosdick calcium dissolution test. • A probe should not be used because a sharp probe can
• Difference is that in Dewar test final pH after 4 hrs is actually damage an incipient carious lesion as per the results
measured and not the calcium content obtained by Kühnisch J et al, 2007 in an SEM study done
• No adequate clinical correlation has been found, hence on intact and incipient carious tooth surfaces.61
uncommonly used. • A probe may carry microorganisms into the lesions and
facilitate spread of caries.
Limitations of Caries Activity Tests
Kidd and Fejerskov suggest that to “jab” a sharp explorer
• None of the tests are highly reliable as indicators of caries into a lesion to determine if it is “sticky” is likely to “cause a
as it is a multifactorial disease. cavity and this will encourage biofilm stagnation and lesion
• Caries activity tests provide an important but indirect progression”.62 They emphasize that the formation of a cavity
evidence associating microbes with dental caries. is a critical moment clinically because the biofilm is protected
80 Essentials of Pediatric Oral Pathology

Caries Assessment Tool (CAT): (Table 2.11)

TABLE 2.11: American Academy of Pediatric Dentistry, Council on Clinical Affairs. Policy on use of a caries risk assessment
tool (CAT) for infants, children and adolescents. Pediatr Dent, 2002; 25: 1853

Caries Low Risk Moderate Risk High Risk

Assessment Tools

1. Clinical conditions No carious teeth in last 24 months Carious teeth in the past 24 months Carious teeth in the past 12 months

No enamel demineralization/white One area of enamel More than one area of enamel
spot lesions demineralization demineralization

No visible plaque; no gingivitis Gingivitis Visible plaque on anterior teeth

Radiographic enamel caries

High titers of mutans streptococci

Wearing dental or orthodontic

appliances

Enamel hypoplasia

2. Environmental Optimal systemic and topical Suboptimal systemic fluoride Suboptimal topical fluoride
characteristics fluoride exposure exposure with optimal topical exposure
fluoride exposure

Consumption of simple sugars Occasional (e.g. 1-2) between Frequent (e.g. 3 or more)
or foods strongly associated meal exposures to simple sugars between meal exposures to
with caries initiation, primarily or foods strongly associated with simple sugars or foods strongly
at mealtimes caries associated with caries

High caregiver socioeconomic Midlevel caregiver socioeconomic Low-level caregiver socio-

status status (e.g. eligible for school lunch economic status (eligible for
program Medicaid)

Regular use of dental care in Irregular use of dental services No usual source of dental care
an established dental home

Active caries present in the mother

3. General health Children with special health care

conditions needs

Conditions impairing saliva

composition/flow

FIGURE 2.22: Visual observation of dental caries FIGURE 2.23: Tactile observation of carious lesions using
diagnostic instruments
Caries in Children 81

in a microcavity and the cavity might have first been created TABLE 2.12: Suggested radiographic protocol for the
by dentists using explorers. In Europe, use of an explorer to pediatric patient with no previous radiographs64
probe carious lesions is even considered unethical. Age Considerations Radiographs
Wilkins suggested that dentists should use a blunt (Years)
periodontal probe and gently run the probe over the surface
3-5 No apparent abnormalities None
with no pressure or the dental explorer can gently be used to
(open contacts)
remove biofilm or debris to assist with visualization.62
No apparent abnormalities 2 posterior bitewings,
(closed contacts) size 0 film
RADIOGRAPHS
Extensive caries 4-film survey (2 bitewings,
• The use of radiographs to examine teeth and other oral size 0 and 2 occlusal
structures for the presence of oral disease remains the “gold projections)
standard”. Deep caries Selected periapical
• Good bitewing radiographs are important in the detection radiographs in addition to
of early carious lesions specially pit and fissure and 4-film survey
proximal caries (Fig. 2.24). Suggested radiographic 6-7 No apparent abnormalities 8-film survey
protocol for the pediatric patient with no previous
Selected periapical
radiographs has been outlined in Table 2.12. radiographs in addition to
The various limitations of the radiographic method are: 8-film survey
• The phenomenon of hidden caries/occult caries, i.e. surface 8-9 No apparent abnormalities 12-film survey
hardening of tooth due to extensive use of fluorides makes or extensive or deep caries
it more impenetrable to exploration, while at the same time 10-12 No apparent abnormalities 12 or 16-film survey,
masking of the carious activity occurs just below the tooth or extensive or deep caries depending on patient size
surface and along the DEJ. In this case, tooth appears caries
free clinically and/or radiographically but is found to be
carious by other diagnostic means. Conventional Radiography 65
• Overlapping of approximal contacts. Conventionally, two types of techniques are employed:
• False diagnosis due to overestimation of lesion depth which • Intraoral periapical radiography and Bitewing radiography.
may appear to be increased due to change in angulation. Periapical radiographs are primarily useful for detecting
• Occlusal lesions imperceptible because of solid buccal and changes about the root and in between the teeth. If paralleling
lingual cusps. technique is used, the usefulness of this projection is increased
• It is a two dimensional image of a three dimensional object. for detecting caries in anterior and posterior teeth. Bitewing
• Cervical burn-out areas may mimic cervical caries. radiographs are more important to detect incipient lesions at
contact points. Also when used as adjuncts, the bitewings
significantly improve the accuracy of pit and fissure caries
diagnosis.

Xeroradiography
This technique simulates the photocopying machine. It is a plate
coated with a layer of selenium particles. When X-rays are
passed onto the film, a latent image is formed which is then
converted to a positive image. The main characteristics of
xeroradiographic technique are the ability to have both positive
and negative prints together.
It is twice as sensitive as conventional D-speed film and a
phenomenon of “Edge Enhancement” is possible. Edge
enhancement means differentiating areas of different densities
especially at the margins or edges. But it also has disadvantages
FIGURE 2.24: Bitewing radiographs showing secondary caries like varying exposure time and development should be within
(arrows) under the amalgam restoration 15 minutes.
82 Essentials of Pediatric Oral Pathology

It also has some disadvantages:

• High cost of the system.
• The life expectancy of CCD is not fixed.
The current evidence suggests that digital systems
performed comparably with film radiography for detection of
dental caries.66

Computer Image Analysis67

Some of the researchers have developed a computer-based
software system that is capable of diagnosing approximal caries
and making decisions about restorative care. These softwares
have been developed for automated interpretation of digital
radiographs in order to standardize image assessment. Software
FIGURE 2.25: Digital imaging of the carious lesion
packages like “Caries Finder” have the potential to raise overall
accuracy by increasing the consistency of treatment decisions
Digital Imaging (Fig. 2.25)
over time.
Dental image acquisition for dental examinations and diagnosis In 1984 Pitts and in 1990 Heaven and others applied such
has been transformed by digital imaging, also known as computer-based software systems for image processing and
computerized digital radiography. Digital radiographic images analysis of approximal caries. The researchers found that the
are created by using the spatial distribution of pixels and the caries finder computer system did not differ from experienced
different shades of gray of each of the pixels. In other words, clinicians in overall accuracy of detecting approximal caries
the digital radiographic imaging devices interface with a and planning restorative therapy. Image analysis systems can
computer to digitize the digital radiographic image into pixels be an important tool in reducing the number of restorations
that are then viewed on a computer monitor. Rather than using placed in intact surfaces. The results of the computer-based
traditional radiographic film, digital radiographs are taken by caries diagnostic system were more precise. Dentists relying
using a sensor that is placed in the same location that dental on a computer-based caries diagnostic system; were less likely
film would be placed. The image can be viewed on a computer to restore non-cavitated approximal surfaces than were dentists
monitor, stored, transmitted to a remote site, or printed. Digital who did not use such a system.
imaging equipment is also available for taking panographic and
Advantages:
cephalometric views extraorally. It has the following advantages
• Observation of smaller lesions.
over traditional radiography:
• It is possible to monitor the lesions.
• Radiation exposure of the patient is significantly reduced.
• Quantification of small lesions is possible.
• Eliminates chemical processing and accompanying errors.
• Hazardous waste and lead foil are eliminated. Disadvantages:
• Same film positioning, no new positioning to learn. • There is always a need for standardization of exposure
• Images can be transferred to other health care professionals geometry.
without loss of original quality. • Sensitivity is higher but specificity is lesser.
There are two types of nonfilm receptors for recording • Time consuming and less economical.
digital images:
1. The Digital image receptor (DIR) which collects the Subtraction Radiography
X-ray directly (DIRECT DIGITAL IMAGING). The most important advantage that digital imaging has for
2. Video camera for forming digital images of a radiograph. dental clinicians is that it can also be used for subtraction
(INDIRECT DIGITAL IMAGING). purposes. It is a technique by which structured noise is reduced
Digital image receptor works on a Charged couple device in order to increase the detectability of changes in the
(CCD), which is electronically connected to a computer. CCD radiographic pattern.
is a semiconductor made up of metal oxides such as silicon It requires two identical images. The subtracted image is a
that is coated with X-ray sensitive phosphorus. CCD is sensitive composite of these two images. The ability of digital subtraction
to both X-rays and visible light. The CCD is placed intraorally to record minimum differences depends on degree of matching
and it captures image, which is stored in computer memory of the two images. Images taken over time can be super-
and can be displaced for viewing. imposed, which makes an excellent method for tracking
Caries in Children 83

progress of carious lesion over time. Likewise dental hygienists Light that shines on a tooth will, in part, penetrate the tooth
can also use digital subtraction radiography to track hidden and and is scattered or absorbed inside.
small carious lesions to determine if fluoride applications and Scattering is the process in which the direction of photon
patient self-care are being effective in remineralizing these is changed without loss of energy. Absorption is the process in
lesions, as more than half of shallow dentinal lesions can be which photons lose their energy, mostly by conversion into heat.
arrested and so operative therapy avoided. Thus it is helpful Since scattering does not cause the light to be lost, scattering
for caries management, not just caries diagnosis. may occur many times consecutively along the path, a
phenomenon called ‘multiple scattering’. After one or more
ELECTRICAL RESISTANCE scatter events, a photon may reach the tooth surface again and
leave the tooth. Back-scatter or volume reflection is the
Sound tooth enamel is a good electrical insulator due to its high
phenomenon where photons leave through the surface by which
inorganic content. Enamel demineralization results in increased
they entered. When photons leave through another surface, the
porosity. Saliva fills these pores and forms conductive pathways
phenomenon is called diffuse transmission.
for electrical current. The electrical conductivity is hence
In a sound tooth, scattering is much more probable than
directly proportional to the amount of demineralization that has
absorption. In dentin both scattering and absorption occur more
occurred. Electrical resistance is measuring the electrical
frequently along the light path than either occurs in the enamel.
conductivity through these pores.
The whitish appearance of teeth is due to the fact that
An instrument called “Van Guard electronic caries detector”
absorption is much lower than scattering.68
has been designed to measure electrical conductivity of the
In white spot carious lesion, scattering is stronger than in
tooth. The electrical conductivity of the tooth is expressed
sound enamel. The penetrating photons change direction more
numerically on a scale from 0 to 9, indicating change from
often in carious enamel and are generally back-scattered before
sound tooth to an increased degree of demineralization.
they reach dentin. Therefore, a lesion observed in reflection
appears whiter than the surrounding sound parts of the tooth.
Advantages
Brown lesions are due to the presence of light-absorbing
• Very effective in detecting early pit and fissure caries. material in the lesion.
• It can monitor the progress of caries during caries control A slight increase in enamel porosity leads to a change in
program. the optical properties of enamel in such a way that light is
increasingly scattered. This is presumed to be primarily due to
Disadvantages the fact that the remaining small mineral particles in the lesion
are embedded in water rather than in mineral rich sound enamel
• It can only recognize demineralized areas and not caries
thereby increasing the difference in refractive index between
specifically. The hypomineralized areas which may be of
the scattering photon and its environment.69
developmental origin or carious origin, will give similar
The refractive index (RI) of enamel apatite is 1.62, and RI
type of readings.
of water and air is 1.33 and 1.0, respectively. Thus, when the
• Presence of enamel cracks may lead to false positive diagnosis.
pores of white spot enamel lesion are filled with water, the light
• A sharp metal explorer is utilized which is pressed into the
scattering is less than when the lesion is dry and the pores are
fissure causing traumatic defects.
filled with air. After dehydration of a caries lesion in enamel,
• Separate measurements are required for different sites
the lesion looks whiter, as a result of more scattered light,
making full mouth examination quite time consuming.
because of the larger difference in RI, 1.62 versus 1.0
A modified form of instrument ‘Electrical Caries Monitor’
not only detects caries at a single point on the tooth but can
Optical Caries Monitor
also screen the whole of the occlusal surface for caries by
covering the surface with a conducting medium before placing White spot lesions look whiter than the surrounding sound
the probe tip. enamel because of the strong scattering of light within the
lesion. This stronger scattering can be quantified with methods
DIAGNOSTIC METHODS BASED ON VISIBLE LIGHT based on fiber technology. The measured quantity is the
‘scattering coefficient.’ An instrument for clinical use, the
Principles of Light Transmission through Teeth
optical caries monitor was constructed comprising a light
Sound enamel consists mainly of hydroxyapatite crystals which source, measuring and reference units and a detection part. The
are very densely packed, giving the enamel glass-like, light is transported through a fiber bundle to the tip of the
translucent appearance. The yellow-white color of teeth is the handpiece. The tip is placed against the tooth surface and the
result of dentin shining through the translucent enamel layer. reflected light is collected by different fibers in the same tip.
84 Essentials of Pediatric Oral Pathology

and specificity values, that is the ability to detect sound surfaces

vary considerably in the studies. For permanent teeth, the
sensitivity of FOTI varies from <50 to 85 percent, while the
specificity is generally high, >95 percent.71
Advantages
1. No hazards of radiation.
2. Simple and comfortable for patients.
FIGURES 2.26A and B: Placement of fiberoptic transillumination 3. Lesions, which cannot be diagnosed radiographically, can
probe on the fissure area and the image formed by the device on be diagnosed by this method.
the computer screen 4. Not time consuming.

The scattering coefficient values are correlated with Disadvantages

histological picture, lesion depth and with mineral loss per unit 1. Permanent records are difficult to maintain as can be kept
area of the lesion surface at the deepest point of the lesion. in radiographs.
The optical caries monitor can be used only for the 2. It is subject to intra and inter observer variations.
quantification of caries lesions on smooth surfaces. After further 3. Difficult to locate the probe in certain areas.
development for clinical application the method may be useful,
Digital Imaging Fiberoptic Transillumination
e.g. for quantitative evaluation of the regression of smooth
surface enamel lesions developed during fixed orthodontic Transillumination has long been used in dental hygiene for the
therapy. detection of carious lesions and especially calculus for over
30 years. The Digital imaging fiberoptic transillumination
Fiber Optic TransIllumination (Figs 2.26A and B) (DIFOTI) system has elevated traditional transillumination to
Fiber Optic Transillumination (FOTI) works on the principle more sophisticated diagnostic levels. The DIFOTI allows for
that since a carious lesion has a lowered index of light images taken from all tooth surfaces to be digitally captured
transmission, an area of caries appears as a darkened shadow by using a digital charged couple device camera and sent to a
that follows the spread of decay through the dentin. FOTI has computer for analysis with dedicated algorithms. The images
been used in common medical procedures since 1960. In are stored for longitudinal evaluation of carious lesions. When
dentistry, it was first used as an improved light source for teeth are transilluminated, the areas of demineralization in
surgical retractors. In 1970, Friedman and Marcus suggested enamel or dentin scatter light, and incipient caries appear as
the use of FOTI in detection of carious lesions.70 darker areas in the captured images (Fig. 2.27). The DIFOTI
It has been introduced as a quantitative diagnostic method varies from the DIAGNOdent in that it has two handpieces,
by which teeth are transilluminated. Fiber optic consists of a one for smooth surface caries detection and one for occlusal
halogen lamp and a rheostat to produce a light of variable caries detection. The system uses a disposable mouthpiece and
intensity. The 150-watt lamp generates a maximum light foot control that allows for selecting the image of concern and
intensity of 4000 lx at the end of a 2 mm diameter cable. Two a computer for capturing and storing the images. Like the QLF
attachments are used; a plane mouth mirror mounted on a steel and DIAGNOdent, the DIFOTI can be used for preventive
cuff and a fiber optic probe 0.5 mm in diameter so that it can management of early carious lesions, and the information
be placed in the embrasure region. It produces a narrow beam gleaned can be used by the dental hygienist to assist patients
of light for transillumination. The rheostat is set to give a light in targeting at risk areas in the mouth. The DIFOTI does not
of maximum intensity. For examination, the tip of the probe is have the capability to determine depths of lesions, but unlike
placed in the embrasure immediately beneath the contact point other alternative caries detection devices, it can detect caries
of proximal surface to be examined either on the buccal or on interproximal surface as well as radiographs can.
lingual surface depending on the tooth. The marginal ridge is
PULSED LASER CARIES DETECTOR—DIAGNODENT
viewed from the occlusal surface.
(FIG. 2.28)
The shadow extending to the dentino-enamel junction
beneath the marginal ridge may be evident if there is a break DIAGNOdent is a battery-powered device that relies on laser
in the integrity of the enamel of marginal ridge. fluorescence to detect caries. The dentist places the tip of
Compared with radiography, the technique is simple and diagnostic probe on the surface of the tooth. A pulsed laser
fast. The majority of clinical studies comparing diagnosis with beam with a wavelength of 655 nm is then transmitted on the
FOTI or unaided clinical diagnosis use radiography as a gold tooth surface through the probe as it scans along the pits and
standard. Sensitivity values, that is the ability to detect caries, fissures. When the penetrating laser beam encounters the
Caries in Children 85

FIGURE 2.27: Differentiating features in images using DIFOTI and conventional radiography

readings at recall appointment can be used to determine if the

DIAGNOdent value has increased, decreased or stabilized.72

Processing (Fig. 2.29)

When the light is absorbed it induces infrared fluorescence of
organic and inorganic materials. This fluorescence is collected
at the top of the handpiece and transmitted back to the
DIAGNOdent unit. The fluorescence is converted into an
acoustic signal and displayed as an integer that ranges from
0-99. An increase in fluorescence is indicative of carious tooth
substance, particularly when the displayed reading is 20.73

Principles of Laser/Light induced Fluorescence

Laser light is composed of electromagnetic waves with equal
wavelengths and equal phases. Some materials possess the
characteristic of fluorescence when illuminated with light.
Fluorescence is a phenomenon by which the wavelength of the
emitted (original) light is changed into a larger wavelength upon
FIGURE 2.28: Processing of pulsed laser caries detector reflectance. When the emitting light is from the visible
spectrum, the fluorescent light has different color to the emitting
presence of carious tooth structure, fluorescent light with an light. By using a filter through which only fluorescent light can
altered wavelength is stimulated. An acoustic signal is set off be passed, the intensity of the fluorescent light can be measured.
as the new wavelength of light is translated back to the power The intensity of the fluorescent light is proportional to the
box, and a numeric reading appears on the monitor. amount of material that causes the fluorescence. The
After calibration, the appropriate probe is selected. For fluorescence of dental hard tissues has been known for a very
smooth surfaces, the probe is gently run over the surface of long time. Three types of fluorescence can be distinguished:
the tooth. For occlusal examinations, the probe should be blue fluorescence is excited in the near ultraviolet, yellow and
moved (e.g. mesially to distally) and swayed buccolingually orange fluorescence is excited in the blue and green, and red
to ensure that all fissures are examined. The device displays fluorescence in the far red and near infrared.
the results in real time. The unit presents current and peak Dental enamel and dentin possess the characteristic of
values from the time when the unit was last reset. Therefore, fluorescence and this natural fluorescence is called auto
the unit should be reset between teeth, so that the peak value fluorescence. Caries lesions, plaque and micro-organisms also
for each tooth can be recorded, along with notes as to the contain fluorescent substances. The difference between the
location on the tooth where the reading was taken. Further fluorescence of sound tooth tissues and that of caries lesions can
86 Essentials of Pediatric Oral Pathology

FIGURE 2.29: Pulsed Laser Caries Detector (DIAGNOdent)

be made visible by the Quantitative laser or light induced Advantages

fluorescence (QLF) method. The measurement of the fluorescence
It is a more sensitive method than the visual-tactile method.
caused by a caries lesion yields a quantitative diagnosis.
The chromophores causing the fluorescence of dental hard
Disadvantages
tissues are not clearly identified. The blue fluorescence was
assigned to dityrosine. It seems likely that most of the yellow • The specificity is a problem between the carious lesion and
fluorescence stems from proteinic chromophores, which the developmental defect.
probably cross-link between chains of structural proteins. The • Still this method has not been developed into a qualitative
red infrared fluorescence has been assigned to protophyrin method.
which is present as a bacterial breakdown product.
QUANTITATIVE LIGHT-INDUCED FLUORESCENCE72
Interpretation of Results (FIGS 2.30A and B)
The numerical scale readings can be interpreted as: Quantitative light induced fluorescence (QLF) enhances early
• 0 to 14—No caries or histological enamel caries limited detection of carious lesions, particularly progression or
to the outer half of enamel thickness. regression of white spots of smooth surface lesions. QLF uses
• 15 to 20—Histological caries extending beyond the outer the intrinsic fluorescence of the teeth within the yellow-green
half, but confined to the enamel. spectrum of visible light. The tooth is illuminated with a blue
• 21 to 99—Histological dentinal caries. light that is emitted from a handpiece and causes the tooth
DIAGNOdent is more so helpful in diagnosing “occult or structure to fluoresce and the image is captured with a color
hidden” caries. microvideo charged couple device camera. The data are then
stored and analyzed by image analysis computer software
Limitations (Inspektor Dental Care, Amsterdam, The Netherlands). The
• The depth of penetration of the light is limited to about tooth is seen on the computer monitor as fluorescent green;
2 mm. dark areas indicate mineral loss. With the computer software,
• The value of the device lies only in detection of occlusal the image can be saved and compared over time to track
involvement; it will not identify interproximal caries. demineralization or remineralization.
QLF is a useful method for visualizing dental biofilm that
ULTRAVIOLET ILLUMINATION is not visible clinically under ordinary lights. It also serves as
an evaluation tool to assess the outcome of preventive self-
Ultraviolet light (UV) has been used to increase the optical
care plans developed for the patient.
contrast between the carious region and the surrounding sound
tissue. The natural fluorescence of tooth enamel, as seen under
ENDOSCOPE/VIDEOSCOPE
UV light illumination is decreased in areas of less mineral
content such as in carious lesions, artificial demineralization The technique is same as that of QLF. In this, tooth is illuminated
or developmental defects. The carious lesion appears as a dark with blue light in the range of 400 to 500 nm. Similarly, a white
spot against a fluorescent background. light source can be connected to an endoscope by a fiber optic
Caries in Children 87

FIGURES 2.30A and B: Quantitative light fluorescence device and the detection of carious lesion by fluorescence light

cable so that teeth can be viewed without a filter. This technique impedance and velocity. Acoustic parameters depend strongly
is referred to as white light endoscopy. on the frequency of the ultrasound as well as other parameters
Additionally, a camera can be used to store the image. The such as temperature.
integration of the camera with the endoscope is called a In dentistry, ultrasound was first used by Ng et al 1988, to
videoscope. A miniature color video camera is mounted in a image the tooth and to find caries lesions on smooth surfaces
custom made metal mirror holder. This is designed in such a of teeth. It was concluded from this initial study that although
way that the image of the surface of enamel can be viewed small lesions could be detected, the method was inappropriate
directly over a television screen. to apply to patients. Moreover, it is not possible to detect
shallow caries lesions. It is observed that there is a definite
Advantages correlation between the mineral content of the body of the
lesion and relative echo amplitude ranges. The sites with a
• It provides a magnified image.
visible cavitation produce echoes with substantially higher
• Clinically feasible.
amplitude. This method is more sensitive than visual-tactile
method; however it is not a quantitative method.
Disadvantages
• Requires meticulous drying and isolation of teeth. DYE PENETRATION METHOD
• Time consuming.
Dye can visualize a subject from its routine background or if
• Very costly.
several objects have a similar appearance, coloring by a dye
may discriminate between them and allow identification. Dyes
ULTRASONIC IMAGING
should fulfill the following criteria before being recommended
Ultrasonic imaging was introduced for detecting early carious for clinical use:
lesions in smooth surfaces. Ultrasound used in ultrasonography • Dyes should be absolutely safe for intraoral use.
is a sound wave with a frequency ranging from 1.6 to about 10 • Dyes should be specific and stain only the tissues they are
MHz. Its diagnostic purpose is to delineate, depict and measure intended to stain.
organs and tissues in the body. In medicine ultrasound is used • Dyes should be specific and easily removed and not lead
to create images of internal organs. These images are produced to permanent staining.
when the sound waves are directed at the organs of the body
and then reflected back to a scanner which measures them. Dyes for Detection of Carious Enamel (Fig. 2.31)
Ultrasound interacts differently with different types of tissue. • ‘Procion’ dyes stain enamel lesions but the staining
The interaction depends upon the acoustic properties of the becomes irreversible because the dye reacts with nitrogen
tissue, such as the attenuation, absorption and scattering, and hydroxyl groups of enamel and acts as a fixative.
88 Essentials of Pediatric Oral Pathology

To apply this method for caries diagnosis remains a

complicated procedure, because for each type of acid attack, a
separate calibration under the same condition is needed.

Disadvantages of Caries Detector Dyes

• Not all dye stainable dentin is infected.
• The absence of stain does not ensure elimination of
bacteria.
• The dyes stain the organic matrix of less well-mineralized
dentin.
• Caries detector dye lacks specificity.
• The dyes neither stain bacteria nor delineate the bacterial
front but stain the collagen associated with less mineralized
organic matrix
• Many dyes, such as procion dyes, produce irreversible
staining that is clinically unacceptable.

FIGURE 2.31: Caries detector dye CONCLUSION

None of these methods of detection is infallible, and none of
• ‘Calcein’ dye makes a complex with calcium and remains them justifies immediate surgical intervention. Each technique
bound to the lesion. ‘Fluorescent dye’ like Zyglo ZL-22 has a place in diagnosis, but, in view of the relatively slow
has been used in vitro which is not suitable in vivo. The progress of the typical carious lesion, an early lesion should
dye is made visible by ultraviolet illumination. be treated with caution. Surgical intervention can only be
• ‘Brilliant blue’ has also been used to enhance the diagnostic properly justified for elimination of actual cavitation.
quality of fiberoptic transillumination.
The use of dyes for diagnosing enamel lesions cannot be CARIES RISK ASSESSMENT
used clinically as yet. If possible it will thus allow remineral-
Only in the absence of disease the restorative dentistry will
ization.
succeed. That's why control is the primary focus. Correct
diagnostic procedures, i.e. Caries Risk Assessment must be
Dyes for Detection of Carious Dentin
carried out for any at risk patient to determine patient’s present
Histopathologically, carious dentin is divided into two layers— and future dental health, with an understanding of the need for
outer layer of decalcification, which is soft and cannot be preventive or surgical intervention.
remineralized and the inner decalcified layer, which is hard and Caries risk may be defined as the probability that a specific
can be remineralized. Dyes have been tried to differentiate number of new lesions will develop and/or a specific number
between these two zones of dentin caries. 0.5 percent basic of existing lesions will progress over a specific period of time.75
fuchsin in propylene glycol has proved to be successful for this The combination of careful visual assessment, patient's
purpose. Thus, it is possible to remove completely the outer factors (e.g. caries risk), and the appearance of a possible lesion
carious zone in dentin caries as it contains denatured collagen. on radiographs is the next level of caries diagnosis, especially
Basic fuchsin dye was considered to be cariogenic; in instances of increased caries infection.76
therefore, it has been replaced by acid red and methylene blue. There are two reasons for assessing caries risk in the current
Methylene blue is also slightly toxic so acid red is preferred. era of reduced caries prevalence:
1. To direct individually based preventive measures to the
A Modified Dye Penetration Method highest risk persons who benefit most from prevention.
2. To identify low risk patients in order to delay restorations
The Iodine penetration for measuring enamel porosity of an
and prevent unnecessary surgical intervention.
incipient carious lesion was developed by Bakhos et al, 1977.74
Potassium iodide is applied for a specific period of time to a
EXAMINATION
well-defined area of enamel and thereafter the excess is
removed. The iodine, which remains in the micropores, is A thorough oral examination should assess the extent and
estimated and that indicates the permeability of enamel. location of incipient lesions, cavitated lesions and restorations.
Caries in Children 89

It should include an assessment of the activity status of the • Using sealants to protect susceptible areas of the tooth that
lesions and condition of the restorations. cannot be kept plaque free by routine oral hygiene measures.
The following evidence should be carefully gathered and • Reducing cariogenic flora so that even in the presence of
recorded: sucrose, acid production will be minimal, e.g. oral hygiene
• Coronal surface: aids, antimicrobial agents.
— Color • Replacement of cariogenic bacteria by low virulence
— Translucency mutants of streptococci that are deficient in lactate
— Cavitation dehydrogenase or glucosyl transferase.
• Root surface
— Texture MANAGEMENT OF CARIES
— Color
• Radiographs Remineralization of early lesions
— Cavitation Restoration
— Pulp Pulp treatment:
— Alveolus Indirect pulp capping
• Transillumination Direct pulp capping
Partial pulpotomy in permanent teeth
— If possible
Vital pulpotomy
• Electronic caries detection
Non-vital pulpotomy
— If possible
Partial pulpectomy in permanent teeth
Pulpectomy in primary teeth
PATIENT'S HISTORY
Apexogenesis and Apexification
It is necessary to take into account the wider range of historical
evidence, including the current status of the major etiological REMINERALIZE EARLY LESIONS77
factors, before deciding if an incipient lesion is able to be arrested
Remineralization should be recognized and utilized as far as
or will progress to cavitation. The following information should
possible for any tooth that has been subject to attack by caries,
be obtained:
because there is no real substitute for natural tooth structure.
• A thorough history of caries, timing when restorations were
Successful remineralization requires intensive patient
placed and frequency of repair or replacement.
education and cooperation. Use of fluoride containing
• An outline of present and past dietary patterns, in particular
dentifrices and mouth rinses has been proven to enhance the
frequency of refined carbohydrate intake and consumption
rate of remineralization of teeth. Mineral will recrystallize in
of acid drinks or foods.
partially demineralized enamel when fluoride, calcium and
• Past and present fluoride contact, both systemic and topical.
phosphate ions are present in adequate proportions.
• Medical or social factors that may affect dental health; for
The major shortcomings of currently available toothpastes,
example, drugs that may affect salivary flow or that contain
mouth rinses and topical applications are the fact that their
high concentration of sweetening agents.
ability to remineralize enamel is limited by the low concen-
• Physical or medical problems that may impair a patient’s
tration of calcium and phosphate ions in saliva. This can be
ability to carry out oral hygiene or that exerts control over
overcome by the use of newer approaches to remineralization
their diet.
which include:
• Emotional or other factors resulting in high levels of stress.
• Illness or organic problems or medications that result in Amorphous Calcium Phosphate
frequent gastric acid regurgitation. (a more soluble form of calcium phosphate)
• Parental dental history. Most children appear to acquire the
It acts as a useful supplement to the calcium and phosphate ions
microorganisms from their mothers.
present in saliva.
Casein phosphopeptides (CPP) are naturally occurring
PREVENTION OF CARIES
molecules which are able to bind calcium and phosphate ions
• Stopping or reducing between meal consumption of and stabilize amorphous calcium phosphate (ACP). Under
carbohydrates, or substituting noncariogenic artificial acidic conditions, CPP are able to release calcium and
sweeteners, e.g. sorbitol, xylitol or lycasin. phosphate ions and thereby maintain a state of supersaturation
• Making the tooth structure less soluble to acid attack by with respect to tooth enamel, reducing demineralization and
using fluorides. enhancing remineralization (Reynolds 1997).78 The delivery
90 Essentials of Pediatric Oral Pathology

has been shown to reduce clinical caries development- in one

study by up to 40 percent.
Shen P et al 2001, found that Recaldent Gum containing
Xylitol and CPP-ACP has enhanced enamel remineralization
considerably, compared to sugar-free chewing gum due to
incorporation of CPP-ACP complexes.79 Recaldent was invented
by Professor Eric Reynolds from Melbourne University.

Use of Xylitol
It is a nonfermentable sweetener and may possess some
properties that promote remineralization. In September 1890,
FIGURE 2.32: GC tooth mousse used for remineralizing
the German chemistry professor Emi Herman Fischer and his
incipient lesions assistant Rudolf Stanel, separated from beech chips a new
compound which was named Xylit, the German word for
of CPP or complexes of CPP-ACP to the plaque fluid can be Xylitol (Makinen, 2000).80
achieved by a range of vehicles, including chewing gums, Xylitol is a naturally occurring polyol which is taken up
dentifrices and topical gels. CPP binds well to dental plaque, by streptococci but not fermentable. Habitual consumption of
and is able to slow or prevent the diffusion of calcium ions xylitol in the diet appears to select for mutans streptococci with
from enamel during episodes of acid challenge, and serves as impaired adhesion properties, i.e. they bind poorly to teeth and
a source of calcium for subsequent remineralization. shed easily from plaque to saliva. Thus, it can be speculated
that the streptococci of mothers in xylitol group had impaired
GC Tooth Mousse (Fig. 2.32) adhesion property leading to reduced mother-child transmission
of mutans streptococci. Use of xylitol chewing gum can retard
A water based, sugar crème containing CPP-ACP (Casein return of oral mutans streptococci after suppression of these
Phosphopeptides—Amorphous Calcium Phosphate ) which cariogenic organisms.
binds tooth surfaces to biofilms, plaque, bacteria, A recent clinical trial demonstrated that chewing a Xylitol
hydroxyapatite and surrounding soft tissue localizing bio- gum three times daily for a minimum of five minutes each time
available calcium and phosphate. Saliva enhances the for three months resulted in a ten fold reduction in salivary
effectiveness of CPP-ACP and flavor helps stimulate saliva. It levels of mutans streptococci.
provides extra protection for teeth. Neutralizes acid challenges
from acidogenic bacteria in plaque and other internal and Use of Polymeric Coatings
external acid sources. It also promotes fluoride uptake. A new technology currently under development for
increasing tooth resistance to decay is the fabrication of thin
Chewing Gum polymeric coatings over tooth crowns and accessible root
A healthy adult produces around 500 ml of saliva per day. For surfaces. According to Mandel, 1996, current research
most of the day the unstimulated flow rate is low (about envisions a two stage process. The initial stage would be
0.2-0.4 ml/min), but saliva can be stimulated by masticatory application of monomer with a controlled degree of water
or gustatory activity. Saliva can be stimulated by any food or solubility that would allow a penetration into hydrated
drink, but the most practical way of stimulating saliva is by organic material and adhere to the tooth substance by
chewing Orbit sugarfree gum. The concentration of ions chemical bonds. A polymeric top coat would then enhance
(calcium and phosphate) which make up the lattice structure durability and aesthetics. 81
of hydroxyapatite are higher in stimulated than in unstimulated
saliva. Consequently, stimulated saliva is more effective at Laser Light
remineralizing enamel crystals damaged by initial caries attack. The ability of laser light to alter the surface of enamel and
The other benefits of sugar-free gum are: increase its resistance to acid challenge has been known for
• Neutralization and buffering of plaque acid. 30 years. A recent study has shown that on use of CO2 laser
• Oral clearance of sugars, acids and food debris from light, it is efficiently absorbed by tooth minerals, is transformed
the mouth. rapidly into heat and forms a ceramic like surface that is highly
The use of sugar-free gum after eating meals and snacks resistant to acid attack, as well as to the initiation of caries as
promotes the in vivo remineralization of enamel lesions, and demonstrated in an in vitro model system.82
Caries in Children 91

TABLE 2.13: Classification of various tooth-cutting

techniques
Category Techniques

Mechanical, rotary Handpiece and burs

Mechanical, non-rotary Hand excavators, air abrasion,
air polishing, ultrasonics,
sonoabrasion.
Chemicomechanical Caridex, carisolv, enzymes
Photo-ablation Lasers FIGURES 2.33A and B: Caries excisional burs

RECENT MODALITIES OF CARIES REMOVAL Air Abrasion

There are many techniques available for cutting tooth tissues Air abrasion is an excellent tool for the minimally invasive
(Table 2.13). Some claim to remove demineralized dentin dentist. Air abrasion cavity design preparation in combination
selectively whereas others are not able to make this distinction with the wide range of currently available low viscosity
and indeed, may not even be able to remove softened tissue adhesive materials forms an ideal alliance for minimally
effectively. invasive dentistry. Air abrasion is an old dental technology that
The ideal cutting instruments should fulfill certain factors is finding a new place in modern, science-based dentistry.
to satisfy both operator and patient. These factors might
Principle: It utilizes kinetic energy or inertia as a means of
include:
rapidly removing tooth structure by incorporating a fine
• Comfort and ease of use in the clinical environment.
abrasive material in a high velocity gaseous propellant.
• The ability to discriminate and remove diseased tissue only.
• Being painless, silent, requiring only minimal pressure for Evolution: As early as 1945, Dr RB Black conceived the
optimal use. concept of application of kinetic energy in the field of dentistry.
• Not generating vibration or heat during operation. Before the invention of air and water turbine hand-piece,
• Being affordable and easy to maintain. “Abrasive Technique” was developed primarily to minimize
patient discomfort. “Air Dent Machine” by SS White
Excavators, Handpieces and Burs (Figs 2.33A and B) Technologies was the first original air-abrasion unit based on
Small round burs Black’s work. The original technique has now been modified
They provide a conservative preparation with good explorer and improved as Kinetic cavity preparation (KCP) system.83
access and require no learning curve. In the early fifties “Abrasive technique” received
They are slow and inefficient in cutting through enamel. considerable interest, but was never popular due to drawbacks
like limited area of good vision, lack of tactile sensation,
Excisional biopsy burs inability to achieve precise margins and angles (to suit the
• The fissurotomy burs are specially designed for recontour- materials available) and possible ill effects of inhalation of
ing the fissures and accessing decay with minimal enamel abrasive particles, etc. In the early sixties, the cavity cutting
removal. procedures were precise and followed the principles of
• They are fast cutting, conservative and inexpensive. mechanical retention and resistance as amalgam and gold were
• No local anesthesia is required. the main restorative materials. The development of “Acid Etch
• The use of the fissurotomy burs is limited to pits, fissures Technique” by Buonocore in 1955 and “Composite Resin” by
and grooves, however, not indicated for treatment of larger Bowen in 1962 revolutionized the concepts of Conservative
decay.
dentistry. Recently, the paradigm shift towards minimal invasive
• The head length of the bur is 2.5 mm, to limit the bur tip
techniques has revived the interest in air abrasion.
to cut just below DEJ, and not further into the dentin.
• The tapered shape of the bur allows the cutting tip to Components of KCP unit
encounter very few dentinal tubules at any given time and • Precision-built device containing a source of suitable
has been designed to minimize heat build-up and vibration. gaseous propellant and means of regulating the pressure
• These burs have been designed anatomically to enlarge the • Reservoirs for containing abrasive materials
fissure and eliminate small caries without removing • Hand-piece with tips made of sintered tungsten carbide with
excessive healthy enamel or dentin. variable inner diameter
92 Essentials of Pediatric Oral Pathology

• Foot control for activation of the machine TABLE 2.14: Amount of enamel removed using air abrasion
• Other components like cabinet, gauges and dials for
Nozzle tip distance Amount of Enamel removed
controls, a suction hood, etc. (mm) (mg)
Abrasive particle: The most commonly used material is
3 12
Aluminium oxide. Magnesium Carbonate (Dolomite) is used, 9 25
though more for prophylaxis, as it is soft and has less weight. 11 30
Aluminium oxide: 27 µm—Intraoral use, comfortable, 15 28
moderately effective cutting
Aluminium oxide: 50 µm—Suitable for coarse surface Amount of enamel removed (50 psi, 0.45 mm diam, 30 sec):
finishing and extraoral microetching procedures. See Table 2.14
Propellant: Compressed air is usually used, but it is not free Technique (Figs 2.34 A to C)
from moisture. Carbon dioxide gas is the propellant of choice. • A setting of 80 psi or less, with 27 µm particle size and
Carbon dioxide-Average pressure: 700-1300 psi, reduced 0.014 inch tip is comfortable and adequate for most
to 45-80 psi at the tip. procedures.
Flow of gas: 1/3 cubic foot/minute • Clean the surface of the tooth and place a caries detection
Recommended propellant pressure dye.
• Cutting enamel: 80 to 90 psi • Place the tip of the nozzle at a right angle and no more
• Cutting dentin: 40 to 60 psi than 1 mm to the surface.
• Stain removal: 40 to 60 psi • Start with a three seconds burst to trace out grooves, fissures
• Calculus removal: 80 to 90 psi and pits. The burst should be interrupted over areas of
Pressure at 80 psi is considered to be the threshold for sound enamel.
sensitivity. So it demands extra caution when using pressures • Observe, diagnose and remove any remaining decay. Use
80 psi or above. short bursts to remove the last stains.
Nozzle diameter: The inner diameter ranges from 0.011 to • As penetration becomes deeper, shorter burst with less air
0.032 inch. The following are commonly used: pressure may be used for patient comfort.
• 0.018 inch (Removal of large lesions, results in wide cone • Stop frequently, observe, diagnose and proceed.
shaped cavity) • Soft, carious dentin absorbs and scatters the particle stream;
• 0.014 inch (Universal use, removal of small lesions) the use of small round bur is recommended for the soft
• 0.011 inch (For precise cutting, preparation of occlusal pits decay.
and fissures, produces narrow deep cavity with nearly • Most cavity preparations take between 30 seconds to 1.5
vertical walls) minutes.
• Standard saliva ejector or high volume intraoral evacuator
Nozzle distance from tooth surface: It also directly influences
should be used.
the shape of the cavity as well as the amount of hard tissue
• For additional powder control 4 × 4 gauze may be placed
removed.
in the operative field.
• 0 to 2 mm—Cavity has nearly vertical walls. The diameter
of the cavity remains approximately the same as the nozzle Patient comfort: Air abrasion is generally well tolerated by
bore. the patients. Most procedures can be performed without the
• 2 to 5 mm—Cavity is more conical. use of local anesthesia. If discomfort is encountered in deeper

FIGURES 2.34A to C: Air abrasion technique

Caries in Children 93

preparations, the use of smaller particle size and lower pressure Consequently the stream spreads at approximately 22° as it
is more comfortable for the patient. The use of warm water exits the nozzle. At approximately 4 or 5 mm past the nozzle,
spray has been advocated to maximize patient comfort. Most the stream becomes too disorganized and carries insufficient
cavity preparations take between 30 seconds to 1.5 minutes. energy to act as a cutting mechanism. A common complaint of
For longer procedures, some patients may experience newcomers to the practice of air abrasion is that the instruments
discomfort from dryness and may wish to have their mouth will not cut. The three most common reasons could be:
rinsed with the water syringe and evacuated with saliva ejector. • Failure to put the nozzle near the surface to be cut—
Spray may be minimized by placing a wet 4 × 4 or 2 × 2 Holding the nozzle too far will only etch and abrade the
gauze square on the tongue and replacing it as necessary. wide area of surface.
• Failure to hold the nozzle steady—The natural tendency is
Precautions
to wave the hand-piece. For cutting to begin, the particle
• Do not touch the surface of the tooth with air abrasion tip.
stream must first cavitate the surface to be cut.
• Do not back up more than 2.5 mm from the surface.
• Failure to use magnification—The dentist simply cannot
• Do not blast the whole surface from far.
see the micro preparations initiated by the particle stream.
• Do not sweep the tip like a brush.
• Emphysema (air embolism) is a possible complication of Safety of KCP: Laboratory tests show that the amount of
all dental treatment, including air abrasion; therefore never alumina inhaled by the dentist and/or auxiliaries in an office
direct the particle stream into an open pulp chamber or into setting to be below detectable limits. The patient inhales just 7
the gingival sulcus. micrograms of alumina during each minute of treatment time.
• Prior to intraoral use, development of skill is essential on At that rate, patient would have to undergo 11.43 minutes of
extracted teeth using caries detecting dye. continued KCP to inhale an amount of alumina equal in weight
to a grain of table salt (80 micrograms). That exposure time is
Indications
equivalent to 28 typical 20 to 30 second KCP preparations.
• Strongly recommended for sealant therapy.
Further, the patient would have to undergo as many as 3.401
• Treatment of suspicious pit.
KCP treatments daily to inhale the amount of alumina particles
• Removal of old composite or amalgam fillings and
that a worker breathes daily in an environment considered safe
fractured porcelain facings.
by Oral safety health administration (OSHA) and American
• Removal of high points on crown.
conference of government industrial hygienist (ACGIH).
• Removal of casting irregularities and modification or
correction of the surface that will receive porcelain (50 µm
Sonic Oscillating Systems
particle size).
• For prophylaxis. The Sonic oscillating systems (SONICSYS) have been
developed for cutting and finishing of proximal “mini” cavities
Contraindications
using an air driven oscillating hand-piece and partially
• Severe dust allergy.
diamond-coated working tips (JG Mount, 2000). 84 This
• Asthma.
preparation method appears to be particularly suitable for
• Chronic pulmonary disease.
cutting small, first intervention cavities in proximal surfaces
• Recent extraction or surgical procedure including
with minimal extensions and good marginal morphology,
periodontal surgery.
without any risk of damaging adjacent teeth.
• Any open wound, lesion or sore, or sutures in the mouth.
Enamel in the proximal and gingival walls of posterior cavity
• Sub-gingival caries removal.
preparations for restoration with composite resin preferably
Dynamics of air abrasion: Many factors are involved in should be beveled, because the direction of the prisms is
developing particle air stream, all conforming to the laws of perpendicular to the surface. However, when using rotary
physics. As the airflow progresses towards the hand-piece, the instruments, much damage can be inflicted to the adjacent tooth.
decrease in inner diameter of the delivery tube generates an Proximal bevels are therefore usually omitted in small box and
increase in the velocity of the particle stream. The particle slot preparations. The introduction of one-sided torpedo-shaped
stream does not reach full velocity until it leaves the tip of the diamond tips in a sonic device allows for beveling of proximal
nozzle by amount of 0.75 mm. When using smaller nozzle (e.g. margins without this risk. When compared to hand instruments,
0.014 inch), the particle stream typically becomes supersonic the sonic tips allow for significantly better finishing of proximal
and can reach twice-supersonic speeds. The stream begins to bevels. The hemispherically shaped tip provides a minimal access
slow at approximately 1 mm past the nozzle. The particle opening to approximal cavities. After access to the lesion is
stream is a divergent stream. It varies from a collimated stream. achieved, caries removal is done with a round bur (low speed).
94 Essentials of Pediatric Oral Pathology

The one-sided sonic tips are excellent tools to finish and bevel TABLE 2.15: Properties of two commonly available
margins in otherwise inaccessible areas in the close vicinity of chemomechanical agents
adjacent surfaces.
Caridex Carisolv

Chemomechanical Caries Removal Solution I 1% NaOCl 0.5% NaOCl

Beeky et al 2000, published an excellent review of the chemo- Solution II 0.1 M Amino butyric 0.1 M Glutamic acid
mechanical means of caries removal. A promising new method acid glycine lycine
0.1 M NaOCl NaOCl
of chemomechanical removal of dental caries is based on the 0.1 NaOH NaOH
principle of MID (Minimal intervention dentistry) and involves
the application of chemicals.85 Two chemicals, Carisolv and Dye — 0.2 Erythrocin (Pink)
Caridex have been extensively evaluated and proven to be pH 11 11
successful (Table 2.15). The principal mode of action is based
on the use of sodium hypochlorite, a non specific proteolytic Consistency Liquid Gel
agent, and the effective interaction of 3-amino acids with Volume needed 100-500 ml 0.2-1.0 ml
carious dentin, removing organic components at room
temperature. The gel is repeatedly applied to the carious dentin Time 5-15 mins 5-15 mins
and softened caries is gently removed with specially designed Equipment Applicator unit None
hand instruments. These are scraping, non cutting instruments
with 90° edges to facilitate the removal of softened dentin Instruments Tips Specially designed
without removing the affected dentin (Figs 2.35A and B). This
method causes less discomfort compared to drilling, but takes The active ingredient in Carisolv is sodium hypochlorite.
longer time (6 mins). The procedure did not require local When mixed with amino acids, it generates chloramines. This
anesthesia to the same extent and dentin caries was effectively results in the chlorination of the partially degraded collagen,
removed without any adverse reactions. and the conversion of hydroxyproline to pyrrole-2-carboxylic
The chemomechanical approach was initially introduced acid, which initiates the disruption of collagen fibers and a
in 1972, in the form of the original GK-101 solution that
selective softening of the outer layer of carious dentin. Due
contains N-monochloroglycine (NMG) and sodium hypo-
to the high pH, only the organic phase of dentin is affected.
chlorite. There were biochemical indications that GK-101 may
disrupt the fibrillar organizations of collagen. In 1984, GK- The high viscosity of Carisolv facilitates accurate placement
101 E was introduced and marketed as Caridex, which contains and decreased volume of the material needed.86
N-monochloro D1, 2 aminobutyric acid (NMAB).
Carisolv is a relatively new material containing two Lasers in Minimal Intervention Dentistry (Fig. 2.36)
solutions. The first contains glutamic acid, leucine, lysine,
sodium chloride, erythrocin, CMC 200-800 cps, purified water Several early workers have described the potential of lasers in
and sodium hydroxide, with a pH of 11. The second solution caries prevention. Lasers may increase caries resistance of
contains sodium hypochlorite 0.5 percent. enamel. Since the first description of their use in this field,

FIGURES 2.35A and B: Procedure for chemomechanical caries removal

Caries in Children 95

The possible use of CO2 laser treatment in the prevention

of lesion progression in dental enamel has also been
demonstrated.
Argon lasers: Argon lasers have been reported to reduce or
prevent demineralization of enamel of extracted teeth using
energy densities of 25 to 100 J/cm2. At these energy densities,
no damage would be expected in pulp or in the surrounding
enamel. Argon irradiation of root surfaces significantly
increases the resistance of cementum and underlying dentin to
continual artificial caries attack. Lased cementum and dentin
seem to have an increased affinity for uptake of fluoride,
phosphorous and calcium ions and there is an apparently
reduced lattice strain in hydroxyapatite following lasing.
These findings reported over many years, have established
beyond doubt, the potential of lasers to enhance caries
resistance. However, clinical trials which might establish the
validity of the experimentally observed effects are lacking. This
is almost entirely due to the lack of official approval for hard
FIGURE 2.36: Lasers used for caries removal tissue applications.
Comparative investigations of the various wavelengths used
nearly all the common laser wavelengths have been are needed to determine the optimum operating regimens to
considered.87 maximize benefit and minimize side effects. The technique of
X-ray microtomography might be of great value in this type of
Nd:YAG lasers: Yamamoto suggested that caries could be
evaluation.
prevented using Nd:YAG laser. Studies have shown that laser
irradiation slightly alters the enamel surface, reducing Er:YAG lasers: Dental enamel is the hardest tissue type in the
subsequent subsurface demineralization. They have found a body. Controlled cutting of enamel with lasers was an elusive
significant difference in solubility between laser-irradiated and goal for many years. Beginning with Stern and Sognnaes in
non-irradiated enamel. 1964, different lasers have been tested for enamel ablation.88
Hashimoto examined the effects of a continuous wave The final breakthrough came in 1997 in the United States with
Nd:YAG laser on the acid resistance of defective rat enamel. the FDA granting marketing clearance for the Erbium:YAG
He found that after irradiation, the defective enamel surface lasers. Initially, the clearances did not include children. By
became smooth and small surface defects disappeared. The 1999, all three Er:YAG lasers were cleared for all ages.
resistance to acid demineralization increased to match that of The explosive interaction between the water molecules and
normal enamel. the Er:YAG laser pulses on the tooth tissue surfaces disrupt
CO2 lasers: Florin has shown that a continuous wave CO2 laser enamel, dentin and decay. The action does not depend on heat.
homogenizes the enamel surface by melting structural elements. A constant spray of air and water is required. Cooling is present,
The tissue in the laser-induced zones of fusion was harder than although secondary to the provision of a water film on the
adjacent, non-irradiated enamel, which Florin suggested would surface for laser interaction. Some ablation of hydroxyapatite,
increase the caries resistance. the mineral of teeth, also occurs. Pulpal tolerance has been
Walsh reported the potential of the CO2 laser for caries found to be better than with burs. The heat of friction with burs
prevention using focused infra red laser radiation on sound endangers the pulp as well as being the prime factor in patient
enamel and early pit and fissure caries. Low power levels discomfort. Almost all laser cavity preparations can be
(2 to 5 W) induced localized melting and re-solidification of performed without anesthesia. Microfractures of enamel at the
enamel with little surface destruction. For sound fissures, fusion margins are not present with laser ablation.
of enamel on the lateral walls of the fissure eliminated the Surface alteration, in addition to enamel removal, is
fissure space, providing a sealant effect. In carious fissures, achieved with Er:YAG lasers. The pulsing action creates a
carious enamel was vaporized and adjacent sound enamel fused surface effect that resembles chemical etching with an expanded
to partially eliminate the defect. Walsh suggested that this surface area and increased surface energy. Micro-retention in
technique had potential applications in sealing pits and fissures undercuts and punctuated surfaces aids bonding strengths.
and producing physicochemical alterations in enamel, which When acid and laser conditioning are combined, the bond
might have preventive benefits. strengths with restorative resins are greater than with either
96 Essentials of Pediatric Oral Pathology

method alone. Low power and pulse settings are the most genius Nikola Tesla patented his first ozone generator and in
appropriate for laser surface conditioning. 1900, he formed the Tesla Ozone Company. In 1898, the
Cavity preparation is approached in a manner similar to Institute for Oxygen Therapy was started in Berlin by
conventional methods. Rubber dam is recommended for proper Thauerkauf and Luth. In 1913, the Eastern Association for
isolation. Topical anesthesia aids rubber dam clamp placement Oxygen Therapy was formed by Dr. Blass and some German
without requiring local anesthesia. The outline form is associates. During World War I, Ozone was used to treat
established at high energy settings for optimal cutting. On wounds, trench foot, gangrene and the effects of poison gas.
accessing dentin and decay, lowered energy parameters are used Dr. Albert Wolff of Berlin also used ozone for colon cancer,
for decay removal and interior contouring. Even procedures cervical cancer and decubitus ulcers in 1915.
near the pulp usually do not cause discomfort to the unanes- In 1920, Dr. Charles Neiswanger, MD and the President
thetized patient. No smear layer is formed and peritubular of the Chicago Hospital College of Medicine, published
dentin may be cut more easily, opening tubules. “Electro Therapeutical Practice” that entitled “Ozone as a
All types of cavity preparations can be performed with Therapeutic Agent”.
Er:YAG lasers (Class I, II, III, IV, V and VI). Crown prepara- Ozone kills more than 99 percent of all bacteria, fungi and
tions and removal of metallic restorations are contraindicated. viruses as this powerful oxidant readily penetrates through
Existing composite materials may be removed. Extraordinarily decayed tissue. Application of ozone to carious tooth surface
large and deep restorations may require anesthesia and may produces a clean and sterile lesion that will remineralize easily
be prepared with bur and laser methods combined, with the and eliminate the need for placing restoration. FDA approves
bur used for enamel contouring and the laser for decay removal the use of ozone in the medical field.
and surface conditioning.
Uses
• Dental cavity disinfections
Use of Lasers in Pit and Fissure Caries
• Root canal disinfections
Pit and fissure caries are ideal for laser treatment. Pit and fissure • Oral candidiasis treatment
caries typically involve the organic part within an enamel • Herpetic treatment
defect. This plug material consists of food debris, bacteria, and • Treatment of aphthous ulcer
remnants of the enamel-forming ameloblasts. Even though, • Stomatitis treatment
there is no absorption of the laser in the enamel, the staining • Cleft lip and palate therapy
of the organic plug makes the material susceptible to the action
Contraindications: In the following cases, the ozone unit
of Nd:YAG lasers. The FDA has granted clearance for an Nd:
should not be used or be used with caution.
YAG laser to be used to detect and remove the caries associated
• Patients using cardiac stimulators (pacemakers).
with pit and fissure lesions. The laser energy gives visual and
• Epileptic patients or those suffering from other serious
acoustic response if organic plug material and caries are
neurological illnesses.
present. Otherwise, there is no interaction with the enamel. The
• Patients suffering from psychological problems.
approach works well with sealant placement. More extensive
• On mucous membrane of infants (under one year old).
caries can be restored with the assistance of bur preparation.
• Patients who are overly sensitive to electric currents.
The pulsing action of the Nd:YAG laser has been found to
• Patients suffering from serious asthma.
reduce tooth sensitivity, reducing the need for anesthesia.
• Pregnant women.
Use of Ozone in Minimally Intervention Dentistry Advantages
Ozone is a strong naturally occurring oxidizing agent in nature. • Good efficacy in disinfection, killing bacteria and healing
It is produced by the action of UV rays on lightning in the wounds.
atmosphere. • High concentration but low volume ozone precisely
O2 2O generated in the focused area.
O + O2 O3 (Ozone) • Disinfection effect of ozone lasts long.
The first ozone generators were developed by Werner von • Eliminates worry of misuse of antibiotics.
Siemens in Germany in 1857. In 1870, the first report on ozone Constitution
being used therapeutically to purify blood was given by C. A : Control Box
Lender in Germany.88 In 1885, the Florida Medical Association B : Ozone Generating Handpiece
published “Ozone” by Dr. Charles J. Kenworth, MD, detailing C : Safety Rod (Ground Wire)
the use of ozone for therapeutic purposes. In 1896, the electrical D : Foot Pedal Switch
Caries in Children 97

E : Power Adapter flex system) to remove stain and debris. DIAGNOdent is used
F : Probe to measure the occlusal areas for fluorescence of bacteria and
indirectly the density of tooth structure and presence of decay.
Probes
A number of units are commercially available that may be used
No. 1 Probe: Pointed probe 10
for dental applications, e.g. HealOzone.
For treatments of gingivitis
A burst of ozone gas at a preset concentration is delivered,
No. 2 Probe: Pointed probe 50
after which the unit vacuums any residual ozone back through
For treatments of gingivitis
a catalyst that converts this ozone back to oxygen within
No. 3 Probe: Flat probe
another ten seconds. To complete the treatment, the HealOzone
For treatments of skin and mucous membrane
pumps a reductant fluid/mineral wash on to the treated site, to
No. 4 Probe: Conical probe
kick-start the remineralization process, which takes five
For alveolar therapies after tooth extraction
seconds. So in just 25 seconds, elimination of microflora is
No. 5 Probe: Pointed probe 10 with conical plastic
achieved and healing starts, thus naturally restoring the tooth
For root canal treatments
tissues. Seal must be achieved around the tooth surface to
Types of ozone generators: Ozone is the only gas that will pick prevent the escape of the ozone, and without this seal the
up and hold electrical energy. In doing so, it becomes HealOzone will not produce ozone gas. An attractive home care
tremendously active and seeks to combine with all other kit, consisting of fluoride dentifrice, a bottle of mouth rinse
substances. The list of substances that are inert to ozone is very and patient information booklet is provided for every patient
short, and includes glass, Teflon, Kevlar, silicone and gold. to encourage remineralization and reversal of carious process.
Therefore, any ozone generator and auxiliary equipment must It is very critical to follow up the patient for 3 to 6 months and
be composed of these substances only. There are several remeasure caries activity with DIAGNOdent.
different techniques used to produce medical grade ozone, Ozone concentration
where freedom from contamination is critical. One type of Medical ozone is produced in varying concentrations. The
generator uses an ultraviolet lamp as its source. It produces a quantity of ozone in comparison with the quantity of oxygen
very small amount of ozone in a narrow frequency bandwidth in the gas stream is called percent concentration. It is measured
of ultraviolet light. Outside of the bandwidth, UV destroys in micrograms of ozone per millimeter (or cc) of the mixture.
ozone. An ultraviolet lamp is unreliable because it is subject A liter of oxygen weighs 1.4 grams.
to degradation over time, causing uncertainty regarding 0.5 percent × 1.4 gm = 7 g/cc
concentration and eventually burns out. 1.0% × 1.4 gm = 14 g/cc
The second method of ozone production is corona 1.5% × 1.4 gm = 21 g/cc
discharge, where a tube with a hot cathode is surrounded by a
5 percent or 70 g/cc is considered to be the upper limit
screen anode. The best ones are called dual-dielectric, because
of concentration for internal use of medical ozone. Dr.
they have a layer of glass separating each component from the
Greenberg has shown, in vitro, that at concentrations of 90 g/
gas stream. This prevents contamination of the ozone in the
cc, there was crimping of red blood cells, which was definitely
best designs, but heat is produced and heat destroys ozone. To
harmful. Experiments by F. Sweet et al have shown inhibition
compensate for the loss in concentration, more electricity is
of growth in healthy cells at concentrations above 72 g/cc. If
used, resulting in more heat and consequent electrical failure.
This produces generators that have short lives. Lack of one stays below that level, there will be few problems.
durability has always beset the ozone generator industry and Ozone also stimulates production of superoxide dismutase,
was one of the major reasons for naturopaths mostly catalase and glutathione peroxide, which are the enzymes in
abandoning ozone therapy during the Thirties. the cell wall, which protect the cell from free radical damage.
A third method of producing clean, medical grade ozone Ozonated water: Ozone can be dispersed in water at 0.5 to 2
is called cold plasma. It uses glass rods filled with a noble gas ppm. Research has shown that water whose bond angle is 101
and an electrostatic plasma field which turns the oxygen into degree is ‘dead’ water bereft of life-giving energy. The highest
ozone. Since there is no appreciable current, no heat is energy obtainable in liquid water is a bond angle of 109.5
produced. Thus the generator will last a very long time, limited degrees, and this is attainable by ozonating water at 4°C.
only by the quality of the power supply. The original cold Ozonated water is not stable for very long, even at 4°C. The
plasma ozone generators were invented by Nikola Tesla in the ozone breaks up into an atom of elementary oxygen and a
1920’s and they still work 75 years later. molecule of oxygen on contact with a substrate. This confers
In ozone treatment, the tooth surfaces (pit and fissure) are it good antibacterial property, because of which it is
cleaned with slurry of sodium bicarbonate and water (Prophy- recommended for use as an endodontic irrigant.
98 Essentials of Pediatric Oral Pathology

Atraumatic Restorative Treatment (ART) rods lie in a different orientation, glass ionomer will still
develop ion exchange adhesion and show acceptable longevity.
ART is a procedure based on excavating soft decalcified tooth
tissue using hand instruments and restoring the cavity with an Site 1–size 1: As the fissure walls become demineralized, the
adhesive filling material. Initially, ART was developed in dentin will become involved as well. Radiographs will not show
Tanzania in mid 1980’s and introduced in a dental clinic setting this early lesion very clearly and laser detector and electrical
in Malawi some years later. It was then evaluated under field impedance machines have limitations. In the presence of strong,
conditions in Thailand in 1991. This was done by Dr. Jo. fluoridated enamel, the occlusal surface entry to the lesion will
Frencken. Formally, the ART technique was released to the remain limited, and bacteria-laden plaque can be forced down
world on 7th April 1994 (i.e. The World Health Day) (Shan into a defective fissure. In these circumstances, dentin involve-
Farhan).90 This procedure was developed because millions of ment can become advanced before symptoms are noted.
people in less industralized countries and special groups like The fissure system is a complex series of pits and fissures;
refugees and people living in deprived communities are able therefore, a carious defect will often be limited to a very
to obtain dental care. restricted area, leaving the remaining fissure system sound and
uninvolved. This means that only the carious defect needs to
Conservative Operative Techniques be instrumented. Minor apparent defects should be explored
in a very conservative manner before sealing the fissure system.
Operative recommendations according to the new classification
are as follows: Site 1–size 2: In this classification, the lesion will either have
progressed to some degree or it may represent replacement of
Size 0: The lesion represents an area of demineralization on a failed Class I restoration. Same conservative principles as in
any of the three surfaces and can be treated by usual preventive site 1– size 1 are applied and there is no need to open up the
measures. While treating a new lesion, minimize the removal remaining fissures any further. If there is any part of the fissure
of tooth structure, thus maintaining the natural strength of the system that is in doubt, it can be explored very conservatively,
crown and keeping the load of the restoration. but it is sufficient to seal the fissures and any carious process
below will be arrested. It will progress no further until there is
Site 1–size 0: 91 The lesion dictates the concept of the fissure
access to the usual nutrients required by the bacteria again
seal, as discussed by Simonsen, 1989. Sealing a deep fissure
(Mertz Fairhurst and others, 1992). If there is any marginal
before it becomes partially occluded by plaque and pellicle,
leakage, there will be further bacterial activity, which is very
and in advance of demineralization into dentin, has an
unlikely when using glass ionomer because of ionic adhesion
acceptable clinical history (Feigal, 1998; Ekstrand and others, and the presence of fluoride release. Instrumentation and
1998). The earliest fissure sealants were unfilled or lightly filled restoration techniques for these lesions will be the same as for
resins, but recent research has shown that there are some doubts a Size 1 lesion. But the occlusal involvement will be more
about the integrity of the acid-etch union between resin and extensive and, if there is any doubt about the ability of the glass
enamel in these regions. It has been shown that a glass ionomer ionomer to withstand the occlusal load, it can be cut back
will successfully occlude such a fissure (Wilson and McLean, conservatively and laminated with resin composite.
1988). This is now being termed “fissure protection” to Glass ionomer is used for restoration of both Size 1 and
differentiate it from a “resin seal”. Size 2 lesions in this category. The restoration is well supported
Anatomy of enamel within a fissure differs from that of by the remaining tooth structure and the ion exchange adhesion
other surfaces in that it is covered with a layer of enamel rods will ensure complete sealing of the remainder of the cavity. If
that appear to run parallel with the surface rather than at right there is any demineralized dentin remaining on the floor of the
angles. When it is etched with orthophosphoric acid, it will
cavity, there will be no further carious activity and there is a
not develop the usual pattern of porous enamel that allows
potential for remineralization (Ngo and others, 2001). It is
penetration of the unfilled resin that is normally relied upon to
possible to use a resin composite for the restoration but that
provide the micromechanical attachment (Burrow, Burrow and
would also mean cleaning the floor down to sound, healthy
Makinson, 2001). The presence of this type of enamel may well
dentin to develop an acid-etch union with fully mineralized
account for the loss of the resin seal in many cases. Neither a
tooth structure. This may mean removing dentin that could
resin nor a glass ionomer will flow into a fissure beyond the
otherwise be remineralized and healed.
point where the fissure narrows down to approximately
200 m in width. So, retention of both materials appears to be Site 2–size 0: 92 Proximal lesions progress very slowly because
dependent on adhesion to enamel at the entrance of the fissure that surface is not under masticatory load and is, to a degree,
rather than mechanical interlocking into the complexities of protected from traumatic damage (Pitts, 1983; Shwartz and
the fissure. Recent work suggests that even though the enamel others, 1984). In contrast to the occlusal fissure lesion, it may
Caries in Children 99

take up to four years to penetrate the full thickness of the

enamel and an additional four years to progress through the
dentin to the pulp. It is necessary to identify Size 0 and Size 1
lesions before any intervention, because it should be possible
to heal the lesion and it is only when cavitation is established
that a surgical technique is required. It is essential to avoid the
use of a probe to explore the proximal surface because this is
the quickest way to actually damage the enamel and cause a
cavity.
Site 2–size 1: In this case, cavitation is established on the
proximal surface and surgical approach becomes essential.
First, determine the position of the damage in relation to the
crest of the marginal ridge. If it is more than 2.5 mm below
the crest, then it may be possible to approach the lesion through
the occlusal fossa and design a “tunnel” cavity (Hasselrot,
1998; Wilson and McLean, 1988). If it is less than this distance,
a tunnel will only undermine the marginal ridge and weaken it
still further. In this case, it is better to design a small box or
“slot” cavity beginning on the outer slope of the ridge, retaining FIGURES 2.37A and B: Tunnel cavity preparation for site
2–size 1 lesion
as much of the enamel as possible. Occasionally, a further
alternative will present itself, when a large Site 2, size 3 or 4 The partial tunnel preparation is indicated in the presence
lesion is being repaired or replaced and a small Size 1 lesion of macroscopically observable cavitations or when the enamel
is revealed on the side of the adjacent tooth. lesion disintegrates during cavity preparation. In such cases,
the enamel may be carefully smoothened around the periphery
Site 2–size 1 “tunnel” (Figs 2.37A and B): Here, the contact
of the opening, leaving the remaining part of the demineralized
area may remain sound and the marginal ridge may be quite
enamel to be remineralized.
strong, provided the lesion is more than 2.5 mm below the crest
In a total tunnel preparation, all demineralized enamel is
of the marginal ridge (Wilson and Mc Lean 1988). Access to
removed.
the lesion through the occlusal surface should be limited to the
The advantages of these preparations include:
extent required to achieve visibility and, where possible, should
• Preservation of tooth structure
be undertaken from an area that is not under direct occlusal
• Maintenance of the marginal ridge and proximal contacts
load (Knight, 1984). For most patients, there is a fossa
• Avoidance of iatrogenic damage to the adjacent tooth
immediately medial to the marginal ridge that is the most
during preparation
suitable position for initial entry and, in a normal occlusion, is
• Provision of fluoride supply to adjacent tooth tissue
often not an area of occlusal contact. Resin composite is not
• A small restorative perimeter and thereby reduced micro
indicated for restoration of these lesions because it will not be
leakage
possible to access the proximal lesion to a sufficient degree to
be able to reliably remove all demineralized enamel. It will Instruments required
not be possible to provide a beveled margin to ensure proper • Small, tapered diamond bur (#206) at intermediate high
adaptation of the resin to the enamel. But glass ionomer will speed (40000 revs/min) with air/water spray, to open
flow readily into a small cavity and has the ability to through the occlusal fossa.
remineralize the enamel margins and any dentin on the axial • Small round burs, sizes 1/011 to 016, for caries removal.
wall that may be demineralized. • Long shank bur for difficult access.
Tunnel preparations can be classified as: • Access for hand instruments is limited, but the MC 1 double
1. Internal bladed chisels may be useful.
2. Partial Site 2–size 1 “slot” (Fig. 2.38): A slot cavity could be used
3. Total when the lesion is less than 2.5 mm below the crest of the
Internal tunnel preparation (or closed tunnel) is actually a marginal ridge and resin is the best material for this cavity
class I cavity, which is appropriate when there are no macro- design. Generally, the lesion will be obvious to visual
scopically observable cavitations. The objective is to obtain examination, particularly when using magnification, because
remineralization of the enamel lesions. of the discoloration under the marginal ridge.
100 Essentials of Pediatric Oral Pathology

incrementally and use a small plastic sponge to tamp the cement

to place. Use an occlusal matrix or a gloved finger, as a matrix
to apply additional pressure.
Cut back the cement and laminate the occlusal entry with
composite resin only if there is doubt about the ability of the
cement to withstand the occlusal load.
Mini box restorations: This preparation was developed in
order to treat caries lesions on proximal surface. Here, the
marginal ridge is removed, which makes it different from tunnel
preparations.
Site 2–Size 1—Proximal approach: A very conservative
approach to restoring a proximal lesion can be achieved on
limited occasions, such as when the proximal surface of a tooth
becomes accessible at the time of cavity preparation in an
FIGURE 2.38: Slot cavity preparation for site 2–size 1 lesion
adjacent tooth. The lesion may have been revealed through
radiographs or may be noted only during cavity preparation.
The outline form will be dictated entirely by the extent of Instruments required
the breakdown of the enamel, removing only that which is • Small, tapered diamond bur (#206) at intermediate high
friable and easily eliminated without applying undue pressure. speed (40000 revs/min) with air/water spray, to open the
Remaining demineralized enamel will generally heal enamel lesion.
satisfactorily. Retention will again be through adhesion so it is
• Small round burs, sizes 1/011-016, for caries removal.
only necessary to clean the walls around the full circumference
• Use a long-shank bur for difficult removal.
of the lesion so there will be adhesion to the dentine as well.
• Access for hand instruments is limited, but the MC 1 double
Leave the axial wall because it will consist of affected dentin
bladed chisel may be useful.
only and will remineralize over a short period of time. Its
removal will be a hazard to the pulp. Preparation and restoration: Enlarge access into the enamel
lesion using a small tapered diamond bur at intermediate high
Instruments required
speed under air/water spray with good illumination and
• Small, tapered diamond (#206) at intermediate high speed
magnification. Remove the infected dentin with small round
(40000 revs/min) with air/water spray, to open the outer
burs at slow speed. Burs with long shanks may be required for
slope of the marginal ridge.
correct bur placement. Make sure the circumference of the
• Small round burs, sizes 1/011-016, for caries removal.
cavity is completely clean to allow for the ion exchange
Preparation and restoration: Use a small tapered diamond bur adhesion to dentin. Leave affected dentin undisturbed on the
at intermediate high speed under air/water spray, and enter from axial wall because it will remineralize and protect the pulp.
the outer slope of the marginal ridge. Open buccally and Condition the cavity, and use a short length of a mylar or
lingually only as far as required to identify the cavitated enamel. metal strip as a matrix, supported as required with a wedge.
Protect the adjacent tooth with a metal matrix band and leave Restore using a high-strength, auto-cure radiopaque glass
this in place during restoration. Remove friable enamel rods ionomer so that it can be monitored radiographically in future.
carefully with a small hand instrument such as the MCI chisel. Contour and polish immediately prior to placing the adjacent
Retain a contact with the adjacent tooth wherever possible. restoration.
Use small round burs at slow speed to remove infected
dentine from the full circumference of the lesion. Leave affected Site 2–Size 2 and beyond (Figs 2.39A and B): These categories
dentine undisturbed on the axial wall because it will recognize the increasing size of the lesion and in many cases
remineralize and protect the pulp. it will represent replacement dentistry where a failed GV Black
Condition the cavity and restore with the selected material. Class II restoration has to be repaired or replaced. There will,
The most suitable material for the restoration will be a high- of course, be neglected new lesions where there is a rather large
strength, auto-cure, radiopaque glass ionomer with the highest proximal lesion and these should be dealt with keeping the same
physical properties. A resin-modified material is satisfactory, principles in MID. Often the occlusal fissure can be left
provided it can be adequately light activated. uninvolved and possibly sealed as part of the restoration.
Place the glass-ionomer with a capsule or a disposable Extension of the cavity margins will need to be sufficient to
syringe to optimize adaptation to the cavity walls. Place allow access to the lesion only to the extent that there will be
Caries in Children 101

Light activate or allow the glass ionomer to set. Check the

excess around the periphery of the matrix to see that it is
properly set. Remove the matrix and immediately apply a
generous coating of a low viscosity resin as a sealant. This is
essential only for the auto-cure material, but is still a good idea
for the resin-modified material. Use a sharp blade to minimally
trim an auto-cure material before light-activating the resin seal.
FIGURES 2.39A and B: Site 2–size 2 lesion restored with Use fine diamonds under air/water spray to contour a resin-
composite resin material modified material and then seal it again with the low viscosity
sufficient sound dentin exposed for proper adhesion around the resin seal.
full circumference. Unsupported enamel will be supported by Polish the restorations after the glass ionomer has matured,
the adhesive materials used for restoration and there is no need only if it is essential. If the matrix was properly applied,
for extension to the so-called “caries free” areas. subsequent polishing is often not required.
When the lesion reaches these dimensions, it is likely that Site 3–size 2: These lesions will generally be cavities resulting
there will be a need to laminate the glass-ionomer base because from active caries. They will vary from the Size 1 lesion only
the occlusal load will be beyond that, which can be safely in relation to their size and they will be more of a challenge to
handled over long term with the cement alone. This suggests restore. The usual instrumentation will be required to remove
that the lamination technique will be the method of choice. the demineralized infected dentin on the surface before
Site 3–size 0: These lesions do not require restoration but they restoring. Clean the walls around the periphery only although,
do need careful diagnosis and treatment planning to ensure they almost certainly, there will be affected dentin remaining on the
will no longer progress. All the potential causes have been floor of the cavity. However, as long as the margin is sealed
discussed above, so effective treatment should not be difficult against micro leakage, the restoration will be effective and the
to achieve. affected dentin will heal.
Instruments required
Site 3–size 1: A common lesion in this category will be the
• For this larger carious lesion it may be necessary to extend
result of advancing erosion, abrasion or abfraction. It is
the margins a little using a small tapered diamond bur
important to both eliminate the cause and seal the lesion before
(# 206)
it becomes too deep. Treatment should be kept very simple
• Use a small round bur to clean the walls sufficient to allow
because a glass ionomer will adhere very effectively to the
ion exchange adhesion.
burnished sclerotic surface of the root through the ion exchange
mechanism. This means that any form of cavity preparation, The preparation is essentially the same as described for the
apart from conditioning, is strictly contraindicated because a site 3–size 1 lesion.
smooth surface is the best for any form of chemical adhesion. Site 3–size 3: 93 These lesions are generally root surface lesions
Instruments required: For the erosion of lesion, there will be on the interproximal surfaces of anterior or posterior teeth.
no instruments required because the cavity need not be prepared Under these circumstances, it will often be prudent and
at all. conservative to enter the lesion from either the buccal or the
For the small carious lesion, use a small round bur only to lingual rather than from the occlusal. The decision concerning
clean the walls sufficient to allow ion exchange adhesion. the side of entry will be dictated primarily by the position of
the lesion and secondarily by the need for access and
Preparation and restoration: For the erosion lesion, clean
lightly with slurry of pumice and water on a small rubber cup convenience. It is, of course, possible in a young patient, to
to remove any plaque on the surface of the lesion and ensure approach an initial lesion that lies immediately under the
complete adaptation of the glass ionomers to the tooth. In the contact area with this design. However, the closer the cavity is
presence of active caries, clean the walls around the full to the marginal ridge, the more likely it is that the ridge will
circumference but leave the axial wall to remineralize under fail at a later date. The lesion in the older patient will be beyond
the glass ionomer. the cementoenamel junction and therefore well below the
Condition the surfaces of the cavity with 10 percent contact area, so remaining tooth structure is less likely to fail
polyacrylic acid for 10 seconds only, wash well and dry lightly. subsequently.
Select a suitable matrix and pre-form as required. Mix the Instruments required
appropriate material. Syringe the glass ionomer on to the tooth • Small, tapered diamond bur (#206) at intermediate high
surface and apply the matrix to adapt the material well and speed (40,000 revs/min) under air/water spray.
positively to the surface of the tooth. • Small round burs, sizes 1/011-016, for caries removal.
102 Essentials of Pediatric Oral Pathology

• Long shank round burs may be required for deep access. • Pulp diagnosis in a child is often imprecise as clinical
• Access for hand instruments is limited. symptoms do not correlate well with histological pulpal
Preparation and restoration: Enter the lesion from the buccal status.
or the lingual as the position of the carious lesion dictates, using • The age and behavior compromise reliability of pain
the small tapered diamond stone at intermediate high speed response in children.
under air/water spray. Use a short length of a metal matrix to • The most important and difficult aspect of pulp therapy is
protect the adjacent tooth while working. Wedge the matrix determining health status of pulp or degree of inflammation.
carefully, when placing the cement. • Treatment goals are developmentally oriented in pediatric
Begin slightly occlusal to the lesion, and move dentistry.
interproximally and gingivally until the lesion is clearly visible.
Sacrifice sufficient tooth structure or old restoration to allow Need of Pulp Therapy in Children
access and convenience form without unduly weakening the • It is very high in developing countries due to a high caries
marginal ridge. Use small round burs at slow speed to remove severity index as also the high DMF/DMFT scores.
all infected dentin and develop clean walls around the entire • Lack of awareness and lack of adequate preventive
circumference. Leave the axial wall, even though it is deminerali- practices add to the high caries rate in developing countries.
zed. If possible, retain the wall at the opposite side from the entry • Dietary patterns, particularly a cariogenic diet also add to
to provide a positive finishing line for the restoration. the above.
Restore using a radiopaque glass ionomer. If access is • Anatomy of primary teeth (thinner enamel layer, more
available for correct placement of the activator light, use a porous dentinal tubules, marked cervical constriction) is a
resin-modified material. Trim and contour carefully after significant factor due to which spread of caries in deciduous
placement to ensure there is no overhang or over-contour. Seal teeth is more rapid and also affects their restorative
with a very low viscosity light-activated resin enamel seal. treatment modality.
Sandwich technique/dentin “replacement”: It is perhaps
difficult to distinguish or delineate between using glass ionomers Objectives of Pulp Therapy in Children
as liners, dentinal adhesives, and the sandwich technique. The “The successful treatment of a pulpally involved tooth is to
sandwich technique gets its name from the fact that, in this retain that tooth in a healthy condition, so it may fulfill its role
particular usage, glass ionomers are “sandwiched” between the as a useful component of the primary and young permanent
tooth surface below and the (other) restorative material above, dentition.” (Lewis and Law)
usually being resin composite. There are a number of articles
promoting the use of this technique, with more limited exposure Effects of Premature Loss of Primary Teeth
to clinical testing of the technique with reported outcomes. The
impressive 91 percent success rate of restorations in the primary • Loss of arch length.
dentition, reported by Mjor, indicated that 9 percent restoration • Insufficient space for erupting permanent teeth.
failure group was represented by a 9 percent failure rate of • Ectopic eruption and impaction of premolars.
amalgam restorations, 8 percent failure rate of traditional glass • Mesial tipping of molar teeth adjacent to primary molar loss.
ionomer cement restorations and 7 percent failure rate of resin- • Extrusion of opposing permanent teeth.
modified glass ionomer cement restorations.94 • Shift of the midline with a possibility of crossbite.
• Development of certain abnormal tongue positions.
MANAGEMENT OF DEEP DENTAL CARIES
IN CHILDREN Effects of Caries on the Pulpodentinal Organ
• Carious process forms three different forms of irritants:
Introduction 1. Biologic irritants.
• Treatment of pulpally inflamed primary and permanent 2. Chemical irritants.
teeth in children presents a unique challenge to the dental 3. Physicomechanical irritants.
clinician because of consideration of a variety of factors • Considerable controversy exists about the depth of carious
such as stage of development of the tooth and anatomy of lesion and pulp status.
the tooth. • M S Duggal95 observed that the response of primary pulp
• Importance of preoperative diagnosis cannot be to carious attack is poorly understood.
overemphasized as without a sound diagnosis, it is impossible • Mortimer, 1970, 96 McDonald, 1994, 97 stated that the
to render adequate treatment for a diseased tooth. anatomy of primary teeth is different in the following ways:
Caries in Children 103

— Smaller dimension. TABLE 2.16: Major differences between

— Thin, permeable, less calcified enamel. affected and infected dentin
— Permeable, thin dentin. Sr. No. Infected Dentin Affected Dentin
— Large pulp chambers and prominent horns.
• These studies revealed that inflammation of pulp in primary 1. Bacterial invasion No bacterial invasion
molars develops at an early stage of proximal caries. 2. Distorted tubules Intact tubules
• Hence, large proximal restorations in primary teeth without
3. Degeneration of Intact odontoblastic
due consideration to the pulp are doomed to failure.
odontoblastic process process
• These diverse results merely confirm that many factors are
involved in the type of reaction of the pulpodentinal organ 4. Not capable of Capable of remineralization
towards caries. remineralization if pulp is vital

Specific Treatment Modalities

Following factors should be considered before deciding upon
a specific treatment modality:
Type of Decay
• More acute caries, less effective reparative mechanism.
Chronic decay is usually accompanied by substantial repair,
provided caries has not extended to the pulp.
• If chronic decay involves pulp, then a destructive reaction
should be expected.
Duration of Decay Process
• Longer duration of acute decay leads to more massive
destruction of tooth structure.
• Longer duration of chronic decay leads to more time for
repair if pulp is not involved.
Depth of Involvement FIGURE 2.40: Infected and affected dentin
• Deeper the caries is, nearer are the sources of irritation to
the pulp with greater possibility of pulpal destruction.
• It is usually found in the deepest portion of the carious
• May be classified into:
cavity.
— Peripheral Involvement.
• Effective Depth = 2 mm or more: healthy reparative reaction.
— Moderate Involvement.
• Effective Depth = 0.8 to 2 mm: unhealthy pulpal reaction.
— Profound Involvement.
• Effective Depth = 0.3 to 0.8 mm: moderate to severe pulp
— Perforating Involvement.
reaction.
Number and Pathogenicity of Microorganisms
Concept of “affected” and “infected” dentin (Table 2.16 and
• Greater the virulence and population of microorganisms,
Fig. 2.40)
greater is the likelihood of pulpal reaction.
• Bacteria never penetrate as far as the advancing front of
• Number of virulent microorganisms found in a caries inactive
the lesion.
mouth is considerably higher than that in a caries active mouth.
• Infected dentin is softened and contaminated with bacteria.
Tooth Resistance • Affected dentin is softened dentin but not yet invaded by
• Thickness of dentin. bacteria.
• Permeability of dentin.
Incidents of macroscopic direct pulp exposure
• Fluoride and calcium content of involved dentin.
• Whenever a carious lesion extends 3 to 4 mm pulpally or
• Susceptibility of tooth—all these factors affect the
axially to DEJ, visible perforation to the pulp chamber can
resistance of a tooth to the caries attack.
be expected in 75 percent cases.
Concept of “Effective Depth” • In younger tooth, the probability of perforation increases.
• It is the area of minimum thickness of sound dentin • Perforation incidents increase, as the lesion occurs more
separating the pulpal tissues from carious lesions. apically on the axial wall.
104 Essentials of Pediatric Oral Pathology

TABLE 2.17: Relation of pulp exposure with

the degree of pulp inflammation

Exposure Peripheral Bleeding Status of Repair

size dentin pulp inflam- ability
mation of pulp

Pin-point Sound Absent Absent Excellent

FIGURE 2.41: Schematic representation of vital
Pin-point Sound Very mild Mild Good
pulp therapy procedures
and
coagulative
Indirect Pulp Capping
>0.5 mm Carious Absent Moderate Borderline
Indirect pulp capping is defined as the application of a
>0.5 mm Carious Profuse Moderate/ Absent
medicament over a thin layer of remaining carious dentin, after
severe
deep excavation, with no exposure of the pulp.
>0.5 mm Soft carious Profuse Severe Absent Indirect pulp capping may be defined as the removal of
with pus
the infected layer of dentin and the placement of a medicament
against the noninfected dentin in order to remineralize the
Incidents of microscopic direct pulp exposure: underlying demineralized tissue.98
• Peripheral pulp tissue is relatively avascular.
Objectives
• Blood vessels are very narrow and tortuous which impedes
• To avoid pulp exposure.
the passage of RBC, WBC.
• To avoid need of more invasive pulp therapy.
• Hence, if these layers are involved in excavating carious dentin,
there will be no hemorrhage observed from perforation. Historical review
• Only evidence of an exposure is oozing of colorless dental • Indirect pulp capping was first described by Pierre
pulp fluid which can be detected microscopically. Fauchard in the 18th century.
• John Tomes in the 19th century put forth the idea of leaving
What is a pulp exposure? (Table 2.17) discolored dentin to avoid sacrifice of a carious tooth.
• An exposure of dental pulp exists when the continuity of • WD Miller put forth the concept of sterilization of dentin.
the dentin surrounding the pulp is broken by physical or • GV Black, however, refused to accept the idea of leaving
bacterial means. behind caries in a tooth irrespective of pulpal exposure.
• It is usually accompanied by symptoms which are most
indicative of the actual condition of pulpodentinal organ. Rationale
• It gives an idea about the repair abilities of the dental pulp. Decalcification of dentin precedes bacterial invasion within
• Lower the ratio of exposure diameter relative to the dentin, hence the removal of dentin infected with bacteria may
dimensions of pulp and root canal, greater will be the provide a suitable environment for the remineralization of the
possibility of repair and healing of the pulpodentinal decalcified dentin.
organ. Difference between outer and inner carious dentinal layers?
• Closer the exposure to anatomical constrictions in pulpal • Outer layer of carious dentin:
chamber, lesser will be the repair possibilities. — Irreversibly denatured
— Infected
PULP TREATMENT PROCEDURES — Incapable of being remineralized
• They can be classified as: • Inner layer of carious dentin:
— Vital pulp therapy (Fig. 2.41). — Reversibly denatured
— Non-vital pulp therapy. — Not infected
• They can be also classified as: — Capable of being remineralized
— Conservative pulp treatment.
Evidence based conclusions
— Radical pulp treatment.
• Cultures of deep layers of remaining carious lesions are
nearly always infected with microorganisms before treatment.
Vital Pulp Therapy
• Cultures of successive layers of carious dentin have
Refer Fig. 2.41 uninfected demineralized dentin below it.
Caries in Children 105

• If only a few bacteria remain in the dentin and find their

way to the pulp, they will be inactivated by healthy pulp.
• However, Whitehead et al stated that only 51 percent
samples were free from signs of organisms after complete
removal of soft dentin.99
• Shovelton observed that primary teeth showed more
bacteria after removal of carious dentin.100
• The above observation was supported by Seltzer and
Bender.101
• Thus, complete clinical removal of carious dentin does not
necessarily ensure that all infected tubules have been
eradicated.
Studies about the coping ability of pulp for minute
contaminations
• Reeves-Stanley102 and Shovelton103 found that when caries
FIGURE 2.42: Placement of calcium hydroxide
is 0.8 mm away from the pulp; no significant inflammatory in indirect pulp capping
changes occur in permanent teeth.
• Rayner and Southam104 observed that in primary teeth this
distance ranges from 0.6-1.5 mm for inflammatory changes Radiographic examination
to occur. • Large carious lesion with apparent pulp exposure
• Interrupted or broken lamina dura
Indirect pulp capping
• Widened periodontal ligament space
Indications
• Radiolucency at the root apices or furcation areas
Case selection should be based upon following findings:
History Two sitting procedure of indirect pulp capping
• Mild discomfort from chemical and thermal stimuli • Anesthetize the tooth and apply the rubber dam.
• Absence of spontaneous pain • Establish the outline form.
• Remove the soft, necrotic, infected dentin with a large
Clinical examination
round bur at slow speed.
• Large carious lesion
• Remove the peripheral dentin with a spoon excavator.
• Absence of lymphadenopathy
• Cover the remaining affected dentin with hard set calcium
• Normal appearance of adjacent gingiva
hydroxide (Dycal, Reocap, Renew) (Fig. 2.42).
• Normal color of tooth • Fill the cavity with intermediate restorative material (IRM).
Radiographic examination • Obtain a bitewing radiograph (Fig. 2.42).
• Large carious lesion in close proximity to the pulp
• Normal lamina dura Second Visit (six to eight weeks later):
• Normal periodontal ligament space • Ask history of pain, obtain a radiograph.
• No interradicular or periapical radiolucency • Anesthetize the tooth and apply the rubber dam.
• Remove all temporary restorative material including
Contraindications calcium hydroxide with caution.
History • Observe the affected dentin; it should be dry, flaky and
• Sharp, penetrating pain that persists after withdrawal of easily removable.
stimulus • Observe the area of potential exposure; it should be white
• Prolonged spontaneous pain, particularly at night and may be soft.
Clinical examination • Do not disturb this predentin.
• Excessive tooth mobility • Irrigate with normal saline and dry with cotton pellets.
• Parulis in the gingiva approximating the roots of the tooth • Cover the entire floor with hard set calcium hydroxide
• Tooth discoloration • Place a suitable base and restore with a permanent
• No response to pulp testing techniques restorative material.
106 Essentials of Pediatric Oral Pathology

One-sitting procedure mended when a mechanical exposure occurs in a dry sterile

• Value of re-entry and re-excavation is questionable. field.98
• There is a potential risk of pulp exposure due to overzealous
Objectives
excavation.
• To seal the pulp against bacterial leakage, encourage the
• Leung et al105 and Fairborne et al106 observed that re-entry
pulp to wall off the exposure site by initiating dentin bridge
is not necessary as long as the tooth is asymptomatic and
formation.
a good seal is maintained.
• To maintain vitality of the pulp.
Indications
Clinical and radiographic criteria of success
• No signs or symptoms of degeneration of the pulpodentinal
• Restoration is intact.
organ.
• Tooth has no mobility.
• Field of operation should be completely aseptic.
• No sensitivity to percussion.
• Exposure should be
• No history of pain after treatment.
— Pin-point size.
• No radiographic evidence of abnormal root resorption.
— No observable hemorrhage.
• No radiographic evidence of radicular disease.
— Dentin at the periphery of the exposure should be
reparable and sound.
Histological Evaluation
— Exposure site should not be at a constricted area in the
• Given by Law and Lewis107
pulp.
1. Irritational dentin formation.
2. Intact zone of Weil. Procedure of direct pulp capping
3. Slight hyperactive pulp with inflammatory cells. • Anesthetize the tooth and apply the rubber dam.
• Establish the outline form.
Conclusion • Remove caries and make a conventional cavity preparation
From these reports (Table 2.18), it appeared that gross caries (which has resulted in a pinpoint exposure).
removal, together with a palliative dressing and proper sealing • Gently clean the preparation with H2O2 (not recommended
of the cavity, will arrest the carious process by eliminating by many investigators).
substrate and bacterial action. • Evaluate quality of hemorrhage and make sure bleeding
stops quickly.
Direct Pulp Capping • Place Calcium Hydroxide (Dycal) directly on exposure.
• Place appropriate base and final restoration.
• Fuks AB: Direct pulp capping involves the placement of a
biocompatible agent on healthy pulp tissue that has been Clinical success is determined by:
inadvertently exposed by caries excavation or a traumatic • Maintenance of pulp vitality.
injury.108 • Absence of pain.
• Direct pulp capping may be defined as the treatment of an • Absence of abnormal radiographic signs
exposure of the dental pulp caused by an accident or in the • Dentin bridge formation.
course of excavating deep caries. It is generally recom- Jeppersen, in a long-term study using a creamy mix of
calcium hydroxide, placed on exposed pulps of primary teeth,
reported a 97.6 percent clinical success and 88.4 percent
TABLE 2.18: Success rate of indirect pulp capping histologic success.109
Sr. Investigators Cases Period Success % Are primary teeth good candidates for direct pulp capping?
No. • DPC in primary teeth has been viewed with skepticism.
1. Sowden,1956 4,000 Up to 7 yr “Very high” • The following are the limitations of direct pulp capping in
primary teeth:
2. Hawes and DiMaggio, 1964 475 Up to 4 yr 97
— Internal resorption.
3. Jordan and Suzuki, 1971 243 10-12 wk 98 — Calcification.
4. Nordstrom et al 1974 64 94 days 84 — Chronic pulp inflammation.
— Necrosis.
5. Nirschl and Avery, 1983 83 6 months 94
— Interradicular involvement.
Caries in Children 107

CALCIUM HYDROXIDE
Calcium hydroxide Table 2.19.
TABLE 2.19: Antibacterial effect of setting
calcium hydroxide paste materials
Sr. Strong effect Medium effect No effect
No.

1. Dycal Dycal MPC Kerr, Romulus,

(Original formula) (New formula) Michigan, USA

2. Reocap Vivadent, Life Kerr, Romulus, Hydrex

Schaan/ Michigan, USA
Liechtenstein

3. Procal Renew SS White Cal- Mer

Co./Dentomax, Experimental
Bradford, UK cement devised by
Laboratory of
Government
FIGURE 2.43: Schematic representation of mechanism
Chemist, Cornwall
of action of Ca(OH)2 cement
house, Stanford St,
London, UK
and coworkers have shown that the pH of untreated teeth with
4. Reolit Vivadent, pulpal necrosis lies between 6.0 and 7.4.113 Teeth with complete
Schaan/ root formation have a pH between 7.4 and 9.6 in the dentin
Liechtenstein
away from the pulp and from 8.0 to 11.1 in the dentin next to
the pulp. These investigators mentioned that the Ca(OH)2
Non-setting Calcium Hydroxide (Table 2.20) neutralizes the acids produced by osteoclasts. Anthony and
coworkers concluded that the alkaline environment would favor
TABLE 2.20: Various non-setting calcium the formation of calcium–phosphate [Ca(PO)4] complexes,
hydroxide materials used which would in turn; serve as a nidus for further calcification.114
Material Vehicle Javelet and coworkers also indicated that the alkalinity is a
significant feature in the ability of Ca(OH)2 to induce hard
Analar Ca(OH)2 Water
tissue formation.115 Other investigators contended that Ca(OH)2
Pulpdent (Pulpdent Corp. of America) Methyl cellulose creates an unfavorable environment for osteoclastic activity or
can activate alkaline phosphatase enzymes. The latter have been
Hypo-Cal (Ellman Dental Methyl cellulose
Manufacturing Co. Int., USA suggested as playing a role in hard tissue formation. It has been
shown that Ca(OH)2 by itself and as a component of root
Reogan Methyl cellulose canal sealers can induce calcification even in a soft tissue
environment.
Role of Calcium Hydroxide (Fig. 2.43) When preparing the Ca(OH) 2 paste using anesthetic
solutions, Stamos and coworkers determined that such a small
Some investigators have proposed that Ca (OH)2 stimulates amount of liquid is used that no appreciable change occurs in
undifferentiated mesenchymal cells to differentiate into the pH.116 Mitchell and Shankwalker concluded that it is
cementoblasts, which in turn initiate cementogenesis at the difficult to ascribe any importance to the pH of the material
apex. Dylewski observed proliferating connective tissue in the when the osteogenic potential is considered. 117 They
apical region that differentiated into calcific material, which rationalized that the use of other materials that have relatively
became continuous with the predentin at the apex.110 Schroder high pH values do not result in consistent had tissue formation.
and Granath mentioned that this calcification process occurs Mc Cormick and coworkers118 mentioned that the reliance
beneath a superficial layer of necrosis.111 They considered that on medication alone to accomplish a pH rise in the periapex
the necrosis was related to the pH of the Ca(OH)2. as proposed by Van Hassell and Natkin,119 is unrealistic. They
Heithersay considered that alkalinity of the material can believed that the other procedures are of considerable
act as a buffer to acidic inflammatory reactions.112 As a result, importance in gaining continued apical development. These
the Ca(OH)2 has a favorable effect on bone healing. Tronstad procedures include:
108 Essentials of Pediatric Oral Pathology

• Adequate preparation of the root canal Alternative Agents to Calcium Hydroxide Suggested for
• Removal of necrotic tissue and substrate Direct Pulp Capping in Primary and Permanent Teeth
• Reduction of microbes (both in terms of numbers and
Zinc Oxide–Eugenol cement: Glass and Zander found that
virulence)
ZOE, in direct contact with the pulp tissue, produced chronic
• A decrease in root canal space.
inflammation, a lack of calcific barrier, and an end result of
Frank concluded that the reduction of contaminates in the
necrosis.125 In spite of the reported lack of success with ZOE
root canal and filling of the root canal space with a temporary
cement, Sveen reported 87 percent success with the capping
resorbable material are more important than which material is
of primary teeth with ZOE in ideal situations of pulp
used.120
exposure.126
Nyborg and other investigators thought that pulpal reactions
to Ca(OH)2 depended on the hydroxyl ion rather than the Corticosteroids and antibiotics: Corticosteroids and/or
calcium ion.121 Nevertheless, in one study, in which magnesium antibiotics were suggested for direct pulp capping in the
hydroxide was used in fourteen apexification procedures, the pretreatment phase and also to be mixed in with calcium
results showed four mild and ten severe inflammatory reactions. hydroxide with the thought of reducing or preventing pulp
Contradictory results were found when magnesium hydroxide inflammation. These agents included neomycin and
was implanted subdermally in rats. Magnesium hydroxide was hydrocortisone,127 Cleocin,128 cortisone,129 Ledermix (calcium
found to have less of an inflammatory reaction than has hydroxide plus prednisolone), 130 penicillin, 131 and Keflin
Ca(OH) 2. In one investigation, barium hydroxide was (cephalothin sodium).132 Although many of these combinations
substituted for Ca(OH)2. Observations from this study included reduced pain for the most part, they were found only to
a lack of bridge formation, a severe foreign body reaction, and preserve chronic inflammation and/or reduce reparative
an acute and chronic inflammation with epithelial proliferation. dentin. Gardner et al found, however, that vancomycin, in
The calcium ion was also thought to be necessary to combination with calcium hydroxide, was somewhat more
decrease capillary leakage and constrict the pre-capillary effective than calcium hydroxide used alone and stimulated a
sphincters. In addition, the calcium ion can also affect the more regular reparative dentin bridge.133
enzyme pyrophosphatase, which is calcium dependent.
Polycarboxylate cements: Negm et al placed calcium
Pyrophosphate is involved in collagen synthesis and therefore
hydroxide and zinc oxide into a 42 percent aqueous
stimulation of this enzyme can increase the defense and repair
polyacrylic acid and used this combination for direct pulp
mechanism.
exposure in patients from 10 to 45 years of age. This mixture
Nevins and coworkers used Ca(PO4) gel to produce
showed faster dentin bridging over the exposures in 88 to 91
mineralized scar around the orifices of polyethylene tubes that
percent of the patients when compared to Dycal as the
were placed subcutaneously in the rats.122 When placed in
control.134
pulp-less open apex teeth in monkeys, the gel resulted in
connective tissue ingrowth. Nevins et al122 postulated that this Inert materials: Inert materials such as isobutyl cyanoacrylate135
could be a step in revitalization of the teeth. In other studies, and tricalcium phosphate ceramic136 have also been investigated
the Nevins group also reported the successful use of collagen as direct pulp-capping materials. Although pulpal responses in
Ca(PO4) gel. In contrast Citrome and coworkers found that the form of reduced inflammation and unpredictable dentin
in dogs the collagen Ca(PO4) gel inhibited the reparative bridging were found, to date, none of these materials have been
process of the initial inflammatory lesion, leading to extensive promoted to the dental profession as a viable technique.
destruction of the periapical tissues. 123 No evidence of
Collagen fibers: Because collagen fibers are known to
apexification was found. They also reported that collagen Ca–
influence mineralization, Dick and Carmichael placed
(PO4) also failed to assist in resolution of instrumentation
modified wet collagen sponges with reduced antigenicity in
trauma and inhibited the repair process. Teeth treated with
pulp-exposed teeth of young dogs.137 Although, the material
Ca(OH)2 in the same animals showed an acceleration of the
was found to be relatively less irritating than calcium hydroxide,
repair process.
and with minimal dentin bridging in eight weeks, it was
Finally, whether or not a complete apical barrier results
concluded that collagen was not as effective in promoting a
from apexification techniques is uncertain. Lieberman and
dentin bridge as was calcium hydroxide.
Trowbridge have shown that even with radiographic and
clinical evidence of a hard tissue barrier, histologic examination Formocresol: Because of the clinical success of formocresol
shows that this barrier is porous.124 Nevertheless, for clinical when used in primary pulp therapy such as pulpotomies and
success, it may not be necessary to have an impermeable hard pulpectomies, several investigators have been intrigued by the
tissue barrier. possibility of its use as a medicament in direct pulp-capping
Caries in Children 109

therapy. Arnold applied full-strength formocresol for 2 minutes PULPECTOMY

over enlarged pulp exposures in primary teeth and found a 97%
Rationale
clinical “success” after 6 months.138
Successful pulp therapy for primary teeth is one of the most
Hybridizing bonding agents: Recent evidence has shown that
valuable services a child patient can receive, since there is
elimination of bacterial microleakage is the most significant
no better space maintainer than the retained primary tooth.
factor affecting restorative material biocompatibility. 139
The practitioner must be aware of the dangers of retaining
Currently, hybridizing dentinal bonding agents (such as
untreated, carious primary molars. A carious molar left
AmalgamBond or C and B MetaBond, Parkell Products, untreated merely invites chronic infection, which may at any
Farmingdale, N.Y.) represent the state of the art in mechanical time become an acute alveolar abscess. The possibility of
adhesion to dentin with resultant microleakage control beneath hypoplasia or hypocalcification in the underlying teeth is also
restorations.140 Miyakoshi et al have shown the effectiveness increased. Deflection of the eruption pathway is also possible.
of 4-META-MMA-TBB adhesives in obtaining an effective Orthodontic implications of premature loss of primary teeth
biologic seal.141 Cox et al demonstrated that pulps sealed with are many.
4-META “showed reparative dentin deposition without
subjacent pulp pathosis.”142 Objectives
Cell-inductive agents: A number of cell-inductive agents have • Successful treatment of a cariously involved pulp.
been proposed as potential direct pulp-capping alternatives to • Maintaining the tooth in the oral cavity in a non pathogenic
calcium hydroxide. These contemporary substances mimic the and healthy condition.
reciprocal inductive activities seen in embryologic development • Endodontically treated teeth should help in mastication and
and tissue healing that are receiving so much attention at this serve as natural space maintainers.
time. • Their retention helps in speech.
• Mineral trioxide aggregate (MTA) (Dentsply, Tulsa; Tulsa, • Help in guidance to the developing dentition.
Okla): MTA was developed at Loma Linda by Torabinejad
for the purposes of root-end filling and furcation perforation Anatomic Differences between Primary and
repair. 143 The material consists of tricalcium silicate, Permanent Teeth
tricalcium aluminate, tricalcium oxide, and silicate oxide. • Pulp chamber anatomy in primary teeth approximates the
It is a hydrophilic material that has a 3-hour setting time in surface shape of the crown more closely than in permanent
the presence of moisture. Major MTA advantages include teeth.
excellent sealing ability, good compressive strength • The pulps of primary teeth are proportionately larger and
(70 MPa) comparable to IRM, and good biocompatibility. the pulp horns extend closer to the outer surfaces of the
Pitt Ford et al documented superior bridge formation and cusps than in permanent teeth.
preservation of pulp vitality with MTA when compared with • The dentin thickness is less than in permanent teeth.
calcium hydroxide in a direct pulp-capping technique.143 • Increased numbers of accessory canals and foramina, as well
They also reported normal cytokine activity in bone and as porosity of pulpal floors (Hibbard and Ireland, 1957).146
cementum regeneration in response to MTA, which is • Roots are longer and more slender.
indicative of its cell-inductive potential.144 • Canals are more ribbon like with multiple pulp filaments
• Calcium phosphate cement: It has been developed for within the more numerous accessory canals.
repairing cranial defects following brain neurosurgery. The • Roots flare outwards to accommodate the succedaneous
components of this material include tetracalcium phosphate tooth bud.
and dicalcium phosphate, which react in an aqueous • Roots tend to undergo physiologic root resorption as soon
environment to form hydroxyapatite, the mineral compo- as root completion occurs, therefore, always in a state of flux.
nent of hard tissues. In contrast to calcium hydroxide,
tetracalcium phosphate cement induced bridge formation Histologically
with no superficial tissue necrosis and significant absence • Increased coronal cellularity and apical vascularity,
of pulp inflammation.145 therefore, increased healing capabilities.
• Fox and Heeley, 1980—No differences structurally between
PULPOTOMY
primary pulp tissue and young permanent pulp tissue with
The procedure has been described in detail in the section on the exception of a cap like zone of reticular and collagenous
apexogenesis/apexification. fibres in the primary coronal pulp.147
110 Essentials of Pediatric Oral Pathology

• Clinicians, however, have noted a difference in the pulp

responses between the two.
• Primary teeth, generally, demonstrate an inflammatory
response as opposed to the calcific scarring of permanent
teeth.
• Bernick, 1959, found that in permanent teeth, pulp nerve
fibres terminate among odontoblasts and even beyond the
predentin. In primary teeth, they terminate in the
odontoblastic layer.148
• Also Rapp et al—Density of the innervation of primary
teeth less than that of permanent teeth.149
• Neural tissue is the first to degenerate when root resorption
begins and the last to mature when the pulp develops.
• McDonald, 1956, reported that the localization of infection
and inflammation is poorer in the primary pulp than that
of permanent teeth.150 FIGURE 2.44: Preoperative radiograph of a deciduous
mandibular second molar indicated for pulpectomy
Partial Pulpectomy
This procedure is called partial pulpectomy as complete Contraindications
negotiation of accessory canals is generally not achievable in • Teeth with non restorable crowns
primary teeth. • Periradicular involvement extending to the permanent tooth
bud
Indications of pulpectomy • Pathologic resorption of >1/3rd root with a fistulous sinus
• History of spontaneous pain at night. tract
• Intraoral swelling/sinus.
• Excessive internal resorption
• History of extraoral swelling.
• Extensive pulp floor opening into the bifurcation
• Extensive bleeding from amputation site of radicular pulp
• Primary teeth with underlying dentigerous or follicular cysts
tissue.
• Young patients with systemic illness like congenital or
• Moderate mobility.
rheumatic heart disease, hepatitis, leukemia, children on
• Clinically evident carious exposure.
long term steroid therapy or immunocompromised.
• Primary teeth with inflammation spreading beyond coronal
pulp but no pathologic resorption of roots and alveolar bone. Historical perspective
• Primary teeth with necrotic pulps, minimum root resorption • Sweet, 1930—introduced the 4 to 5 step technique using
and minimum bony destruction in the bifurcation area. formocresol for pulpless teeth with or without fistulae.151
• Pulpless primary teeth: • Rabinowitz, 1953—recommended 4 to 17 visits comprising
— Without permanent successor 2 to 3 day application of formocresol in nonvital primary
— In hemophiliacs molars followed by precipitation of silver nitrate and a
— Before eruption of 1st permanent molar sealer of ZOE cement into the canals.152
— When speech, crowded arches or esthetics are a factor • Gould, 1970—worked upon primary molars in 3½ to 8½
— Next to the line of a palatal cleft year children. CMCP was placed in the pulp chamber for
— Supporting orthodontic appliances 5 minutes. ZOE was placed in the canals. 83% clinical and
— When arch length is deficient radiographic success was reported.156
— When space maintainers or continued supervision is not • Hobson, 1970—experimented with necrotic primary teeth
feasible (handicapped/isolated children) whose canals were not debrided. Beechwood creosote was
Radiographic indications (Fig. 2.44) used for 2 weeks, then pulp chamber was filled with
• Deep carious lesions extending upto the pulp confirmed ZOE.153
by clinical evaluation • Vander Wall et al, 1972—stated that formocresol is more
• Interradicular radiolucency evident on radiographs effective than CMCP or Cresatin as a root canal medica-
• Pathologic root resorption not involving more than 1/3rd ment for inhibiting bacterial growth.160
root • Lewis and Law, 1973—practised conventional endodontics
• Minimal destruction of bone support with sodium hypochlorite irrigation. Canals were medicated
Caries in Children 111

for 3 to 7 days with eugenol, camphorated parachlorophenol Procedure

or formocresol. During the second visit, canals were filled
• Adequate anesthesia is given.
with ZOE or ZOE mixed with iodoform crystals.154
• Rubber dam is applied.
• Frigoletto, 1973—stated that in asymptomatic necrotic • Dental caries removed completely.
primary teeth, canals should be debrided and irrigated with • Enamel overhanging the coronal pulp removed for adequate
sodium hypochlorite. Canals were filled with root canal access.
paste using a specially designed pressure syringe.157 • Access cavity completed.
• Starkey, 1980—recommended a one appointment method • Coronal pulp amputated upto the root canal orifice using a
for vital pulp—partial pulpectomy removing coronal aspect spoon excavator (e.g. API, Germany).
of the radicular pulp. He recommended a multiappointment • Radicular pulp is removed with a fine barbed broach.
method for necrotic pulps and periradicular involvement. • H file or barbed broach for removal of pulp tissue remnants.
Formocresol or CMCP was sealed with IRM for one week. • Cleaning and shaping of canals preferably with K files as
Then canals are filled with ZOE.158 H files tend to fracture easily.
• Coll et al 1985—reported an 80 to 86 percent success rate • Working length (Fig. 2.45)
with the one sitting technique.159 — (1 to 1.5 mm)—Damle, 2006164
• Barr et al 1986—Observation period—40.2 months — (2 to 3 mm)—Dummett and Kopel165 short of the radio-
(approx. 3½ years)—85.5 percent success rate of primary graphic apex.
molar pulpectomies. 88 percent show complete ZOE paste • Starting with a small sized file (#10), proceed in a
resorption. 25.8 percent reduction of preoperative sequential manner to a larger size (#35).
radiolucencies.161 • Care should be taken that instruments do not extend
• Flaitz et al 1989—Observation period —37 months— 68.5 periapically.
percent successful pulpotomized anterior teeth and 84 • Frequent irrigation is a must to allow debris to be flushed
percent successful pulpectomized anterior teeth.162 out.
• Yacobi and Kenny, 1991—Observation period—2 years— • Irrigation with distilled water, saline, three percent hydrogen
83 percent success rate for pulpectomized anterior teeth peroxide followed by 5.2 percent sodium hypochlorite.
and 90 percent success rate for pulpectomized posterior • Canals dried using absorbent points.
teeth, probably due to more microleakage from composites • Canals filled with ZOE paste using incremental technique.
Canals may be filled using a reamer and plugger
in anterior teeth.163
— Absorbent points or a jiffy tube
• Judd and Kenny, 1991—Recommended that H files
— A lentulospiral
(usually #20) be used for vital pulp extirpation and canals
— Pressure syringes (Pulpdent Corporation, America).
be filled with thin mix (toothpaste viscosity) of ZOE using
If pressure syringes are not available, a syringe with
a lentulospiral.155
a tuberculin needle can be used.
• IOPA taken to evaluate the filling (Fig. 2.46).
General Treatment Considerations • Restoration with miracle mix or amalgam followed by a
• Patient should be healthy and cooperative. In case of stainless steel crown.
systemic disorders compromising a child’s responses, the
child’s physician should be consulted.
• Informed consent should be obtained.

Dental Considerations
• Tooth must be restorable after root canal treatment.
• Chronologic and dental age must be evaluated to rule out
teeth with imminent exfoliation.
• Psychological or cosmetic factors must be considered (may
be more important to the parent).
• Number of teeth to be treated and strategic importance to
the developing occlusion must be evaluated.
• Primary molar root anatomy, along with proximity of the FIGURES 2.45A and B: Diagnostic instruments placed in the
underlying succedaneous tooth should be evaluated. canals
112 Essentials of Pediatric Oral Pathology

Iodoform Paste
Commonly available:
Kri paste
Iodoform 80.8 percent
Camphor 4.86 percent
Parachlorophenol 2.025 percent
Menthol 1.215 percent
Maisto paste
Zinc oxide 14 gm
Iodoform 42 gm
Thymol 2 gm
Chlorophenol camphor 3 cc
Lanolin 0.5 gm
FIGURE 2.46: Canals obturated with zinc oxide eugenol
Barker and Lockett, 1971—identified the potential benefits of
Kri paste, an iodoform compound.170 Advantages are long
ROOT FILLING MATERIALS lasting bactericidal properties, excellent resorbability and no
undesirable effects on succedaneous teeth when used as a pulp
Ideal Requirements canal medicament in abscessed primary teeth. It was noted that
• Should resorb at a similar rate as the primary root. this paste would resorb within two weeks if found in the
• Should be harmless to the periapical tissues and to the periradicular or furcation areas. An ingress of connective tissue
permanent tooth germ and should resorb readily if pressed was seen in the apical portions of the treated canals.
beyond the apex. Since iodoform paste does not set into a hard mass, it can
• Should have a stable disinfecting power. be removed if retreatment is required.
• Should be inserted easily into the canal and be removed Holan and Fuks, 1993—clinically and radiographically
easily if necessary. compared Kri paste with ZOE after 48 months. 84 percent
• Should adhere to the walls of the canal and should not shrink. success with the Kri paste group v/s 65 percent success with
• Should not be soluble in water. the ZOE group was found.171
• Should not discolor the tooth.
• Should be radiopaque. Garcia-Godoy, 1987—95.6 percent success clinically and
No material has been found till date which fulfills all of radiographically with Kri paste during a 24 month period for
these criteria. 43 teeth.172
Commonly used materials are:
• Zinc oxide eugenol Calcium Hydroxide
• Iodoform paste
• Calcium hydroxide Several clinical and histopathologic investigations of calcium
hydroxide and iodoform mixture have been published.
Zinc Oxide Eugenol Vitapex (Neo Dental Chemical Products Co, Tokyo),
Most commonly used filling material for primary teeth. Camp, Endoflas (Sanlon Laboratories, Columbia), Metapex are
1984,166 introduced endodontic pressure syringe to overcome available.
the problem of underfilling, although, reports mention that as Vitapex— Calcium hydroxide
far back as 1961, Greenberg developed the pressure syringe Iodoform
for filling primary canals and this technique was described in Oily additives
detail by Spedding, 1977 167 and Krakow et al, 1981.168 It is harmless, and any overfilled material resorbs. It has
Underfilling is frequently clinically acceptable, as been found to resorb at a slightly faster rate than the root.
overfilling may cause a mild foreign body reaction. It has antiseptic properties, is radiopaque and can be easily
Another disadvantage of ZOE is the difference between its removed in case of retreatment. It can be considered to be
rate of resorption and that of the tooth root. a nearly ideal primary tooth filling material. However, it is
Coll et al 1985, found that ZOE particle retention occurred not generally recommended in vital pulp therapy in primary
in 8 of 17 patients followed till the time of premolar eruption.169 teeth.
Caries in Children 113

Chawla et al 1998, have carried out a pilot study in the • Periapical surgery with beveling of the apex for adequate
mandibular primary molars using calcium hydroxide paste as reverse amalgam placement further compromises an already
a root canal filling material and found it to be a success.173 increased crown-root ratio.
Tandon S et al observed almost a 100 percent clinical • Arens, 1977, has pointed out that the cementum of
success in 10 endodontically treated primary molars which were underdeveloped root tips is so weak and flimsy that it
filled with Vitapex (Calcium hydroxidised iodoform).174 shatters like glass when it is contacted by a surgical bur.174
• Psychological trauma of a surgical procedure.
Gutta Percha
APEXOGENESIS
Since gutta percha is not a resorbable material, it is
contraindicated in the primary teeth. Definitions

Recall Weine, 1996—A pulpotomy procedure is indicated in the tooth

with an open apex to allow completion of apical closure as
• Rate of success is generally high. long as the apical pulp remains vital. This is known as
• Tooth should be periodically checked for normal resorption apexogenesis.176
without interfering with the eruption of the permanent tooth. Walton and Torabinejad, 1989—Apexogenesis is defined as
• The primary tooth should remain asymptomatic, firm in the a treatment of vital pulp by pulp capping or pulpotomy in order
alveolus and free from pathosis. If evidence of pathosis is to permit continued growth of root and continued closure of
detected, extraction and conventional space maintenance open apex.177
are recommended. Arens, 1977—Apexogenesis is defined as physiological root
• Occasionally, tooth may be overretained. Generally, large end development and formation.175
amount of ZOE in the pulp chamber may impair resorption.
Management consists of simple removal of the crown and Indications for Apexogenesis
allowing the permanent tooth to erupt.
• Reversible pulpitis from a deep carious lesion resulting in
Criteria for Success a mechanical exposure in a tooth with incompletely formed
root apex.
• Radiographically • Irreversible pulpitis in a case of traumatic pulp exposure
— No enlargement of periapical or furcation radiolucency or a deep carious lesion involving the pulp of a young
— No pathologic resorption permanent tooth with an incompletely formed root but
— Maintenance of normal PdL space and lamina dura having a vital apical portion of the pulp.
— No damage to crypt of succedaneous tooth.
• Clinically Contraindications for Apexogenesis
— Patient asymptomatic
— No extraoral/intraoral swelling/sinus (gumboil) • Tooth is sensitive to percussion or has a long history of
— No pathologic mobility pain indicates a long standing chronically inflamed pulp.
— No sensitivity on percussion/mastication. • Totally necrotic pulp or presence of sinus associated with
the infected tooth.
APEXOGENESIS AND APEXIFICATION
Conventional Pulpotomy with Calcium Hydroxide
Introduction
Technique
For many years, open apices, attributable to lack of maturation • The tooth is anesthetized and isolated with rubber dam.
or resorption, have been treated inadequately by root canal • A conventional access is prepared with a high speed bur
fillings or by periapical surgery with a reverse amalgam filling. under aseptic conditions, using an air water coolant to
This situation is most frequently encountered in children and minimize heat damage to the underlying pulp.
young adults. It is often best to treat immature teeth with a non- • Use of copious water spray and reduction of dentin in the
surgical approach because of the following reasons: floor of the access preparation to paper thinness before
• In the surgical procedure, there is increased potential for penetrating the pulp will minimize embedding of the
interference with adjacent vital structures as this situation dentinal debris in the remaining pulp tissues.
is most frequently encountered in children and young • After leaving paper thinness of the dentin, the roof of the
adults. pulp chamber is removed into its entirety with a sharp long
114 Essentials of Pediatric Oral Pathology

shank excavator in posterior teeth and a rotary instrument symptoms does not necessarily reflect the true state of the
in anterior teeth. pulp. In instances of pulp capping and high (partial)
• The cavity and the pulpal wound at its base are gently pulpotomies, pulp testing may aid in determining the pulp's
irrigated with sterile saline, sterile water or local anesthetic viability. Where a temporary crown has been placed, a small
solution for 2 to 3 minutes until hemorrhage ceases. This hole may be placed slightly incisally from the facial gingival
will help prevent an extrapulpal blood clot forming over margin to allow for pulp testing. However, in evaluating
the wound surface as this has been shown to interfere with most pulpotomies, radiographic interpretation of pulpal and
pulpal healing and detract from the prognosis. periapical pathosis or clinical symptoms of spontaneous
• It may be necessary to saturate a cotton pellete with irrigant pain, swelling, unusual discomfort from percussion and
and apply to the surface with gentle pressure to control palpation and/or sinus tract formation are the major means
bleeding. Racemic epinephrine or any caustic chemical or of suggesting the status of the pulp. Caution should be
medicament should not be used because the objective is to observed in interpreting the normal radiographic
maintain the health of the remaining pulp. radiolucency of the root sheath as periapical involvement.
• A suitable medicament or dressing (Calcium hydroxide) is It has been suggested by Camp, that on numerous
placed on the remaining tissue in an attempt to promote occasions, the electric pulp tester gave no response when
healing and retention of this vital tissue. Calcium hydroxide testing uninvolved, incompletely developed permanent
hard setting pastes (commercially available as Life, Dycal, teeth.178 Therefore, only a positive response is usually
Pulpdent, etc.) are applied to the amputation site, if the considered as valid.
amputation site is at the cervical line of the tooth. However,
for deeper amputation, calcium hydroxide powder carried Shallow/Partial Pulpotomy with Calcium Hydroxide
to the tooth in an amalgam carrier is the easiest method of The shallow pulpotomy is the treatment of choice for impact
application. trauma exposure on an anterior tooth. Cvek has reported that
• A layer of intermediate restorative material (IRM) is placed pulp capping and partial pulpotomy with calcium hydroxide
over the calcium hydroxide. on traumatically exposed pulps are successful 96 percent of
• A permanent restoration is essential to the success of the the time.179
apexogenesis procedure as temporary fillings invariably The major advantages compared with total pulpotomy
washout and leak over a period of time, resulting in operation are:
bacterial contamination, often causing death of the pulp. • It conserves tooth structure
• A radiograph of the tooth is taken to serve as a basis for • It permits continued formation of dentin coronally
comparison with later films. • It is simpler to perform
Recall procedure Technique
• Includes the clinicoradiographic evaluation of the tooth. • The tooth is anesthetized and isolated with rubber dam.
• The total time taken for achievements of the goals of the • The exposed dentin is washed with a weak solution of
apexogenesis pulpotomy ranges between 1 and 2 years, sodium hypochlorite (or saline or anesthetic solution).
depending primarily upon the extent of tooth development • The granulation tissue from the site of pulp exposure is
at the time of pulpotomy procedure. removed.
• The apexogenesis procedure differs from the apexification • Gently and gradually, wipe away pulp tissue with a rotating,
treatment in that the paste is not to be changed in the former water-cooled round diamond bur in a high speed handpiece.
whereas the paste may need to be changed numerous times Begin at the site of exposure and proceed to a depth of
in the latter. 2 mm but avoid the cervical level of pulp, if possible. The
• The patient should be recalled at a minimum of three month pulp in that area is important for dentin production, which
intervals after apexogenesis therapy in order to determine will help strengthen the tooth and help prevent future
the vitality of the pulp and the extent of apical maturation. cervical crown fracture.
• It is important to remember that the formation of the • Gently wash the wound with saline and place a moist cotton
calcification is not always indicative of the success of the pellet on the wound until bleeding stops.
treatment and that, in some cases, apical maturation occurs • Dress the wound with a hard setting calcium hydroxide
in the absence of the calcific barrier. It is important not to liner. Also cover the exposed dentin with the same liner.
allow the calcific barrier to enlarge in size and partially or • Place a thin mix of hard setting zinc phosphate cement over
totally obliterate the root canal, because it would then be the liner.
difficult to gain access to the root canal and would impede • When the cement has set, the tooth can be restored with
endodontic treatment at a later date. An absence of acid etched composite.
Caries in Children 115

• Clinico-radiographic examination on recall should be Technique

followed diligently. • Anesthetize the tooth and isolate it using rubber dam (Fig.
2.47).
Prognosis: The prognosis of partial pulpotomy is uncertain
• Place a no. 330 bur in a high speed handpiece and gain
because of the difficulty of diagnosing the state of the pulp
occlusal access to the pulp. It is better to make too large
and of maintaining the temporary restoration in some cases.
an opening than one that is too small. Remove all
However, partial pulpotomy gives better results than a direct
overhanging enamel.
pulp capping.
• Using a sterile no. 4 or no. 8 round bur (slow speed), remove
all carious dentin, if possible, before exposing the pulp horns.
Formocresol Pulpotomy
• With a slow or high speed bur connect the pulp horns and
Formalin containing compounds have been used in pulp therapy expose the pulp.
since the early days of the twentieth century. Formocresol was • Excise the pulp tissue to the orifices of the root canals.
introduced in 1904 by Buckley, who contended that equal parts • Use a large spoon excavator to remove any remaining
of formalin and tricresol would react chemically with the pulpal tissue. Gently wash out the debris with a water
intermediate and end products of pulp inflammation to form a syringe (Fig. 2.48).
new, colorless and non-infective compound of harmless • Evaluate the hemorrhage. A vital pulp with minimal chronic
nature.180 The formocresol pulpotomy technique used today is inflammation should achieve hemostasis in 3 to 5 min. If
a modification of the original method reported by Sweet in there is chronic hemorrhage, check for remaining pulpal
1930. 181 This formula of Buckley consists of 35 percent tissue in the pulp chamber. If there is purulent exudate,
tricresol, 19 percent formaldehyde, 15 percent water and fibrotic pulp or uncontrollable hemorrhage, consider
glycerine. alternative pulp procedures.
• Place a sterile cotton pellet moistened (not saturated) with
Mechanism of action
20 percent dilution formocresol on the exposed pulp
• Formocresol is an efficient bactericide.
stumps. Place a dry pellet over the first pellet to maximize
• It was also found to have the ability to prevent autolysis of
contact with the pulpal tissue and leave for 2 min (Fig.
tissue by the complex chemical binding of formaldehyde
2.49).
with protein which may be reversible.
• On removal, the pulp stump should appear blackish brown.
• Massler and Mansukhani, 1959, conducted a detailed
If there is bleeding, check for residual pulpal tissue.
histologic investigation of the effect of formocresol on
Reapply formocresol for 2 min.
human pulps of primary and permanent teeth.182 Fixation
• Fill the chamber to about half its volume with the thick
of the tissue directly under medicament was apparent
mixture of zinc oxide eugenol. Some clinicians advocate
shortly after application (7 to 14 days). The pulps developed
adding equal drops of formocresol to the eugenol, then
three distinctive zones:
mixing with zinc oxide powder.
— A broad eosinophilic zone of fixation
— A broad pale staining zone with poor cellular definition
— A zone of inflammation diffusing apically into normal
pulpal tissue.
• Formocresol pulpotomy was earlier regarded as non-vital
or mummification method but subsequent clinical and
histologic studies have raised a doubt about labeling the
method as non-vital. Quiet a few investigators term the
technique as vital (upto 5 min) or non-vital (3 days)
according to the length of time of the application.
• The formocresol pulpotomy procedure has appeal because
there is a lack of calcification of the remaining pulp tissue
as is sometimes seen with calcium hydroxide pulpotomy.
• Hence, even though the calcium hydroxide pulpotomy has
been recommended as the treatment of choice in the
pulpally involved permanent tooth, evidence of continued
apical development following formocresol pulpotomy
procedures on young permanent teeth with incompletely FIGURE 2.47: Preoperative view of a deciduous mandibular
developed apices has been reported. second molar indicated for pulpotomy
116 Essentials of Pediatric Oral Pathology

FIGURE 2.50: Radiographic view of formocresol pulpotomy after

placement of stainless steel crown

development of the roots of an incompletely formed tooth in

FIGURE 2.48: Bleeding points seen after which the pulp is no longer vital.
removal of coronal pulp Nicholls, 1992—Apexification is a method of inducing
apical closure through formation of mineralized tissue in the
apical pulp region of a non-vital pulp with an incompletely
formed apex.

Technique
First appointment
• In all patients in whom acute clinical symptoms are present,
emergency endodontic procedures should be carried out
before starting root induction. Once the patient is
asymptomatic, the following treatment sequence can be
used.
• Anesthetize the tooth and isolate using the rubber dam.
• Establish a conventional access.
• Debride the coronal two thirds of the canal with broaches
and large Hedstrom files.
FIGURE 2.49: Pulp stumps seen after • Irrigate the canal thoroughly with an acceptable endodontic
application of formocresol irrigant such as 2.5 percent sodium hypochlorite.
• Dry the canal that is approached with files, using large
• A permanent restoration of amalgam or stainless steel crown sterile absorbent points.
is of choice (Fig. 2.50). • Medicate the canal with camphorated monochlorophenol
Other materials used for pulpotomy are cresatin, Ledermix, (CMCP).
glutaraldehyde. • Temporarily seal the chamber with Cavit.
Second appointment
APEXIFICATION • Place a rubber dam and remove the temporary filling.
• Irrigate the canal thoroughly.
Definitions
• Establish the working length. Since the canal walls in the
American Association of Endodontists, 1981—Apexification apical region may be paper thin, there is probably some
is a method of inducing apical closure by the formation of advantage to establishing the working length approximately
osteocementum or a similar hard tissue or the continued apical 2 mm coronal to the most apical root edge. By working at
Caries in Children 117

this slightly coronal level, there is less likelihood that the

thin apical root structure will be torn by files. By restricting
filing to within the root canal, there is also less likelihood
that periapical tissue that may still have the potential to
participate in further root development will be damaged;
hence, additional root structure may form apical to the level
to which one ultimately places calcium hydroxide.
• Instrument the canal; large sized (# 70 to #90) Hedstrom
files) are recommended. Instrumentation in these instances
should be thought of as planing of all walls of the canal
without an attempt to increase the size of the canal.
• Dry the canal with coarse sterile paper points, held at the
working length so that the point does not go beyond the
canal.
• Medicate the canal with CMCP. FIGURES 2.51A and B: Radiographic appearance of apexification
• The access cavity is then sealed with Cavit. after placement of calcium hydroxide in a maxillary central incisor
and after obturation with gutta-percha
Third appointment
• Place the rubber dam and remove the Cavit and medicated have the radiodensity of conventional root canal filling
pellet. materials, you should begin to lose the clarity of the canal
• Freshen the canal walls with the same size file used at the outline when the material has been properly condensed
second appointment, and then thoroughly irrigate the canal (Figs 2.51A and B).
again to remove debris. • Excess paste should be removed from the chamber with a
• Dry the canal with absorbent points. Some bleeding may spoon excavator and a suitable intermediate restorative
be encountered due to ingrowth of perapical inflammatory material should be placed in the access cavity.
tissue. This may be controlled by irrigation and assuring • Another technique for the paste placement by Teplitsky,
that the paper points do not go beyond the working length. 1986, using the compactor, as originally used by
Slight bleeding that continues to dampen the apical canals McSpadden for canal filling has been described.183
should not, however, be of concern.
• Prepare the calcium hydroxide paste on a sterile glass slab, Refilling Procedure
mixing USP calcium hydroxide powder with camphorated
• For a more predictable result, the calcium hydroxide would
monochlorophenol. When mixed to proper consistency, the
be changed routinely at the first 6-week observation visit.
mass should have putty like thickness and should hold its
• Holland et al, 1979, demonstrated better results with the
shape when mounted on the slab. The paste can be carried
change of paste. They speculated that the refilling process
into the canal with a sterile amalgam carrier. Alternatively,
caused contact with a developed connective tissue rather
special syringes can be used to introduce the calcium paste
than with the blood clot, whereas previous researchers
into the canal.
demonstrated unsuccessful cases when a blood clot was
• Begin moving the paste into the apical canal with the long
present between the calcium hydroxide and vital pulp
plugger that can be introduced to within 2 to 3 mm of the
tissue.184
working length without binding on any of the canal walls.
• The paste is removed with an endodontic instrument
Although material that is forced beyond the canal into
approximately half the diameter of the canal with a reaming
periapical tissue will generally resorb, it is desirable to
motion followed subsequently by copious irrigation with
attempt to confine the calcium hydroxide paste to the root
sterile water or saline.
canal.
• Often the incisal half of paste filling is dry but the apical
• Then, additional paste can be placed in the canal and the
half is wet (mushy).
condensation procedure repeated. As the canal begins to
• After drying the canal a new calcium hydroxide paste is
fill it may be helpful to use a larger size plugger than the
vertically condensed exercising care to confine the paste
one you used for the initial condensation. Although pluggers
to the canal system.
work well, some operators prefer to tamp the paste in place
with the butt ends of large sized paper points. Recall
• A radiograph should be taken to assess the quality of the • Ultimately, a bridge of hard tissue in the apical 1 to 2 mm
obturation. Although the calcium hydroxide paste does not is desired in apexification therapy.
118 Essentials of Pediatric Oral Pathology

• As a general rule, the patient should be recalled 6 weeks — Confirmation of a calcific deposit by light finger
following the second paste placement and approximately pressure with smaller files.
2 to 3 months thereafter until a calcified deposition is — Drying of the canal system with paper points eliciting
complete. no hemorrhage or tissue fluids.
• The total time of treatment averages 12 to 18 months but • Obturation of the root canal system with gutta-percha can
may vary from a few months to a couple of years, be accomplished successfully with either of the following
depending primarily on the status of the root closure at the techniques:
time of initial treatment. — The Warm (Vertical) Gutta-percha technique (Schilder,
• If there appears to be a dilution of paste in the canal (e.g. 1983)—the large canal system and the blunderbuss apex
it becomes more radiolucent) when checked on recall, the in many of these teeth may indicate that the incremental
calcium hydroxide should be changed. warm gutta-percha technique is more desirable.185
• At six months, the patient is recalled for radiographic — The Lateral Condensation technique—As many of these
examination again. One of the following five apical canals are irregular and larger than the largest size gutta-
conditions will be found: percha cone available (140), a customized gutta percha
— No radiographic change is apparent but if an instrument cone will need to be fabricated. Ingle, 1976, suggests
is inserted, a blockage at the apex will be encountered. rolling two or more gutta-percha cones together
— Radiographic evidence of a calcified material is seen between a cool glass slab and one that is heated to create
at or near the apex. In some cases degree of calcification the desired size and shape of the master cone.186
might be extremely extensive. • Because the calcific deposit is of porous nature, extrusion
— Apex closes without any change in the canal space. of the root canal cement may be seen as a result of proper
— Apex continues to develop with closure of the canal. condensation pressure applied to the gutta-percha filling.
— No radiographic evidence of change is seen and clinical • However, the bridging will confine the gutta-percha to the
symptoms and/or development of periapical lesion root canal system.
occur. • As with all endodontic fillings, the patient should be
recalled at 6 and 12 month intervals for reevaluation.
Final filling of the root canal system
• Remove the calcium hydroxide paste and irrigate the canals.
• Instrumentations should extend only till the calcific barrier REFERENCES
which may be 1 to 2 mm short of the initial treatment length 1. Bowen WH, Sylvia PK, Rosalen PL, Miguel JO, Shih AY.
because of the thickness of the barrier. Assessing the cariogenic potential of some infant formulas, milk
• Canal may be enlarged by two instrument sizes to remove and sugar solutions. JADA 1997;128:865-71.
calcium hydroxide remnants, critically judging this step 2. Sivapathasundharam B, Raghu AR. Chapter 9: Dental caries.
with the relative thinness of dentinal walls of immature In: Rajendran, Sivapathasundharam Eds. Shafer’s Textbook of
teeth. oral pathology. 5th edn, Elsevier, 2007;567.
• The final determination of readiness to obturate with gutta- 3. Newbrun E. Chapter 1: History and early theories of the etiology
percha is predicted on the following criteria: of caries. In: Newbrun E (Ed). Cariology. Quintessence Books,
3rd edn, 1989;13.
— Symptomless tooth with healing of any previous sinus
4. Adair SM. Chapter 12: The dynamics of change: Epidemiology
tract or fistula.
and mechanisms of dental disease. In: Pinkham JR,
— Radiographic observation of osseous deposition in the
Casamassimo P, McTigue DJ, Fields HW, Nowak AJ (Eds).
periapical or lateral defect, if originally present. Often Pediatric dentistry. Infancy through adolescence. Saunders, an
the calcific bridging does not occur until after the imprint of Elsevier, 4th edn, 2005;199.
osteogenic healing which provides a matrix for hard 5. Newbrun E. Chapter 1: History and early theories of the etiology
tissue deposition. of caries. In: Newbrun E (Ed). Cariology. Quintessence Books,
— Radiographic observation of hard tissue deposition at 3rd edn, 1989;13-28.
the apex, barium sulfate is often incorporated into this 6. Jenkins GN. A critique of the proteolysis—chelation theory of
apical bridging, mimicking the paste filling. caries. Br Dent J 1961;111:311-30.
— Finding the calcium hydroxide paste to be dry when 7. Bachrach FH, Young M. A comparison of the degree of
tested by probing with an endodontic instrument or resemblance in dental characters shown in pairs of twins of
explorer, suggesting that there is minimal fluid identical and fraternal types. Brit DJ 1927;48:1293-1304.
exchange between the root canal system and the 8. Goldberg S. The dental arches of identical twins. Dental Cosmos
periodontium. 1930;72:869-81.
Caries in Children 119

9. Mansbridge JN. Heredity and dental caries. J Dent Res 1959; with nursing bottle caries, rampant caries, healthy children with
38:337-47. 3-5 dmft/DMFT and healthy caries free children. JISPPD 2002;
10. Lehner T, Lamb JR, Welsh KL, Batchelor RJ. Association 20(1):1-5.
between HLA-DR antigens and helper cell activity in the control 28. Barsamian-Wunsch P, Park JH, Watson MR, Tinanoff N, Minah
of dental caries. Nature 1981;292:770-2. GE. Microbiological screening for cariogenic bacteria in
11. de Vries RR, Zeylemaker P, van Palenstein Helderman WH, children 9 to 36 months of age. Pediatric Dentistry 2004;26(3):
Huis in't Veld JH. Lack of association between HLA-DR 231-8.
antigens and dental caries. Tissue Antigens, 1985;25:173-4. 29. Ersin NK, Eronat N, Cogulu D, Uzel A, Aksit S. Association of
12. Senpuku H, Yanagi, K, Nisizawa T. Identification of maternal child characteristics as a factor in early childhood caries
Streptococcus mutans PAc peptide motif binding with human and salivary bacterial counts. J Dent Child 2006;73(2): 105-11.
MHC class II molecules (DRB1-0802, 1101, 1401 and 1405). 30. Gustafsson Be, Quensel Ce, Lanke Ls, Lundqvist C, Grahnen
Immunology 1998;95:322-30. H, Bonow Be, et al. The Vipeholm dental caries study; the effect
13. Acton RT, Dasanyake AP, Harrison RA, et al. Association of of different levels of carbohydrate intake on caries activity in
MHC genes with levels of caries-inducing organisms and caries 436 individuals observed for five years. Acta Odontol Scand
severity in African-American women. Human Immunol 1999; 1954;11(3-4):232-64.
60:984-9. 31. Sullivan HR, Goldsworthy NE. Review and correlation of the
14. Newbrun E. Chapter 2. Current concepts in caries etiology. In: data presented in papers 1-6 (Hopewood House Study). Aust
Newbrun E (Ed). Cariology. Quintessence Books, 3rd edn, 1989. Dent J 1958;3:395-8.
15. McGhee JR, Michalek SM. Immunobiology of dental caries. 32. Robert A, Downs DDS, MSPH, Muriel M, Dunn RDH, Eleanor
Microbial aspects and local immunity. Annu Rev Microbiol L, Richie MA. Report of dental findings of seventh-day
1981;35:595-638. adventist students as compared to comparable students in other
16. Gibbons RJ. Bacterial adhesion to oral tissues: a model for schools. American Journal of Orthodontics 1959;45(2):141-3.
infectious diseases. J Dent Res 1989;68:750-60. 33. Scheinin A, Mackinen KK. Turku sugar studies I-XXI, Acta
17. Loesche WJ, Syed SA. Bacteriology of human experimental Odont Scand 1975;33(Sup 70).
gingivitis: Effect of plaque and gingivitis score. Infect Immun 34. Theodore M, Roberson Harald O, Heymann Edward J Swift.
1978;21(3):830-9. GV Blacks classification. Sturdevant's Art and Science of
18. Bowen WH, Cohen B, Cole MF, Colman G. Immunization Operative Dentistry. Elsevier Mosby, 5th edn, 2006.
against dental caries. Br Dent J 1975;139:45-58. 35. Mount GJ, Hume WR. A revised classification of carious lesions
19. Loesche WJ, Rowan J, Straffon LH, Loos PJ. The association by site and size. Quintesscence International 1997;28(5):301-3.
of streptococcus mutans with human dental decay. Infect and 36. Tinanoff N. Introduction to the Early Chilhood caries
Immunol 1975;11(6):1252-60. conference: initial description and current understanding. Comm
20. Loesche WJ, Straffon LH. Longitudinal investigation of the role Dent Oral Epidemiol 1998;26(Suppl 1):5-7.
of Streptococcus mutans in human fissure decay. Infect Immun 37. Ruhl S, Rayment SA, Schmalz G, Hiller KA, Troxler RF.
1979;26:498-507. Proteins in whole saliva during the first year of infancy. J Dent
21. Billings RJ, Brown LR, Kaster AG. Contemporary treatment Res 2005;84(1):29-34.
strategies for root surface dental caries. Gerodontics. 1985;1(1): 38. Mahejabeen R, Sudha P, Kulkarni SS, Anegundi R. Dental
20-7. caries prevalence among preschool children of Hubli: Dharwad
22. Brown LR, Billings RJ, Kaster AG. Quantitative comparisons city 2006;24(1):19-22.
of potentially cariogenic microorganisms cultured from non- 39. Saravanan S, Madivanan I, Subashini B, Felix JW. Prevalence
carious and carious root and coronal tooth surfaces. Infect pattern of dental caries in the primary dentition among school
Immune 1986;51(3):765-70. children. Indian J Dent Res 2005;16(4):140-6.
23. Bowden GHW. Microbiology of Root Surface Caries in 40. Jenkins GN, Ferguson DB. Milk and dental caries. British
Humans. Journal of Dental Research 1990;69(5):1205-10. Dental Journal 1966;472-7.
24. Nyvad B, Kilian M. Microbiology of the early colonization of 41. Bhat SS, Dubey A. Acidogenic potential of soya infant formula
human enamel and root surfaces in vivo. Scand J Dent Res in comparison with regular infant formula and bovine milk: A
1987;95:369-80. plaque pH study. JISPPD, 2003;21(1):30-4.
25. Schupbach P, Osterwalder V, Guggenheim B. Human root 42. Danchaivijitr A, Nakornchai S, Thaweeboon B, Leelataweewud
caries: microbiota of a limited number of root caries lesions. P, Phonghanyudh A, Kiatprajak C, et al. The effect of different
Caries Res 1996;30:52-64. milk formulas on dental plaque pH. Int Journ of Pediatric Dent
26. Mattee MIN, et al. Mutans streptococci and lactobacilli in breast- 2006;16:192-8.
fed children with rampant caries. Caries Res 1992; 26:183-7. 43. Kelly M, Bruerd B. The prevalence of baby bottle tooth decay
27. Krishnakumar R, Singh S, Subba Reddy VV. Comparison of among two native American populations. J Public Health Dent
the levels of mutans Streptococci and lactobacilli in children spring 1987;47(2):94-7.
120 Essentials of Pediatric Oral Pathology

44. Ripa LW. Nursing caries: A comprehensive review. Pediatr Dent 66. White SC, Pharoah MJ. Specialized radiographic technique. In:
1988;10:268-82. Chapter 12, Oral radiology- principles and interpretation.
45. Davies GN. Early childhood caries - a synopsis. Comm Dent Mosby publications, 4 edn. 217-241.
Oral Epidemiol 1998;26(Suppl 1):106-16. 67. Duncan RC, Heaven T. Using computers to diagnose and plan
46. American Academy of Pediatric Dentistry: Reference manual treatment of approximal caries detected in radiographs. JADA
2003-2004. Pediatr Dent 2003;25:54. 1995;873-82.
47. Moynihan PJ. Dietary advice in dental practice. Brit Dent J, 68. Fejereskov O, Kidd E. Advanced methods of caries diagnosis
Nov 2002;193(10)563-68. and quantification. In: Chapter 8, Dental caries: The disease
48. Wyne Amjad Husain. Early childhood caries: a review. Indian and its clinical management, Oxford (UK): Blackwell and
J Dent Res, 1996;7(1) 7-9. Munksgaard, 2003;127-39.
49. Ismail AI. Prevention of early childhood caries. Community 69. Angmar-Mansson B et al. Strategies for improving the
Dent Oral Epidemiol, 1998;26 (Suppl 1):49-61. assessment of dental fluorosis: focus upon optical techniques.
50. Weintraub JA. Prevention of early childhood caries: a public Adv Dent Res 1994;8:75-79.
health perspective. Community Dent Oral Epidemiol 1998; 26 70. Friedman J, Marcus MI. Transillumination of the oral cavity
(Suppl 1):62-6. with use of fiber optics. JADA 1970;80(4):801-9.
51. Suhonen J, Sener B, Bucher W, Lutz F. Release of preventive 71. Fejereskov O, Kidd E. Clinical and radiographic diagnosis. In:
agents from pacifiers in vitro. An introduction to a novel Chapter 7, Dental caries: The disease and its clinical
preventive measure. Schweiz Monastsschr Zahnmed management, Oxford (UK): Blackwell and Munksgaard,
1994;104:946-51. 2003;111-28.
52. Frostell G. A colorimetric screening test for evaluation of the 72. Pretty IA, Maupome G. A closer look at diagnosis in clinical
buffer capacity of saliva. Swedish Dental Journal 1980;4(3):81- dental practice: Part 5. Emerging technologies for caries
86. detection and diagnosis. J Can Dent Assoc 2004;70(8):540.
53. American Academy of Pediatric Dentistry, Council on Clinical 73. Barnes CM. Dental hygiene participation in managing incipient
Affairs. Policy on use of a caries risk assessment tool (CAT) and hidden caries. Dent Clin of N Am 2005;49(2005):795-813.
for infants, children and adolescents. Pediatr Dent 2002;25:18. 74. Bakhos, Y., Brudevold, F, Aasenden, R. In vivo estimation of
54. Ericson D, Bratthall D. Simplified method to estimate salivary the permeability of surface human enamel. Arch Oral Biol
buffering capacity. Scand J Dent Res 1989;97:405-7. 1977;22:599-603.
55. Hadley, F.P. A quantitative method for estimating Bacillus 75. Anusavice KJ. Caries risk assessment. Oper Dentistry 2001,
acidophilus in saliva. J dent Res 1933;13:415-28. supplement 6:19-26.
56. Snyder, ML, Clarke, M K Evaluation of the colorimetric 76. Ricketts DNJ, Kid EAM. Operative and microbiological
(Snyder) Test. J Dent Res 1950;29:298. validation of visual, radiographic and electronic diagnosis of
57. Grainger, RM, Jarrett, M, and Honey, L. An epidemiological occlusal caries in non-cavitated teeth judged to be in need of
study of the awab Test, J Canad Dent Ass 1965;31:515-26. operative care. Br Dent J 1995;179:214-20.
58. Alban A. An improved Snyder test. J Dent Res 1970;49(3):641. 77. Mount GJ, Hume MI. A new concept for operative dentistry,
59. Fosdick LS, Calandra JC, Blackwell RQ, Burrill JH. A new Quintessence Int 2000;31(8):527-33.
approach to the problem of dental caries control. J Dent Res. 78. Reynolds EC. Remineralization of enamel subsurface lesions
1953;32:486. by casein phosphopeptides stabilized calcium phosphate
60. Dewar, Margaret. Tests for Susceptibility to Dental Caries. Part solutions. J Dent Research 1997;76(9):1587-95.
I. Australian J. Dent., 1949; 21:519. 79. Shen P et al. Remineralization of enamel subsurface lesions by
61. Kühnisch J, Dietz W, Stosser L, Hickel R, Heinrich-Weltzien sugar-free chewing gum containing casein phospeptide—
R. Effects of dental probing on occlusal surfaces - a scanning Amorphous calcium phosphate, J Dent Research 2001;
electron microscopy evaluation. Caries Res 2007;41:43-48. 80(12);2066-2070.
62. Kidd EA, Fejerskov O. What constitute dental caries? 80. Makinen KK. The rocky road of xylitol to its clinical
Histopathology of carious enamel and dentin related to the applications. J Dent Research 2000;79(6):1352-55.
action of cariogenic biofilms [special issue]. J Dent Res 2004; 81. ID Mandel. Caries prevention: Current strategies, new
83(C): C15-17. directions. JADA 1996;10;1477-88.
63. Wilkins E. Clinical practice of the dental hygienist. 9th edn, 82. Featherstone JDB, Zhang SH, Shariati M, McCormac SM.
Philadelphia: Lippincott, Williams and Wilkins, 2004. Carbon dioxide laser effects on caries-like lesions of dental
64. Miles DA, Parks ET. Chapter 5: Radiographic techniques. In: enamel. Proc Soc Photo-Optical Instrum Engineers
McDonald RE, Avery DR, Dentistry for the child and adolescent 1991;1424:145-9.
Eds. Harcourt India Pvt. Ltd. 7th edn, 2001:67. 83. Goldstein RE, Parkins FM. Air abrasive technology: its new
65. Lussi A. Comparison of different methods for the diagnosis role in restorative dentistry. JADA 1997;551-557.
of fissure caries without cavitation. Caries Research 84. Mount GJ, et al. MID—A review—FDI commission project—
1993;27:409-16. 2000. Int. Dent J 50:1-12.
Caries in Children 121

85. Beeky JA, Yip HK, Stevenson AG. Chemomechanical caries 109. Jeppersen K. Direct pulp capping on primary teeth—a long-
removal. A review of techniques and latest development. . Br term investigation. Int Assoc Dent Child 1971;12:10.
Dent J, 2000;188:427-30. 110. Dylewski JJ. Apical closure of non-vital teeth, Oral Surg 32:
86. George M, Maragakis, Petra Hahn, Freiburg EH. 1971;82-9.
Chemomechanical caries removal a comprehensive review of 111. Schröder U, Granath LE. On internal dentin resorption in
literature. Int Dent J 2001;51:291-299. deciduous molars treated by pulpotomy and capped with
87. Robert. Lasers and light amplification in dentistry. Dent Clin calcium hydroxide. Odontol Revy 1971;22:179.
of N Am 2000;44. 112. Heithersay GS.Calcium hydroxide in the treatment of pulpless
88. Stern, RH, Sognnaes, RF. Laser beam effect on dental hard teeth with associated pathology. J Brit Endo Soc, 1975;8:74-93.
tissues, J Dent Res 1964;43:873. 113. Tronstad L, Andreasen JO, Hasselgren G, Riis I. pH changes in
89. Mount GJ. Minimal intervention dentistry: rationale of cavity dental tissues after root canal filling with calcium hydroxide.J
design. Oper Dent 2003;28:92-9. Endod 1981;7:17-21.
90. Shan Farhan: Filling without drilling—Not a myth but a reality, 114. Anthony DR, Senia S. The use of calcium hydroxide as
JIDA 2003;4;563-64. temporary paste fill. Texas Dental J 1981;8:6-10.
91. Mount GJ. Minimal intervention: site # 1 lesions Indian Dentist 115. Javelet J, Torabinejad M, Bakland L. Comparison of two pH
Research and Review 2005;3:7-8. levels for the induction of apical barriers in immature teeth of
92. Mount GJ. Minimal intervention: site # 2 lesions, Indian Dentist monkeys. J Endod 11:375-78, 1985.
Research and Review, March 2005,3-4. 116. Stamos DG, Haasch GC, Gerstein H. The pH of local anesthetic/
calcium hydroxide solutions. J Endod 1985;11:264-5.
93. Mount GJ, Hien Ngo. Minimal intervention: advanced lesions.
Quintessence Int. 2000;31(9):621-29. 117. Mitchell OF, Shankwalker GB. Osteogenic potencial of calcium
hydroxide and other materials in soft tissue and bone wounds.
94. Mjor IA. Placement and replacement of restorations. Oper, Dent
J Dent Res 1958,37:1157-63.
1981;6;49-54.
118. Mc Cormick. Calcium-hydroxide pulpotomy: report of a case
95. Monty S Duggal, Curzon MEJ, Fayle SA, MA Pollard
observed for nineteen years. ASDC J Dent Child
restorative techniques in paediatric dentistry 2002;30.
1981;48(3):222-5.
96. Mortimer KV. The relationship of deciduous enamel structure
119. Van Hassel HJ, Natkin E. Induction of root end closure. Journal
to dental disease. Caries Res 1970;4:202-223.
of Dentistry for Children 1970;37:57-9.
97. McDonald RE, Avery DR. Dentistry for child and adolescent.
120. Frank AL. Therapy for the divergent pulpless tooth by continued
St. Louis, MO: CV Mosby Company, 6th ed, 1994;377-78.
apical formation. Journal of American Dental Association
98. SN Frankl: Pulp therapy in pedodontics. Oral Surg Oral Med 1966;72:87-93.
Oral Pathol 1972;34(2):293-309. 121. Nyborg H. Capping of the pulp. The processes involved and
99. Whitehead FI, et al. The relationship of bacterial invasion of their outcome. Odontol Tidskr 1958;66:296.
softening of the dentin in permanent and deciduous teeth. Br 122. Nevins AJ, et al. Pulpotomy and partial pulpectomy procedures
Dent J 1960;108:261. in monkey teeth using cross-linked collagen-calcium phosphate
100. Shovelton DS. Studies of dentin and pulp in deep caries. Int gel. Oral Surg 1980;49:360.
Dent J 1970;20:283. 123. Citrome GP, Kaminski EJ, Heuer MA. A comparative study of
101. Seltzer S, Bender IB. The dental pulp. Philadelphia: JB tooth apexification in the dog. Journal of Endodontics October
Lippincott, 1965;184-98. 1979; 5(10):290-297.
102. Reeves R, Stanley HR. The relationship of bacterial penetration 124. Lieberman J, Trowbridge H. Apical closure of nonvital
and pulpal pathosis in carious teeth. Oral Surg 1966;22:59. permanent incisor teeth where no treatment was performed: case
103. Shovelton DS. Studies of dentin and pulp in deep caries. Int report. J Endod 1983;9:257-60.
Dent J 1970;20:283. 125. Glass RL, Zander HA. Pulp healing. J Dent Res 1949;28:97.
104. Rayner J, Southam JC. Pulp changes in deciduous teeth 126. Sveen OB. Pulp capping of primary teeth with zinc oxide-
associated with deep carious dentin. J Dent 1979;7:39-42. eugenol. Odontol Tidskr 1969;77:427.
105. Leung RL, et al. Effect of Dycal on bacteria in deep carious 127. Brosch JW. Capping pulps with a compound of calcium
lesions. J Am Dent Assoc 1980;10:193. phosphate, neomycin and hydrocortisone. J Dent Child
106. Fairbourn DR, et al. Effect of improved Dycal and IRM on 1966;33:42.
bacteria in deep carious lesions. J Am Dent Assoc 1980;100:547. 128. Soldali GD. Pulp capping with antibiotics. Fla Dent J
107. Law DB, Lewis TM. The effects of calcium hydroxide on deep 1975;46:18.
carious lesions. Colorado Dent J 1964;42:27. 129. Bhaskar SH, et al. Tissue response to cortisone-containing and
108. Fuks AB. Pulp therapy for the primary dentition. In: Pinkham cortisone-free calcium hydroxide. J Dent Child 1969;36:1.
JR, Cassamassimo PS, Fields HW, et al, Eds. Pediatric dentistry- 130. Ulmansky M, Sela J. Response of pulpotomy wounds in normal
infancy through adolescence. WB Saunders, 2nd ed. 1988;326- human teeth to successively applied Ledermix and Calxyl. Arch
38. Oral Biol 1971;16:1393.
122 Essentials of Pediatric Oral Pathology

131. Haskell EW, et al. Direct pulp capping treatment: A long-term 154. Lewis TM, Law DC. Pulpal treatment of primary teeth. In: Finn
follow-up. J Am Dent Assoc 1978;97:607. SB, Ed. Clinical pedodontics. Philadelphia: WB Saunders 4th
132. McWalter G, et al. Long-term study of pulp capping in monkeys edm;1973.
with three agents. J Am Dent Assoc 1976;933:105. 155. Judd P, Kenny D. Non-aldehyde pulpectomy technique for
133. Gardner DE, Mitchell DF, McDonald RE. Treatment of pulps primary teeth. Ontario Dentist 1991;68:25.
of monkeys with vancomycin and calcium hydroxide. JDR 156. Gould JM. Root canal therapy for infected primary molar teeth
1971;50:1273. - preliminary report. J Dent Child 1970;34:23.
134. Negm M, et al. Clinical and histologic study of human pulpal 157. Frigoletto RT. Pulp therapy in pedodontics. J Am Dent Assoc
response to new cements containing calcium hydroxide. Oral 1973;86:1344.
Surg 1980;50:462. 158. Starkey P. Treatment of pulpally involved primary molars. In:
135. Bhaskar SH, et al. Human pulp capping with McDonald RE, et al, Eds. Current therapy in dentistry. St. Louis:
isobutylcyanoacrylate. J Dent Res 1972;51:50. CV Mosby, 1980;414.
136. Heys DR, Cox CF, Avery JK. Histological considerations of 159. Coll JA, Josell S, Casper JS. Evaluation of a one appointment
direct pulp capping agents. J Dent Res 1981;68:1371. formocresol pulpectomy technique for primary molars. Pediatr
137. Dick HM, Carmichael DJ. Reconstituted antigen poor collagen Dent 1985;7:123.
preparations as potential pulp capping agents. JOE 1980;6:641. 160. Vander Wall GL, et al. Endodontic medicaments. Oral Surg
138. Arnold DS. The use of formocresol as a pulp capping agent in 1972;25:238.
human primary teeth [thesis]. Lincoln, NE: Univ. of Nebraska 161. Barr ES, et al. Radiographic evaluation of primary molar
School of Dentistry; 1970. pulpectomies in a pediatric dental practice [abstract]. Pediatr
139. Bergenholtz G, et al. Bacterial leakage around dental Dent 1986;8:180.
restorations: its effect on the dental pulp. J Oral Pathol 162. Flaitz CM, et al. Radiographic evaluation of pulpal therapy for
1982;11:439. primary anterior teeth. J Dent Child 1989;56:182.
140. Cox CF. Effects of adhesive resins and various dental cements 163. Yacobi R, et al. Evolving primary pulp therapy techniques. J
on the pulp. Oper Dent 1992;55:165. Am Dent Assoc 1991;122:83.
141. Miyakoshi S, et al. Interfacial interaction of 4-META-MMA- 164. Damle SG. Text book of pediatric dentistry. 2006;3rd edn, Arya
TBB resin and the pulp [abstract]. J Dent Res 1993;72:220. (Medi) Publishing House,
142. Cox CF, et al. Pulp response following in vivo etching and 4- 165. Dummett CO, Kopel HM. Pediatric endodontics. In: Ingle JI,
META bonding. J Dent Res 1993;72:213. Bakland LK, Endodontics. BC Decker Inc., 5th ed, Ontario,
143. Torabinejad M, et al. Physical and chemical properties of a new 2002.
root-end filling material. JOE 1995;21:349. 166. Camp J. Pulp therapy for primary and young permanent teeth.
144. Pitt Ford TR, Torabinejad M, Abedi HR, et al. Using mineral Dent Clin North Am 1984;28:651.
trioxide aggregate as a pulp capping material. J Am Dent Assoc 167. Spedding R. Endodontic treatment of primary molars. In:
1996;127:1491. Goldman HM, et al., Eds. Current therapy in dentistry. St. Louis:
145. Yoshimine Y, Maeda K. Histologic evaluation of tetracalcium CV Mosby, 1977;558-69.
phosphate-based cement as a direct pulp-capping agent. Oral 168. Krakow A, et al. Advanced endodontics in pedodontics. In:
Surg Oral Med Oral Pathol Oral Radiol Endod 1995;79:351. White G, Ed. Clinical oral pediatrics. Chicago: Quintessence,
146. Hibbard ED, Ireland RL. Morphology of the root canals of the 1981;248-64.
primary molar teeth. J Dent Child 1957;24:250. 169. Coll JA, Josell S, Casper JS. Evaluation of a one appointment
147. Fox AG, Heeley JD. Histologic study of human primary teeth. formocresol pulpectomy technique for primary molars. Pediatr
Arch Oral Biol 1980;25:103. Dent 1985;7:123.
148. Bernick S. Innervation of the teeth and periodontium. Dent Clin 170. Barker BC, Lockett BC. Endodontic experiments with
North Am 1959;503. resorbable paste. Aust Dent J 1971;16:364.
149. Rapp R, et al. The distribution of nerves in human primary teeth. 171. Holan G, Fuks AB. A comparison of pulpectomies using ZOE
Anat Rec 1967;159:89. and Kri Paste in primary molars: a retrospective study. Pediatr
150. McDonald RE. Diagnostic aids and vital pulp therapy for Dent 1993;15:403.
deciduous teeth. J Am Dent Assoc 1956;53:14. 172. Garcia-Godoy F. Evaluation of an iodoform paste in root canal
151. Sweet CA. Procedure for treatment of exposed and pulpless therapy for infected primary teeth. J Dent Child 1987;54:30.
deciduous teeth. J Am Dent Assoc 1930;17:1150. 173. Chawla HS, Mani SA, Tewari A. Calcium hydroxide as a root
152. Rabinowitz BZ. Pulp management of primary teeth. Oral Surg canal filling material in primary teeth; a pilot study. J Ind Soc
1953;6:542. of Pedo and Prev Dent 1998;16(3)90-2.
153. Hobson P. Pulp treatment of deciduous teeth. Br Dent J 174. Tandon S. Textbook of pedodontics. Paras Medical Publisher,
1970;128:275. 1st edn, 2001. Reprint march, 2002.
Caries in Children 123

175. Arens, DE. Apexification, pulp capping and pulpotomy. In: 181. Sweet CA. Procedure for treatment of exposed and pulpless
Clinical Dentistry, Harper and Row, Maryland, 1977;4:215. deciduous teeth. J Am Dent Assoc 1930;17:1150.
176. Weine FS, McCormick JE, Maggio JD. Tissue pH of developing 182. Massler M, Mansukhani H. Effects of formocresol on the dental
periapical lesions in dogs . J Am Dent Assoc 1996;127:1491-4. pulp. J Dent Child 1959;26:277.
177. Walton RE, Torabinejad M Cleaning and shaping. In: Pedersen 183. Teplitsky P McSpadden compactor. Vertical condensation
P, Ed. Principles and practice of endodontics. Philadelphia: technique to deliver calcium hydroxide. Journal of the Canadian
Saunders, 1989. Dental Association 1986;52:779-81.
178. Camp J, Barrett EJ, Pulver F. Pediatric Endodontics: Endodontic 184. Holland, R, Souza, V, Nery, M, J. Mello W, Bernabé, PFE,
treatment for the primary and young permanent dentition. In: Otoboni Filho JA. A histological study of the effect of calcium
Cohen S and Burns RC, Pathways of the pulp. Chapter 23. hydroxide in the treatment of pulpless teeth of dogs. J. Brito
Mosby Co., St. Louis, 8th Ed., 2002. Endod Soe 1979;12:15-23.
179. Cvek M. A clinical report on partial pulpotomy and capping 185. Schilder H. Vertical compaction of warm gutta-percha. In:
with calcium hydroxide in permanent incisors with complicated Gerstein H, Ed. Techniques in clinical endodontics.
crown fracture. J Endod 1978;4(8):232-7. Philadelphia, PA: WB Saunders; 1983:76-98.
180. Buckley JP. The chemistry of pulp decomposition with a rational 186. Ingle JI and Leif K. Bakland: Textbook of endodontics:
treatment for this condition and its sequelae. J Am Dent Assoc Obturation of the radicular space. BC, Decker Inc, 2002, 5th
1904;3:764. Edn, 2002;598-636.
3
Pulp Pathologies
in Children
Shweta Dixit Chaudhary, Mayur Chaudhary

CHAPTER OVERVIEW
Introduction Acute pulpitis
Need to learn pulp pathology Chronic pulpalgia (Subacute pulpitis)
Concept of pain Acute pulpitis with apical periodontitis
Neuroanatomy of pain Nonpainful pulpitis
Behavior of dental pains
Pulpal granuloma
Causes of pulp pathology
Chronic hyperplastic pulpitis
Pulpal reaction to an insult
Reversible pulpitis Irreversible pulpitis
Hypersensitivity Pulp degeneration
Cracked tooth syndrome Pulp calcifications
Focal reversible pulpitis (Pulp hyperemia) Pulp necrosis

INTRODUCTION to devitalize pulps. The third half-century, 1876 to 1926, was

highlighted by the discovery and development of the X-ray,
The pulp has been described both as a highly resistant organ
the advent of local anesthetics, and the acceptance of
as well as an organ with little resistance or recuperating ability.
antisepsis as a part of endodontic therapy. Beginning about
The desirability of maintaining a vital pulp and protecting it
1912, dentistry in general and endodontics in particular were
from injury has been recognized by early practitioners.
set back by the wide acceptance of the theory of focal
Dr. Grossman chronicled the historical events impacting on root
canal therapy. He divided the 200 years between 1776 and 1976 infection. Wholesale extraction of both vital and pulpless teeth
into four 50-year periods.1 During the first period, 1776 to 1826, took place. The professions were not to recover their senses
he noted that treatment was crude - abscessed teeth were until well after World War II. The final 50-year period, 1926 to
treated with leeches or toasted fig poultices and pulps were 1976, saw improvements in radiographs, anesthetics and
cauterized with red-hot wires. Nevertheless, it was during this procedures as well as the introduction of new methods and
same period that root canals were being filled from apex to agents. Calcium hydroxide made its appearance, as did
crown with gold foil. The second half-century, 1826 to 1876, ethylenediaminetetraacetic acid (EDTA) for chelation. This
was marked by the founding of the first dental journal and the period also witnessed the rise and decline of the silver root
first dental school, the introduction of general anesthesia, canal point. A more sensible attitude towards endodontic
rubber dam, gutta-percha, root canal points and the barbed surgery developed.
broach, as well as three- and four-sided tapering broaches for During this period, the American Association of
cleaning and enlarging root canals, intracanal antiseptics, and Endodontists (AAE) was formed, followed by the American
oxyphosphate of zinc cement. At the same time, however, pulps Board of Endodontics. Continuing education in endodontics
were still being removed by driving wooden pegs into the canal, widely disseminated information, skills and techniques to an
and crowns of the teeth were also being "snipped" off at the eager profession. The prevention of pulp disease began to play
gingival level to cure toothache. Arsenicals were still being used a more important role in dental practice. There was a decline
Pulp Pathologies in Children 125

in dental caries, in part because of fluoridation. Research into • Accurate knowledge of pain arising from dental or
the causes and biology of dental trauma led to improved nondental sources is of utmost importance. Let us remember
awareness and treatment of dental injuries. Antibiotics greatly that there is no single cookbook therapy which is effective
improved the profession's ability to control infection, while new for pain; its management differs not only with the structural
anesthetics and injection techniques increased control over origin of pain but also with the psychological condition of
pain. The high-speed air rotor handpiece added to patient the patient.
comfort and the speed and ease of operation, as did • Pain involves the following processes:2
prepackaged sterilized supplies. The mandatory use of masks, — Transduction
gloves and better sterilizing methods rapidly emerged with the — Transmission
spread of Human immunodeficiency virus/Acquired immune — Modulation and
deficiency syndrome (HIV/AIDS) and hepatitis. The — Perception.
widespread use of auxiliaries expanded dental services. All in
all, the new millennium should prove exciting and profitable FUNCTIONAL NEUROANATOMY OF PAIN2 (FIG. 3.1)
for the profession and patients alike. • The primary afferent neuron receives stimulus from the
sensory receptor.
NEED TO LEARN PULP PATHOLOGY • This is carried to the CNS by way of the dorsal root to
synapse in the dorsal horn of the spinal cord.
• To effectively treat endodontic infections, clinicians must
• Cell bodies of all the primary afferent neurons are located
recognize the cause and effect of microbial invasion of the
in the dorsal root ganglia.
dental pulp space and the surrounding periradicular tissues.
• Second order neuron then carries it across to the
• Knowledge of the microorganisms associated with
anterolateral spinothalamic pathway.
endodontic disease is necessary to develop a basic
• Between the first and second order neuron, multiple
understanding of the disease process and a sound rationale
interneurons may be present.
for effective management of patients with endodontic
infections. THE FIRST ORDER NEURON
• Proper diagnosis is the discipline that separates the really
competent dentist from the merely mechanical; hence the • Each receptor is attached to a first order neuron.
extensive coverage of the subject. • Clark et al 1935, gave the relationship between diameter
of nerve fibers and their conduction velocities.3
• Larger fibers conduct more rapidly than the smaller ones
CONCEPT OF PAIN
• Around 21.8 percent adults in US report with orofacial pain Type A Nerve Fibers
within a span of six months. In fact, it is the most common • Alpha fibers
factor for seeking treatment. 13–20 m in diameter
• At the same time, fear remains the most common factor 70–120 m/s velocity
for avoiding treatment.
• It remains our duty as clinicians to diagnose the cause and
source of orofacial pain, allay patient fears and render
treatment suitable for the individual.

DEFINITIONS OF PAIN
• An unpleasant emotional and sensory experience
associated with actual or potential tissue damage. Defines
both physiologic and psychologic components.
• A more or less localized sensation of discomfort, distress,
or agony, resulting from the stimulation of specialized
nerve endings. It serves as a protective mechanism in so
far as it induces the sufferer to remove or withdraw from
the source.
• An unpleasant emotional experience usually initiated by a
noxious stimulus and transmitted over a specialized neural
network to the CNS where it is interpreted as such. FIGURE 3.1: Functional neuroanatomy of pain
126 Essentials of Pediatric Oral Pathology

• Beta fibers • Impulses by A-delta fibers synapse in lamina I of the

6–13 m in diameter subnucleus caudalis.
40–70 m/s velocity • They then travel through the neospinothalamic tract to the
• Gamma fibers thalamus.
3–8 m in diameter • This results in fast pain.
15–40 m/s velocity • Impulses by C fibers synapse in Laminae II, III and V.
• Delta fibers • They are carried through paleospinothalamic tract.
1–5 m in diameter • They travel through interneurons into the reticular
5–15 m/s velocity formation of the brain stem.
• From here, they travel to the thalamus.
Type C Nerve Fibers • Impulse can be changed or modulated before it reaches the
thalamus.
• 0.5–1 m in diameter • This results in slow pain.
• 0.5–2 m/s velocity • Sharp pain—Easily localized, quick response.
Probable relationship between the fiber size and the type • Slow pain—Suffering.
of impulse transmitted: • C fibers contain substance P as the major neurotransmitter.
• A-alpha, A-beta, A-gamma carry impulses for tactile and • Substance P persists at the synapse; hence progressive
proprioceptive responses. increase in intensity of slow chronic pain occurs.
• A-delta and C fibers conduct pain, but are not specific
for it. PERCEPTION
• Determined by interaction of the cortex, thalamus,
TYPES OF PAIN hypothalamus and limbic structures.
• Pricking pain • Till now we have seen a mechanistic approach to pain.
• Burning pain • If treatment fails to alleviate pain, should the patient be
• A-delta fibers also conduct touch, warmth and cold considered neurotic? Such a situation probably indicates
• C fibers also conduct itch, warmth and cold. that researchers have still not completely unraveled the
mystery of the human brain and its networking, nor that
FIBERS DEMONSTRATE SPECIFICITY OF of pain perception.
FUNCTION • In perception occurs the addition of instincts, drives and
emotions.
• Fine peripheral nerve fibers can convey touch, pain, warmth • This is influenced by past experiences and present
and cold. environmental conditions.
• Larger peripheral nerve fibers have specificity of function. • Hypothalamus prepares the body through responses of
the ANS.
NOCICEPTIVE AFFERENT NEURONS
• Three classes:
1. Mechanothermal afferents—A-delta fibers—12–18 m/s.
High degree of discriminative information. Peculiar to
primates.
2. Polymodal afferents—C fibers—0.5 m/s. Seen in all
mammals.
3. High threshold mechanoreceptive afferents—A-delta
fibers. Present in all mammals. Can be sensitized by
algogenic substances and responds to noxious heat.

MODULATION (FIGS 3.2 AND 3.3)

• The nociceptive specific second order neuron will cross
the brain stem and ascend in the anterolateral tract on the FIGURE 3.2: Cross section of the spinal cord
opposite side. showing various laminae
Pulp Pathologies in Children 127

FIGURES 3.3A and B: Pain signals are transmitted to the brain by two main pathways. The lateral system (A) is made up of long thick fibers
that transmit information about the onset of injury, and its precise location and intensity. They are designed to carry a rapid flow of pain signals
to the thalamus to stimulate an immediate antinociceptive response. The medial system (B) is composed of phylogenetically older fibers that
carry slower signals and probably transmit information related to the persistence of injury and level of response induced

• Hence, experience of pain is individual and personal, • Pain behavior, hence directly depends on the perception
• Bio—From somatic tissues (Figs 3.4 and 3.5). of pain and suffering of an individual.
• Psychosocial—interaction between thalamus, cortex and
limbic structures (Figs 3.4 and 3.5). SUBSTANCE P
• All neural function is based on neurotransmitter activity.
• There are two schools of thought here: • Polypeptide with 11 amino acids.
1. Bio and psychological aspects are totally independent • Released at the central terminals of primary nociceptive
2. All activity has an organic neural basis. neurons.
• Suffering depends on an individual's psychology and may • Found at the distal terminals also.
not be proportionally related to nociception or pain. • Excitatory neurotransmitter for nociceptive impulses.
128 Essentials of Pediatric Oral Pathology

FIGURE 3.4: Biopsychosocial model of pain

PULPAL PAIN
• Visceral character
• Threshold type of pain
• Responds to impact, shock, thermal and chemical irritants,
direct exploration
• Not to ordinary masticatory function
• Nonlocalizable by the subject.

CLASSIFICATION OF PULPAL PAIN

• Acute
• Chronic
FIGURE 3.5: Diagrammatic representation of • Recurrent
biopsychosocial model of pain • Mixed with periodontal elements
• Nondental origin.
• Released from spinal cord cells by stimulation of A-delta
Acute Pulpal Pain
and C fiber afferents.
• Excites neurons in the dorsal horn. • Most typical of visceral pain
• Involved in neurogenic inflammatory phenomena. • Completely nonlocalizable
• Content is highest in the most severely inflammed joints. • Objective evidence required for diagnosis
• Protection is offered by tooth structure
BEHAVIOR OF DENTAL PAIN • Only extreme surface irritation sensed as pain
• For example, split tooth results in acute pulpal pain.
• The extreme variability of toothache affects the behaviour
of dental pains. Causes
• A good rule for any examiner is to consider all pains about • Threshold stimulation of the intact surface
the mouth and face to be of dental origin until proved • Cervical exposure due to gingival recession
otherwise. • Splitting or fracture of the tooth
Pulp Pathologies in Children 129

• Repeated thermal shocks transmitted by metallic fillings Dental Pains of Periodontal Origin
• Thinning by erosion or abrasion • Deep somatic pain of the musculoskeletal type
• Traumatic shock • More localized in nature
• Dental caries. • Responds in graduated increments
Sequelae • Precise localization
• Inflammatory changes occur • May be due to primary periodontal inflammatory condition
• May be reversible or congestion • May be due to dental therapy
 • May spread from pulpal inflammation
Pulpal necrosis • May extend from a nearby inflammatory condition.

Gangrene Toothaches of Nondental Origin
• Multirooted teeth present a confusing symptom picture. • Dental pain is the most common orofacial pain. But
• Pain threshold is lowered by inflammation. heterotopic pains of variable etiology also do occur.
• Hypersensitivity also produces acute dental pain. • Due to central sensitization or excitation of the second order
• Acute pulpal pain may range from occasional hyper- neurons produced by a constant barrage of nociceptive
sensitivity to spontaneous violent throbbing toothache of input from deep structures.
intolerable intensity. • Local provocation, selective use of local anesthetic agents
• It is increased by both heat and cold. help in localization of pain.
OR • Muscular toothache: Usually pain from the muscles of
Increased by heat, relieved by cold. mastication has a tendency to be referred to the teeth. Pain
Recovery usually increases with mastication and appears as non-
• Pain ceases spontaneously. pulsatile, diffuse, dull and constant. Palpation of the muscle
• This could be due to transition from pulpal to periodontal at specific trigger points may help in diagnosis of pain.
involvement. • Neurovascular toothache: Neurovascular toothache is
• If pulpal inflammation is due to infection, mixed pulpal and usually brought on by stress. Most common type is migraine
periodontal pains may occur due to development of an although tension type or cluster headache may be seen. Pain
acute periodontal abscess. simulates pulpal pain with unilateral occurrence and
• If pulpal inflammation is sterile, a painless periapical periods of remission.
granuloma or radicular cyst may develop. • Cardiac toothache: Cardiac pain is clinically characterized
by heaviness, tightness or throbbing pain in the substernal
Chronic Pulpal Pain region which commonly radiates to the left shoulder, arm,
neck and mandible. It is the referred pain felt in the jaws
• Inflammatory changes go into a chronic phase.
with its origin in the cardiac region.
• There is neither resolution of the pulpal lesion nor necrosis.
• Neuropathic toothache: Neuropathic pain is usually
• Chronic pulpitis may develop.
caused by abnormalities in the neural structures. It may
• Usually due to trauma to a young permanent tooth.
occasionally be misdiagnosed as psychogenic pain as local
• Less responsive but positive to the pulp tester.
factors cannot be visualized. Neuropathic pain may be seen
• Internal resorption may occur. as neuralgia, neuritis or neuropathy.
• Pain may be severe or mild. • Sinus or nasal mucosal toothache: Sinus or nasal mucosal
Recurrent Pulpal Pain toothache is usually expressed as pain in the maxillary and/
or mandibular teeth. Signs and symptoms of sinusitis help
• Recurrent pulpal pain rarely occurs. in diagnosis along with aids like the paranasal sinus view,
• Inflammation usually follows either of two pathways: either computed tomography imaging and nasal ultrasound.
it may resolve and pain may cease or it may change in nature • Psychogenic toothache: In psychogenic toothache no
leading to pulp necrosis, gangrene or periapical abscess. damage to any local tissue is evident. It is included in
• Examples are partially split tooth opened by some unusual the category of mental disorders in which a patient may
occlusal stress. complain of a physical condition without the presence
• Recurrent hypersensitivity is a true example of recurrent of any physical signs. All possible diagnoses must be
pulpal pain. ruled out before making the diagnosis of psychogenic
• So called menstrual toothache and high altitude toothache. toothache.
130 Essentials of Pediatric Oral Pathology

CLASSIFICATION OF PULPAL PATHOSES — Abnormally responsive to heat

— Chronic
LOYOLA UNIVERSITY — Asymptomatic with pulp exposure
1. Inflammatory changes — Hyperplastic pulpitis
• Hyperalgesia — Internal resorption
— Hypersensitive dentin 2. Pulp degeneration
— Hyperaemia • Calcific (Radiographic diagnosis)
• Acute pulpalgia (acute pulpitis) • Others (Histologic diagnosis)
• Chronic pulpalgia (subacute pulpitis) 3. Necrosis.
• Chronic pulpitis
• Chronic hyperplastic pulpitis CAUSES OF PULP PATHOLOGY 4
• Pulp necrosis 1. Bacterial
2. Retrogressive (degenerative changes) A. Coronal ingress
• Atrophy • Caries
• Dystrophic calcification • Fracture
— Complete
INGLE’S CLASSIFICATION — Incomplete (cracks, infraction)
1. Inflammatory changes • Nonfracture trauma
• Hyperreactive pulpalgia • Anomalous tract
— Hypersensitivity — Dens invaginatus (aka dens in dente)
— Hyperemia — Dens evaginatus
• Acute pulpalgia—incipient, moderate, advanced — Radicular lingual groove (aka palatogingival
• Chronic pulpalgia groove)
B. Radicular ingress
• Hyperplastic pulpitis
• Caries
• Pulp necrosis
• Retrogenic infection
2. Retrogressive (degenerative changes)
— Periodontal pocket
• Atrophic pulposis
— Periodontal abscess
• Calcific pulposis
• Hematogenic
2. Traumatic
SELTZER AND BENDER’S CLASSIFICATION A. Acute
1. Inflammatory changes • Coronal fracture
• Intact pulp with scattered chronic inflammatory cells • Radicular fracture
• Acute pulpitis • Vascular stasis
• Chronic partial pulpitis with partial necrosis • Luxation
• Chronic partial pulpitis with partial liquefaction necrosis • Avulsion
• Chronic partial pulpitis (hyperplastic form) B. Chronic
• Pulp necrosis • Adolescent female bruxism
2. Retrogressive (degenerative changes) • Traumatism
• Atrophic pulp • Attrition or abrasion
• Dystrophic mineralization • Erosion
3. Iatral
GROSSMAN’S CLASSIFI CATION A. Cavity preparation
• Heat of preparation
1. Pulpitides • Depth of preparation
• Reversible • Dehydration
— Symptomatic • Pulp horn extensions
— Asymptomatic • Pulp hemorrhage
• Irreversible • Pulp exposure
— Acute • Pin insertion
— Abnormally responsive to cold • Impression taking
Pulp Pathologies in Children 131

B. Restoration • This results in vasodilatation due to increased vascular

• Insertion permeability.
• Fracture • Decreased flow resistance occurs.
— Complete • Protein filtration occurs.
— Incomplete • Tissue becomes edematous.
• Force of cementing • Venules are compressed.
• Heat of polishing • Blood flow becomes sluggish.
C. Intentional extirpation and root canal filling • Rheologic changes occur.
D. Orthodontic movement • Hypoxia prevails leading to impaired waste removal.
E. Periodontal curettage • Van Hassel5 has explained the circumferential spread of
F. Electrosurgery inflammation.
G. Laser burn
H. Periradicular curettage COMPENSATORY MECHANISM
I. Rhinoplasty
J. Osteotomy • AVA vessels open and shunt the blood.
K. Intubation for general anesthesia • Increase in tissue pressure occurs.
4. Chemical • Macromolecules are pushed back into the bloodstream in
A. Restorative materials a healthy region.
• Cements • Tissue pressure thus decreases back to the normal range.
• Plastics • Restoration of normal blood flow occurs.
• Etching agents Response of the pulp to any insult depends on:
• Cavity liners • Strength and duration of irritant
• Dentin bonding agents • Extent of pulp tissue affected
• Tubule blockage agents • Prior health status of the pulp.
B. Disinfectants
• Silver nitrate CORONAL INGRESS OF BACTERIA
• Phenol
• Sodium fluoride • Coronal caries is the most common means of ingress to
C. Desiccants the dental pulp.
• Alcohol • Langeland, 1959, noted pulp reactions in superficial fissure
• Ether caries also.6
• Others • Brannstrom and Lind, 1965, also noted pulp reaction in initial
5. Idiopathic enamel caries with no evidence of radiographic penetration.7
A. Aging • Only 60 percent caries is actually detected by radiographs,
B. Internal resorption due to a variety of factors: either inherent limitations in
C. External resorption the technique or operator related deficiencies.
D. Hereditary hypophosphatemia
E. Sickle cell anemia Contradiction
F. Herpes zoster infection
G.. Human immunodeficiency virus (HIV) and acquired • Reeves and Stanley, 1966, stated that little inflammation
immune deficiency syndrome (AIDS). was observed with bacteria within 1.1 mm of pulp.8
• Inflammation increases when lesion reaches within 0.5 mm
of the pulp.
PULPAL REACTION TO AN INSULT
• Generalized systemic bacteremia or anachoresis may occur
• Initial insult results in profound decrease in pulpal blood with diffusion of the bacteria into the blood stream.
flow to the site of insult.
• Increase in flow of blood through arteriovenous anasto- Permeability
moses (AVA) occurs to reduce the amount of blood at the
site of insult. • Dentin acts as a barrier but is permeable
• Localized inflammatory lesions in the pulp occur (Fig. 3.6). • Most permeable—Dead tract dentin (Figs 3.7 and 3.8)
• Chemical mediators of inflammation are released in the • Next—Primary dentin
vicinity. • Less permeable—Irritation dentin.
132 Essentials of Pediatric Oral Pathology

FIGURE 3.6: Circumferential spread of inflammation

FIGURE 3.8: Dentin permeability after formation of

dead tracts, sclerotic dentin, reparative dentin
FIGURE 3.7: Bacteria penetrating through dentinal tubules
Pulp Pathologies in Children 133

Pulpal diffusion of microbial products depends on:

• Thickness of remaining dentin
• Surface area of exposed, permeable dentin
• Presence of a smear layer
• Potency of the microbial products
• Rate of pulpal blood flow.

PHYSICAL AGENTS AFFECTING THE PULP

• Stanley, 1959, stated that speeds of 50,000 rpm and greater
are acceptable.
• 3000 to 30,000 rpm are most harmful to the pulp.9
• Effects of pulpal irritation are reduced by the production FIGURE 3.9: Diagrammatic representation of the interface between
of irritational dentin. restoration and tooth
• A remaining dentin thickness (RDT) of 2 mm is most
suitable.
• 20 fold increase in dentin permeability from RDT–3 mm
to RDT–0.5 mm.
• Shortly after cutting there is an appreciable decrease in
dentin permeability.
• Kim, 1985, stated that periodontal ligament injection
should not be administered for anesthesia for restorative
procedures in vital teeth as it alters the blood flow to the
pulp and hampers the compensatory mechanism of the
pulp.10
Thermal agents causing an insult to the pulp:
• Friction from cutting tools
— Degree of frictional heat depends on:
i. Size and type of cutting tool
ii. Speed of rotation FIGURE 3.10: Factors affecting pulp reaction to a restoration
iii. Cavity depth
iv. Effectiveness of coolant PULPAL REACTION TO RESTORATION
• Metal fillings without proper insulation (FIGS 3.9 AND 3.10)
• Heat during setting of cement
• During polishing of a restoration. • Chemical effects due to acidity of the restorative material.
• Microleakage through miniscule gaps in the restoration in
CHEMICAL AGENTS INSULTING THE PULP which bacteria grow.
• Full crown restorations also exhibit some amount of
• Disinfecting chemical applied to exposed dentin causes leakage.
severe damage when RDT = 0.6 mm. • Silicate > composite > amalgam > zinc oxide eugenol in
Hydrogen peroxide may cause emboli and even arrest terms of irritation to the pulpal tissue.
pulpal circulation, and hence is not currently recommended • ZOE most effective sealing agent against bacterial leakage.
for disinfecting a prepared cavity. • Factors affecting pulpal reaction to restoration:
• Dentin-conditioning and bonding agents — Law of diffusion as applied to the dentin affects the
• Etchants reaction of the pulp to a restorative material.
• Acid-liquid components of cements — Microleakage with a restoration.
— GIC luting cement has a longer period of acidity than — Contraction gap of the restoration.
zinc phosphate or polycarboxylate but is more — Tab on age of restoration.
biocompatible to the pulp due to the larger size of
polyacrylic molecules. Brannstrom, 1984, gave the following recommendations:11
• Acrylic monomer • Smear layer formed on dentinal cutting fosters bacteria,
• Eugenol diffusion into the tubules may be beneficial or hence antibacterial detergent should be used to remove
irritating. superficial smear layer.
134 Essentials of Pediatric Oral Pathology

• Residual smear plugs to be retained resulting in reduced HYPERSENSITIVITY

permeability.
• Hydrophilic liner under restoration to be placed to DEFINITION
counteract contraction gap. • Dentin hypersensitivity is characterized by short, sharp
pain arising from exposed dentin in response to stimuli,
REVERSIBLE PULPITIS typically thermal, evaporative, tactile, osmotic or chemical
DEFINITION and which cannot be ascribed to any other dental defect
or pathology.
Mild to moderate inflammatory condition of the pulp caused • Graf and Galasse, 1977, reported that hypersensitivity
by noxious stimuli in which the pulp is capable of returning to affects 1 of 7 adult dental patients.12
an uninflammed state following removal of stimuli.
ETIOPATHOGENESIS
ETIOLOGY
• It is caused due to exposed dentinal tubules, leading to
Any noxious stimulus caused by trauma, disturbed occlusal activation of A—delta fibers.
relationship, thermal shock, etc.
• The hydrodynamic theory is an excellent attempt at
explaining the pathogenesis of hypersensitivity. This
PATHOGENESIS theory states that sensitivity is proportional to the
• Acute inflammatory reactions are limited to the hydraulic conductance of dentin.
odontoblastic and sub-odontoblastic regions adjacent to the — Hydrodynamic theory: The dentinal tubules contain
dentinal tubules involved. fluid called dental lymph. Fluid movement in the
• The odontoblastic nuclei may be displaced into the tubules dentinal tubules stimulates the pain mechanism by
due to increased local tissue pressure. mechanical disturbance of the nerve closely associated
• The pulp tissue after repair is less vascular, less cellular with the odontoblastic process. When dentin is
and more fibrous than before, hence less able to withstand exposed for the very first time, very small blebs of fluid
a subsequent insult. are visible on the pulpal wall of the cavity or the wall
of freshly cut dentin which is nearest to the pulp. If
CLI NICAL FEATU RES the cavity is dried using an air spray, more fluid is lost
• Sharp pain lasting for a moment. resulting in more fluid movement leading to more pain.
• More often by cold food and beverages and cold air. Due to profuse branching of tubules at the dentino-
• Pain is not spontaneous in nature. enamel junction, the sensitivity is more. This is the
• Stops as soon as the cause is removed. most accepted theory of pain transmission through
• Tooth responds to electric pulp testing at a lower current. dentin.
• Pashley, 1986, stated that the most important variable is
HISTOPATHOLOGIC FEATURES the condition of tubule aperture.13
• Intradental nerves may be affected with excess Na or K
• Ranges from hyperemia to mild to moderate inflammatory around the nerve terminal.
changes in the area of the involved dentinal tubules. • This results in sensitivity to cold, air and heat presenting
• Reparative dentin, disruption of the odontoblastic layer, as pulpal pain.
dilated blood vessels, extravasation of fluids, immuno- • Inflammation of pulp occurs with excitation of C fibers
logical response may be seen.
leading to a release of CGRP (calcitonin gene related
• Chronic inflammatory cells with a few acute inflammatory
peptide) and substance P.
cells are seen.
• Increase in blood flow and capillary permeability occurs.
Management • Low compliance results in an increase in tissue pressure.
• Due to this, mild changes in pressure will excite the
1. Removal of noxious stimulus. intradental nerves resulting in a lowered threshold.
2. In case of caries, restoration of the tooth with an
• In fact, Trowbridge, 1985, stated that hypersensitivity is
appropriate restorative material is indicated.
the equivalent of sunburned skin.14
Pulp Pathologies in Children 135

• Dentin sensitivity rather than hypersensitivity would be a FOCAL REVERSIBLE PULPITIS / PULP HYPEREMIA
more appropriate term, although both terms could be
suitable. • Excessive accumulation of blood within the pulp tissue
leading to vascular congestion.
Management
• Capillary bed engorgement with a predisposition to edema
due to prolonged vasodilatation.
Certain chemical and physical agents have been used
in the management of dentinal hypersensitivity. PATHOPHYSIOLOGY (FIG. 3.11)
• Chemical agents • Dentin is the permeable imtermediary between the pulp and
– Corticosteroids the enamel and cementum.
– Silver nitrate • Due to the bidirectional patency of the tubules,
– Strontium chloride permeability is a major factor in the etiology of hyperaemia.
– Formaldehyde • Increase in the number of open dentinal tubules increases
– Calcium hydroxide the dentin permeability.
– Potassium nitrate • Permeability  r4, where r = radius of a dentinal tubule.
– Fluorides • Permeability 1/ length of the dentinal tubule.
– Sodium citrate • Pulpal disease state
– Iontophoresis with 2 percent sodium fluoride
Number of organisms × Virulence
– Potassium oxalate. = ______________________________________
• Physical agents Tissue resistance
– Composites
– Resins
• Sarnat and Massler, 1965, stated that caries may be active
– Varnishes or arrested. Arrested caries usually has a sclerotic zone in
– Sealants dentin. Hence shows a less incidence of pulp hyperemia.15
– Soft tissue grafts • Pulp hyperemia is a potentially reversible response.
– Glass-ionomer cements
– Lasers.

CRACKED TOOTH SYNDROME

• Why is it justified to call cracked tooth condition as a
syndrome?
• Syndrome can be understood as syn means together and
dromos means running along. Put together, it means an
appearance of two or more features. Cracked tooth syndrome
shows the following features:
— Sharp, momentary pain on release of pressure.
— Patient complains of pain ranging from mild to
excruciating, at the initiation or release of biting pressure.
— Crack in the tooth may involve enamel and dentin or
may even involve pulp.
— Pain mostly due to fluid movement in dentinal tubules
causing stimulation of sub-odontoblastic nerve fibers.
• Crack can be visualized by using a dye or by trans-illuminat-
ing the tooth with fiber-optic light or by selective biting
pressure on cotton roll or small wooden stick or rubber.

Management
1. Splinting of the offending cusp with cusp protecting
restoration.
2. Removing the split cusp and restoring the tooth.
FIGURE 3.11: Pathophysiology of pulp hyperemia
136 Essentials of Pediatric Oral Pathology

CLI NICAL FEATU RES • Pulp exposure caused by a faulty cavity preparation.
(SIMILAR TO REVERSIBLE PULPITIS) • Trauma to tooth or cracked tooth syndrome.
• Also may be due to acute exacerbation of chronic
• Sensitive to thermal changes, mainly cold.
inflammatory process.
• Pain disappears on removal of cold stimulus.
• Tooth responds to electric pulp testing at a lower current. CLI NICAL FEATU RES
• Tooth usually shows a deep carious lesion or a large
• Severe pain due to thermal changes.
metallic restoration or a restoration with defective margins.
• Pain does not subside on removal of the stimulus.
HISTOPATHOLOGIC FEATURES • Severity of pain increases with time, may be described as
lancinating.
• Acute extravasation of RBCs and some diapedesis of • Intensity of pain increases on lying down.
WBCs. • Application of heat may cause acute exacerbation of pain.
• Slowing of blood flow and hemoconcentration may result • Tooth reacts to the electronic pulp tester at a lower level
in thrombosis. of current.
• Engorged pulpal vessels (Fig. 3.12). • Pain present due to lack of escape of the inflammatory
• Cell free zone obscured by inflammatory cells. exudate and increase in intrapulpal pressure.
• Small hemorrhages present in the pulp. • Patient extremely uncomfortable and mildly ill.
• Dentinoblastic layer partially disrupted. • Pain from a vital pulp without tenderness to percussion.
• If prolonged insult, predentin is decreased in width.
HISTOPATHOLOGIC FEATURES
Management • Pulp may show chronic nature.
Removal of pulp irritants before the pulp is severely • Pulp could be in a serous stage, suppurative stage or a
damaged. combination.
• Exudation closest to the point of irritation results in the
ACUTE PULPITIS acute symptoms.
• Vasodilatation, fluid exudation and leukocyte infiltration.
Extensive inflammation of the pulp, it is a frequent sequel of • Inflammation confined to coronal pulp.
focal reversible pulpitis. • Localized destruction of pulp tissue and formation of pulp
ETIOLOGY abscess.
• Pulp abscess seen as a small void surrounded by a dense
• Usually occurs in a tooth with a large carious lesion or a band of leukocytic infiltration.
defective restoration or secondary caries. • Odontoblasts near the abscess undergo degeneration.

Management
1. Depending on whether the pathologic process is
reversible or irreversible, treatment is palliative or
invasive.
2. Treatment required is judged by knowledge of
inflammatory spread in pulp and its non-compliant
nature; this may include a vital or nonvital
pulpotomy or a pulpectomy.

CHRONIC PULPALGIA (SUBACUTE PULPITIS)

CLI NICAL FEATU RES

• Intermittent episodes of mild to moderate pain by transient
pressures from the exudative zone.
• Sometimes described as a smouldering inflammatory
response.
• Word "grumble" used when describing discomfort from
FIGURE 3.12: Pulp hyperemia showing engorged blood vessels chronic pulpalgia.
Pulp Pathologies in Children 137

• Throbbing pain due to pulsations. The exudative products:

• Only peaks of pulses reach suprathreshold levels. – Are absorbed into circulation.
• Later spontaneous, constant pain. – Spread/point into adjacent connective tissue.
• If trigger is present, pain continues on removal of the irritant. – Drain into the carious lesion.
– A combination of the above.
HISTOPATHOLOGIC FEATURES
• Prolonged dilation and engorgement—hyperemia. CLI NICAL FEATU RES
• Increased vascular permeability. • Pain is not a prominent feature, but if present, is dull
• Fluid exudation. aching in nature, more often intermittent than continuous.
• Increased protein content in tissue spaces. • Response to electric pulp vitality tester is reduced.
• Leukocyte infiltration. • Pulp tissue may be exposed.
• Localized abscess formation.
• Injured and dead cells and products of proteolysis act as HISTOPATHOLOGIC FEATURES
secondary irritants of the pulp.
• Sclerotic and irritation dentin produced.
• Phagocytosis and autolysis occurs.
• Slow moving caries starts destroying the dentinal barrier.
• Suppurative core is formed.
• Pulp finally exposed.
• By this stage partial necrosis occurs. • Similar process of inflammation.
• Zones of necrosis/infection, contamination, irritation/
Management proliferation are seen (Fig. 3.13).
1. Depending on whether the pathologic process is • Healing response shows formation of repair tissue.
reversible or irreversible, treatment is palliative or
invasive. Management
2. In initial conditions, removal of the causative factor Pulpectomy or root canal treatment followed by crown
may result in resolution of the pain. restoration.
3. In later stages, pulp therapy may be required.
PULPAL GRANULOMA
ACUTE PULPITIS WITH APICAL PERIODONTITIS • Granulomatous tissue formed, not just granulation tissue.
• Kronfeld's mountain pass concept, 1939—mainly for
CLI NICAL FEATU RES periapical granuloma.17 Kronfeld has explained that a
• Tooth tender to percussion. granuloma does not favour the growth of bacteria. He
• Heat causes intense pain. compared the bacteria in the root canal with an army
• Cold relieves the pain. entrenched behind 'high and inaccessible mountains', the
• May be sensitive to both heat and cold. foramina serving as mountain passes. The exudative and
• Inflammatory response extends to the periapex.
• Both pain and pressure receptors respond with pain.
• Periapex may be normal; frequently, widening of PDL space
seen.

Management

Complete pulpectomy required or pulp near the apex

must be removed to relieve pain.

NONPAINFUL PULPITIS
In nonpainful pulpitis, proliferative/chronic forces are
hyperactive. There is decreased exudative inflammatory FIGURE 3.13: Zones of inflammation in
activity, hence decrease in intrapulpal pressure. the pulp described by Fish16
138 Essentials of Pediatric Oral Pathology

granulomatous tissue of the granuloma represents a mobilized

army defending the plains (periapex) from the invaders
(bacteria). When a few invaders enter the plain through the
mountain pass, they are destroyed by the defenders
(leucocytes). A mass attack of invaders results in a major battle
analogous to acute inflammation. Only complete elimination
of the invaders from their mountainous entrenchment will
eliminate the need for a defence force in the 'plains'. Once
this is accomplished, the defending army of leucocytes
withdraws, the local destruction created by the battle is
repaired, and the environment returns to its normal pattern.

CHRONIC HYPERPLASTIC PULPITIS FIGURE 3.14: Deciduous mandibular right second molar
showing a pulp polyp
• Also called as “pulp polyp” or “pulpitis aperta”.
• Productive pulpal inflammation due to an extensive carious
exposure of young pulp.
• Characterized by the development of granulation tissue,
covered at times by epithelium and resulting from long
standing low grade infection.

ETIOLOGY
• Slow progressive carious exposure of pulp.
• A large open cavity, young resistant pulp and chronic low
grade stimulus is necessary.
• Mechanical irritation from chewing and bacterial infection
also provides stimuli.

CLI NICAL FEATU RES

• Most commonly involved are deciduous molars and first
permanent molars as they have an excellent blood supply
because of large root opening, coupled with high tissue
resistance and reactivity in young persons.
• Seen in children and young adults.
• Appears as a red fleshy pulpal mass filling the pulp
chamber or cavity or extending beyond the confines of FIGURE 3.15: Specimen of a deciduous
tooth (Figs 3.14 and 3.15). mandibular molar with a pulp polyp
• Relatively nonpainful to touch.
• May cause discomfort during mastication.
• Bleeds easily due to rich network of blood vessels. • Due to absence of nerve endings, pain occurs only on
HISTOPATHOLOGIC FEATURES pressure from mastication.

• Surface covered by stratified squamous epithelium. Management

• Epithelium derived from
— Gingiva Depending on the progress of the pathologic process,
extirpation of pulp or extraction of the tooth may be
— Freshly desquamated epithelial cells of oral mucosa or
required.
tongue.
• Granulation tissue projects from the pulp into carious lesion.
IRREVERSIBLE PULPITIS
• Granulation tissue is vascular containing collagen fibers,
blood vessels, inflammatory cell infiltrate chiefly consisting Persistent inflammatory condition of the pulp, which may be
of neutrophils, plasma cells and lymphocytes (Fig. 3.16). symptomatic or asymptomatic and is caused by noxious stimuli.
Pulp Pathologies in Children 139

Management
Complete removal of pulp and placement of intracanal
medicaments like cresatin, eugenol or formocresol.

PULP DEGENERATION
Degeneration is usually present in older aged people.

ETIOLOGY
Result of persistent, mild irritation in the teeth.

CLINI CAL FEATU RES

• Early stages—No symptoms.
• Later stages—Discoloration of tooth. No response of pulp
to stimuli.

TYPES
FIGURE 3.16: Carious dentin on the edges of soft tissue growth
projecting from the carious lesion, the pulp chamber being highly • Calcific degeneration
vascular and has abundant chronic inflammatory cell infiltrate — Part of pulp tissue replaced by calcific material, i.e.
formation of pulp stones and denticles
ETIOLOGY — Calcific material has onion skin appearance, lies
• Bacterial involvement of pulp through caries. unattached within body of pulp
• Chemical, thermal or mechanical injury may also be the cause. — In another type, calcific material is attached to the wall
of the pulp cavity called as diffuse calcification
CLI NICAL FEATU RES • Atrophic degeneration
— Observed in older people
• Pain may be caused by sudden temperature changes like — Pulp tissue less sensitive than normal
cold, sweet and acid foodstuffs. • Fibrous degeneration
• Pain continues after removal of cause. — Replacement of cellular elements by fibrous connective
• Pain is sharp, piercing or shooting. Pain may be intermittent tissue
or continuous. – Pulp has characteristic appearance of a leathery fiber
• Change of position or posture exacerbates the pain due to • Pulp artefacts
change in intrapulpal pressure. Patient may stay awake at — Vacuolization of odontoblasts was once thought to be
night due to pain brought about by change in posture. a type of pulp degeneration characterized by empty
• Pain may radiate to sinuses in case of upper teeth spaces occupied by odontoblasts
involvement or to the angle of mandible if lower teeth are
— It is an artefact caused by poor fixation of the tissue
involved.
specimen
• In later stages, pain is more severe and generally gnawing
• Tumor metastasis
or throbbing.
— Rare
• Pain increased by heat and relieved by cold.
— May occur due to direct local extension from the jaw.
HISTOPATHOLOGIC FEATURES
PULP CALCIFICATIONS
• Chronic inflammatory response may be seen in pulpal
tissue. • May be located in pulp chambers or in root canals
• Postcapillary venules get engorged, necrosis of pulp. • No sex predilection
• Necrotic areas attract polymorphonuclear leukocytes. • May occur in any teeth in either of the dental arches
• Necrotic debris, dead cells form pus. • Basically of two types:
• Various areas of microabscesses are formed lined by 1. Discrete pulp stones (denticles, pulp nodules)
fibrous wall. 2. Diffuse calcification.
140 Essentials of Pediatric Oral Pathology

FIGURE 3.18: Free denticle within the pulp, lined by dentin

— They are larger than true denticles and mostly occupy

pulp chamber
— They are also classified as free or attached.
• Interstitial denticles
— After deposition of calcified material, a stage comes
when this material is in apposition with the dentinal wall.
— This material is then surrounded by secondary dentin
FIGURE 3.17: Schematic representation of formation and is then called as interstitial denticle.
of pulp stones
DIFFUSE CALCIFICATION
ETIOLOGY • Also called calcific degeneration
Refer Fig. 3.17. • Amorphous unorganized linear strands or columns parallel
with blood vessels and nerves of the pulp (Fig. 3.19).
DISCRETE PULP STONES
RETICULAR ATROPHY OF PULP
They are of following types:
• True denticles • Degenerative or regressive change of pulp.
— Made of local masses of calcified tissue • Tooth is symptomless and responds normally to the vitality
— Resemble dentin due to their tubular structure test.
— More common in pulp chamber than root canals. • Presence of large vacuolated spaces in the pulp with
They are subdivided into two: reduction in number of cellular elements.
1. Free denticle: Lying entirely within pulp tissue and is • Degeneration and disappearance of odontoblasts.
not attached to the dentinal wall (Fig. 3.18).
2. Attached denticle: Continuous with dentinal wall. PULP NECROSIS
• False denticles May be partial or complete.
— Composed of localized mass of calcified material
— Composed of concentric layers or lamellae deposited ETIOLOGY
around a central nidus • Sequelae of inflammation
— Composed of cells surrounded by reticular fibers that • May occur following trauma in which pulp is destroyed
calcify before an inflammatory reaction.
Pulp Pathologies in Children 141

R EFER EN CES
1. Grossman LI. Endodontics 1776-1976: a bicentennial history
against the background of general dentistry. J Am Dent Assoc
1976;93:78.
2. Okeson JP. Bell’s Orofacial Pains: the clinical management of
orofacial pain. Quintessence Books, sixth edition, 2006.
3. Clark GL, Schmitt FO, Bear RS. Structure of nerve fibres.
Radiology 1935;25:131.
4. Sim on J HS, Walton R E, P ash ley DH , D ow den WE ,
Bakland LK. Pulpal pathology. In: Ingle JI, Bakland LK,
editors. Endodontics. 4th ed. Baltimore: Williams and
Wilkins, 1994.
5. Van Hassel HJ, Brown AC. Effect of temperature changes on
intrapulpal pressure and hydraulic permeability in dogs. Arch
Oral Biol, 1969;14:301.
FIGURE 3.19: Diffuse calcification within the pulp, 6. Langeland K. Histologic evaluation of pulp reactions to
surrounded by dentin on both sides operative procedures. Oral Surg, 1959;12:1235.
7. Brännström M, Lind PO. Pulpal response to early caries. J
TYPES
Dent Res 1965;44:1045.
• Coagulation necrosis: 8. Reeves R, Stanley HR. The relationship of bacterial penetration
Soluble portion of tissue is precipitated or converted into and pulpal pathosis in carious teeth. Oral Surg, 1966;22:59.
solid material. 9. Stanley HR. Traumatic capacity of high speed and ultrasonic
• Liquefaction necrosis: dental ... early human pulp reactions to full crown preparations,
Results when proteolytic enzymes convert the tissue into JADA 1959;59.
softened mass, liquid or amorphous debris.
10. Kim S. Ligament injection: a physiological explanation of its
CLI NICAL FEATU RES efficacy. J Endod 1986;12:486.
11. Brannstrom. Communication between the oral cavity and the
• No painful symptoms.
dental pulp associated with restorative treatment. Operative
• May cause swelling or distension of periapical tissue.
Dent 1984;9:57.
• Discoloration of tooth.
• Tooth with partial necrosis may respond to thermal changes 12. Graf H, Galasse R. Morbidity, prevalence and intraoral
owing to few vital nerve fibers. distribution of hypersensitive teeth. J Dent Res, Special issue
• History of severe pain lasting from few minutes to few hours A 1977;162:2
followed by cessation of pain. 13. Pashley DH. Dentin permeability, dentin sensitivity, and
treatment through tubule occlusion. J Endod 1986;12:465.
HISTOPATHOLOGIC FEATURES 14. Trowbridge J. Hypersensitivity. J of Endodontics 1985;11:489:
497.
• Necrotic pulp tissue, cellular debris and microorganisms.
15. Sarnat H, Massler M. Microstructure of active and arrested
• Periapical tissue may be normal or there is slight inflammation.
dentinal caries. J Dent Res 1965;44:1389-1401.
16. Fish EW. The pathology of the dentine and the dental pulp.
Management
Br Dent J 1933;53:351.
1. Complete debridement necessary. 17. Castellucci A. Chapter 8: Periapical disease. In: Castellucci A,
2. Root canal therapy. Blumenkranz U (Eds). Tridente 1:160.
4
142 Essentials of Pediatric Oral Pathology

Sequelae of Pulp
Pathologies in Children
Shweta Dixit Chaudhary, Mayur Chaudhary

CHAPTER OVERVIEW
Introduction Osteomyelitis:
Classification Acute suppurative osteomyelitis
Parulis Infantile osteomyelitis
Acute alveolar abscess Chronic focal sclerosing osteomyelitis
Acute apical periodontitis Chronic osteomyelitis with proliferative periostitis
Acute exacerbation of chronic lesion Cellulitis (phlegmon)
Chronic alveolar abscess Ludwig's angina
Granuloma Intracranial complication of dental infections:
Apical periodontal cyst Cavernous sinus thrombosis/thrombophlebitis
External root resorption Focal infection

INTRODUCTION TABLE 4.1: WHO 1995.1 Classification of diseases

of periapical tissues
The reaction of the pulp to initial insult has been discussed
Code No. Category
in the previous section. On persistence of the insult, the pulp
may not be able to recuperate, leading to one of the sequelae K04.4 Acute apical periodontitis
of pulpal pathology which will be discussed in the present K04.5 Chronic apical periodontitis (Apical granuloma)
section. K04.6 Periapical abscess with sinus (Dentoalveolar abscess
with sinus, Periodontal abscess of pulpal origin)
K04.60 Periapical abscess with sinus to maxillary antrum
CLASSIFICATION OF DISEASES OF
K04.61 Periapical abscess with sinus to nasal cavity
PERIRADICULAR TISSUES K04.62 Periapical abscess with sinus to oral cavity
• WHO classification does not take into consideration the K04.63 Periapical abscess with sinus to skin
structural aspects of diseased tissues (Table 4.1). K04.7 Periapical abscess without sinus (Dental abscess
without sinus, dentoalveolar abscess without sinus,
• It is a histopathologic classification based on: periodontal abscess of pulpal origin without sinus)
— Distribution of inflammatory cells within the lesion, K04.8 Radicular cyst (Apical periodontal cyst, periapical cyst)
— The presence or absence of epithelial cells, K04.80 Apical and lateral cyst
— Whether the lesion has been transformed into a cyst, K04.81 Residual cyst
and K04.82 Inflammatory paradental cyst
— The relationship of the cyst cavity to the root canal
of the affected tooth. ACUTE ALVEOLAR ABSCESS (FIG. 4.1)
• Grossman, 1991, proposed a classification based on the An acute alveolar abscess is a localized collection of pus in
clinical aspects of the diseased tissues (Table 4.2). the alveolar bone at the root apex of a tooth following death
Sequelae of Pulp Pathologies in Children 143

TABLE 4.2: Grossman's classification, 1991, of BACTERIOLOGY

diseases of periradicular tissues2
• Mixed microflora is seen with approximately 32 percent
1. Acute periradicular disease anaerobes3
• Acute alveolar abscess • Various species of streptococci, staphylococci, Bacteroides
• Acute apical periodontitis melaninogenicus have been seen
– Vital • Purulent discharge may be sterile with dead leukocytes and
– Nonvital dead bacteria.
2. Chronic periradicular diseases with areas of rarefaction
• Chronic alveolar abscess CLINICAL FEATURES
• Granuloma • Systemic reactions like fever, chills, malaise, headache, loss
• Cyst of sleep may be present
3. Condensing osteitis • Severe, throbbing pain in the region with localization of
4. External root resorption
pain
5. Diseases of periradicular tissues of non-odontogenic origin
• Swelling of the adjacent tissues
• Mobility of the involved tooth
• If left unattended, this may progress to osteitis, cellulitis
or osteomyelitis
• Sinus opening may be seen on the labial or buccal mucosa
with respect to the affected tooth, skin of patient's face,
neck, antrum or nasal cavity
• Sinus opening usually seen through the labial alveolar plate
in the maxillary arch
• Suppuration from the maxillary lateral incisor or from the
palatal root of deciduous molars and permanent maxillary
molars expresses palatally as the root of the maxillary
lateral incisor and palatal roots of maxillary molars lie
closer to the palatal plate of bone
• In the mandibular arch, suppuration usually expresses
FIGURE 4.1: Acute alveolar abscess in deciduous molar through the buccal alveolar plate
• May also occur through the lingual alveolar plate in the
of the pulp with extension of the infection through the apical mandibular molars because of close proximity of the
foramen into the periradicular tissues. roots.
Also commonly known as acute abscess, acute apical
abscess, acute dentoalveolar abscess, acute periapical abscess PARULIS
and acute radicular abscess. • At the intraoral opening of a sinus tract, often there is a
mass of subacutely inflamed granulation tissue known as
ETIOPATHOGENESIS parulis (gumboil)
• Trauma, mechanical irritation, chemical irritation • No symptoms of pain may be present if a non-vital tooth
• Carious tooth leading to bacterial invasion with necrosis is associated with parulis making it difficult to determine
of the pulp leading to a periapical abscess the causative tooth
• Infection extends in the direction of least resistance, i.e. • Gutta-percha point insertion into the tract during
through apical foramen radiographic examination can aid in the diagnosis.
• Contained pus may break through and a sinus tract opening
in the labial or buccal mucosa, skin of patient’s face, neck, RADIOGRAPHIC FEATURES
antrum or nasal cavity may be seen • As lesion is present for short period of time and confined
• At times, there is liquefaction of pus due to enzymes trypsin to medullary bone, no destruction of alveolar bone seen
and cathepsin • Slight widening of periodontal ligament membrane is seen
• Point at which pus breaks in the mouth depends on the • In long standing cases, periapical rarefaction will be
thickness of alveolar bone and the overlying soft tissues. evident.
144 Essentials of Pediatric Oral Pathology

BACTERIOLOGY
• Microorganisms mostly involved are streptococci and
staphylococci.
• Sundqvist used anaerobic culture methods and detected
Bacteroides melaninogenicus in cultures.4

HISTOPATHOLOGIC FEATURES
• Area of suppuration composed of :
— Central area of disintegrating polymorphonuclear
leukocytes (Fig. 4.2)
— Dilatation of blood vessels
— Tissue around area of suppuration contains serous
exudate.
FIGURE 4.2: Acute alveolar abscess showing numerous
Management polymorphonuclear leukocytes and dilated blood vessels

1. Immediate establishment of drainage. • External pressure on tooth forces edema fluid against
2. Pulpectomy or extraction as indicated of the already sensitized nerve endings and results in pain.
concerned tooth.
3. Control of systemic reactions.
RADIOGRAPHIC FEATURES
ACUTE APICAL PERIODONTITIS • In vital tooth:
— Little variation ranging from normal to widening of
Acute apical periodontitis is a painful inflammation of the
periodontal ligament.
periodontium as a result of trauma, irritation or infection • In non-vital tooth:
through the root canal, regardless of whether the pulp is vital — Changes range from widening of periodontal ligament
or non-vital. space and resorption of lamina dura due to destruction
Acute apical periodontitis is said to be primary when of apical bone resulting in well-demarcated radiolucency.
inflammation is of short duration, initiated within healthy
periodontium in response to irritants, and considered secondary HISTOPATHOLOGIC FEATURES
when acute response occurs in already existing chronic apical
periodontitis lesion (periapical flare-up, 'phoenix abscess'). • Dilated blood vessels.
• Presence of distinct polymorphonuclear leukocytes
ETIOLOGY (Fig. 4.3).
• Accumulation of serous exudate.
• In vital tooth due to: • Distension of periodontal ligament.
— Wedging of foreign object between teeth • Severe irritation leads to activation of osteoclasts and
— Trauma (overcontoured restoration) resorption of periapical bone.
• In non-vital tooth due to:
— Diffusion of bacteria and noxious products from Management
inflamed or necrotic pulp.
1. Determining the cause and relieving of symptoms.
— Iatrogenic cause: Forcing of bacteria or of irritating 2. Conservative treatment by removing the cause of
medicaments through apical foramen, over-instrumen- periodontitis in a vital tooth.
tation during cleaning and shaping of root canal, 3. Pulpectomy while avoiding the use of irritating
extension of root canal material through apical medicaments, overinstrumentation and overobtura-
foramen. tion.

CLINICAL FEATURES ACUTE EXACERBATION OF CHRONIC LESION

• Patient gives previous history of pulpitis. Acute exacerbation of a chronic lesion is also known as
• Tooth slightly elevated from the socket (due to edema). phoenix abscess after the bird phoenix which had arisen from
• Patient feels tenderness while biting. the ashes according to Greek mythology.
Sequelae of Pulp Pathologies in Children 145

FIGURE 4.3: Acute apical periodontitis showing dilated blood FIGURE 4.4: Phoenix abscess showing well-defined
vessels and presence of polymorphonuclear leukocytes periradicular radiolucency

This condition is an acute inflammatory reaction

superimposed on an existing chronic lesion, such as cyst or
granuloma.

ETIOLOGY
• Influx of necrotic products from diseased pulp.
• Bacteria and their toxins.
• Reactivation of dormant lesions such as granulomas and
cysts.

CLINICAL FEATURES
• Tooth tender on percussion.
• Tooth elevated from the socket.
• Mucosa over radicular area swollen and red.
FIGURE 4.5: Phoenix abscess showing necrotic area with
RADIOGRAPHIC FEATURES numerous inflammatory cells infiltrate

Well-defined periradicular radiolucent lesion (Fig. 4.4). Management

1. Establishment of drainage.
BACTERIOLOGY 2. Control of systemic reactions.
3. Pulpectomy.
Microorganisms are generally not seen in this lesion, if present,
are transient microorganisms.5 It is appropriate at this stage to discuss why intratreatment
pain occurs:
HISTOPATHOLOGIC FEATURES Intratreatment pain may be due to the following factors:
• Apical periodontitis secondary to treatment
• Areas of liquefaction necrosis with disintegrating — Throbbing, gnawing and/or pounding pain.
polymorphonuclear leukocytes and cellular debris — Cause is frequently overinstrumentation causing
• Areas of necrosis are surrounded by infiltration of pushing of necrotic debris and microbial by-products
macrophages and lymphocytes (Fig. 4.5). as well as microbes through the apical foramen.
146 Essentials of Pediatric Oral Pathology

— May be due to overmedication, overirrigation causing

irritation of the periapical tissues.
• Incomplete removal of pulp tissue
— Pain returns, after removal of coronal tissue only, during
an emergency treatment.
• Recrudescence of chronic apical periodontitis
— Also called phoenix abscess
— Exact reason not known
— May be due to change in environment
— May be due to organisms or at times may occur in
absence of organisms.

CHRONIC ALVEOLAR ABSCESS

Chronic alveolar abscess is also known as chronic suppurative
apical periodontitis. A chronic alveolar abscess is a long-
FIGURE 4.6: Gumboil
standing, low-grade infection of the periradicular alveolar bone.

ETIOLOGY
• Sequela of death of the pulp.
• Resulting from pre-existing acute alveolar abscess.

CLINICAL FEATURES
• History of sudden, sharp pain that subsided and did not
recur or history of a traumatic injury
• Generally asymptomatic
• Presence of sinus tract
• Sometimes healing of sinus tract may occur with elevation
of mucosa and “gumboil” (Fig. 4.6)
• Sinus tract in lower anterior teeth opens near symphysis
• In lower molars, it opens along the lower border of mandible
• The purulent material in deciduous teeth may pass into the FIGURE 4.7: Chronic alveolar abscess showing a mixture of
gingival sulcus giving the impression of a periodontal polymorphonuclear leukocytes, lymphocytes and plasma cells
pocket.
• Lymphocytes and plasma cells are seen at the periphery of
RADIOGRAPHIC FEATURES the lesion
• Fibroblasts may start to form a capsule at the periphery
• Diffuse area of bone rarefaction • Sinus tract generally lined by granulation tissue
• Widening of periodontal ligament space. • Harrison JW, Larson WJ, 1976, found that 1 in 10 sinus
tracts were lined by epithelium7
BACTERIOLOGY
• Grossman, however, examined tissue in the tract as well
• Block RM et al 1976, examined 230 periapical specimens as adjacent to the tract and found no evidence of epithelial
and found that bacteria were infrequently present6 lining of the tract.5
• Bacteria mostly involved are alpha-hemolytic streptococci
and obligate anaerobes. Management
1. Elimination of the infection of the root canal by
HISTOPATHOLOGIC FEATURES pulpectomy, thorough debridement and irrigation of
• Toxic products diffuse through apical foramen leading to the root canals.
detachment of periodontal ligament fibers at the root apex 2. Sinus tract heals by granulation tissue formation.
• Polymorphonuclear leukocytes are seen at the center of the 3. If healing is delayed, thorough curettage of the tract
is prescribed.
lesion (Fig. 4.7).
Sequelae of Pulp Pathologies in Children 147

GRANULOMA plasma cells, while non-immune granulomas show more

macrophages and giant cells
Granuloma may also be referred to as chronic apical periodonti- • When numerous plasma cells are present, scattered
tis. A dental granuloma is a growth of granulomatous tissue eosinophilic globules of gamma globulin called Russel
continuous with the periodontal ligament resulting from death bodies may be seen
of the pulp and diffusion of bacteria and bacterial toxins from • Clusters of light basophilic particles also may be present
the root canal into the surrounding periradicular tissues through in association with plasmacytic infiltrate called Pyronine
the apical and lateral foramina. bodies
The term is a misnomer as its tissue is principally • Researchers have found a vast majority of lymphocytes in
granulomatous comprising mostly of chronic inflammatory cell a granuloma but surprisingly, no immunoglobulin
infiltrate in the fibrous connective tissue stroma of the production was seen
granulation tissue. • Non-B lymphocytes of T-cell origin are seen making the
lesion an expression of delayed hypersensitivity
ETIOLOGY • T-cell activity results in expression of cytotoxic lymphokines,
• Death of pulp followed by mild irritation or infection of collagenase and mostly Osteoclast Activating Factor (OAF)
periapical tissue resulting in resorption of bone
• In some cases, it is preceded by chronic alveolar abscess • Macrophages and foreign body giant cells may also be
• Stern, 1979, demonstrated that a granuloma is a cell- present
mediated response to pulpal bacterial products.8 • Adams DO, 1976, stated that compared with granulomatous
inflammation, banal chronic inflammation is a diffuse
CLINICAL FEATURES heterogeneous collection of cells, usually dominated by
mononuclear cells other than macrophages, i.e. it lacks
• Nobuhara and del Rio showed that 59.3 percent of the
organization13
periradicular lesions were granulomas, 22 percent cysts, 12
• In some granulomas, a large number of phagocytes ingest
percent apical scars and 6.7 percent other pathoses9
lipid material and are called as foam cells.
• Involved tooth usually non-vital
• Deposition of cholesterol as well as hemosiderin is often
• May be slightly tender on percussion
present
• Mild pain on biting or chewing
• Cholesterol crystals appear microscopically as needle-like
• In some cases, the tooth is elongated into its socket.
spaces or clefts owing to dissolving of cholesterol during
processing of tissues
BACTERIOLOGY
• Connective tissue activity is prominent at the periphery of
• Post-extraction cultures were done by many authors and the lesion
they found the specimen to be positive, possibly due to • Condensation of bundles of collagen fibers is seen.
contamination of the specimen
• Burket L, 1938, found that a pre-extraction culture of
bacteria through the root canal or alveolar plate showed
the presence of Streptococcus viridans, Streptococcus
hemolyticus, non-hemolytic streptococci, Staphylococus
aureus, Staphylococcus albus, E. coli, pneumococci.10

HISTOPATHOLOGIC FEATURES
• Granulomatous tissue replaces alveolar bone and perio-
dontal ligament
• Rich vascular network, fibroblasts derived from periodontal
ligament
• The new capillaries formed are lined by swollen endothelial
cells
• Moderate infiltration of chronic inflammatory cell infiltrate
mainly composed of lymphocytes and plasma cells (Fig. 4.8)
• Athanassiades and Spears,11 Page and his associates12 stated FIGURE 4.8: Periapical granuloma showing moderate infiltration
that immune granulomas show more lymphocytes and of chronic inflammatory cell infiltrate
148 Essentials of Pediatric Oral Pathology

• Slow expansion of soft tissue mass leads to formation of • Chen SY et al 1981, by immunoperoxidase procedure
continuous capsule and separates granulation tissue from identified hyaline bodies as endogenous in origin.21
the bone
• Another important feature is the presence of epithelium, Management
which is mostly derived from epithelial cell rests of 1. Resolution of inflammation by removal of cause.
Malassez 2. Pulpectomy.
• In some instances, it arises from: 3. If not responding to treatment, extraction.
— Respiratory epithelium of the maxillary sinus in cases
where lesion has perforated the sinus wall Cellulose Granuloma
— Oral epithelium growing in through a fistulous tract
— Oral epithelium perforating apically from a periodontal • White EW, 1968, reported that particles of predominantly
pocket, or bifurcation or trifurcation involvement by cellulose containing material used in endodontic practice,
periodontal disease also with apical perforation. medicated cotton wool for apical seal, endodontic paper
• In early periapical granulomas, epithelium is confined to points for microbial sampling and drying of root canals may
the immediate vicinity of periodontal ligament get dislodged at the periapex.22
• Later, it may proliferate by inflammatory stimuli and wall • Sedgley CM, Messer H, 1993, stated that these particles,
off the irritants coming into the periapical region through when not degraded by body cells, result in a foreign body
the apical foramen reaction.23
• The epithelium forms sheets and anastomosing cords which
are mainly responsible for formation of periodontal cyst Gutta-percha
• Dunlap CL and Barker BF, 1977, coined the term giant • Gutta-percha is a root canal sealant used for obturation of
cell hyaline angiopathy for periapical granuloma14 the root canal during endodontic therapy
• It consists of inflammatory cell infiltrate, collection of • Gutta-percha cones get contaminated with tissue irritating
foreign body giant cells and ringlike structures called substances leading to a foreign body reaction
Rushton bodies (eosinophilic material resembling • Nair PNR et al 1990, stated that the most striking feature
hyalinized collagen). is the presence of multinucleated giant cells and birefringent
inclusion bodies24
FOREIGN BODY REACTIONS • Energy dispersive x-ray microanalysis demonstrated the
Oral Pulse Granuloma presence of magnesium and silicon in inclusion bodies.

• It denotes a foreign body reaction to particles of vegetable Other Foreign Materials

food materials; e.g.: leguminous seeds that get lodged in
oral tissues • Amalgam
• King,15 1978, and Mincer HH et al,16 1979, coined the term • Endodontic sealants
pulse granuloma • Calcium salts derived from periapically extruded calcium
• Also referred to as giant cell hyaline angiopathy, vegetable hydroxide.
granuloma and food induced granuloma
• Head MA, 1956, has reported pulse granuloma in lungs,17 APICAL PERIODONTAL CYST
Sherman FE, Moran TJ, 1954, have reported it in stomach Apical periodontal cyst is a slowly growing epithelial sac at
walls and peritoneal cavities18 the apex of a tooth that lines a pathologic cavity in the alveolar
• Associated with teeth grossly damaged by caries and history bone. It is also called as radicular cyst, periapical cyst and root
of endodontic therapy end cyst.
• Characterized by presence of intensely iodine and PAS
positive hyaline rings or bodies, surrounded by giant cells ETIOLOGY
and inflammatory cells
• Knoblich R, 1969, identified granuloma inducing agents • Physical, chemical or bacterial injury resulting in death of
to be cellulose, antigenic proteins and mitogenic phyto- the pulp followed by stimulation of epithelial cell rests of
hemagglutinins19 Malassez.
• Simon JHS, 1982, stated that vegetable food materials reach • In some instances, it arises from:
the periapex via root canals of teeth exposed to oral cavity, — Respiratory epithelium of the maxillary sinus in cases
by trauma, carious damage or endodontic procedures20 where lesion has perforated the sinus wall.
Sequelae of Pulp Pathologies in Children 149

— Oral epithelium growing in through a fistulous tract. discontinuities in the linings of apical cysts, this theory does
— Oral epithelium perforating apically from a periodontal not fully explain cyst formation.26
pocket, or bifurcation or trifurcation involvement by
periodontal disease along with apical perforation. Osmotic Pressure Theory
• This is the most widely accepted theory
PATHOGENESIS • Skaug, 1974, 1977, reported that plasma protein exudate,
• Initial reaction is proliferation of epithelial rests in hyaluronic acid, as well as products of cell breakdown
periapical area involved by granuloma. contribute to the high osmotic pressure of the cystic fluid27
• Proliferation is induced by keratinocyte growth factor • This osmotic pressure attracts more fluid and leads to cystic
elaborated by periodontal stromal cells. cavity formation and cyst expansion.
• Gao et al speculated that activated T-cells in periapical Immune Mediated Theory
granuloma caused lymphokine production leading to
proliferation of epithelial cell rests of Malassez. Altered • Development of cavities in proliferating epithelium is
differentiation of these cell rests leads to cyst formation.25 mediated by an immunologic reaction
• This epithelial proliferation displays an irregular pattern. • Immunocompetent cells in proliferating epithelium of
• Basal cell layer of epithelium proliferates resulting in the periradicular lesion, presence of immunoglobulins in cystic
central portion of the mass being separated from nutrition. fluids and discontinuity of epithelial lining in most of the
• Degeneration, necrosis and liquefaction of the central mass apical cysts are seen
of cells occur. • Activated epithelial cell rests of Malassez can obtain
• Thus, an epithelium-lined cavity filled with fluid is formed antigenicity and become recognized as antigens.
called as an apical periodontal cyst.
• The cyst increases in size by osmosis, fibrinolysis and CLINICAL FEATURES
continued epithelial proliferation. • Majority of cases are symptomatic and may result in a
prolonged, extremely troublesome and disconcerted course
THEORIES OF CYST FORMATION of events
• Nutritional deficiency theory • Commonly seen between 20 and 60 years but may occur
• Abscess theory at any age
• Osmotic pressure theory • Most commonly involved teeth are the maxillary anteriors
• Immune mediated theory. • Associated teeth are either non-vital, show a carious lesion
or may have undergone treatment
Nutritional Deficiency Theory • Sometimes, it undergoes acute exacerbation leading to a
• Central cells of epithelial strands removed from their source phoenix abscess which may proceed to cellulitis or may
of nutrition result in necrosis and liquefactive degeneration form a draining fistula.
• Accumulating products attract neutrophils in the necrotic
area RADIOGRAPHIC FEATURES
• Microcavities containing degenerating epithelial cells, • Localized radiolucent area surrounding the root of the
infiltrating leukocytes and tissue exudate coalesce to form involved tooth (Fig. 4.9)
a cystic cavity lined by stratified squamous epithelium • Occasionally, a thin radiopaque line around the periphery
• Torabinejad M, Walton RE stated that because there is no of radiolucent area is present indicating the reaction of bone
evidence of lack of blood supply and proliferating to a slowly expanding mass
epithelium is usually invaginated by connective tissue, this • Priebe WA et al 1954, decided on the clinical criteria for
theory is somewhat unsatisfactory.26 diagnosis, that an endodontically treated tooth if heals can
be called as a granuloma, if not or if it takes a longer time
Abscess Theory to heal can be called as a cyst.28
• Proliferating epithelium surrounds an abscess formed by
tissue necrosis and lysis because of the inherent nature of HISTOPATHOLOGIC FEATURES
epithelial cells to cover exposed connective tissue surfaces. • Similar to granuloma except for epithelium lined lumen.
• Torabinejad M, Walton RE stated that because of inherent • Epithelial lining is usually stratified squamous epithelium.
differences between epithelial cell rests of Malassez and • In a newly formed cyst, epithelial thickness is uneven and
epithelial cells of skin, and because of numerous often shows hyperplasia.
150 Essentials of Pediatric Oral Pathology

Cholesterol Crystals/Clefts
• Cholesterol stands for Chloe-stereos = “bile solid”.
• Cholesterol is a lipid of the steroid family. It is present in
almost all tissues, abundant in myelin and other membrane
rich tissues and cells.
• Cholesterol clefts are associated with multinucleated giant
cells.
• In histopathologic sections, narrow, elongated clefts are
seen because crystals dissolve in solvents during processing
leaving behind clefts.
• Crystals are believed to be formed from cholesterol released
by:
— Disintegrating erythrocytes of stagnant blood vessels
within the lesion.
— Lymphocytes, plasma cells and macrophages
disintegrate in chronic apical lesion.
— Circulating plasma lipids.
• Cholesterol crystals erode the epithelium and are found in
cystic lumen, such movement of crystals from epithelium
FIGURE 4.9: Periapical cyst showing localized to the cystic lumen is termed as glacier-like movement.
radiolucent area surrounding the root of the tooth • Cystic fluid when held in sunlight shows a golden colored
reflection, visible by naked eye. Such reflection is termed
• In an established cyst, epithelium has a regular appearance as shimmering effect.
and a fairly even thickness. • Accumulation of cholesterol crystals in body is called as
• Gardener AF, 1969, reported the possibility of development cholesteatoma.
of epithelial lining of this cyst into a carcinoma.29 • Nair PNR et al 1990, coined the term apical cholesteatoma
• Periapical true cyst is an apical inflammatory cyst with a to distinguish deposition of cholesterol crystals in periapex
distinct pathologic cavity, completely enclosed in an from accumulation of cholesterol crystals in other body
epithelial lining with no communication to root canal. organs.24
• Cholesterol crystals are hydrophobic in nature and thus
• Periapical pocket cyst is an apical inflammatory cyst
need to be esterified to mobilize into lipid droplets and
containing a sac-like epithelium-lined cavity which is open disperse into surrounding tissue fluids. Giant cells and
and continuous with the root canal. macrophages have the property of esterification by
• Interesting and peculiar structures seen are the Rushton incorporating phospholipids and lipoproteins into crystals.
bodies in epithelium. They are tiny, linear or arc shaped Thus, they are found around the cholesterol clefts.
bodies, associated with lining epithelium and are
amorphous in reaction and brittle in nature. Management
• Allison RT, 1974, reported the frequency of occurrence of
1. Nair PNR et al 1990, stated that “the presence of vast
Rushton bodies between 2.6 and 9.5 percent.30 number of cholesterol crystals would be sufficient to
• Sedano HO and Gorlin RJ, 1968, reported morphologic and sustain the lesion indefinitely”. So removal of such
histochemical similarity between these bodies and red blood crystals might resolve the lesion.24
cells.31 Rushton bodies arise from thrombus formation in 2. Root canal therapy with apicectomy in permanent
small capillaries—a Rouleau phenomenon. teeth; pulpectomy in deciduous teeth. Apicectomy is
• Connective tissue made up of parallel bundles of compressed not recommended in deciduous teeth as the apices
collagen fibers, numerous fibroblasts, blood vessels and of these teeth are usually in a state of resorption.
inflammatory cell infiltrate consisting chiefly of lymphocytes 3. Extraction of the involved tooth with curettage.
and plasma cells makes up the wall of the cyst.
• Dystrophic calcification may also be seen. EXTERNAL ROOT RESORPTION
• Contents of lumen vary from watery, straw colored, blood- External root resorption is a lytic process occurring in the
tinged fluid to semisolid materials. cementum or cementum and dentin of the roots of teeth. It is
• Low concentration of proteins is seen in this cyst. also called as odontoclasia.
Sequelae of Pulp Pathologies in Children 151

ETIOLOGY Management
• Periradicular inflammation due to trauma. 1. Management varies with the etiologic factor.
• Excessive masticatory forces. 2. If pulpal disease extends into supporting tissues, root
• Granuloma, cyst, central jaw tumors. canal therapy is recommended.
• Impaction of teeth. 3. If due to excessive orthodontic forces, reduction of
• Iatrogenic causes like excessive orthodontic forces. forces is recommended.
• Idiopathic causes. 4. Removal of impacted tooth should be done as soon
as resorption due to the impacted tooth is discovered
CLINICAL FEATURES to avoid further increase in the crown root ratio.

• Tooth involved may be asymptomatic. OSTEOMYELITIS

• It may be mobile.
• If resorption extends to the crown, it will give appearance Osteomyelitis is defined as inflammation of bone and its
of “pink tooth” seen in internal resorption. marrow contents. It is considered as an inflammatory condition
• If tooth gets ankylosed, it will remain in infraocclusion of bone that usually begins as an infection of the medullary
(“submerged tooth”). cavity, rapidly involves the Haversian system and quickly
extends to the periosteum (Fig. 4.12).
RADIOGRAPHIC FEATURES
CLASSIFICATION
• Concave or ragged areas on the root surface seen as a
radiolucent area (Fig. 4.10). • Suppurative osteomyelitis
• Blunting of apex. — Acute suppurative
• In case of ankylosis, no periodontal ligament space will be — Chronic suppurative
seen. a. Primary—No acute phase preceding
• In case of tumor, root resorption can be seen adjacent to b. Secondary—Follows acute phase
the radiolucent area. — Infantile
• Non-Suppurative osteomyelitis
HISTOPATHOLOGIC FEATURES — Diffuse sclerosing osteomyelitis
— Focal sclerosing osteomyelitis (condensing osteitis)
• Varies from small areas of cementum resorption replaced — Osteomyelitis with proliferative periostitis (Garré's
by connective tissue or repaired by new cementum, to large osteomyelitis)
areas of resorption replaced by osseous tissue, to “scooped — Osteoradionecrosis.
out” areas of resorption replaced by inflammatory or
neoplastic tissues. Multinucleated giant cells, i.e.
odontoclasts are seen resorbing the root surface (Fig. 4.11).

FIGURE 4.10: External root resorption showing FIGURE 4.11: External root resorption showing
radiolucencies in the root and adjacent bone multinucleated giant cells resorbing the root surface
152 Essentials of Pediatric Oral Pathology

Commonly seen in the pediatric population (first and Clinical Features

second decades of life) are acute suppurative osteomyelitis, • Common in the first and second decades with slight male
focal sclerosing osteomyelitis and osteomyelitis with predilection
proliferative periostitis. • May involve maxilla or mandible
• Usually localized in maxilla and diffuse and widespread
PREDISPOSING FACTORS in the mandible
• Trauma, jaw fractures • More common in posterior mandible in children and
• Gunshot wounds anterior maxilla in infants
• Radiation damage • Neonatal maxillitis, occurs in infants and young children
• Paget's disease • Occasionally caused by bacteremia
• Osteopetrosis • Local oral infection following minor trauma
• Systemic conditions (malnutrition, uncontrolled diabetes • Severe pain and trismus may be present
mellitus, sickle cell anemia, acute leukemia and chronic • Paraesthesia of lower lip occasionally may be present
alcoholism). • Elevation of temperature with regional lymphadenopathy
• Elevated white blood cell count
Acute Suppurative Osteomyelitis • Involved teeth become loose
• It is a serious sequela of periapical infection. • Exudation of pus may be seen usually near the lower border
• Diffuse spread of infection throughout medullary spaces. of mandible in children.
• Necrosis of variable amount of bone. Radiographic Features
• Dental infection is a frequent cause. • Little radiographic evidence until two weeks
• Usually of polymicrobial origin. • Individual trabeculae become fuzzy and indistinct
• Microorganisms involved are Staphylococcus aureus,
• Ill-defined margins with moth eaten appearance.
Staphylococcus albus, streptococci, various anaerobes such
as Bacteroides, Porphyromonas or Prevotella, Eikenella
corrodens, Mycobacterium tuberculosis, Treponema HISTOPATHOLOGIC FEATURES
pallidum, Actinomyces species. • Medullary spaces filled with inflammatory exudates, chiefly
• Also more common in patients of tuberculosis, syphilis, polymorphonuclear leukocytes, occasionally plasma cells
actinomycosis. and lymphocytes (Fig. 4.13).
• Osteoblasts bordering bony trabeculae are destroyed.
• Trabeculae may loose viability and undergo resorption with
time.

FIGURE 4.13: Acute suppurative osteomyelitis showing devitalized

lamellar bone with scalloped edges and absence of stainable
osteocytes and osteoblasts, edema and granulocytic infiltration of
FIGURE 4.12: Mechanism of formation of osteomyelitis surrounding tissues
Sequelae of Pulp Pathologies in Children 153

Management Chronic Focal Sclerosing Osteomyelitis

1. Debridement of necrotic tissue. • Also called as condensing osteitis.
2. Establishment of drainage through tooth or through • It is an unusual reaction of healthy bone to infection where
the area where it is pointing. tissues react to infection by proliferation rather than
3. Antibiotic therapy with culture and sensitivity testing destruction.
alongwith antipyretics. • Reaction to mild bacterial infection through a carious tooth
4. Adequate diet and avoidance of dehydration in or bacteria with low virulence.
children.
• Infection acts as a stimulus rather than an irritant.
5. Sequestrectomy and saucerization of bone.
Sequestrectomy has to be performed with utmost Clinical Features
caution because of risk of damage to the underlying • Most commonly seen in children and young adults chiefly
tooth bud. in the first and second decades with no gender predilection.
6. Decortication of bone. • Most commonly involved tooth is a carious mandibular first
7. Immediate intervention is recommended. molar.
• Patient complains of mild pain associated with an infected
Sequelae pulp.
Sequelae like periosteitis, soft tissue abscess, cellulitis and • Sclerotic bone at the periapex may impede the eruption of
pathologic fracture are not uncommon. succedaneous tooth.
Radiographic Features
Infantile Osteomyelitis
• Well-circumscribed radiopaque mass of sclerotic bone
• Fortunately it is uncommon with risks of involvement of surrounding and extending below the apex of one or both
eye, extension to dural sinuses and potential for facial roots, with margins blending into the surrounding bone.
deformities. • Intact lamina dura.
• Usually occurs a few weeks after birth. • Widened periodontal ligament space.
• Maxilla affected commonly. • This is the most common periapical radiopacity.
• Before the advent of antibiotics, mortality rate was Histopathologic Features
thought to be 30 percent, which has reduced drastically • Dense mass of bony trabeculae with little interstitial marrow
currently. tissue (Fig. 4.14).
• Believed to occur by hematogenous route or perinatal • Empty lacunae are seen.
trauma of oral mucosa from obstetrician's finger.
Clinical Features
• Malaise, hyperpyrexia, anorexia and dehydration.
• Inner and outer canthal swelling, palpebral edema, closure
of eye and proptosis may result.
• Maxilla on the affected side is swollen both buccally and
palatally.
• Staphylococcus aureus is the most common offending
organism. Streptococci have also been found in cultures.

Management

1. Intravenous antibiotics and drainage of abscess.

2. Antipyretics for management of elevated temperature.
3. Proper diet and hydration. Dehydration is often the
cause of fatality.
4. Sequestrectomy less preferred due to damage to
tooth bud. FIGURE 4.14: Dense mass of bony trabeculae with empty
lacunae and little interstitial marrow tissue
154 Essentials of Pediatric Oral Pathology

• Interstitial soft tissue is fibrotic with an abundance of Spread of Infection

lymphocytes. • An oral infection may originate in the dental pulp and
• Osteoblastic activity may have completely subsided by the extend through root canals into the periapical tissues. It may
time of microscopic study. then become localized or extend diffusely.
• The routes by which infection can spread are:
Management
— Lymphatic system
1. No treatment of bony lesion is required. — Blood streams
2. Manage the odontogenic infection by pulpectomy, root — Directly through the tissues.
canal therapy.
• Factors affecting the ability of infection to spread are:
3. Lesion may regress after treatment or may result in
a bony scar which may impede eruption of the — Type and virulence of organisms.
succedaneous tooth. — General health of the patient.
— Anatomical site of initial infection which decides
Chronic Osteomyelitis with Proliferative Periostitis drainage of pus.
— Immune status of the patient.
• Also called as Garré's chronic non-suppurative sclerosing
osteitis or periostitis ossificans. • Odontogenic infections often spread through natural
• First described by Carl Garré in 1893. pathways into potential tissue spaces situated between
• It is an irritation induced focal thickening of the periosteum different planes of fascia.
and cortical bone of the tibia. • Infections into the various tissue spaces are known as
space infections. Such infections in the vicinity of the
Clinical Features jaw bones can be divided into two broad groups, namely
• Occurs primarily in children, young adults and occasionally
those related to the maxilla and those related to the
in older individuals with no gender predilection.
mandible.
• Localized, hard, non-tender, unilateral bony swelling of
lateral and inferior aspects of mandible. • Examples of space infections are Ludwigs angina, cellulitis,
• Mild tenderness may occasionally be present on palpation. canine fossa infection, palatal space infection, infra
• Skin overlying the lesion appears normal. temporal space infection, submandibular space infection,
• Occurs in bicuspid and molar region commonly. etc.
• Maxilla is seldom affected.
• Patient complains of toothache and bony hard swelling on CELLULITIS (PHLEGMON) (FIG. 4.15)
the outer surface of the jaw. Cellulitis is a diffuse inflammation of soft tissues which is not
Radiographic Features circumscribed or confined to one area, but which in contra-
• Occlusal radiograph shows a focal overgrowth of bone on distinction to abscess, tends to spread through tissue spaces
the outer surface of the cortex. and along fascial planes.
• Diffuse, poorly-defined mixed radiopaque and lucent This type of reaction occurs as a result of infection by
expansile lesion. microorganisms that produce significant amounts of
• Duplication of cortical layer of bone (“onion skin streptokinase, hyaluronidase and fibrinolysins.
appearance”) (laminated appearance) is evident.
• Associated radiolucency with the involved tooth is seen. ETIOLOGY
Histopathologic Features • Streptococci are a common causative organism in case of
• Abundance of reactive osteoid tissue. cellulitis.
• Osteoblasts bordering many of the trabeculae are seen. • Cellulitis of face and neck most commonly results from
• Trabeculae are arranged perpendicular to the cortex. dental infection and infection following a dental
• Fibrous connective tissue shows diffuse or patchy sprinkling extraction.
of lymphocytes and plasma cells.
CLINICAL FEATURES
Management
• Patient is moderately ill.
1. Endodontic treatment or extraction of the involved • Temperature is elevated.
tooth. • Leukocytosis.
2. Antibiotic therapy.
• Painful swelling of soft tissues that is firm and brawny.
3. Expanded bone usually remodels without surgical
recontouring.
• Much of swelling is due to inflammatory edema.
• Regional lymphadenitis.
Sequelae of Pulp Pathologies in Children 155

• In younger children, dehydration is quick to develop.

• Infection may spread to parapharyngeal spaces, to the
carotid sheath or to the pterygopalatine fossa.
• Most of the cases are mixed infections.

Management

1. Antibiotic therapy.
2. Surgical drainage by stab incision.
3. Emergency tracheostomy to prevent suffocation.
4. Sedation to relax the associated muscles in
spasm.

INTRACRANIAL COMPLICATION OF DENTAL

INFECTIONS
• Haymaker has given a series of 28 fatal infections occurring
after tooth extraction.32
• The infection process may proceed along fascial planes to
the base of the skull and then traverse the skull by one or
more routes to spread into the intracranial cavity.

FIGURE 4.15: Periorbital cellulitis CAVERNOUS SINUS THROMBOSIS/

THROMBOPHLEBITIS

HISTOPATHOLOGIC FEATURES Cavernous sinus thrombosis occurs due to formation of

thrombus in the cavernous sinus or it's communicating
Shows diffuse exudation of polymorphonuclear leukocytes and branches.
occasional lymphocytosis. Infection of head, face and intraoral structures above the
maxilla are prone to cavernous sinus thrombosis.
Management

1. Broad spectrum antibiotics in combination with anti- ROUTES OF SPREAD OF INFECTION

anaerobic antibiotics.
2. Surgical intervention if necessary. 1. External route: Facial and angular veins carry infection
from the face and lip. It is a very rapid mode of spread of
Ludwig's Angina infection because these large veins directly lead to the
Ludwig's angina can be defined as severe cellulitis, beginning cavernous sinus.
usually in the submaxillary space and secondarily involving 2. Internal route: Pterygoid plexus carries infection especially
sublingual and submental space as well. from maxillary and mandibular 3rd molar. It is much slower
because infection has to pass through a small and twisted
CLINICAL FEATURES venous passage.

• 2nd and 3rd molars are the primary sites acting as a source
CLINICAL FEATURES
of infection.
• It appears as a rapidly developing board-like swelling of • Headache, nausea, vomiting, pain, chills and fever are
the floor of the mouth and consequent elevation of the present at initial stages.
tongue. • Exophthalmos with edema of eyelid and chemosis.
• Swelling is firm, painful and diffuse, showing no evidence • Paralysis of external ocular muscle with impairment of
of localization. vision and sometimes photophobia and lacrimation due to
• Difficulty in breathing, eating and swallowing present. occlusion of ophthalmic vein.
• Patients usually have high fever, increased pulse rate, • Complete paralysis of 3rd, 4th and 6th cranial nerve.
increased respiratory rate and moderate leukocytosis. • Death may occur due to brain abscess and meningitis.
156 Essentials of Pediatric Oral Pathology

Management REFERENCES
1. Antibiotic therapy. 1. Nair PNR. Pathobiology of the Periapex. In: Cohen S, Burns
RC, editors. Pathways of the Pulp. 8th Edition. St. Louis: Mosby
2. Drainage. Inc, 2002.
3. Anticoagulant therapy.
2. Louis I Grossman, Seymour Oliet, Carlos E del Rio. Endodontic
Practice. 11th Edition. Lea and Febiger, Varghese Publishing
FOCAL INFECTION House, 1991.
3. Matusow RJ. Oral Surg 1979;48:70.
Focal infection is a localized or generalized infection caused
4. Sundqvist G. Bacteriologic studies of necrotic dental pulps. In:
by the dissemination of microorganisms or toxic products from Umea University Odontological Dissertations. Umea, Sweden
a focus of infection. 1976;5.
Focus of infection is a circumscribed area of tissue which 5. Grossman LI. Origin of microorganisms in traumatized,
is infected with exogenous pathogenic microorganisms and is pulpless, sound teeth. J Dent Res 1967;46:551.
usually located near mucous or cutaneous surfaces. 6. Block RM, Bushell A, Rodrigues HS, Langlend K. A
histopathologic, histobacteriologic and radiographic study of
periapical endodontic surgical specimens. Oral Surg Oral Med
SPREAD OF INFECTION
Oral Pathol 1976;42:656.
Two accepted mechanisms of spread of infection are: 7. Harrison JW, Larson WJ. The epithelialized oral sinus tract. Oral
1. Metastases of microorganisms from an infected focus by Surg 1976;42:511.
hematogenous or lymphogenous spread. 8. Stern MH, et al. Cell mediated immune responses in human
apical granulomas. J Dent Res 1979;150:130.
2. Toxins or toxic products may be carried through the blood
9. Nobuhara WK, del Rio CE. Incidence of periradicular pathoses
or lymphatic channels from a focus to a distant site where in endodontic treatment failures. JOE 1993;19:315.
they may incite a hypersensitive reaction in the tissues. 10. Burket L. Recent studies relating to periapical infection,
Oral foci of infection are generally: including data obtained from human necropsy studies. J Am
Dent Assoc 1938;25:260.
• Infected periapical lesion.
11. Athanassiades TJ, Spears RS. Granuloma induction in the
• Teeth with infected root canals. peritoneal cavity: a model for the study of inflammation and
• Periodontal diseases with special reference to extraction plasma-cytopoiesis in nonlymphatic organs. J Reticuloendothel
or manipulation. Soc 1972;11:60.
• According to Fish and MacLean, pumping action during 12. Page RC, Davies P, Allison AC. Pathogenesis of the chronic
extraction may force microorganisms from the gingival inflammatory lesion induced by hroup: a streptococcal cell wall.
crevice into capillaries of gingiva and pulp.33 Lab Invest 1974;30:568.
• Evidence indicates that extraction of teeth and minor oral 13. Adams DO. The granulomatous inflammatory response. Am J
Pathol 1976;84:164.
procedures produce transient bacteremia that persists for
14. Dunlap CL, Barker BF. Giant cell hyaline angiopathy. Oral Surg
over 30 min. Oral Med Oral Pathol 1977;44:587.
15. King OH. “Giant cell hyaline angiopathy”: Pulse granuloma by
SIGNIFICANCE OF ORAL FOCI OF INFECTION another name? Paper presented at the meeting of the American
Oral foci of infection may cause or aggravate many systemic Academy of Oral Pathologists, Fort Lauderdale, Fla 1978;23-9.
16. Mincer HH, McCoy JM, Turner JE. Pulse granuloma of the
diseases like:
alveolar ridge. Oral Surg Oral Med Oral Pathol 1979;48:126.
1. Arthritis: It has been proven that failure of removal of oral 17. Head MA. Foreign body reaction to inhalation of lentil soup:
foci results in aggravation of rheumatoid arthritis. giant cell pneumonia. J Clin Pathol 1956;9:295.
2. Valvular heart diseases: Symptoms of infective endocarditis 18. Sherman FE, Moran TJ. Granulomas of stomach. Response to
are often seen after extraction of teeth. injury of muscle and fibrous tissue of wall of human stomach.
3. Gastrointestinal diseases: Frequent swallowing of Am J Cl Pathol 1954;24:415.
microorganisms may lead to many gastrointestinal diseases. 19. Knoblich R. Pulmonary granulomatosis caused by vegetable
4. Ocular diseases particles. So called lentil pulse granuloma. Am Rev Respir Dis
1969;99:380.
5. Skin diseases: Eczema and urticaria are related to oral
20. Simon JHS, Chimenti Z, Mintz G. Clinical significance of the
infection. pulse granuloma. J Endod 1982;8:116.
6. Renal diseases. 21. Chen SY, Fantasia JE, Miller AS. Hyaline bodies in the
Luckily, occurrence of metastatic infection from mouth to connective tissue wall of odontogenic cysts. J Oral Path 1981;
a distant bodily site is not very common. 10:147.
Sequelae of Pulp Pathologies in Children 157

22. White EW. Paper point in mental foramen. Oral Surg Oral Med permeability and clearance of cyst protein. J Oral Pathol 1974;
Oral Pathol 1968;25:630. 3:47.
23. Sedgley CM, Messer H. Long term retention of a paper point 28. Priebe WA, Lazansky JP, Wuehrmann AH. Oral Surgery, Oral
in the periapical tissues: a case report. Endod Dent Traumatol Medicine and Oral Pathology 1954;7:979.
1993;9:120. 29. Gardener AF. The odontogenic cyst as a potential carcinoma: a
24. Nair PNR, Sjögren, Krey G, Sundqvist G. Therapy-resistant clinicopathologic appraisal. Journal of the American Dental
giant-cell granuloma at the periapex of a root-filled human tooth. Association 1969;78:740-55
J Endod 1990;16:589.
30. Allison RT. Electron microscopic study of 'Rushton' hyaline
25. Gao Z, Flaitz CM, Mackenzie IC. Expression of keratinocyte
bodies in cyst linings. Brit Dent J 1974;137:102.
growth factor in periapical lesions. Journal of Dental Research
1996;75(9):1658-63. 31. Sedano HO, Gorlin RJ. Hyaline bodies of Rushton: some
26. Torabinejad M, Walton RE. Periradicular lesions. In: Ingle JI, histochemical considerations concerning their etiology. Oral
Bakland LK, (Eds.). Endodontics. 5th ed. Hamilton, Ont: BC Surg 1968;26:198.
Decker Inc. 2002;175-201. 32. Haymaker W. Fatal infections of the central nervous system
27. Skaug N. Proteins in fluids from non-keratinizing jaw cysts. 4. and meninges after tooth extraction. Am J Orthodont
Concentrations of immunoglobulins (IgG, IgA, IgM) and some 1945;31:117-88.
non immunoglobulin proteins: relevance to concepts of cyst wall 33. Fish EW, MacLean. Brit Dent J 1936;61:336.
5
158 Essentials of Pediatric Oral Pathology

Gingival and Periodontal

Diseases in Children
Shweta Dixit Chaudhary, Mayur Chaudhary

CHAPTER OVERVIEW
Introduction Puberty gingivitis
Prevalence of gingivitis in children Fibromatosis
Classification of periodontal diseases and conditions Genetic form
Gingiva Pharmacologically induced gingival overgrowth
Simple gingivitis Scorbutic gingivitis
Gingivitis associated with poor oral hygiene (local irritants) Periodontal diseases in children
Allergy and gingival inflammation Early onset periodontitis
Acute gingival disease Localized aggressive periodontitis
Herpes simplex virus (HSV) infection Generalized aggressive periodontitis
Recurrent aphthous ulcer Prepubertal periodontitis
Acute necrotizing ulcerative gingivitis Localized early onset periodontitis (localized juvenile
Acute candidiasis periodontitis)
Acute bacterial infections Papillon-Lefèvre syndrome
Chronic nonspecific gingivitis Cyclic neutropenia
Desquamative gingivitis Gingival recession
Conditioned gingival enlargement Abnormal frenum attachment

INTRODUCTION CLASSIFICATION OF PERIODONTAL DISEASES

1 2 AND CONDITIONS
McCall, 1938, and Baer, 1974, suggested a possible
connection between incipient periodontal diseases in children At the 1999 International Workshop for the classification of the
and severe disease processes and subsequent loss of teeth found periodontal diseases organized by the American Academy of
in adults. Periodontal disease has long been recognized in Periodontology, the following classification was internationally
children, but due to its incipient nature, has not received the accepted by common consensus on the opinion of the diseases
kind of attention commanded by dental caries. Children and and conditions affecting the periodontium3 (Table 5.3).
adolescents can have any of the several forms of periodontitis
as described in the proceedings of the 1999 International
GINGIVA
Workshop for a Classification of Periodontal Diseases and
Conditions. However, chronic periodontitis is more common The gingiva, as described by Löe, Listgarten and Terranova,
in adults, while aggressive periodontitis may be more common 1990, is the part of the oral mucous membrane that covers the
in children and adolescents.3 alveolar processes and the cervical portions of the teeth.5
The gingival tissues are normally light pink, though the
PREVALENCE OF GINGIVITIS IN CHILDREN color may be related to the complexion of the person, the
V. Dhar et al used the Löe and Silness Index to score thickness of the tissue and the degree of keratinization. The
gingivitis and found a slightly higher prevalence in girls4 surface of the gingiva has a stippled appearance, varying from
(Tables 5.1 and 5.2). fine to coarsely grained. In the healthy adult, the marginal
Gingival and Periodontal Diseases in Children 159

TABLE 5.1: Epidemiologic studies of gingival diseases in children

Sr. Year Investigators Group studied No. of Age group % affected

No. children by gingivitis

1. 1940 Marshal-Day and Tandon Middle class children in Lahore 756 13 68

2. 1940 Marshal-Day Fluoride endemic area in northern India 203 5-18 59.6

3. 1944 Marshal-Day Boys in Kangra district 200 13 81

4. 1947 Marshal-Day and Shourie Low to middle class male school children 1054 9-17 99.4
in Lahore

5. 1947 Marshal-Day and Shourie Girls of high socioeconomic level at Lahore 179 9-17 72.3

6. 1960 Greene School boys in low economic area of India 1613 11-17 96.9

7. 1965 Dutta Boys and girls in Calcutta 1424 6-12 89.8

8. 2007 Dhar V, Jain A, Government school children of Udaipur district 1,587 5-14 84.37
Van Dyke TE, Kohli A

TABLE 5.2: Prevalence of gingivitis in children TABLE 5.3: Classification of periodontal diseases and
conditions
Sr. Age group Overall Mild Moderate Severe
No. prevalence gingivitis gingivitis gingivitis Gingival diseases:
of gingivitis • Plaque induced gingival diseases
• Nonplaque induced gingival diseases
1. 5-7 years 78.72% 64.89% 11.17% 2.66%
Chronic periodontitis:
2. 8-10 years 85.01% 61.16% 19.08% 4.77% • Localized
3. 11-14 years 85.22% 53.45% 28.57% 3.2% • Generalized

Periodontitis as a manifestation of systemic diseases:

gingiva has a sharp, knifelike edge; during the period of
Necrotizing periodontal diseases:
eruption in a child, however, the gingivae are thicker and have • Necrotizing ulcerative gingivitis (NUG)
rounded margins. • Necrotizing ulcerative periodontitis (NUP)

SIMPLE GINGIVITIS Abscesses of the periodontium:

• Gingival abscess
Gingivitis refers to inflammation limited to the soft tissues that • Periodontal abscess
surround the teeth (Fig. 5.1). • Pericoronal abscess

Periodontitis associated with endodontic lesions:

Clinical Features • Endodontic-periodontal lesion
• Periodontal endodontic lesion
Usually, clinically detectable inflammatory changes of the
• Combined lesion
gingiva begin in childhood and increase with age. The
frequency of gingivitis peaks between the ages of 9 and 14 Developmental or acquired deformities and conditions:
years, with a decline thereafter till the age of 17 years, after • Localized tooth-related factors that predispose to plaque
which a steady and slow increase in the prevalence of gingivitis induced gingival diseases or periodontitis
is seen till the sixth decade of life. It may be diffuse or confined • Mucogingival deformities and conditions around teeth
• Mucogingival deformities and conditions on edentulous ridges
to the free gingival margins or the interdental papilla. Earliest
• Occlusal trauma
signs of gingivitis include loss of stippling and bleeding on
gentle probing.
Stage I: Initial Lesion
Histopathologic Features The changes seen are:
There are four stages, three of which are confined to the gingiva • Dilatation of capillaries and venules causing increased
whereas, there occurs periodontal breakdown in the fourth stage. blood flow in the area
160 Essentials of Pediatric Oral Pathology

FIGURE 5.1: Gingivitis showing inflamed interdental gingiva FIGURE 5.2: Eruption gingivitis showing
inflammation in upper anterior region

• Margination of neutrophils • Usually subsides after the teeth emerge into the oral cavity
• Increase in gingival crevicular fluid • Maximum incidence in the 6 to 7 year age group when the
• This stage may resolve or progress to the next stage with permanent teeth begin to erupt.
the appearance of lymphocytes and macrophages.
Etiopathogenesis
Stage II: Early Lesion
The changes seen are: The gingivitis apparently occurs because the gingival margin
• Destruction of dentogingival and circular fibers receives no protection from the coronal contour of the tooth
• Predominant cell type is lymphocyte during the early stage of active eruption, and the continual
• Similar changes occur as seen in stage I, but with increased impingement of food on the gingivae causes the inflammatory
severity. process. Local irritants like plaque, material alba and food debris
often collect around and beneath the free tissue and cause
Stage III: Established Lesion development of the inflammatory process. The condition may
The changes seen are: be painful and may develop into a pericoronitis or a pericoronal
• Dilated blood vessels become engorged giving a bluish abscess.
color to the gingiva
• Preponderance of plasma cells deep into the connective tissue Radiographic Features
• Granular cell debris within widened intercellular space of
junctional epithelium No radiographic changes are evident.
• Elongated rete pegs of junctional epithelium
• Infiltrate of plasma cells, neutrophils, lymphocytes and mast Histopathologic Features
cells Usually a mild inflammatory reaction is present showing an
• Destruction of collagen fibers infiltrate of lymphocytes, plasma cells, neutrophils, mast cells
Stage IV: Advanced Lesion and macrophages. Destruction of collagen is also evident.
There occurs periodontal breakdown.
Management
ERUPTION GINGIVITIS (FIG. 5.2) • No treatment for mild eruption gingivitis
It is a temporary type of gingivitis observed in children when • Improvement of oral hygiene
teeth are erupting. • Irrigation with counter-irritant such as peroxyl
(Colgate-Palmolive Co, New York) in cases of painful
Clinical Features pericoronitis
• Antibiotic therapy for pericoronitis accompanied by
• Associated with erupting teeth
swelling and lymph node involvement.
• May be associated with difficult eruption
Gingival and Periodontal Diseases in Children 161

GINGIVITIS ASSOCIATED WITH POOR ORAL Matsson and Moller, 1990, studied the degree of seasonal
HYGIENE (LOCAL IRRITANTS) variation of gingival inflammation in children with allergies to
birch pollen. Their results indicated an enhanced gingival
This type of gingival inflammation is associated with local
inflammatory reaction in the allergic children during the pollen
factors like calculus, food impaction, faulty or irritating
seasons.8
restorations or appliances, mouth breathing, tooth malposition,
chemical and drug application, untreated carious lesions, etc.
Lindhe and Axelsson, 1973, examined the effects of various ACUTE GINGIVAL DISEASE
preventive measures on gingivitis in Swedish children. The HERPES SIMPLEX VIRUS INFECTION
plaque indices of the test group improved markedly over a 72
month period.6 Herpes simplex virus (HSV) infections are common vesicular
Poulsen et al, 1976, demonstrated a reduction of gingival eruptions of the skin and mucosa. They occur in two forms-
inflammation in Danish children seven years of age as a result systemic or primary and may be localized or secondary in
of professional cleaning every two weeks for 72 months.7 nature. Both forms are self-limited but recurrences of secondary
Adequate mouth hygiene and cleanliness of teeth are related forms are common because the virus can be sequestered within
to frequency of brushing and the thoroughness with which ganglionic tissue in the latent stage.
bacterial plaque is removed from the teeth. Favorable occlusion Control rather than cure is the usual goal of treatment.
and the chewing of coarse, detergent-type foods such as raw
carrots, celery and apples have a beneficial effect on oral Pathogenesis (Fig. 5.4)
cleanliness. Physical contact with an infected individual is the typical route
Gingivitis associated with poor oral hygiene is usually of HSV inoculation for a seronegative individual. The virus
classified as early, moderate, advanced. binds to the cell surface epithelium via heparan sulfate followed
Early gingivitis is quickly reversible and can be treated with by the sequential activation of specific genes during the lytic
good oral prophylaxis and through teaching good tooth phase of infection.
brushing and flossing techniques to keep the teeth free of The incubation period after exposure ranges from several
bacterial plaque. days to two weeks. In overt primary disease, primary
gingivostomatis or a vesiculoulcerative eruption occurs in the
ALLERGY AND GINGIVAL INFLAMMATION oral and perioral tissues. After resolution of primary herpetic
(FIG. 5.3) gingivostomatis, the virus migrates along the periaxon sheath
Although it is difficult to assess the significance of gingival of the trigeminal nerve to the trigeminal ganglion, where it is
reaction during short allergic seasons, it has been suggested capable of remaining in a latent or sequestered state. No major
that patients with complex allergies who have symptoms for histocompatibility (major histocompatibility complex)
longer periods may be at higher risk for more significant antigens are expressed, so there is no T-cell response during
adverse periodontal changes. latency.

FIGURE 5.3: Gingival inflammation in upper and

lower anterior region due to allergy FIGURE 5.4: Pathogenesis of herpes simplex infection
162 Essentials of Pediatric Oral Pathology

• The ulcers may be observed on any area of mucous

membrane including buccal mucosa, tongue, lips, hard and
soft palate and the tonsillar areas.
• Four-fold rise of serum antibodies to HSV-1.
• Lesion culture will be positive to HSV-1.

Histopathologic Features
• Intraepithelial vesicles containing exudates, inflammatory
cells and characteristic virus-infected epithelial cells are
seen
• Virus-infected keratinocytes contain one or more
homogeneous, glassy nuclear inclusions
• HSV-1 cannot be differentiated from HSV-2 histologically.
FIGURE 5.5: Herpes simplex infection showing
multiple ulcers around the face
Secondary Herpes Simplex
Reactivation of latent herpes simplex virus type 1 may occur
due to triggering factors like exposure to sunlight, cold, trauma,
stress or immunocompression. Lesions on the lip may also
appear after dental treatment and may be related to irritation
from rubber dam material or even routine daily procedures.
Reactivation is common, although frequency decreases with
aging. Prodromal symptoms like tingling and burning may be
present. Sites affected are the same as in primary herpes
simplex and the disease is self-limiting.

Management

FIGURE 5.6: Herpetic gingivostomatitis 1. Natural course of 10 to 14 days.

2. Basically symptomatic treatment is recommended.
Reactivation of virus may follow exposure to sunlight 3. Fluid and nutritional intake should be maintained.
("fever blisters"), exposure to cold ("cold sores"), trauma, stress 4. Application of mild topical anesthetic such as 0.5
or immunocompression. percent dyclonine hydrochloride (Dyclone) or lidocaine
(Xylocaine Viscous) before meal time will temporarily
Clinical Features relieve the pain and allow the child to take a soft diet.
5. Schaff, 1984, recommends a mixture of equal parts of
• The primary infection usually occurs in a child under six diphenhydramine (Benadryl) elixir and kaopectate as
years of age. an alternative to the anesthetic. The diphenhydramine
• Most infections are subclinical (99%). has mild analgesic and anti-inflammatory properties,
• Oral and perioral vesicles rupture forming ulcers (Fig. 5.5). whereas the kaolin-pectin compound coats the lesion.9
6. A vitamin supplement is indicated during the course of
• Intra-oral lesions on any surface.
the disease as fruit juices will be irritating to the ulcerated
• Self-limited; symptomatic care. area.
• Immunocompromized patients have more severe disease 7. Zunt, 1999, suggested that the mainstay of definite
• The symptoms of the disease develop suddenly and include therapy is regular doses of specific systemic antiviral
in addition to the fiery red gingival tissues, malaise, medication combined with systemic analgesics.10
irritability, headache, and pain associated with the intake 8. Acyclovir (Zovirax)—Five daily doses to equal 1000 mg
of food and liquids of acid contents. per day for ten days. Available as capsules or
• Characteristic oral finding is the presence of yellow or white suspension has been successfully used in infants and
children.
liquid filled vesicles.
9. Famciclovir (Famvir) and valacyclovir (Valtrex) are
• In a few days the vesicles rupture and form painful ulcers, newer and possibly more potent antiviral agents but
1 to 3 mm in diameter, covered with the whitish-gray their use has not yet been adequately studied in
membrane and having a circumscribed area of inflammation pediatric populations.
(Fig. 5.6).
Gingival and Periodontal Diseases in Children 163

10. Valacyclovir should not be prescribed for immuno- epithelium. He showed both IgG and IgM binding by
compromised patients. epithelial cells of the spinous layer of oral mucosa in
11. Bed rest and isolation from other children in the family patients suffering from RAU using a fluorescent antibody
are recommended. technique.13
12. Sunscreen can prevent sun induced recurrences. • Iron, vitamin B12, folic acid deficiency: There has been
13. Most effective treatment for recurrences is the use of some evidence that nutritional deficiencies might be of
specific, systemic antiviral medications immediately some significance in the etiology of RAU. In patients with
after the prodromal symptoms of recurrence. The
iron, vitamin B12, folic acid deficiency, a prompt response
dosage remains the same while the course of treatment
usually ends after five days. to replacement therapy suggested a direct action on oral
14. A topical antiviral agent, penciclovir cream (Denavir) mucosa, although, it has been reasonably postulated that
is available for extraoral lesions (not to be used the presence of a deficiency allows the expression of an
intraorally), to be applied every two hours while awake unrelated underlying tendency to ulceration and that the
for four days. It is not to be used in children younger deficiency itself does not play a primary role.
than two years of age.
15. Another remedy for the infection is the amino acid lysine Precipitating Factors
based on its antagonistic effect on amino acid arginine.
L-Lysine monohydrochloride is available in capsule A variety of factors have been repeatedly identified immediately
form or tablets containing 100 or 312 mg of L-Lysine. preceding the outbreak of aphthous ulcers:
This therapy resulted in control of disease. • Trauma: Self-inflicted bites, oral surgical procedures, tooth-
16. Avoidance of foods containing arginine, e.g. cereals,
brushing, dental injections and dental trauma.
seeds, nuts and chocolate and selection of foods with
adequate lysine, e.g. dairy products, yeast are • Endocrine conditions: Premenstrual, menstrual, postmen-
recommended. This may explain the low incidence of strual, post-ovulation. Rarely occurs during menarche and
herpes in infants before they are weaned from a menopause.
predominantly milk diet. • Psychic factors: Acute psychologic problems associated
with precipitation of attacks.
RECURRENT APHTHOUS ULCER • Allergic factors: History of asthma, hay fever or food or
drug allergies.
Recurrent aphthous ulcers (RAU) also referred to as Recurrent
aphthous stomatitis (RAS) or canker sore is an unfortunately
Clinical Features
common condition characterized by painful ulceration on the
mucous membrane that occurs in school aged children and The pediatric age group for RAU is in first and second decades
adults. Because of the similarity between this disease and of life. There is a female predilection. They occur mostly in
Herpes simplex infection, with respect to precipitating factors non-keratinized mucosa, e.g. buccal and labial mucosa.
leading to development of lesions, certain aspects of clinical RAU has been classified on the basis of clinical appearance
appearance of lesions, duration of lesions, chronic recurrence and the time taken for healing of an ulcer as:
and general failure of response to any form of therapy, the
two diseases have been generally confused until only a short Minor Aphthous Ulcers
time ago. • Recurrent painful ulcers
Etiology • Superficial
• Oval in appearance
Numerous etiological factors have been suggested: • Less than 1cm in diameter
• Bacterial infection: Barile, 1963,11 Graykowski, 1966,12 • Resolves in 7 to 10 days.
have strongly implicated a pleomorphic, transitional L-form
of an -hemolytic streptococcus, Streptococcus sanguis, Major Aphthous Ulcers (Fig. 5.7)
as the causative agent of the disease. Graykowski et al have • Multiple, deep ulcers
also shown that patients with recurrent aphthous ulcers,
• Greater than 1 cm in diameter
when tested with a streptococcus vaccine, give a positive
• Resolve in 2 to 6 weeks.
delayed type of hypersensitive skin reaction in contrast to
patients with no history of aphthae, who give a less frequent
Herpetiform Variant
and less severe response.
• Immunologic abnormalities: Lehner, 1969, suggested that • First described by Cooke in 1960.14
RAU is a result of an autoimmune response of the oral • Showers of multiple small ulcers.
164 Essentials of Pediatric Oral Pathology

for 5-7 days) is also useful, but the suspension should

not be swallowed.
3. Chlorhexidine mouthwash may also help in alleviating
the symptoms of RAU.
4. 0.1 percent triamcinolone acetonide (Ledercort
ointment, Kenalog in Orabase) may be applied to the
surface of the lesions before meals to facilitate intake
of food. This is a potent topical steroid. Moderately
potent steroids like glucocorticoid may be
administered or mildly potent corticosteroids may also
be administered.
5. Meiller et al 1991, have reported that the duration and
the severity of the lesions can be reduced significantly
by vigorous rinsing with an antimicrobial mouthwash
(Listerine Antiseptic) twice daily.17
6. Aphthasol, a topical paste containing 5 percent
FIGURE 5.7: Major apthae in labial vestibule amlexanox to be applied 4 times daily after meals and
at bedtime till the ulcer heals, is effective in reducing
• May be confused with herpes simplex but Brook and Sapp, pain and accelerating the healing of RAU ulcers.
1976, showed that there was no herpes simplex as seen by 7. Xylocaine/Lidocaine, silver nitrate, benadryl, camphor
cytologic, microscopic, immunofluorescent and serologic phenol may be applied topically to alleviate the
techniques.15 symptoms of the ulcer.
8. Diet supplementation with vitamin B12, folic acid, iron
Associated with Behçet's Syndrome and zinc sulphate has been advocated.
• Range in size from several millimeters to a centimeter in 9. Immune enhancement with the help of levamisole or
diameter vaccine, are modalities that are being tried.
• Erythematous border
• Ulcer covered by a gray or yellow exudate. ACUTE NECROTIZING ULCERATIVE GINGIVITIS
Acute necrotizing ulcerative gingivitis (ANUG), also called as
Histopathologic Features "trench mouth", was described during World War I where this
Hematoxylin and eosin stained biopsy sections of the lesional condition was prevalent amongst the allied troops staying in
tissue shows a fibropurulent membrane covering an ulcerated trenches for long duration.
area. There is break in the continuity of epithelium. Superficial
colonies of microorganisms may be present in this membrane. Etiology
Connective tissue stroma consists of intense inflammatory cell Reade, 1963, described a case of ulcerative gingivitis in a 15
infiltrate mostly consisting of neutrophils and lymphocytes. month old infant and demonstrated Treponema vincentii and
A characteristic change of elongated nuclei containing a gram negative fusiform bacilli.18 It is generally an endogenous
linear bar of chromatin extending towards the nuclear membrane fuso-spirochetal infection caused by predisposing factors. Some
around aphthous ulcer taken by cytologic smear was reported investigators believe that this infection may be caused by vibrio
by Wood and his associates, 1975.16 The cells showing such and coccal forms. Many experiments were conducted to find
change were termed as Antischkow cells. They are not out the etiological agent pertaining to this infection by various
pathognomonic of this lesion and may also be seen in sickle cell investigators. Mc Donald et al 1963, in their experiments
disease, megaloblastic anemia, iron deficiency anemia, children concluded that Bacteroides melaninogenicus was the true
receiving cancer chemotherapy and even in normal people. causative agent of necrotizing ulcerative gingivitis.19 Goldhaber
Management and Giddon, 1964, have published an excellent review of this
disease.20
1. Management is primarily symptomatic as it is a self-
limiting disease with uneventful healing with rarely a
scar formation except in unusually large ulcers. PREDISPOSING FACTORS
2. Topical application of tetracycline to the ulcers is often • Psychological stress
helpful in reducing the pain and shortening the course • Immunosuppression
of the disease. Mouth rinses with a suspension
• Smoking
containing tetracycline (250 mg/5 ml 4 times a day
• Upper respiratory tract infection
Gingival and Periodontal Diseases in Children 165

TABLE 5.4: Difference between ANUG and acute

herpetic gingivostomatitis

Sr. ANUG Acute herpetic

No. gingivostomatitis

1. Most frequent in young adults Most frequent in pre-

in a mouth in which irritants and school children
poor oral hygiene are present

2. Develops over a longer period Rapid onset

3. Usually limited to the gingivae Usually gingivae, lips and

cheeks are involved

4. Therapeutic trial cleaning will No change in signs or

bring along a favorable response symptoms seen after
therapeutic trial cleaning

5. Therapeutic trial antibiotics will No change in signs or

reduce the acute symptoms symptoms seen after
FIGURE 5.8: Acute necrotizing ulcerative gingivitis involving therapeutic trial antibiotics
upper and lower gingivae

• Local trauma • Early stages of conditions like Hand-Schüller-Christian

• Poor nutritional status disease or Letterer-Siewe disease are associated with many
• Poor oral hygiene. of the symptoms of vincent infection.
• Differences between ANUG and acute herpetic gingivo-
Clinical Features stomatitis are cited in Table. 5.4.
• This inflammatory condition involves the free gingival
Histopathologic Features
margin, the crest of gingiva and the interdental papillae.
• Rarely the lesions spread to soft palate and tonsillar Hematoxylin and eosin stained sections of the lesional tissue
areas; in such instances, the condition is called 'Vincent's show an ulcerated stratified squamous epithelium replaced by
angina'. thick fibrinous exudates. The connective tissue stroma shows
• It occasionally occurs in children 6 to 12 years old, and is an intense hyperemia and dense inflammatory infiltrate chiefly
common in young adults. consisting of polymorphonuclear leukocytes. Since the
• Characterized by development of painful hyperemic gingiva histopathological picture is non-specific, the diagnosis is based
purely on clinical examination.
and sharply punched out crater-like erosions of the
interdental papillae of sudden onset (Fig. 5.8). Microscopic Smear Examination
• Bleeding on probing is seen.
Smear material from this disease shows fusiform bacilli and
• Ulcers become covered by a grayish-green necrotic
spirochetes. Other microorganisms observed are filamentous
pseudomembrane.
microorganisms, vibrios, cocci. Along with the microorganisms
• Usually begins at a single isolated focus but tends to spread
seen, there are desquamated epithelial cells and polymorpho-
and may eventually involve all gingival margins. nuclear leukocytes. Generally major causative microorganisms
• Fetid odor. responsible for producing ANUG are:
• Inability to eat due to gingival pain and bleeding.
• Nature of pain—Superficial 'pressure'. Fusiform Bacillus
• Headache, malaise, low grade fever. • Elongated rod with tapered ends
• Excessive salivation with metallic taste to the saliva. • 5 to 14 microns in length
• Regional lymphadenopathy. • 0.5 to 1 microns in diameter
• In severe infection, systemic manifestations including • Gram-positive, nonmotile, anaerobic, occurs singly or
leukocytosis, gastrointestinal disturbances, and tachycardia inclusters.
may be seen.
• Healing results in an area which is hollowed out due to Borrelia vincentii
destruction of the crests of the interdental papillae which • Gram-negative spirochete with 3 to 6 long, loose spirals
retain debris and microorganisms and serve as an • 10 to 15 microns in length
"incubation zone" where recurrence of ANUG may occur. • Motile, anaerobic.
166 Essentials of Pediatric Oral Pathology

The bacterial smear may be of value as an aid in diagnosis

of ANUG. However, it has been suggested that the presence of
microorganisms in the tissues is due to surgical artifact. Schaffer,
1953, using light microscopy failed to find bacteria penetrating
vital tissues.21 Listgarten 1967, however, with the help of electron
microscopic techniques, could identify spirochetes between
viable epithelial cells.22

Management
1. Subgingival curettage, debridement, use of mild
oxidizing solutions (diluted hydrogen peroxide) will
help in a dramatic improvement in the symptoms of
the disease.
2. Antibiotic therapy is indicated for patients having FIGURE 5.9: Acute pseudomembranous candidiasis
extensive inflammation. showing white, curd-like patches on tongue
3. Improved oral hygiene, use of mild oxidizing mouth-
rinses after every meal, twice daily chlorhexidine
mouthrinses will aid in overcoming the infection. • The plaques consist of masses of fungal hyphae with
4. Recontouring of gingiva (gingivoplasty) if required. intermingled desquamated epithelium, keratin, fibrin,
necrotic debris, leukocytes.
ACUTE CANDIDIASIS • The patches show a radial pattern of growth varying in size
and shape.
Candida albicans is a common inhabitant of the oral cavity • After removal of these white patches, there is bleeding of
but may be responsible for the opportunistic infection of oral the underlying surface.
cavity following decline in host resistance. The word candida • Mild burning sensation is experienced.
is of Latin origin meaning glowing white or glistening, because
• Neonatal candidiasis, occurs during passage through the
of the appearance of glistening colonies on culture media. It is
vagina and erupts clinically during the first two weeks of
a yeast-like fungus causing an infection termed candidiasis,
life.
also referred to as thrush, candidosis, moniliasis.
• Infants may have diaper rash.
[Monile (L) = necklace—large group of moulds/fungi
called fruit moulds. Few pathogenic ones were later separated
ACUTE ATROPHIC CANDIDIASIS
and classified as candida.]
Candida occurs in three forms pseudohyphae, yeast and • Lesions appear red or erythematous rather than white, thus,
chlamydospore. appearing pseudomembranous where membrane had been
Frequently classified into two types: wiped off.
1. Mucocutaneous candidiasis (oral or oropharyngeal, • Lehner, 1964, states that this is the only type of candidiasis
intestinal, esophageal, etc.) which is painful.23
2. Systemic candidiasis (involves eyes, kidneys and other • Usually involve the palate and dorsum of the tongue.
visceral organs). • Lesions are sometimes ulcerated and crusted.
The most common forms in children are pseudomemb- • More common in children with a lip sucking habit, which
ranous and erythematous candidiasis. may lead to spread of the infection to the adjacent perioral
skin.
ACUTE PSEUDOMEMBRANOUS CANDIDIASIS • Accumulation of saliva in deep folds of the corners of the
Clinical Features mouth allows for the colonization of fungal and bacterial
• Occurs mainly in debilitated or chronically ill individuals. organisms (angular cheilitis).
Common in children receiving oncology treatment, during
periods of severe immunosuppression and neutropenia. HISTOPATHOLOGIC FEATURES
• May occur in young children after antibiotic therapy. Oral smear may be prepared by macerating fragments of the
• No gender predilection. plaque material with 20 percent potassium hydroxide and
• Oral lesions are soft, white to yellow, slightly elevated, furry examined for the typical hyphae.
plaques occurring on buccal mucosa, tongue, gingiva, floor Periodic acid-schiff (PAS) stained section of the biopsy
of mouth (Fig. 5.9). shows yeast cells and hyphae or mycelia in the superficial and
Gingival and Periodontal Diseases in Children 167

the deeper layers of the involved epithelium. Chlamydospores 9. Chlorhexidine gluconate 0.12 percent can be used as
are seldom seen in histological section. antimicrobial rinse and is most useful for maintenance
purposes.
DIAGNOSIS 10. Antifungal ointments and creams include nystatin,
clotrimazole, myconizole and ketoconazole.
A detailed description about diagnosis has been given in the 11. For chronic cases of angular cheilitis, Mycolog II is the
chapter on Infectious diseases in children. best choice when applied to the corners of the mouth
three times a day for five days.
Management
1. Improved oral hygiene is of importance which includes ACUTE BACTERIAL INFECTIONS
control of caries, keeping pacifiers and appliances Acute bacterial infections have been commonly known to affect
clean, replacing contaminated tooth brushes.
the oral cavities of children although the exact epidemiology
2. Topical antifungal agents like compounded clotrimazole
suspension (10 mg/ml) and nystatin oral suspension
is still debatable. Acute streptococcal gingivitis has been
(100,000 U per ml) may be swished for 2 min. and reported causing painful, vivid, red gingivae that bleed easily.
swallowed/expectorated four times daily for two Ulceration of the gingivae, gingival abscesses and enlarged
weeks. The patient should not eat or drink for 30 papillae have been observed. Diagnosis requires laboratory
minutes afterwards. Adolescents can use 1 to 2 tests. Management usually consists of broad spectrum
pastilles (200,000 U) slowly dissolved in the mouth five antibiotics, chlorhexidine and antimicrobial mouthwashes and
times daily. improved oral hygiene.
3. All antifungal agents formulated for topical use contain This topic is dealt with in detail in the section on bacterial,
sweeteners which may promote caries if used for an viral and fungal infections.
extended period. Nystatin solution contains 30 to 50
percent of sucrose. Daily use of topical fluoride is
recommended to reduce the caries potential. CHRONIC NONSPECIFIC GINGIVITIS
4. Clotrimazole troches (10 mg) also very rich in sucrose It is a long standing condition common in the preteenage and
can be used by slowly dissolving in mouth, one troch
teenage period. Dietary inadequacies have been pointed out
every 3 hours while awake (5 per day) for 14 days. The
child must be of age and maturity to comprehend and
by various researchers as one of the contributing factors.
follow instructions to use troch vehicle. Liver toxicity Presence of malocclusion, mouthbreathing, etc. also lead to an
has been reported in patients using clotrimazole. accumulation of local irritants. It usually occurs due to the
Further clinical studies are required to establish the presence of a chronic local irritant. May be localized or
safety of drug in children less than 3 years. generalized. The local irritant variously results in gingival
5. Systemic antifungal drugs are advantageous when inflammation, enlargement and ulceration with fiery red
other topically delivered medications are administered gingivae and further leads to a conducive environment for
concurrently. It is usually reserved for children either accumulation of plaque and materia alba. Histopathological
not tolerating or failing topical treatment or those at risk features are similar to gingivitis except more number of plasma
of systemic infections.
cells and lymphocytes are present.
6. Systemic agents include clotrimazole 6 mg/kg every
12 to 24 hours for 5 to 7 days; adolescents can use a
200 mg loading dose and then 100 to 200 mg once a DESQUAMATIVE GINGIVITIS
day for about a week. This is a type of chronic nonspecific gingivitis. The term
7. Ketoconazole may also be used in children at 5 to 10 "desquamative gingivitis" is more of a clinical description and
mg/kg every 12 to 24 hours and in adolescents 200 to
has also been referred to as gingivosis. The term chronic
400 mg every 24 hours for 5 to 7 days. It is highly
effective and has the advantage of good patient desquamative gingivitis was coined in 1932 by Prinz.24
compliance. Fluconazole is generally preferred over
ketoconazole which has a greater risk of associated
Etiology
hepatotoxicity. Itraconazole and voriconazole are two The exact etiology has not been established but McCarthy
additional azoles with excellent activitiy against et al 1960, have suggested the following etiological factors:25
candida. • Hormonal influences
8. Common side effects with systemic use include • Certain dermatoses
nausea, vomiting, pruritis, skin rash, abdominal
• Abnormal responses to irritation
discomfort, headache, abnormal liver function test and
• Chronic infections
drug induced hepatitis.
• Idiopathic.
168 Essentials of Pediatric Oral Pathology

Clinical Features • Mouth breathing could lead to chronically dehydrated

gingivae in the maxillary labial area leading to localized
• Mainly in the teenage group
gingivitis.
• Gingiva appears intensely, fiery red with a characteristic
• Marginal gingival enlargement is seen characterized by
desquamation of the surface epithelium.
prominent bulbous interproximal papillae far greater than
• There may be swelling and glossy appearance of the
gingival enlargements associated with local factors.
gingivae due to loss of stippling.
• Usually only the anterior segment of one arch affected. The
• Multiple vesicles and denuded areas may be seen.
lingual gingival tissue generally remains unaffected.
Nikolsky's sign is positive which indicates slipping or • A longitudinal study carried by Mombelli A et al 1990 on
peeling of the tissue at the dermal-epidermal junction under subgingival microbiota of children between age group
slight lateral pressure 11 to 14 years had implicated Capnocytophaga species in
• Buccal mucosa may be involved at times. initiation of pubertal gingivitis.26
HISTOPATHOLOGIC FEATURES Histopathologic Features
Histopathologic features depend upon the condition, i.e. either The overlying epithelium may show blunt rete ridges.
mucous membrane pemphigoid or lichenoid reaction. The Connective tissue stroma is loose, edematous with chronic
overlying epithelium may be atrophic or with blunt rete ridges. inflammatory cell infiltrate.
The connective tissue stroma is fibrous with chronic
inflammatory cell infiltrate mostly consisting of lymphocytes Management
and plasma cells. Sometimes clefting may be seen below the
1. Thorough oral prophylaxis.
basement membrane.
2. Motivating towards better practice of home oral
hygiene measures.
Management
3. Removal of all local irritants, restoration of carious
1. Thorough oral prophylaxis. teeth, dietary prescriptions to ensure adequate
2. Elimination of predisposing factors like malocclusion, nutritional status.
overhanging restorations, gingival and periodontal 4. 500 mg ascorbic acid may be administered orally daily
defects which predispose to the accumulation of local for approximately four weeks for improvement in
irritants. gingival inflammation.
3. Correction of dietary inadequacies. 5. Surgical management involves gingivoplasty and
4. Evaluation of hormonal imbalance and correction of removal of the thickened fibrotic marginal and
the same if required. interproximal tissue.
5. Doxycycline monohydrate 100 g daily for 4 to 11
6. Maintenance of adequate oral hygiene ensures
weeks.
minimal recurrence rate.
6. Topical corticosteroid such as triamcenolone 0.1
percent or fluocinone 0.05 percent applied daily.

CONDITIONED GINGIVAL ENLARGEMENT

(FIG. 5.10)
PUBERTY GINGIVITIS
Gingivitis is common in children and adolescents, especially
around puberty. It is believed to be related to an increase in
steroidal hormone. Granulomatous changes of the gingivae
similar to those occurring in pregnancy may be visible.

Clinical Features
• Peak prevalence is 10 years in girls and 13 years in boys.
• Crowded teeth and orthodontic appliances may be
important contributors as they render difficulty in practicing FIGURE 5.10: Conditioned gingival enlargement showing
oral hygiene measures. prominent bulbous interproximal papillae
Gingival and Periodontal Diseases in Children 169

FIBROMATOSIS
Fibromatosis is a slow, progressive, diffuse, benign, fibrous
enlargement of gingiva. Basically it has been shown to occur
in two forms:
1. Genetic and
2. Pharmacologically induced.

Genetic Form
Hereditary gingival fibromatosis, the most common genetic
form of this lesion has been reviewed by Zackin and
Weisberger, 1961, who reported a family of 11 affected and
10 normal children. It usually occurs as an autosomal dominant
trait. 27 It may also be idiopathic. Hart et al, 1998, have
identified the first polymorphic marker for this condition in
chromosome 2p21.28 This rare type of gingivitis has been FIGURE 5.11: Gingival fibromatosis almost
referred to as elephantiasis gingivae. completely covering the teeth
Clinical Features
• Gingival tissues appear normal at birth but begin to enlarge
with eruption of primary teeth.
• More common in the first and second decades.
• No sex predilection is seen.
• Affects both the dentitions.
• Usually affects the attached gingiva and maxillary
tuberosity.
• Diffuse, firm, smooth to stippled or nodular overgrowth of
gingiva in one or both the arches (Fig. 5.11).
• Pale in color.
• Pain is not the feature unless the overgrowth covers the
teeth completely and then gets traumatized during
mastication.
• Mobility and displacement of the teeth may be evident
FIGURE 5.12: Hyperkeratotic epithelium with thickened and
sometimes because of resorption of bone caused by
elongated rete pegs and connective tissue stroma is dense and
pressure induced by the overgrowth fibrous
• Tooth eruption may be impeded due to the excessive tissue.
Radiographic Features differentiate procollagens, intermediate and pathological
Resorption of bone may be evident on orthopantomogram collagen fibers. This has been shown to be of value in skin
(OPG) as a result of pressure induced by the overgrowth. lesions, hyperplastic gingival and odontogenic tumors.
Histopathologic Features Syndromes Associated with Gingival Fibromatosis
Zackin and Weisberger, 1961, described the features of gingival • Byars-Jurkiewicz syndrome: Gingival fibromatosis,
fibromatosis similar to gingival hyperplasia. The epithelium is hypertrichosis, giant fibroadenomas of the breast and
hyperkeratotic with thickened and elongated rete pegs kyphosis
(Fig. 5.12). Connective tissue stroma is dense and fibrous, filled • Cross syndrome: Gingival fibromatosis, microphthalmia,
with collagen fibers and interspersed blood vessels and mental retardation, athetosis and hypopigmentation
fibroblasts between them. • Jones-Hartsfield syndrome: Gingival fibromatosis and
Picrosirus red staining followed by polarizing microscopy sensorineural hearing loss
can selectively demonstrate collagen. Differences in • Laband syndrome: Gingival fibromatosis; ear, nose, bone
polarization colors are caused by fiber thickness, as well as by and nail defects; and hepatosplenomegaly
packing of collagen. Examination of collagen fibers of known • Rutherford syndrome: Gingival fibromatosis and corneal
thickness by this method can serve as a procedure to dystrophy.
170 Essentials of Pediatric Oral Pathology

Management • The severity of the enlargement is affected by adequacy of

oral hygiene and the gingival concentration of the
1. Surgical removal of the hyperplastic tissue is recom-
medication
mended, however recurrences are common.
2. Good oral hygiene helps in delaying the recurrence
• The most severe cases of gingival enlargement usually
of the growth. occur in mentally retarded patients in part due to poor oral
3. Although the tissue usually appears pale and firm, the hygiene.
surgical procedure is accompanied by excessive
Histopathologic Features
hemorrhage. Hence, quadrant dentistry is recom-
mended.
The epithelium is hyperkeratotic with thickened and elongated
4. Apically positioned flap surgery and CO 2 laser rete pegs. Connective tissue stroma is dense and fibrous, filled
evaporation have variously been used to reduce the with collagen fibers and interspersed blood vessels and
gingival tissue. fibroblasts between them. Connective tissue stroma is dense
5. When tooth eruption is impeded, surgical removal of and fibrous due to proliferation of fibroblast-like cells and
the excessive tissue and exposure of the teeth are decrease in the collagen degradation and chronic inflammatory
recommended. cell infiltrate (Fig. 5.14).
6. Tooth extraction alone can cause the tissues to shrink
almost to normal and recurrence can be prevented
by this means.

Pharmacologically Induced Gingival Overgrowth

• Most commonly seen is phenytoin induced gingival
overgrowth. Phenytoin was introduced by Merrit and
Putnam in 1938.29 Phenytoin (Dilantin diphenylhydatoin)
is a major anticonvulsant agent used in the treatment of
epilepsy. Kimball in 1939 first described varying degrees
of gingival hyperplasia as a common side effect of
phenytoin therapy.30 Dilantin sodium induces gingival
enlargements in 3 to 84.5 percent of patients receiving the
drug. Other hydantoins known to induce gingival
enlargements are ethotoin and mephenytoin. Gingival
enlargements can occur after therapy with immuno-
suppressant cyclosporine or calcium channel blockers.
Cyclosporine is used to control host rejection of
transplanted organs and to manage autoimmune diseases. FIGURE 5.13: Localized gingival fibromatosis in lower
Cyclosporine has been reported to produce gingival anteriors due to dilantin sodium intake
enlargement in 30 percent of the patients receiving the
drugs. Calcium channel blockers such as nifedipine and
nitrendipine are cardiac drugs that are sometimes prescribed
in children to control hypertension. Nifedipine induces
gingival enlargements in 20 percent of the cases. There
appears to be a genetic component to susceptibility to
gingival enlargement.
Clinical Features
• Painless overgrowth, differs from chronic inflammatory
enlargement in that it is fibrous, firm, dense, resilient,
insensitive and pale pink often with little tendency to bleed.
• The enlargement first occurs in the interdental region and
may appear lobular.
• It gradually spreads to the gingival margin.
• There is increased stippling which ultimately results in a
roughened or pebbled surface with lobulations (Fig. 5.13).
• The overgrowth may sometimes cover the crown of entire FIGURE 5.14: Localized gingival fibromatosis showing
teeth and interfere with eruption or occlusion. hyperplastic epithelium and chronic inflammatory cell infiltrate
Gingival and Periodontal Diseases in Children 171

Management periodontitis. It is characterized by periodontal destruction that

becomes clinically significant during adolescence or early
1. It is similar to gingival fibromatosis, although the tissue
adulthood. Ranney, 1993, classified the disease in localized and
does not show the tendency to bleed.
generalized form.31
2. Some regression of the enlargement may result if the
use of the drug is discontinued. 1. Localized aggressive periodontitis is characterized by bone
3. If medication cannot be discontinued or changed, the loss around the first molars and incisors.
enlargement can be surgically removed but it will 2. Generalized aggressive periodontitis is characterized by a
recur. more widespread pattern of periodontal destruction.
4. Surgery is indicated when
• Appearance of the gingiva is unacceptable to the Localized Aggressive Periodontitis (LAP)
patient.
• Enlargement interferes with comfortable function. Etiology
• Enlargement results in a periodontal pocket that • A strong association between LAP and a unique bacterial
cannot be maintained in a healthy state. microbiota dominated by actinobacillus actinomycetemco-
5. Post-operative discomfort after gingivectomy should mitans was reported by Newman, 1979 32 and Nelson,
be carefully weighed against potential benefits for 1999.33
patients who may not be able to give fully informed • Other microorganisms reported to be associated with
consent. LAP include P. gingivalis, E. corrodens, C. rectus, F.
nucleatum, Bacillus capillus, Capnocytophaga species and
SCORBUTIC GINGIVITIS Spirochetes.
This is gingivitis associated with vitamin C deficiency. • It has been found that the humoral immune response to A.a.
is elevated in patients with LAP. Prevalence of A.a. is six
Clinical Features times greater in LAP than in healthy patients.
• Prevalence of A.a. is greater in younger LAP patients who
• Severe pain and spontaneous hemorrhage. show a more destructive disease course than in the older
• Only the marginal tissues and gingival papillae are involved. ones. This could correlate with the disease activity.
• Although severe forms are rare in children, they may occur • Prevalence of A.a. is less or absent at healthy site.
when a child is allergic to fruit juices. • A.a. has been known to be quite virulent as it produces a
• In mild forms, usually only inflammation and enlargement leukotoxin, collagenase, phosphatases, bone resorbing
of the marginal gingival tissue and papillae occur. factors, as well as other factors important in invasion of
host tissues, evasion of host defences, immunosuppression
HISTOPATHOLOGIC FEATURES and destruction of periodontal tissues.
The overlying epithelium shows blunt rete ridges. The • A positive correlation has been found by Socransky and
connective tissue stroma is loose, edematous with collagen Haffajee, 1991, between the elimination of A.a. from
degeneration and engorged capillaries. Chronic inflammatory subgingival flora and successful clinical treatment of LAP.34
cell infiltrate consisting mainly of plasma cells and lymphocytes • Page, 1985, believes that transmission could be autosomal
may be seen. recessive while some others believe it to be an X-linked
dominant mode of transmission.35
Management Clinical Features
1. Dramatic response has been seen to a daily • The classic form of localized aggressive periodontitis was
administration of 250 to 500 mg of ascorbic acid for initially referred to as periodontosis and then as Localized
four weeks. juvenile periodontitis (LJP).
2. Thorough oral prophylaxis alongwith improved oral • Onset around the time of puberty in an otherwise healthy
hygiene measures will greatly improve the gingival individual.
condition. • Aggressive periodontal destruction localized almost
exclusively to the incisors and first molars; less than 30
PERIODONTAL DISEASES IN CHILDREN percent of sites are involved.
• Familial pattern of occurrence.
EARLY ONSET PERIODONTITIS
• Low incidence from 0.1 to 2.3 percent
Recent terminology dictates the use of the term "Aggressive • Females are affected more often than males; blacks are
periodontitis" instead of former term of early onset affected more than whites.
172 Essentials of Pediatric Oral Pathology

• Abnormality in phagocyte function. Etiology

• Self-arresting disease progression. • Actinobacillus actinomycetemcomitans along with a diverse
• One of the earliest signs is sudden symmetric pathologic microbiota.
drifting of teeth in a patient having a relatively good oral • Defective neutrophil or monocyte function.
hygiene.
• The affected teeth become mobile very soon followed by Clinical Features
pocket formation. • Usually affects individuals under 30 years of age
• Progression of bone loss is 3 to 5 times faster than adult • Generalized involvement of the permanent teeth; more than
periodontitis. 30 percent of sites are involved.
• Bone loss around the primary teeth can be an early finding • Generalized interproximal attachment loss affecting at least
in this disease. three permanent teeth other than first molars and incisors.
• May also include patients previously characterized as
Radiographic Features having rapidly progressive periodontitis.
• A localized vertical bone loss with widening of periodontal • Subgingival tissues show the presence of gram-negative
ligament (PDL) space. rods including P. gingivalis and exhibit suppressed
• Arc shaped bone destruction starting from the distal margin neutrophil chemotaxis.
of the second premolar and extending up to the mesial • Qualitatively P. gingivalis, A. actinomycetemcomitans and
margin of the second molar. Tannerella forsythia are frequently detected in the scant
Histopathologic Features plaque that is present.
The features appear similar to chronic periodontitis. Soft tissue • Compared to LAP a weaker antibody response to A.a. is
areas of periodontitis show a hyperplastic crevicular epithelium seen which supports the hypothesis that antibodies function
with exocytosis of acute inflammatory cells. Adjacent to limit the disease process.
connective tissue shows chronic inflammatory cells consisting • The destruction often appears episodically, with periods of
of lymphocytes and plasma cells. advanced destruction followed by stages of quiescence of
variable length (weeks to months or years).
Management • Patients often have small amounts of bacterial plaque
1. Early diagnosis aids in successful treatment. associated with the affected teeth. However, the amount of
2. Antibiotics can be provided against infective micro- plaque seems inconsistent with the amount of periodontal
organisms- tetracycline sometimes in combination destruction.
with metronidazole. • The gingival tissue could be severe, acutely inflamed, pro-
3. Surgical treatment includes raising a modified Widman liferating, ulcerated and fiery red with spontaneous bleeding
flap, removal of the infected crevicular epithelium and and suppuration. This usually occurs in the destructive stage
debridement of root surfaces with a simultaneous 14 in which attachment and bone are actively lost.
day course of 1g/day doxycycline hyclate. • Another gingival response shows absence of inflammation
4. DNA probe test for A a may be used for establishing
and presence of some degree of stippling, although with
the risk of aggressive periodontitis. Retesting after the
deep pockets. This response coincides with periods of
completion of antibiotic therapy will detect the patients
response to the treatment. quiescence in which the bone level remains stationary.
5. Keyes technique has been described by Rams, Keyes • Some patients may also have systemic manifestations such
and Wright, 1985.36 This technique involves: as weight loss, mental depression and general malaise.
• Meticulous scaling and root planing of all teeth.
Radiographic Features
• Concomitant irrigation to probing depth of satu-
• The radiographic picture may range from severe bone loss
rated inorganic salt solutions and one percent
chloramine-T. associated with the minimal number of teeth to advanced
• 1 g/day tetracycline for 14 days systemically. bone loss affecting the majority of the teeth.
• Home care including daily application of sodium • Rapidity of the bone loss indicated by radiographs taken
bicarbonate or three percent hydrogen peroxide at different times illustrates the aggressive nature of this
paste and inorganic salt irrigations. disease.

Generalized Aggressive Periodontitis (GAP) Risk Factors

• Microbiologic factors: Increase in levels of A. actino-
It is assumed that few of the individuals previously classified mycetemcomitans.
as having rapidly aggressive periodontitis would be considered • Immunologic factors: Human leukocyte antigens mainly
to be having GAP. HLA A9 (candidate markers) and B15 antigens are
Gingival and Periodontal Diseases in Children 173

consistently associated with aggressive periodontitis.

Functional defects of polymorphonuclear leukocytes, 9. Full mouth disinfection: Quirynen et al, 1995, described
this concept. It consists of full mouth debridement
monocytes or both may be seen.
completed in two appointments within a 24 hour period.
• Genetic factors: Segregational and linkage analyses of
In addition to this, the tongue is brushed with a
families with a genetic predisposition suggest that a major chlorhexidine gel (1 percent) for 1 minute, the mouth
gene transmitted through an autosomal dominant mode of is rinsed with a chlorhexidine solution (0.2%) for 2
inheritance plays a role in generalized aggressive perio- minutes and periodontal pockets are irrigated with a
dontitis. chlorhexidine solution (1%).39
• Environmental factors: Schenkin et al 1995, have suggested 10. Host modulation: The use of sub-antimicrobial dose of
that although smoking is implicated it may not have the same doxycycline (SDD) may help to prevent the destruction
impact on attachment levels in younger patients.37 of the periodontal attachment by controlling the
activation of matrix metalloproteinases, primarily
Histopathologic Features collagenase and gelatinase, from both infiltrating cells
Features are same as for localized aggressive periodontitis but and resident cells of the periodontium, primarily the
the area of destruction is extensive in this form of gingivitis neutrophils.
and more amount of chronic inflammatory cell infiltrate is seen.
PREPUBERTAL PERIODONTITIS
Management
Earlier thought to be a rare form of periodontitis found to affect
1. Conventional periodontal therapy: Patient education,
the primary dentition. Now classified as periodontitis as a
oral hygiene improvement, scaling and root planing,
flap surgery and regular recall and maintenance. manifestation of systemic disease because most children with
However, response has been limited and unpredictable. severe periodontal destruction also demonstrate profound
2. Local irrigation using Keyes technique may be done. immunologic abnormalities. The underlying immune deficiency
Irrigation with inorganic salt solutions and one percent may vary and includes neutrophil defects and leukocyte
chloramine T. Home care may include daily application adhesion defects. Some cases of severe periodontal destruction
of a paste of sodium bicarbonate and three percent are associated with the mutation in the cathepsin C gene in
hydrogen peroxide.
affected children. The subgingival bacterial flora is not much
3. Early diagnosis results in a better outcome than those
who are diagnosed at an advanced stage of different than that in other forms of periodontal disease. The
destruction. occurrence of severe destruction at an early age is a reflection
4. Surgical resective therapy: Mainly used to reduce or of increased host susceptibility, resulting from systemic disease.
eliminate pocket depth. However, a less than ideal
outcome must be taken into consideration before CLINICAL FEATURES
deciding to treat increased pocket depth surgically.
5. Regenerative therapy: Regenerative materials like bone • Usually seen in children less than 11 years of age.
grafts, barrier membranes and wound healing agents • Presence of calculus is scant.
have often been used for intrabony defects, particularly • Disease progression is rapid and destructive primarily
vertical defects with multiple osseous walls. affecting the primary molars and incisors.
6. Antimicrobial therapy: Walker and Karpinia, 2002,
suggested adjunctive antibiotic treatment frequently
• It tends to show familial occurrence.
results in a more favorable clinical response than • Usually associated with systemic disease. Infact,
mechanical therapy alone.38 identification of severe periodontal destruction in a child
7. Microbial testing: Some investigators advocate may be one of the first signs of systemic disease.
microbial testing as a necessary means of identifying
the specific periodontal pathogens responsible for Histopathologic Features
disease and to select an appropriate antibiotic based
on sensitivity and resistance. Features are same as for localized aggressive periodontitis but
8. Local delivery of antibiotics: Primary advantage of local the area of destruction is extensive in this form of gingivitis and
therapy is that smaller total dosages of topical agents more amount of chronic inflammatory cell infiltrate is seen.
can be delivered inside the pocket, avoiding the side
effects of systemic antibacterial agents while increasing
Management
the exposure of the target microorganisms to higher
concentrations. Many different forms of local delivery 1. The primary modality of treatment is similar to that of
agents like solutions, gels, fibers and chips are generalized aggressive periodontitis.
available. 2. Response to treatment is generally found to be poor.
174 Essentials of Pediatric Oral Pathology

LOCALIZED EARLY ONSET PERIODONTITIS

(LOCALIZED JUVENILE PERIODONTITIS) (FIG. 5.15)
As has been described previously, the newer terminology
recommends the use of the term aggressive periodontitis for
periodontal destruction that becomes clinically significant during
adolescence or early adulthood. This disease has been classified
into the localized and generalized types. Other terms found in
literature used to describe the same pathologic conditions include
juvenile, localized juvenile, generalized juvenile, rapidly
progressive, severe and prepubertal periodontitis. It is more
common in girls and it is usually characterized by a lack of the
common clinical signs of periodontal disease (inflammation,
bleeding, heavy plaque). There may be a genetic predisposition
to juvenile periodontitis and it has been linked with some
problems of the immune system. It is mostly caused by
Actinobacillus actinomycetemcomitans. Some genetic and
inherited disorders associated with aggressive periodontitis FIGURE 5.15: Localized early onset periodontitis
have been elaborated in Table 5.5. involving the first molar

RADIOGRAPHIC FEATURES TABLE 5.5: Genetic and inherited disorders associated

with aggressive periodontitis
There occur vertical defects of bone. Arc shaped bone loss is
seen around the central incisors and permanent first molars. Sr. Disorder Protein or Tissue defect
No.
Management 1. Leukocyte adhesion CD18 ( -2 integrin chain of
1. Successful treatment of early onset periodontitis deficiency Type I leukocyte function-associated
molecule)
depends on early diagnosis. Children, as well as
adults, should receive routine and thorough 2. Leukocyte adhesion CD15 (neutrophil ligand for E
periodontal evaluations. deficiency Type II and P selectins); inborn error
2. Treatment of early onset periodontitis begins with the in fucose metabolism
elimination of the bacteria that cause the disease and
3. Acatalasia Catalase enzyme
the control of risk factors.
3. As in adults, treatment consists of scaling and root 4. Chronic and cyclic Unknown
planing and thorough oral hygiene instructions that neutropenias
include brushing and flossing.
4. Periodontal surgery and systemic antibiotics may be 5. Chédiak-Higashi Abnormal transport of vesicles
needed. syndrome to and from neutrophil lysosomes
caused by mutations in lysosomal
5. After active treatment, patients should be on a strict
trafficking regulator gene
supportive periodontal maintenance schedule. This
will help prevent the recurrence and progression of 6. Ehler-Danlos syndrome Type III collagen for EDS type
periodontal disease. (EDS types IV and VIII) IV, unknown for type VIII

7. Papillion-Lefèvre Cathepsin C (dipeptidyl

PAPILLON-LEFÈVRE SYNDROME syndrome aminopeptidase I)
It is an inherited disease and tends to form an autosomal 8. Hypophosphatasia Tissue-nonspecific alkaline
recessive pattern. Parents are not affected but both must carry phosphatase
the autosomal genes for the syndrome to appear in the offspring.
9. Trisomy 21 Multiple; critical trisomic
Clinical Features region at least 5 Mb long

10. Prepubertal periodontitis Cathepsin C

• Males and females are equally affected.
(nonsyndromic)
• Disease is usually manifested in childhood.
• Characteristic features include hyperkeratotic skin lesion. 11. Kindler syndrome Defect in actin-extracellular
severe destruction of periodontium and in some cases, matrix linkage caused by loss
of function mutations in KIND-1
calcification of the dura.
Gingival and Periodontal Diseases in Children 175

• Early inflammatory changes followed by rapid bone loss

results in exfoliation of teeth (Fig. 5.16).
• The skin lesions consist of hyperkeratosis and icthyosis of
localized areas on palms, soles, knees and elbows (Figs 5.17
A and B).
• All the primary teeth may be lost by 5 to 6 years of age.
• Permanent dentition erupts normally, but they too are lost
within a few years due to destructive periodontal disease.

Histopathologic Features
• Marked chronic inflammation of the lateral wall of the
pocket with a predominantly plasma cell infiltrate,
considerable osteoclastic activity and apparent lack of
osteoblastic activity and an extremely thin cementum.
• Composition of bacterial flora is similar to that of chronic
periodontitis.
• Spirochete-rich zones in the apical portion of the pockets
as well as spirochete adherence to the cementum and
microcolony formation of mycoplasma species have been
reported.
• Gram-negative cocci and rods appear at the apical border
of the plaque.
• No significant alterations have been found in peripheral
blood lymphocytes and polymorphonuclear leukocytes.
Hypophosphatasia
• Rare genetic disease manifested by bone pain with
spontaneous fractures.
• Low alkaline phosphatase.
FIGURES 5.17A and B: Palmar and plantar keratosis in
• Excretion of phosphoethanolamine. Papillon-Lefèvre syndrome
• Elevation of serum phosphorus.
Clinical Features
Types • Premature loss of primary teeth (Fig. 5.18).
Infantile, childhood and adult . • No gingival inflammation.
• Loss of alveolar bone.
• Absence of cementum.

CYCLIC NEUTROPENIA
• Neutropenia is a rare disorder that causes children to have
lower than normal levels of neutrophils, a type of white
blood cell that destroys bacteria in the blood.
• Periodic episodes of fever and oral ulcerations. When it
occurs in periodic cycles, it is termed as cyclic neutropenia.
The cycle generally occurs over a period of 21 days.
• Periods of profound neutropenia.
• Onset by 10 years of age.
• 19 to 21 day cycle.
• Course: Usually benign.
FIGURE 5.16: Pathologic migration of teeth due to bone loss in Clinical and radiographic pictures of cyclic neutropenia
Papillon-Lefèvre syndrome have been presented in Figures 5.19 and 5.20.
176 Essentials of Pediatric Oral Pathology

A more detailed description about this disorder is

mentioned in the chapter on Blood pathologies in children.

GINGIVAL RECESSION

SELF MUTILATION
Self mutilation may be described as repetitive acts that result
in physical damage to the person. It is extremely rare in normal
children. However, its incidence in the mentally retarded
population is between 10 percent and 20 percent.40
Self-mutilation is a learned behavior as it is reliably
reinforced by gaining attention each time the behavior is
practiced. Any child who willfully inflicts pain or damage on
FIGURE 5.18: Premature loss of primary teeth himself or herself should be considered psychologically
abnormal. Self-mutilation may serve as an escape from reality.
Fisher, 1958, reported that unhappiness and conflict in the home
can be hidden more easily from a 15-year-old child than from
a 15 week old child.41
Self-mutilation has also been associated with biochemical
disorders such as Lesch-Nyhan and de Lange's syndromes.

Clinical Features
• A frequent manifestation of self-mutilation is biting of the
lips, tongue and oral mucosa.
• Self mutilation probably occurs more frequently than is
realized because relatively few children will admit the act
unless they are observed practicing it. Hence diagnosis is
critical.
FIGURE 5.19: Severe gingivitis and ulceration • Children have been observed to traumatize the free and
without evidence of inflammation attached gingival tissues with the finger nail, occasionally
to the extent that the supporting alveolar bone has been
destroyed.
• Stripping of the gingival tissues unilaterally or bilaterally
has been observed, usually with the finger nail or with
articles like a pencil or a bobby pin.
• Chronic cheek biting may be practiced occasionally
producing large necrotic areas.
• An unconventional sucking habit observed by Stewart and
Kernohan, 1973, involves the production of traumatic
gingival recession in infants by a segment of the plastic
shield of a pacifier being embraced by the lower lip such
that the inner surface of the shield bears against the labial
aspect of the incisors and the gingival tissues.42 This results
in an abrasive action during sucking, resulting in gingival
injury, recession and loss of alveolar bone.

Management
1. Behavior modification is the primary mode of
approach in a child displaying self mutilative behavior.
FIGURES 5.20A to D: Marked destruction of bone in cyclic The family can be directed to competent counseling
neutropenia services to treat the emotional problems.
Gingival and Periodontal Diseases in Children 177

2. An attempt should be made to determine the cause. Management

3. If it is found to be the result of local dental factors, it
1. Surgical treatment is indicated only if there is stripping
should be corrected.
of gingival tissue, inability to keep the area clean,
4. Use of restraints, protective padding and sedatives
inflammation, pocket, persistent midline diastema or
may be used.
impedance in speech.
5. If the use of restraints and protective padding is
2. Surgical treatment involves a frenotomy or a
unsuccessful, extraction of the selected teeth may be
frenectomy.
necessary.
3. Frenotomy involves incision of the periosteal fiber
attachment and suturing of the frenum to the
ABNORMAL FRENUM ATTACHMENT periosteum at the base of the vestibule.
4. Frenectomy involves complete excision of the frenum
Henry, Levin and Tsaknis, 1976, describe a frenum as a mucous
and its periosteal attachment. It is indicated when
membrane fold containing epithelium and connective tissue large, fleshy frenums are involved.
fibers, but no muscle.43 A normal frenum attaches apically to
the free gingival margin so as not to exert a pull on the zone of
REFERENCES
the attached gingiva, usually terminating at the mucogingival
junction. 1. McCall JO. Gingival and periodontal disease in children. J
Most commonly freni are found on the facial gingival Periodontol 1938;9:7.
surface of the anterior midline of the maxilla, on the facial and 2. Baer PN. Periodontal disease in children and adolescents.
Philadelphia: JB Lippincott Co, 1974.
lingual gingival surfaces of the anterior midline of the mandible
3. Armitage GC. Development of a classification system for
and on the mandibular and maxillary premolar facial area. Some
periodontal diseases and conditions. Ann Periodontol 1999;4:
frena have bifid and trifid attachment to the alveolar process. 1-6.
4. Dhar V, et al. Prevalence of gingival diseases, malocclusion and
CLINICAL FEATURES fluorosis in school going children of rural areas in Udaipur. J
• An abnormal or high frenum is present when there is Ind Soc Pedodontics and Preventive Dent, 2007.
inadequate attached gingiva in the terminal insertion area. 5. Löe H, Listgarten MA, Terranova VP. The gingiva: structure
and function. In: Genco RJ, Goldman HM, Cohen DW, editors.
• The abnormal frenum attachment is most often observed
Contemporary Periodontics. St. Louis: Mosby, 1990.
in the central incisor area though it may involve the labial
6. Lindhe J, Axelsson P. The effect of controlled oral hygiene and
tissue in the canine areas. topical fluoride application in caries and gingivitis in Swedish
• A close attachment of the frenum to the gingival margin school children. Community Dent Oral Epidemiol 1973;1:9.
may interfere with proper toothbrush placement, may cause 7. Poulsen S, Agerback N, Melson B, et al. The effect of
opening of the gingival crevice during function, or may professional tooth cleaning in gingivitis and dental caries in
interfere with the speech. children after 1 year Community Dent Oral Epidemiol
• Abnormal frenum attachments may also be associated with 1976;4:195.
isolated gingival recessions and diastemas, though a cause 8. Matsson L, Moller C. Gingival inflammatory reactions in
and effect relationship may or may not be involved. children with rhinoconjunctivitis due to birch pollinosis. Scand
• Isolated gingival recession may also lead to pocket J Dent Res 1990;98:504-9.
formation. 9. Schaff JE. Non-dental pharmacotherapeutics. Alumni Bull Sch
• Lip movements may cause the abnormal frenum to pull on Dent Indiana Univ. Spring, 1984.
the fibers inserting into the free gingival margin. This could 10. Zunt SL. Personal communication, 1999.
11. Barile MF, Graykowski EA, Driscoll EJ, Riggs DB. L form of
lead to food accumulation, inflammation, pocket
bacteria isolated from recurrent aphthous stomatitis lesions. Oral
development between the labial surface of the tooth and
Surg 1963;16:1395.
the vestibular mucosa.
12. Graykowski EA, Barile MF, Lee WB, Stanley HR. Recurrent
• Eventually, continued stripping of the labial tissue may aphthous stomatitis, clinical, therapeutic, histopathologic and
occur resulting in subsequent loss of alveolar bone and hypersensitivity aspects. JAMA 1966;196:637.
possibly even the tooth. 13. Lehner T. Pathology of recurrent oral ulceration and oral
• Impedance of speech may occur, especially the /t/, /d/ ulceration in Beçhet's syndrome: light, electron and fluorescence
and /l/ sounds due to a short mandibular lingual frenum microscopy. J Pathol 1969;97:481.
inhibiting the tongue from touching the maxillary central 14. Cooke BED. Recurrent Mikulicz's aphthae. Dent Pract
incisors. 1961;12:116.
178 Essentials of Pediatric Oral Pathology

15. Brooke RI, Sapp JP. Herpetiform ulceration. Oral Surg 30. Kimball OP. Treatment of epilepsy with sodium diphenyl-
1976;42:182. hydantoinate. JAMA 1939;112:1244-5.
16. Wood TA Jr, DeWitt SH, Chu EW, Rabson AS, Graykoswi EA. 31. Ranney R. Classification of periodontal diseases. Periodontol
Anitschkow nuclear changes observed in oral smears. Acta Cytol 2000;1993(2):13.
1975;19:434. 32. Newman MG, Sims TN. The predominant cultivable microbiota
17. Meiller TF, et al. Effect of antimicrobial mouthrinse on recurrent of the periodontal abscess. J Periodontol 1979;50:350.
aphthous ulcerations. Oral Surg Oral Med Oral Pathol 33. Nelson D, Potempa J, Kordula T, Travis J. Purification and
1991;72:425-9. characterization of a novel cysteine proteinase (Periodontain)
18. Reade PC. Infantile acute ulcerative gingivitis: a case report. J from Prophyromonas gingivalis. J Biol Chem 1999;274:122-45.
Periodontol 1963;34:387-90. 34. Socransky SS, Haffajee AD. Microbial mechanisms in the
19. McDonald JB, Socansky S, Gibbons RJ. Aspects of the pathogenesis of destructive periodontal diseases: a critical
pathogenesis of mixed anaerobic infections of mucous assessment. J Periodontol Res 1991;26:195.
membranes. J Dent Res 1963;42:529-44. 35. Page RC, et al. Clinical laboratory studies of a family with a
20. Goldhaber P, Giddon DB. Present concepts concerning the high prevalence of juvenile periodontitis. J Periodontol
etiology and treatment of acute necrotizing ulcerative gingivitis. 1985;56:602-10.
Int Dent J 1964;14:468. 36. Rams TE, Keyes PH, Wright WE. Treatment of juvenile
21. Schaffer EM. Biopsy studies of necrotizing ulcerative gingivitis. periodontitis with microbiologically modulated periodontal
J Periodontol 1953;24:22. therapy (Keyes technique). Pediatr Dent 1985;7:259-70.
22. Listgarten MA, Lewis DW. The distribution of spirochetes in 37. Schenkin HA, Gunsolley JC, Koertge TE, et al. Smoking and
the lesion of acute necrotizing ulcerative gingivitis: an electron its effects on early onset periodontitis. J Am Dent Assoc
microscopic and statistical survey. J Periodontol 1967;38:379. 1995;126:1107.
23. Lehner T. Oral thrush or acute pseudomembranous candidiasis. 38. Walker C, Karpinia K. Rationale for use of antibiotics in
A clinicopathologic study of 44 cases. Oral Surg 1964;18:27. periodontics. J Periodontol 2002;73:1188.
24. Prinz H. Chronic diffuse desquamative gingivitis. Dent Cosmos 39. Quirynen M, Bollen CN, Vandekerckhove BN, et al. Full vs
1932;74:331. partial mouth disinfection in the treatment of periodontal
25. McCarthy FP, McCarthy PL, Shklar G. Chronic desquamative infections: short term clinical and microbiological observations.
gingivitis: a reconsideration. Oral Surg 1960;13:1300. J Dent Res 1995;74:1459.
26. Mombelli A, Lang NP, Burgin WB, et al. Microbial changes 40. DenBesten PK, McIver FT. Oral self-mutilation in a child with
associated with the development of puberty gingivitis. J congenital toxoplasmosis: a clinical report. Pediatr Dent 1984;
Periodontol Res 1990;25:331. 6:98-101.
27. Zackin SJ, Weisberger D. Hereditary gingival fibromatosis: 41. Fisher GD. Growth and development of child. J Dent Child
report of a family. Oral Surg Oral Med Oral Pathol 1961;14:825- 1958;25:69-83.
35. 42. Stewert DJ, Kernohan DC. Traumatic gingival recession in
28. Hart TC, et al. Genetic linkage of hereditary gingival fibromato- infants: the result of dummy sucking habit. Br Dent J 1973;
sis to chromosome 2p21. Am J Hum Genet 1998;62:876-83. 135:157-8.
29. Merrit HH, Putnam TJ. Sodium diphenylhydantoinate in 43. Henry SW, Levin MP, Tsaknis PJ. Histological features of
treatment of convulsive disorders. JAMA 1938;111:1068-73. superior labial frenum. J Periodontol 1976;47:25-8.
6 Cysts in the Pediatric Population 179

Cysts in the
Pediatric Population
Mayur Chaudhary, Shweta Dixit Chaudhary

CHAPTER OVERVIEW
Introduction Dermoid cyst
Definition Epidermoid cyst
Classification Teratoid cyst
Odontogenic keratocyst Thyroglossal tract cyst
Dentigerous cyst Intraoral lymphoepithelial cysts
Eruption cyst Lingual cyst of foregut origin
Gingival cyst of infant Cystic hygroma
Midpalatal raphae cyst Parasitic cysts:
Radicular cyst Hydatid cyst
Residual cyst Cysticercus cellulosae
Solitary bone cyst Cysts of salivary glands:
Aneurysmal bone cyst Mucoceles
Calcifying odontogenic cyst Ranula

INTRODUCTION ODONTOGENIC KERATOCYST

Human teeth, which are so casually used each time we eat, talk, Philipsen, 1956,3 first described the term odontogenic keratocyst
swallow, etc. are formed by the complex process of (OKC). It is named odontogenic keratocyst because the epithelial
odontogenesis, where normal tooth structure is formed from lining of cyst produces keratin that gets filled within the cystic
the odontogenic apparatus. Deviation from the normal process lumen. They are aggressive in nature and thus Toller, 1967,4
of odontogenesis leads to formation of cysts and tumors. Cysts suggested that they may be regarded as benign cystic neoplasms.
of odontogenic origin in children are one of the aberrations
from the normal process of odontogenesis which form a diverse PATHOGENESIS
group of lesions. Varying theories regarding its pathogenesis exist:
• Arises from odontogenic epithelium—Dental lamina rests
DEFINITION • Arises from basal cells from the overlying oral epithelium
• Derived from enamel organ in its early stages of
Kramer, 1974 described cyst as a pathological cavity having
development by degeneration of stellate reticulum before
fluid, semifluid or gaseous contents and which is not created calcification of any hard tissue of teeth
by the accumulation of pus; it is frequently but not always lined • Satellite/daughter cyst—Arises from dental lamina rests.
by epithelium.1
TYPES OF ODONTOGENIC KERATOCYST (FIG. 6.2)
CLASSIFICATION2
Main, 1970, described an 'envelopmental' variety of cyst
See Figure 6.1. radiographically where an OKC embraces an adjacent unerupted
180 Essentials of Pediatric Oral Pathology
Cysts in the Pediatric Population 181

FIGURE 6.2: Types of OKC (Main-1970)5

FIGURE 6.3: Follicular keratocyst

FIGURE 6.1: Classification of cysts2

tooth.5 The cysts that were formed in place of tooth were termed
as 'replacement' variety. Those that occurred in ascending ramus
were named as 'extraneous' and those that occurred adjacent to
the tooth roots were termed as 'collateral' variety.
Replacemental variety is also called as primordial cyst. FIGURE 6.4: Odontogenic keratocyst presenting as a painful
One more variety of OKC is follicular keratocyst (Fig. 6.3). swelling in the lower left posterior region of the jaw
A tooth surrounded by its follicle erupts into a keratocyst
cavity in the same way as it erupts in the oral cavity.
• Seen more often in males as compared to females
• Occurrence in mandible is more than in maxilla
CLINICAL FEATURES
— In mandible more common in posterior region (Fig. 6.4)
• Seen in 1st to 9th decade of life (more often in the 2nd, — In the mandibular posterior region, more often seen in
3rd and 5th decades) ramus/angle of mandible.
Meara et al, 1996, made a pediatric institutional review • Signs and symptoms associated with the cyst often present
of OKC’s. Their ages ranged from 8 to 18 years. They as pain, swelling, discharge and paresthesia of lower lip
found OKC’s in 11 children6 • OKC extends in the medullary cavity—Hence observable
• Incidence is 12 to 13 percent of total jaw cysts expansion of bone occurs late
182 Essentials of Pediatric Oral Pathology

Connective Tissue Wall

• Fibrous capsule of the cyst is usually thin
• Epithelium—connective tissue attachment is weak and
tends to separate from each other in many areas
• Few to many daughter/satellite cysts are seen.
Rarities: Cholesterol clefts, melanin pigment, hyaline bodies,
mast cells, mucous metaplasia.
Recurrence rate is very high in OKC (20–60%)
Why does OKC Recur?
FIG URE 6. 5: Od ontog enic k erat oc y s t with mult iloc u lar
radiolucent area and well-defined anterior margin extending
• Tendency to multiplicity is due to satellite cyst formation
towards premolar region on left side and towards the canine • Incomplete removal of lining may occur because lining is
region on the right side thin and fragile and attachment between the two is weak
• There is a particular predisposition to form OKC from
dental lamina rests
In Maxilla—Buccal cortical plate expansion is more • Proliferation of basal cells of oral epithelium.
In Mandible—Both buccal and lingual cortical plate
expansion is seen Expansion of cyst is due to the following factors:
• Syndrome associated with odontogenic keratocyst: Naevoid • Hydrostatic pressure
basal cell carcinoma syndrome (Gorlin-Goltz syndrome). • Active epithelial growth
Its salient features are: • Production of bone resorbing factor by the cystic capsule/
— Multiple OKCs wall
— Multiple basal cell carcinomas • Accumulation of certain proteins, e.g. keratin.
— Abnormalities of Ca++ and PO4– metabolism which are
Management
manifested by calcification of falx cerebri, bifid rib and
vertebral deformities. 1. Ghali and Connor, 2003, suggested surgical
enucleation of the lesion.7
2. Schmidt, 2003, suggested the use of liquid nitrogen
RADIOGRAPHIC FEATURES (Fig. 6.5)
cryotherapy for management of OKC’s.8
• Small, round or ovoid radiolucent areas demarcated with 3. Stoleinga, 2003, suggested excision of the overlying,
sclerotic margins attached mucosa in conjunction with cyst enucleation
• Unilocular more often than multilocular and treatment of bony defect with Carnoy solution.9
• Scalloped margins are present which are mistaken for 4. Pogrel, 2003, suggested decompression and
marsupialization.10
multilocularity
• Downward displacement of inferior alveolar canal
• Resorption of lower cortical plate of mandible as well as
perforation of bone
• Fracture of the mandible may occur
• Displacement of roots but resorption of roots is rarely
seen.

HISTOPATHOLOGIC FEATURES

Epithelium
• Parakeratinized (80%) or Orthokeratinized (20%) stratified
squamous epithelium
• Corrugated epithelium (Fig. 6.6)
• 5 to 8 cell layer thick
• No rete ridges (rete pegs) FIGURE 6.6: Histopathologic picture of odontogenic keratocyst
• Basal cells are columnar to cuboidal and show palisading. showing a corrugated epithelium, 6 to 8 cells thick
Cysts in the Pediatric Population 183

FIGURE 6.8: Types of dentigerous cysts

FIGURE 6.7: Radicular cyst mimicking dentigerous cyst

DENTIGEROUS CYST (FOLLICULAR CYST)

Browne has explained the meaning of dentigerous as 'tooth
bearing'.11

PATHOGENESIS
• Occurs due to accumulation of fluid
— Either between the reduced enamel epithelium and the
enamel or
— Within the enamel organ itself. FIGURE 6.9: High power view of dentigerous cyst showing a
Lustmann and Shear, 1985,12 Wood et al, 1988,13 suggested thin, regular lining of non-keratinized squamous epithelium
that sometimes radicular cyst (periapical cyst) may mimic a
dentigerous cyst (Fig. 6.7). • Well-defined sclerotic margins unless infected
• Rarely resorption of lower cortical plate of mandible
CLINICAL FEATURES • Displacement of roots may be seen but rarely is resorption
• Incidence is 16 to 18 percent of total jaw cysts (2nd highest of roots seen
in occurrence) • Radiographically, three different varieties of dentigerous
• Seen in 1st to 3rd decade of life cysts have been described (Fig. 6.8):
• Seen more often in males than females — In central variety, the crown is enveloped symmetrically.
• Occurrence in mandible is more than in maxilla with the — Dilatation of the follicle on one aspect of crown results
ratio of 2:1. Incidence in tooth no.: (38, 48) > (13, 23) > in lateral variety of dentigerous cyst.
(34, 35, 44 and 45) > (18, 28) (FDI system) — In circumferential variety, the entire tooth is enveloped
• Seen more often in whites as compared to blacks by the cyst.
• Bodner, 2002, in a series of 69 pediatric patients with cystic
lesions of jaws found 31 were dentigerous cysts14 HISTOPATHOLOGIC FEATURES
• In the presence of a dentigerous cyst, the tooth of the Cyst is always attached to the neck (CEJ) of the tooth.
permanent series is missing (impacted)
• At times, a supernumerary tooth or cystic odontome may Epithelium
be involved in cyst formation, then the complement of teeth • Is in fact reduced enamel epithelium (Fig. 6.9)
in the arch remains the same • Lining of the cyst is thin—2 to 3 layers of flat or cuboidal cells
• Patient does not present with pain unless infected • Non-keratinized
• Swelling is rarely seen (uncommon). • Occasionally, the lining might form keratin by metaplasia.
RADIOGRAPHIC FEATURES Connective Tissue Wall
• Small round, ovoid unilocular radiolucent areas associated • Thin fibrous cyst wall (which is derived from dental
with crowns of unerupted teeth follicle) is present
184 Essentials of Pediatric Oral Pathology

• Consists of young fibroblasts widely separated by stroma

and ground substance
• Shibata et al, 2004, in their study on 70 patients under the
age of 16 years found that inflammatory change at the apex
of a deciduous tooth may be responsible for initiating a
dentigerous cyst of the permanent successor.15
Rarities: Mucous metaplasia of the lining, cholesterol clefts
are seen.
Complications
• Ameloblastoma
• Mucoepidermoid carcinoma
• Squamous cell carcinoma.
FIGURE 6.10: Eruption cyst presenting as a painless swelling in
Management the maxillary anterior region
1. Conservative surgical removal (enucleation) of the
cyst followed by orthodontic treatment.
• Cyst produces a smooth swelling over the erupting tooth,
2. Hyomoto et al, 2003, investigated dentigerous cysts
involving 47 mandibular premolars and 11 maxillary
which may be of normal color or slightly bluish in color
canines in pre-adolescent children and suggested that (Fig. 6.10)
a period of 100 days after marsupialization is the • Swelling is soft, fluctuant and painless
critical time for deciding whether to extract or to use • Deciduous anterior and molar region, permanent anterior
traction.16 and premolar region are the common sites where eruption
cyst is often observed.
ERUPTION CYST
RADIOGRAPHIC FEATURES
It is a type of dentigerous cyst occuring in the soft tissues.
Cyst may throw a soft tissue shadow, but there is usually no
PATHOGENESIS bone involvement; except that the dilated and open crypt may
be seen on the radiograph.
Similar to dentigerous cyst.
HISTOPATHOLOGIC FEATURES (Fig. 6.11)
CLINICAL FEATURES
• Hematoxylin and eosin stained section of the lesional tissue
• Bodner, 2002, in a series of 69 pediatric patients with cystic shows a covering of keratinized stratified squamous
lesions of jaws reported 15 eruption cysts indicating a epithelium
higher frequency of cystic lesions of jaws in children with • Underlying connective tissue is fibrous
these cysts14 • 2 to 4 cells thick stratified squamous epithelium resembling
• Aguilo et al, 1998, in a series of 27 patients reported 36 reduced enamel epithelium is sometimes seen with cystic
eruption cysts17 space.
• Bodner et al, 2004, reported the cysts associated with natal
teeth in 2 cases. The primary mandibular central incisors Management
and permanent first molars were the teeth most frequently
involved, and boys were affected twice as often as girls18 1. No treatment is required in most cases. The cyst bursts
• Ramon Boj and Garcia-Godoy, 2000, reported a case of on its own and the tooth erupts into the oral cavity.
a 15 month old child with 6 eruption cysts19 2. Marsupialization: The dome of the cyst is excised,
• Kuczek et al, 2003, reported that eruption cysts developed exposing the crown of the tooth which is allowed to
erupt.
in a boy treated with cyclosporin A following a heart
transplant20
• They are seen more frequently clinically and some burst GINGIVAL CYST OF INFANT (GCOI) AND
spontaneously MIDPALATAL RAPHAE CYST (MPRC)
• These are usually not excised and are therefore not Both these entities are discussed together as they share the same
submitted for histological examination clinical features.
Cysts in the Pediatric Population 185

FIGURE 6.11: Histopathologic picture of eruption cyst showing a covering FIGURE 6.12: Epstein's pearls
of keratinized stratified squamous epithelium, underlying fibrous
connective tissue and 2 to 4 cells thick reduced enamel epithelium

But gingival cyst of infant is odontogenic in origin and

midpalatal raphae cyst (MPRC) is non-odontogenic in origin.

PATHOGENESIS
• Gingival cyst of infant arises from dental lamina rests (some
open into the oral cavity and some are involved with teeth).
• Midpalatal raphae cyst—Arises from epithelial inclusions
at the line of fusion of palatal folds and nasal processes
(after birth these inclusions usually atrophy and become
resorbed).

CLINICAL FEATURES
• Gingival cyst of infant is mainly seen in newborn infants,
but they are rarely seen after three months of age
• Most of them either undergo involution and disappear or
rupture through the surface epithelium
• Nodular growths seen on the midpalatal raphae region are
FIGURE 6.13: Bohn's nodules
termed as Epstein's pearls (Fig. 6.12) and on alveolar
ridges (buccal and lingual) are known as Bohn's nodules • Basal cells are flat
(Fig. 6.13) • As a result of cystic pressure on the oral epithelium, the
• Ikemura et al, 1983, in their study on 541 Japanese neonates, latter might appear as atrophic.
reported a frequency of 89 percent of these cysts21
• Dilley et al, 1991, reported a frequency of 50 percent of Management
palatal and alveolar cysts in neonates22
No indication for treatment. Once their contents are
• Liu and Huang, 2004, reported a frequency of 94 percent
in 420 Taiwanese neonates.23 expelled, they atrophy and disappear.

HISTOPATHOLOGIC FEATURES RADICULAR CYST (PERIAPICAL CYST)

(SAME FOR BOTH)
Radicular cysts are the most common inflammatory cysts and
• Thin lining of stratified squamous epithelium with arise from the epithelial residues in the periodontal ligament
parakeratotic surface and cystic cavity (lumen) filled with as a result of periapical periodontitis following death and
keratin necrosis of the pulp.
186 Essentials of Pediatric Oral Pathology

PATHOGENESIS
• Radicular cyst arises from odontogenic epithelial residues
in the periodontal ligament.
• Caries Inflammation of pulp Death and necrosis of
pulp Cyst at the apex of root.

THREE PHASES

Phase of Initiation
Meghji et al, 1996, showed that higher levels of bacterial
endotoxins initiate the proliferation of epithelial cell rests of
Malassez.24

Phase of Cyst Formation

The most widely accepted theory, postulates that a cyst cavity
forms within a proliferating epithelial mass in an apical FIGURE 6.14: Radicular cyst, associated with carious primary
molars. Deviated eruption of maxillary first premolar can be seen
granuloma by degeneration and necrosis of the cells in the center.

Phase of Enlargement
Toller, 1970, hypothesized that osmosis is responsible for
growth and enlargement of the radicular cyst.25

CLINICAL FEATURES
• Incidence of 50 to 55 percent of total jaw cysts (highest
occurrence) FIGURE 6.15: Enlargement of radicular cyst
• Occurs most frequently in 3rd to 4th decade of life (but
any age group may be affected) • In maxilla, buccal and/or palatal expansion of bone is seen,
• More common in males than females whereas, in mandible, labial/buccal expansion but rarely
• Occurrence in maxilla is more as compared to mandible. lingual expansion is seen
In maxilla, occurrence in anterior region is most • Sinus formation from the cyst cavity onto the surface of
common. the oral mucosa or skin is occasionally seen.
• Deciduous teeth involvement is very rare. 0.5% of all radicular
cysts as recorded in the University of Witswatersrand over a RADIOGRAPHIC FEATURES (Fig. 6.16)
period of 25 years • Round, ovoid radiolucency surrounded by a narrow radio-
• Deciduous teeth when involved are usually the primary molars opaque (sclerotic) margin which extends from the lamina
with caries being the most common etiological factor, as reported dura of the involved tooth
by Lustmann and Shear, 1985 (Fig. 6.14).12 In 23 cases of • In rapidly enlarging cyst or highly infected cyst the radio-
the age range 4 to 12 years, they reported 9 cases with buccal opaque margin may not be present
expansion and in 8 cases permanent tooth buds were displaced • Root resorption may occur but is rare, displacement of
• Lustmann and Shear, 1985, reported only 28 cases of adjacent tooth roots may be seen.
radicular cyst in 1898 patients12
• Radicular cyst is usually symptomless, found on routine HISTOPATHOLOGIC FEATURES
radiographic examination of non-vital pulp
• At first the enlargement is bony hard but as the cystic cavity Epithelium
increases in size the covering bone becomes very thin and • Non-keratinized stratified squamous (1-50 cell layer thick)
the swelling exhibits springiness (Fig. 6.15) (Fig. 6.17)
• Only when the cyst completely erodes the bone, will the • Proliferation of epithelial lining with arcading pattern
lesion appear fluctuant (Fig. 6.18)
Cysts in the Pediatric Population 187

FIGURE 6.16: Radiographic picture of a radicular cyst, FIGURE 6.18: High power view of radicular cyst showing
associated with carious primary molars arcading pattern of the epithelium

Rushton Bodies
• Present in epithelium (rarely in connective tissue)
• Measure about 0.1 mm
• Linear, straight, curved or hair-pin shaped and sometimes
concentrically laminated
• Brittle and fracture frequently during histological sectioning
• Circular hyaline bodies are also seen with clear outer layer
surrounding a central granular body.
What are Rushton bodies?
• They are keratinized secondary enamel cuticle
• They are secretory products of odontogenic epithelium
• Thrombi in the venules of connective tissue.
Types of Rushton bodies
• Type I—Has no central granular component.
FIGURE 6.17: Low power view of radicular cyst showing arcading • Type II—Fine grained matrix which encloses the coarse-
pattern of the epithelium. Connective tissue shows blood vessels, grained foreign material and undergoes a different degree
collagen fibers and inflammatory cells of homogenization.
Cholesterol crystals are found in most of the mature radicular
• Inflammatory cells are present, mostly neutrophils cysts (Fig. 6.19).
• Mucous cells were observed in 40% of the cases (incidence
increases with age) Source of Cholesterol
• Hyaline bodies, called as Rushton's Bodies (rarely seen • Released from disintegrating RBCs
in connective tissue wall). • Degeneration and disintegration of lymphocytes, plasma
cells and macrophages
• Circulating plasma lipids
Connective Tissue Wall
Once the cholesterol crystals are deposited—It evokes a
Composed of three layers: foreign body giant cell reaction.
1. Inner granulomatous layer Cholesterol crystals in the connective tissue of radicular
2. Intermediate layer cysts get dissolved in the chemical reagents used during tissue
3. Outer fibrous connective tissue layer. processing, leaving empty spaces as seen within the connective
188 Essentials of Pediatric Oral Pathology

FIGURE 6.21: Formation of a residual cyst

SOLITARY BONE CYST (SBC)

(Traumatic, Simple, Hemorrhagic Bone Cyst)
It is a pseudo cyst, as these categories of lesions are usually
fluid filled or empty intraosseous lesions found most commonly
FIGURE 6.19: Radicular cyst focused on a cholesterol crystal in the proximal metaphyseal region of the long bones in
children and adolescents.

HOWE'S CRITERIA FOR PSEUDOCYST

• Cyst should be single
• Should have no epithelial lining
• Should show no evidence of acute or chronic infection
• Should contain principally fluid and not soft tissue.

PATHOGENESIS
• Not known
• Various hypotheses have been put forth to explain the
pathogenesis of a solitary bone cyst:
— Trauma to bone causes intermedullary hemorrhage.
Failure of early organization of hematoma in the
marrow spaces and subsequent liquefaction of the clot
FIGURE 6.20: Histopathologic picture of radicular cyst
showing cholesterol clefts
leads to formation of solitary bone cyst.
— Mesenchymal cells which usually form bone or cartilage
tissue in hematoxylin and eosin stained section. These empty fail to do so. Instead of forming bone or cartilage, mesen-
spaces are then termed as cholesterol clefts (Fig. 6.20). chymal cells form synovial tissue. Thus, solitary bone
cyst might originate as multiple synovial cavities which
Complications: Very rarely, may undergo a carcinomatous change. later coalesce to form a larger lined defect.

Management CLINICAL FEATURES

1. Endodontic treatment of the offending tooth often
• Diagnosed by chance on routine examination
results in regression of the cyst.
• Occurs in age range 2 to 35 years but more common in
2. Surgical removal with/without extraction of the
offending tooth
2nd decade
• In Howe's analysis, 1965, the patients ranged in age from
2.5 to 35 years27
RESIDUAL CYST (Fig. 6.21)
• Sheffield's analysis of 36 cases showed the presence of 53
Quite often a radicular cyst remains behind in the jaws after percent of the lesions in children less than 16 years of age28
removal of the offending tooth. • Occurs with equal frequency in males and females
High and Hirschmann, 1986, showed that ages of patients • Occurrence in mandible is more compared to maxilla.
varied from 1 month to 20 years.26 Mandibular posterior region is most often involved
Cysts in the Pediatric Population 189

FIGURE 6.22: Radiographic picture of a solitary bone cyst showing FIGURE 6.23: Aneurysmal bone cyst showing the
a radiolucent area with an irregular but definite edge. Lesion typical blow out of the bone
remained undiagnosed since childhood

• Swelling, pain, labial paresthesia may occur but are rare • Cyst may result from vascular disturbances in the form of
• Previous history of trauma may be present sudden venous occlusion
• All related teeth are vital. • Pre-existing lesions (fibrous dysplasia, ossifying fibroma,
giant cell tumor, giant cell granuloma, osteoblastoma,
RADIOGRAPHIC FEATURES (FIG. 6.22) solitary bone cyst, chondroblastoma and myxofibroma) may
• Cyst appears as a radiolucent area with an irregular but initiate an arterio-venous malformation which may lead to
definite edge the formation of aneurysmal bone cyst.
• Scalloping is a prominent feature.
CLINICAL FEATURES
HISTOPATHOLOGIC FEATURES • Occurs between 1st-3rd decade of life
• No epithelial lining • Jones and Franklin, 2006, reported only 11 cases of
• Consists of loose vascular fibrous tissue membrane of aneurysmal bone cyst over a period of 30 years, in the
variable thickness Sheffield series. This was a mere 0.15 percent of the 7224
• Fibrin with RBCs are seen jaw cysts. The lesion was relatively more common in
• Hemorrhage and hemosiderin pigments are usually present children accounting for 0.7 percent of the 590 jaw cysts in
• The adjacent bone when included in the specimen shows patients lower than 16 years old28
osteoclastic resorption. • Seen most commonly in females as compared to males
• Occurrence in mandibular posterior region is more common
Management than in maxilla
1. Surgery is the treatment of choice. • Presents as swelling of the jaws, rarely painful
2. Cyst lumen is opened to reveal an empty cavity, the cyst • Enlargement is rapid and malocclusion frequently becomes
wall is then curetted. In few cases, blood or sero- progressively worse
sanguinous fluid may be present. Precaution is required • History of displacement of teeth may be present
to prevent damage to the inferior alveolar nerve. • If lesion perforates the cortex then egg-shell crackling is
evident.
ANEURYSMAL BONE CYST (ABC)
This is also a pseudo cyst. The term "aneurysmal bone cyst" HISTOPATHOLOGIC FEATURES
was suggested by Jaffe and Lichtenstein, 1942, to describe • Lesion consists of many capillaries and blood filled spaces
the characteristic blow out of the bone (Fig. 6.23).29 (not lined by endothelial cells) of varying sizes lined by
flat spindle cells, separated by delicate loose-textured
PATHOGENESIS fibrous tissue (Fig. 6.24)
• Pathogenesis of aneurysmal bone cyst is controversial • Blood filled spaces have no endothelial lining, no-elastic
• May be due to trauma to the bone, although there is little or smooth muscle around them
evidence to support this • Giant cells are seen surrounding the blood spaces
190 Essentials of Pediatric Oral Pathology

Dentinoid or odontome may be found in the cyst wall, induced

by the lining epithelium.

CLASSSIFICATION {PRAETORIUS et al (1981)}31

Type I A. Simple unicystic type
B. Odontome producing type
C. Ameloblastomatous proliferating type
Type II Neoplasm with some histopathological features of
COC.

CLINICAL FEATURES
• Praetorius, 2006, reported the youngest patient with
calcifying odontogenic cyst of one year and the oldest patient
of 82 years indicating a wide age-range with an impressively
high peak in the second decade
• Incidence of 1 to 2 percent of total jaw cysts
• Males are affected more often as compared to females
• Occurs with equal frequency in mandible and maxilla.
Anterior part of the jaw is affected more often
FIGURE 6.24: Histopathologic picture of an aneurysmal bone
cyst showing blood filled spaces surrounded by giant cells • Swelling is the most frequent complaint
• Rarely is the swelling accompanied by pain
• Osteoid bone formation in few areas is also evident • Lingual expansion of cortical bone is noted
• Fibroblasts, histiocytes and hemosiderin pigment are also • Occasionally the COC may perforate the cortical plate and
seen in areas extend into the soft tissue.
• Solid areas of cyst may have the appearance of a primary
RADIOGRAPHIC FEATURES
lesion.
• Regular outlined radiolucent lesion with well demarcated
Management margins
• Lesion is usually unilocular/occasionally multilocular.
1. According to El-Deeb et al, 1980, thorough curettage • Irregular opacities in the radiolucency may be seen
with removal of primary tumor if any is the preferred
(calcified bodies)
treatment.30
• Displacement of teeth is a common finding
2. According to El-Deeb et al, 1980, recurrence is up to
26 percent. It depends on the primary tumor (Ossifying
• Resorption of adjacent roots is a frequent finding
fibroma cases have shown high recurrence rate).30 • Local expansion sometimes occurs and perforation of the
cortical plate may be evident.
CALCIFYING ODONTOGENIC CYST
HISTOPATHOLOGIC FEATURES (FIG. 6.25)
(Gorlin cyst; Odontogenic ghost cell tumor)
• Epithelium may be regular, 6 to 8 cells thick
Calcifying odontogenic cyst (COC) was considered to be quite • The epithelial lining has characteristic odontogenic features
a confusing entity. Praetorius et al, 1981, concluded that what with a prominent basal layer consisting of palisaded
had previously been regarded as a calcifying odontogenic cyst columnar or cuboidal cells and hyperchromatic nuclei
actually comprised two entities: a cyst and a neoplasm.31 In which are polarized away from the basement membrane
the latest WHO publication on odontogenic tumors (Praetorius • Sometimes the epithelium represents squamous cells and
and Ledesma-Montes, 2005), it was classified as a benign sometimes stellate reticulum like cells
odontogenic tumor and was renamed calcifying cystic • Ghost cells are seen in the epithelial lining. These cells
odontogenic tumor (CCOT).32 sometimes penetrate the connective tissue wall and evoke
a foreign body giant cell reaction.
PATHOGENESIS • Ghost cells:
It may arise from: — They are pale and eosinophilic and although the cell
• Reduced enamel epithelium outlines are usually well-defined, they may sometimes
• Remnants of odontogenic epithelium. be blurred so that groups of them may fuse
Cysts in the Pediatric Population 191

FIGURE 6.25: Histopathologic picture of calcifying odontogenic FIGURE 6.26: Histopathologic picture of dermoid cyst showing a
cyst showing ghost cells in the epithelial lining lining of stratified squamous epithelium. Numerous sebaceous
glands are also evident

— A few ghost cells may contain nuclear remnants but • When located above mylohyoid muscle, it displaces the
these are in various stages of degeneration and in the tongue superiorly and posteriorly
majority, all traces of chromatin have disappeared • Below mylohyoid, a midline swelling of neck occurs
leaving only a faint outline of the original nucleus. • The cyst generally appears as painless and slow growing
— Ghost cells represent an abnormal keratinization and • It does not have a particular sex predilection
have an affinity for calcification. • On palpation, the cyst is soft and doughy because of keratin
— Some workers believe that it is amelogenin like protein and sebum in the lumen.
and not keratin.
— Ghost cells are also seen in odontoma, ameloblastic HISTOPATHOLOGIC FEATURES (FIG. 6.26)
fibro-odontome.
• The cyst is lined by stratified squamous epithelium
Management supported by fibrous connective tissue
• Lumen shows keratin or sebum
1. Surgical enucleation.
• Numerous secondary skin structures, including hair
2. If associated with any other odontogenic tumor, wider
follicles, sebaceous glands and sweat glands, may be found.
excision is required.
3. Conservative approach if associated with complex
odontome. Management
1. Surgical excision. Those cysts located above the
DERMOID CYST geniohyoid muscle can be removed by the intraoral
approach.
It is a developmental lesion occurring in many areas of the 2. Below geniohyoid muscle, the extraoral approach
body. It is considered to be a hamartomatous tumor containing should be employed.
multiple sebaceous glands and almost all skin adenexa. It may
also contain substances such as nails and dental, cartilage-like EPIDERMOID CYST
and bone-like structures. (Epidermal Inclusion Cyst) (FIG. 6.27)
ETIOLOGY The term epidermoid cyst is used in a general context, in that,
• Developmental entrapment of multipotential cells irrespective of the source of the epithelium, the term persists.
• Sequestration of skin and subsequent implantation of Was also called as a sebaceous cyst, but this is a misnomer as
epithelium along the lines of embryonic closure. this cyst is not of sebaceous origin.

CLINICAL FEATURES ETIOLOGY

• Yoshimura et al, 1970,33 and Yeschua et al, 1977,34 reported • Sequestration or implantation of epidermal rests during
an age incidence between 15-35 years with a wide age- embryonal period
range from birth to senility • Occlusion of the pilosebaceous unit
192 Essentials of Pediatric Oral Pathology

FIGURE 6.27: Epidermoid cyst presenting as a soft fluctuant FIGURE 6.28: Histopathologic picture of epidermoid cyst
swelling near the outer canthus of eye showing a keratin filled cystic cavity

• Iatrogenic or surgical implantation of epithelium into the Management

jaw mesenchyme.
Surgical excision is the treatment of choice.
CLINICAL FEATURES
TERATOID CYST
• It is indolent in nature and slow to progress
• Remains asymptomatic until secondarily infected Occasionally, cysts may be encountered that may have elements
• Presents with pain and tenderness when infected derived from ectoderm, endoderm and mesoderm. These are
• Twice more common in men than in women termed teratoid cysts and are the rarest of the developmental
• Most common in third and fourth decades cysts of the oral cavity.
• May present with discharge of a cheese-like foul smelling
CLINICAL FEATURES
material
• When located orally, may cause difficulty in feeding, • It is a rare entity
swallowing or speaking • Arises in the floor of the mouth
• Appears as firm, round, mobile, flesh-colored to yellow or • Harada et al, 1995,35 and Bonilla et al, 1996,36 report that
white subcutaneous nodules of variable size the cyst usually presents in neonates, although a case was
• Central pore or punctum may or may not be present reported in a 5 year old boy by Ohishi et al, 1985.37
• May be located on face, scalp, neck, trunk, extremities
HISTOPATHOLOGIC FEATURES
• Associated syndromes:
— Gardener syndrome • Cysts are lined by keratinized stratified squamous
— Basal cell nevus syndrome epithelium, gastrointestinal epithelium or respiratory
— Pachyonychia congenita. epithelium.
• Presence of sebaceous glands, sweat glands, cartilage,
HISTOPATHOLOGIC FEATURES smooth muscle, striated muscle, neural tissue or bone in
the cyst wall.
• Cystic lining comprises of stratified squamous epithelium
with glandular differentiation Management
• Cyst is filled with desquamated keratin disposed in a
Surgical excision.
laminar pattern (Fig. 6.28)
• Dystrophic calcification and reactive foreign body reaction
THYROGLOSSAL TRACT CYST (Fig. 6.29)
is associated with the cystic capsule
• Malignant transformation of the cystic capsule may Luna and Pfaltz, 2001,38 and Koch, 2005,39 reported the
occur. thyroglossal cyst to be the most common developmental cyst
Cysts in the Pediatric Population 193

Management
1. Surgical excision is the treatment of choice.
2. The Sistrunk operation involves removal of a 1cm
block of tissue surrounding the duct and a 1 to 2cm
portion of the central part of the hyoid bone. The
thyroglossal tract should also be traced down to the
pyramidal lobe of thyroid gland and to the foramen
cecum at the base of the tongue.

INTRAORAL LYMPHOEPITHELIAL CYSTS

Intraoral lymphoepithelial cysts develop within a benign
lymphoid aggregate or accessory tonsil of the oral or
pharyngeal mucosa. This cyst was first reported by Parmentier
in 1857 as a hydatid cyst.
FIGURE 6.29: Thyroglossal duct cyst presenting
as a midline swelling in the neck
PATHOGENESIS
• May arise from epithelial inclusions within oral lymphoid
of neck accounting for 90 percent of the congenital abnormalities tissues
in the neck in children. • May arise as a result of cystic dilatation of tonsillar crypts
or superficial ducts of minor salivary glands.
PATHOGENESIS
Development of thyroid CLINICAL FEATURES
 • Mostly occur in the floor of the mouth and tongue
Thyroid anlage grows downwards in neck • Giunta and Cataldo, 1973, reported a series of 21 patients
 ranging from 7 to 65 years, including 12 females and 9
Residues of epithelial elements that do not atrophy males. Of these 17 (80%) involved the floor of the mouth,
completely give rise to cysts. two were in the soft palate and one each in the retromolar
area and the mandibular labial vestibule41
CLINICAL FEATURES • Allard, 1982, reviewed 105 cases in which he found an
age range of 7 to 81 years with a peak frequency of 42
• Allard, 1982, in a sample of 1316 cases reported 32% cases percent in the third decade.40
under the age of 10 years40 64 percent cases involved males
• Cyst may be found anywhere from posterior portion of 36 percent cases involved females
tongue to the midline of the neck 69 percent cases involved the floor of the mouth
• Majority of cases occur below the level of hyoid bone 23 percent cases involved the tongue
• When attached below hyoid bone and tongue, the cyst may 8 percent cases involved the soft palate, buccal
retract on swallowing vestibule and the anterior pillar of fauces.
• If infected, a draining sinus tract may occur.
HISTOPATHOLOGIC FEATURES
HISTOPATHOLOGIC FEATURES
• Buchner and Hansen, 1980, suggested that the cyst is lined
Findings vary according to location of cysts: by keratinized (parakeratinized and occasionally
• Lesions above level of hyoid bone demonstrate a lining orthokeratinized) stratified squamous epithelium devoid of
chiefly of stratified squamous epithelium. rete ridges42
• A ciliated or columnar type of epithelium is usually • Lymphoid tissue is found surrounding the cyst. In a few
found in cysts occurring below the hyoid bone. cases this is present only in a part of the cyst wall
• Thyroid tissue may be seen within the connective tissue • Lymphoid tissue shows a germinal center, sometimes with
wall. only a dense infiltrate of lymphocytes.
194 Essentials of Pediatric Oral Pathology

Management Management
Surgical excision is the treatment of choice. 1. Surgical excision. If acute infection occurs prior to
resection, surgery should be delayed for at least 3
LINGUAL CYST OF FOREGUT ORIGIN months.
2. Laser therapy is a recent advancement in the
The term lingual cyst is a descriptive term for cysts of foregut treatment of microcystic lesions.
origin that arise within the tongue. 3. Magnetic resonance—controlled laser-induced inter-
stitial thermotherapy is a novel therapy that has been
proposed for treatment of lymphangiomas.
CLINICAL FEATURES 4. The medical treatment of cystic hygroma consists
• Arise within the tongue. of the administration of sclerosing agents. Sclerosing
agents include OK-432 (an inactive strain of group
• Allard, 1982, reported that 12 out of 18 cases of this lesion
A Streptococcus pyogenes ), bleomycin, pure
occurred in the first year of life, 3 cases were between the ethanol, bleomycin, sodium tetradecyl sulfate and
ages 2 to 11 years and two were adult patients where lesion doxycycline.
was present since childhood40
• Manor et al, 1999, found most of the lesions on the dorsal
PARASITIC CYSTS
surface of the tongue in the anterior midline area.43
HYDATID CYST
HISTOPATHOLOGIC FEATURES
Hydatid cysts occur in hydatid disease or echinococcosis.
Cysts are lined by respiratory epithelium, gastric epithelium,
intestinal epithelium or combinations of two or three of these Etiology
types. It is caused by the larvae of E. granulosus, the dog tapeworm,
which resides in the intestinal tract of the dog. The ova may
Management be accidentally ingested by the host. These ova hatch in the
Surgical excision. upper gastrointestinal tract and are dispersed through the blood
vessels to all parts of the body.
CYSTIC HYGROMA Clinical Features
Cystic hygroma is a developmental abnormality in which there • Ataoglu et al, 2002, reported a case of a 6-year-old boy
is a progressive dilatation of the lymphatic channels. It is showing a parasitic cyst in the maxillary sinus45
currently designated as cavernous type of lymphangioma. • Most of the cases occur in salivary glands, pterygopalatine
or temporal fossa areas
CLINICAL FEATURES • Bouckaert et al, 2000, reported two cases of this lesion.
One case was reported in submandibular gland of 20-year-
• Bayer and Hardman, 1976, reported that the lesion is often
old female, the second case occurred in the buccal mucosa
present at birth; most cases are diagnosed before the age
of a 6-year-old boy.46
of 2 years.44
• In the head and neck region, it produces a swelling, which Histopathologic Features (Fig. 6.30)
is often painless and usually compressible.
• The lesion is usually unilateral but the entire side of the • The cyst consists of three layers:
neck and lower face may be involved. 1. Outer layer of host origin lined by fibrous tissue
• The overlying skin may be blue. showing chronic inflammatory cell infiltrate,
• The swelling tests positive to transillumination. eosinophils and giant cells.
2. Intermediate layer of parasitic origin, whitish, non-
nucleated, consisting of delicate laminations.
HISTOPATHOLOGIC FEATURES
3. Inner nucleated germinal layer of parasitic origin.
Cystic hygroma consists of dilated cystic spaces lined by • Clear, albumin-free cyst fluid containing the so-called
endothelial cells. ‘hydatid sand’ consisting of brood capsules and scolices.
Cysts in the Pediatric Population 195

of the pork tapeworm Taenia solium. They can act both as the
intermediate and the definitive host.

Clinical Features
• Ostrofsky and Baker, 1975, reported 3 cases of this lesion
of which one occurred as a swelling on the dorsum of the
tongue in a 7-year-old child47
• Lustmann and Copelyn, 1981, reported 2 cases of this
lesion of which one case occurred in the tongue of an 11-
year-old boy48
• The cystic mass contains clear, watery fluid and a coiled
white structure apparently attached to the inner aspect of the
cyst.
FIGURE 6.30: Histopathologic features of hydatid cyst showing
brood capsules and solices lined by fibrous connective tissue
Histopathologic Features
containing inflammatory cell infiltrate • It shows presence of a dense fibrous outer capsule derived
from host tissue
• These brood capsules develop originally as minute • The capsule consists of a dense inflammatory cell infiltrate
projections of the germinative layer which develop central consisting predominantly of lymphocytes, plasma cells and
vesicles and become minute cysts. histiocytes
• Scolices of the head of the worm develop in the inner • On the inner aspect of the capsule, the infiltrate shows a
aspects of the brood capsules. dense aggregation of eosinophils and neutrophils
• A delicate double layered membrane within the capsule
Management consists of an outer acellular hyaline eosinophilic layer and
1. Better forms of chemotherapy and newer methods, an inner sparsely cellular layer
such as the puncture, aspiration, injection and • This membrane can be readily separated from the capsule
reaspiration (PAIR) technique are now available but because of its loose attachment to the capsule
need to be tested. • This membrane contains the cyst as also the larval form of
2. Two benzimidazoles are used, albendazole and T. solium
mebendazole. Albendazole is administered in several • At cephalic extremity of the larva, the scolex with the
1-month oral doses (10 to 15 mg/kg/day) separated
rostellum, bothria (suckers) and hooklets may be identified.
by 14-day intervals. Mebendazole is administered 40
to 50 mg/kg/day PO for 3 to 6 mo (primary mode of Management
therapy) or for 4 days prior to surgery and then 1
month postoperatively as adjunct. 1. It is harmless to the oral tissues.
3. PAIR technique is performed using either ultrasound 2. Over a period of time, localization in the brain, heart
or CT guidance and involves aspiration of the valves and orbit may produce important functional
contents via a special cannula, followed by injection derangements.
of a scolicidal agent (e.g. formalin, hydrogen peroxide, 3. Death of the parasite may occur after many years
hypertonic saline, chlorhexidine, absolute alcohol and resulting in a granulomatous reaction around the
cetrimide) for at least 15 minutes and then reaspira- parasite which then calcifies.
tion of the cystic contents. This is repeated until the
return is clear. The cyst is then filled with isotonic CYSTS OF SALIVARY GLANDS
sodium chloride solution. Perioperative treatment with
a benzimidazole is mandatory (4 days prior to the MUCOCELES (FIG. 6.31)
procedure and 1 to 3 months after).
Mucous extravasation cysts and mucous retention cysts are
CYSTICERCUS CELLULOSAE often referred to collectively as mucoceles. Generally, the term
'mucous extravasation cyst' is reserved for those lesions in
Cysticercus cellulosae is a parasitic cyst occurring in humans which mucous has extravasated into the connective tissues and
with cysticercosis. Cysticercosis occurs through the larval form in which there is no epithelial lining. And the term 'mucous
196 Essentials of Pediatric Oral Pathology

retention cyst' is employed to describe mucoceles that result

from dilatation of the ducts and which are lined by epithelium.
It is mainly classified as:
• Mucous extravasation cysts
• Mucous retention cysts.

Pathogenesis
• Obstruction of salivary gland duct due to a stone or some
other cause may lead to dilatation of the duct proximal to
the obstruction and results in formation of retention cyst
lined by epithelium.
• Trauma to the duct or secretary acini may lead to extra-
vasation of mucus in the connective tissue resulting in
formation of mucus extravasation cyst.
FIGURE 6.32: Histopathologic picture of mucocele showing a lining
Clinical Features of stratified squamous epithelium and underlying connective tissue
with eosinophilic mucinous material surrounded by inflammatory
• Harrison, 1975, reviewed 400 cases from literature and cell infiltrate
reported that mucous extravasation cysts most commonly
occur in adolescents and children, whereas, mucous
HISTOPATHOLOGIC FEATURES (FIG. 6.32)
retention cysts most commonly occur in adults49
• In one study on 151 South African patients, the youngest • Robinson and Hjørting-Hansen, 1964, defined three
patient found was 8 months old distinct patterns of mucoceles53
• Standish and Shafer, 1959, reported a case of this lesion • Mucus extravasation cysts show two patterns, one which
present at birth50 consists of numerous irregular pools of eosinophilic
• Poker and Hopper, 1990, reported a case of such a lesion mucinous material and vacuolated macrophages termed as
occurring in the tongue of a 10-week-old girl51 'mucinophages', these were termed as poorly defined cysts.
• Cataldo and Mosadomi, 1970, in their study of 594 cases Others termed as well-defined cysts were further divided
reported the occurrence of this lesion in only 2.7 percent into two groups and differed only in that the periphery of
of infants less than 1-year-old52 one consisted of granulation tissue and was infiltrated by
• Most of the lesions occur as a round or oval, smooth and vacuolated macrophages, lymphocytes, eosinophils, etc.
fluctuant swelling of the lower lip, followed by floor of • Mucous retention group was partially or completely lined
the mouth, tongue, palate, buccal mucosa and upper lip. by epithelium that was either flattened consisting of one
• Superficial lesions appear bluish in color whereas, deep or two layers of cells of stratified squamous epithelium.
lesions appear as normal mucosa. Management
1. Mucoceles of smaller size require no specific treatment.
2. Larger lesions require surgical removal along with the
removal of associated lobule of the salivary gland
involved.

RANULA
The term ranula is used to describe mucoceles occurring on
the floor of mouth.
Classification
They are of two types: superficial and plunging.
1. Superficial type develops as an extravasation or retention
type mostly associated with trauma to one or more excretory
ducts of submandibular or sublingual salivary glands
2. Plunging ranulas are a result of extravasation of sublingual
glands, which ramify diffusely into the neck (Figs 6.33
FIGURE 6.31: Mucocele on lower lip and 6.34).
Cysts in the Pediatric Population 197

FIGURE 6.33: Ranula in the floor of mouth FIGURE 6.35: Histopathologic picture of a ranula showing a huge
collection of mixed salivary gland acini. The connective tissue
separating the lobules is delicate, loosely arranged with numerous
mature and immature blood vessels. There is also a huge collection
of mucoid material

Clinical Features
• They are mostly found associated with submandibular or
sublingual glands
• They are usually unilateral
• Incidence in children and young adults is more common
• Occurrence in the floor of the mouth is common, where
they appear translucent blue in color, that resembles a frog's
belly, hence the name
• They are small fluctuant swellings
• Larger lesions may lift the tongue and cause difficulty in
swallowing and speech
• The swelling increases in size during meals due to increased
secretory activity as a part of gustatory stimulation
• Herniated projections of sublingual gland as a result of
hiatus or deficiency between anterior and posterior parts
of mylohyoid muscle had been reported as etiological
factors for plunging ranulas.

Histopathologic features (Fig. 6.35)

• Histopathological features are same as mucoceles
• Majority of ranulas show lack of epithelial lining.

Management
1. Surgical removal of sublingual salivary gland.
2. Rho et al, 2006, proposed the use of a sclerosing
and immune system stimulating agent OK-432. This
agent is prepared from lyophilized Streptococcus
pyogenes.54
FIGURES 6.34A and B: Plunging ranula (arrows)
198 Essentials of Pediatric Oral Pathology

REFERENCES 17. Aguilo L, Cibrian R, Bagan JV, Gandia JL. Eruption cysts:
retrospective clinical study of 36 cases. ASDC Journal of
1. Kramer IRH. Changing views on oral disease. Proceedings of Dentistry for Children 1998;65:102-6.
the Royal Society of the Medicine 1974;67:271-6. 18. Bodner L, Goldstein J, Sarnat H. Eruption cysts: a clinical report
2. Shear Mervin, Speight Paul (Eds). Chapter 1: classification and of 24 new cases. Journal of Clinical Pediatric Dentistry 2004;
frequency of cysts of the oral and maxillofacial tissues. In: Cysts 28:183-5.
of the oral and maxillofacial region. Blackwell Munksgaard, 19. Ramon Boj J, Garcia-Godoy F. Multiple eruption cysts: report
4th ed, 1-2. of a case. ASDC Journal of Dentistry for Children 2000;67:
3. Phillipsen H. On keratocysts in the jaws. Tandleagebladet 1956; 282-4.
60:963. 20. Kuczek A, Beiklar T, Herbst H, Flemmig TF. Eruption cyst
4. Toller P. Origin and growth of cysts of the jaws. Ann R Coll formation associated with cyclosporine A. Journal of Clinical
Surg Engl 1967;40(5):306-36. Periodontology 2003;30:462-6.
5. Main DMG. Epithelial jaw cysts: a clinicopathological 21. Ikemura K, Kakinoki Y, Nishio K, Suenaga Y. Cysts of the oral
reappraisal. Br J Oral Surg 1970;8:114-9. mucosa in newborns: a clinical observation. Journal of the
6. Meara JG, Li KK, Shah SS, Cunningham MJ. Odontogenic University of Occupational and Environmental Health 1983;
keratocysts in the pediatric population. Arch of Otolaryngology 5:163-8.
Head and Neck Surgery 1996;122:725-8. 22. Dilley DC, Siegel MA, Budnick S. Diagnosing and treating
7. Ghali GE, Connor MS. Surgical management of the odontogenic common oral pathologies. Pediatric Clinics of North America
keratocyst. In: Pogrel MA, Schmidt BL (Eds). Oral and 1991;38:1227-64.
maxillofacial clinics of North America. The odontogenic 23. Liu MH, Huang WH. Oral abnormalities in Taiwanese
keratocyst. WB Saunders Co 2003;15:383-92. newborns. Journal of Dentistry for Children 2004;71:118-20.
8. Schmidt BL. The use of liquid nitrogen cryotherapy in the 24. Meghji S, Qureshi W, Henderson B, Harris M. The role of
management of odontogenic keratocyst. In: Pogrel MA, Schmidt endotoxin and cytokines in the pathogenesis of odontogenic
BL (Eds). Oral and maxillofacial clinics of North America. The cysts. Archives of Oral Biology 1996;41:523-31.
Odontogenic Keratocyst. WB Saunders Co 2003;15:393-404. 25. Toller PA. The osmolality of fluids from cysts of the jaws. British
9. Stoelinga PJW. Excision of the overlying, attached mucosa, in Dental Journal 1970(b);129:275-8.
conjunction with cyst enucleation and treatment of the bony defect 26. High AS, Hirschmann PN. Age changes in residual radicular
with carnoy solution. In: Pogrel MA, Schmidt BL (Eds). Oral cysts. Journal of Oral Pathology 1986;15:524-8.
and maxillofacial clinics of North America. The Odontogenic 27. Howe GL. ‘Hemorrhagic cysts’ of the mandible. British Journal
Keratocyst. WB Saunders Co 2003(b);15:407-14. of Oral Surgery 1965;3:55-75, 77-91.
10. Pogrel MA. Decompression and marsupialization as a treatment 28. Jones AV, Franklin CD. An analysis of oral and maxillofacial
for the odontogenic keratocyst. In: Pogrel MA, Schmidt BL pathology found in children over a 30 year period. International
(Eds). Oral and maxillofacial clinics of North America. The Journal of Pediatric Dentistry 2006;16:19-30.
Odontogenic Keratocyst. Philadelphia: WB Saunders Co 2003 29. Jaffe HL, Lichtenstein L. Solitary unicameral bone cyst with
(b);15:311-5, 415-27. emphasis on roentgen picture, the pathologic appearance and
11. Browne RM. The pathogenesis of odontogenic cysts: a review. the pathogenesis. Archives of Surgery 1942;44:1004-25.
Journal of Oral Pathology 1975;4:31-6. 30. El-Deeb M, Sedano HO, Waite DE. Aneurysmal bone cyst of
12. Lustmann J, Shear M. Radicular cysts arising from deciduous the jaws. Report of a case associated with fibrous dysplasia and
teeth. Review of the literature and report of 23 cases. review of the literature. International Journal of Oral Surgery
International Journal of Oral Surgery 1985;14:153-61. 1980;9:301-11.
13. Wood RE, Nortje CJ, Padayachee A, Grotepass F. Radicular 31. Praetorius F, Hjørting-Hansen E, Gorlin RJ, Vickers RA.
cysts of primary teeth mimicking premolar dentigerous cysts: Calcifying odontogenic cyst. Range, variations and neoplastic
report of three cases. Journal of Dentistry for Children 1988; potential. Acta Odontologica Scandinavica 1981;39:227-40.
55:288-90. 32. Praetorius F, Ledesma-Montes C. Calcifying cystic odontogenic
14. Bodner L. Cystic lesions of the jaws in children. International tumour. In: Barnes L, Eveson JW, Reichart PA, Sidransky D
Journal of Pediatric Otorhinolaryngology 2002;62:25-9. (Eds). World Health Organization Classification of Tumours.
15. Shibata Y, Asaumi J, Yanagi Y, et al. Radiographic examination Pathology and genetics of tumours of the head and neck. Lyon:
of dentigerous cysts in the transitional dentition. Dentomaxillo- IARC 2005;313.
facial Radiology 2004;33:17-20. 33. Yoshimura Y, Takada K, Takeda M, Mimua T, Mori M.
16. Hyomoto M, Kawakami M, Inoue N, Kirita T. Clinical Congenital dermoid cysts of the sublingual region. Report of a
conditions for eruption of maxillary canines and mandibular case. Journal of Oral Surgery 1970;28:366-70.
premolars associated with dentigerous cysts. American Journal 34. Yeschua R, Bab IA, Wexler MR, Neuman Z. Dermoid cyst of
of Orthodontics and Dentofacial Orthopedics 2003;124:515- the floor of the mouth in an infant. Journal of Maxillofacial
20. Surgery 1977;5:211-3.
Cysts in the Pediatric Population 199

35. Harada H, Kusukawa J, Kameyama T. Congenital teratoid cyst 44. Bayer RA, Hardman FG. Intraoral surgical management of cystic
of the floor of the mouth: a case report. International Journal hygroma. British Journal of Oral Surgery 1976;14:36-40.
of Oral and Maxillofacial Surgery 1995;24:361-2. 45. Ataoglu H, Uckan S, Oz G, Altinors N. Maxillofacial hydatid
36. Bonilla JA, Szeremeta W, Yellon RF, Nazif MM. Teratoid cyst cyst. Journal of Oral and Maxillofacial Surgery 2002;60:454-6.
of the floor of the mouth. International Journal of Pediatric 46. Bouckaert MM, Raubenheimer EJ, Jacobs FJ. Maxillofacial
Otorhinolaryngology 1996;38:71-5. hydatid cysts. Oral Surgery, Oral Medicine, Oral Pathology, Oral
37. Ohishi M, Ishii T, Shinohara M, Horinouchi Y. Dermoid cyst Radiology and Endodontics 2000;89:338-42.
of the floor of the mouth: lateral teratoid cyst with sinus tract 47. Ostrofsky MK, Baker MAA. Oral cysticercosis. Journal of the
in an infant. Oral Surgery, Oral Medicine, Oral Pathology 1985; Dental Association of South Africa 1975;30:535-7.
60:191-4.
48. Lustmann J, Copelyn M. Oral cysticercosis. Review of the
38. Luna MA, Pfaltz M. Cysts of the neck, unknown primary literature and report of two cases. International Journal of Oral
tumour and neck dissection. In: Gnepp D (Ed). Diagnostic Surgery 1981;10:371-5.
surgical pathology of the head and neck. Oxford: Saunders
49. Harrison JD. Salivary mucoceles. Oral Surgery, Oral Medicine,
2001;650-80.
Oral Pathology 1975;39:268-78.
39. Koch BL. Cystic malformations of the neck in children.
50. Standish SM, Shafer WG. The mucus retention phenomenon.
Pediatric Radiology 2005;35:463-77.
Journal of Oral Surgery 1959;17:15-22.
40. Allard RHB. Non-odontogenic cysts of the oral regions. MD
thesis. Free University of Amsterdam, Naarden: Drukkerrij Los 51. Poker ID, Hopper C. Salivary extravasation cyst of the tongue.
BV 1982. British Journal of Oral and Maxillofacial Surgery 1990;28:176-7.
41. Giunta J, Cataldo E. Lymphoepithelial cysts of the oral mucosa. 52. Cataldo E, Mosadomi A. Mucoceles of the oral mucous
Oral Surgery, Oral Medicine, Oral Pathology 1973;35:77-84. membrane. Archives of Otolaryngology 1970;91:360-5.
42. Buchner A, Hansen LS. Lymphoepithelial cysts of the oral 53. Robinson L, Hjørting-Hansen E. Pathologic changes associated
cavity. A clinicopathologic study of 38 cases. Oral Surgery, Oral with mucus retention cysts of minor salivary glands. Oral
Medicine, Oral Pathology 1980;50:441-9. Surgery, Oral Medicine, Oral Pathology 1964;18:191-205.
43. Manor Y, Buchner A, Peleg M, Taicher S. Lingual cyst with 54. Rho MH, Kim DW, Kwon JS, et al. OK-432 sclerotherapy of
respiratory epithelium: an entity of debatable histogenesis. plunging ranula in 21 patients: it can be a substitute for surgery.
Journal of Oral and Maxillofacial Surgery 1999;57:124-7. Americal Journal of Neuroradiology 2006;27:1090-5.
7
200 Essentials of Pediatric Oral Pathology

Odontogenic Tumors
in Children
Mayur Chaudhary, Shweta Dixit Chaudhary, Anuraag B Chaudhary

CHAPTER OVERVIEW
Introduction Compound odontoma
Classification Odontoameloblastoma
Solid/multicystic ameloblastoma Calcifying cystic odontogenic tumor
Adenomatoid odontogenic tumor Dentinogenic ghost cell tumor
Calcifying epithelial odontogenic tumor Odontogenic fibromas (epithelium poor and epithelium rich)
Squamous odontogenic tumor Odontogenic myxoma/fibromyxoma
Ameloblastic fibroma Metastasizing ameloblastoma
Ameloblastic fibrodentinoma Ameloblastic carcinoma
Ameloblastic fibro-odontoma Primary intraosseous squamous cell carcinoma
Complex odontoma Ameloblastic fibrosarcoma

INTRODUCTION periodontal ligament. The enamel organ then involutes to a

monolayer, which becomes squamoid and ultimately fuses with
Odontogenic tumors are lesions derived from epithelial or the gingiva during eruption.
ectomesenchymal tissues or both, which are part of the tooth- Any aberration from this normal process of tooth formation
forming apparatus. They usually comprise of about one may either give rise to a cyst or a tumor formation. This chapter
percent of all jaw tumors. Before proceeding towards various focuses on the most common odontogenic tumors occurring
odontogenic tumors, a basic understanding of the histogenesis in children. Few lesions though being rare fall in the pediatric
of the tooth and a clear difference between a tumor and age group and thus are also discussed here.
hamartoma is necessary.
Tooth development begins when the dental lamina projects DIFFERENCE BETWEEN A TUMOR AND A
into the underlying ectomesenchyme. The cells of dental lamina HAMARTOMA
differentiate into a layered cap with an inner and outer enamel
epithelium, which contain the inner stratum intermedium and • Tumor: A group of heterogeneous lesions that range from
stellate reticulum layers. Changes also occur in the underlying hamartomatous or non-neoplastic tissue proliferations to
ectomesenchyme forming the dental follicle and dental papilla. malignant neoplasms with metastatic capabilities.
Mesenchymally derived odontoblasts form along the dental • Hamartoma: Tumor like malformation, developmental in
papilla and secrete dentin, which induces the inner enamel origin, with the tissue being native to the site.
epithelium to become ameloblasts. Ameloblasts are responsible
for enamel production and eventual crown formation. CLASSI FI CATI ON
Cementoblasts and fibroblasts from the dental follicle • Odontogenic tumors are lesions derived from epithelial,
mesenchyme deposit cementum on the root surface and form ectomesenchymal and/or mesenchymal elements that are
the periodontal membrane, respectively. The penetration of or have been a part of the tooth forming apparatus.
these cells through Hertwig's sheath at the edge of the enamel • French physician Broca, 1867, first proposed a classifica-
organ gives rise to epithelial rests of Malassez within the tion of tumors originating from dental tissues.
Odontogenic Tumors in Children 201

• In 1992, WHO revised the classification of odontogenic is now included in malignant group of odontogenic
tumors. The current classification was approved at a carcinomas.
consensus conference held at Lyon (France) in July 2003.
BENIGN
DIFFERENCE BETWEEN WHO CLASSIFICATION SOLID/ MULTICYSTI C AMELOBLASTOMA
AND THE CURRENTLY ACCEPTED CLASSIFICATION
French physician Broca, 1868, first gave a detailed description
• Adenomatoid odontogenic tumor is included in the group of solid multicystic ameloblastoma. 1 Malassez, 1884-85,
of tumors with odontogenic epithelium with mature, fibrous described it as epithelioma adamantin.2,3 Churchill, 1934, first
stroma: odontogenic ectomesenchyme not present gave the term “Ameloblastoma”.4 Adamantinoma is a misnomer
(previously it was placed in group odontogenic epithelium as cells of tumor islands are not true ameloblasts.
with odontogenic ectomesenchyme with or without dental Robinson defined ameloblastoma as “Usually unicentric,
hard tissue formation). non-functional, intermittent in growth, anatomically benign and
• Odontogenic keratocyst (Keratinizing cystic odontogenic clinically persistent”.5
tumor) is also included in the group of tumors with
odontogenic epithelium with mature, fibrous stroma: Etiology
odontogenic ectomesenchyme not present. The following etiological factors have been implicated:
• Clear cell odontogenic tumor which was included in the • Irritation
group of tumors with odontogenic epithelium with mature, • Infection
fibrous stroma: odontogenic ectomesenchyme not present • Trauma

WORLD HEALTH ORGANIZATION (WHO) CLASSIFICATION OF ODONTOGENIC TUMORS (1992)

BENIGN
1. Odontogenic Epithelium without Odontogenic Ectomesenchyme:
• Ameloblastoma
• Squamous odontogenic tumor
• Calcifying epithelial odontogenic tumor (Pindborg’s tumor)
• Clear cell odontogenic tumor
2. Odontogenic Epithelium with Odontogenic Ectomesenchyme, with or without Dental Hard Tissue Formation:
• Ameloblastic fibroma
• Ameloblastic fibro-dentinoma and Ameloblastic fibro-odontoma
• Odontoameloblastoma
• Adenomatoid odontogenic tumor
• Calcifying odontogenic cyst
• Complex and compound odontoma
3. Odontogenic Ectomesenchyme, with or without Included Odontogenic Epithelium:
• Odontogenic fibroma
• Myxoma (Odontogenic myxoma, myxofibroma)
• Benign cementoblastoma

MALIGNANT
1. Odontogenic Carcinomas
• Malignant ameloblastoma
• Primary intra-osseous carcinoma
• Malignant variants of other odontogenic tumors
• Malignant changes in odontogenic cysts
2. Odontogenic Sarcomas
• Ameloblastic fibrosarcoma (ameloblastic sarcoma)
• Ameloblastic fibro-dentinosarcoma and ameloblastic fibro-odontosarcoma
3. Odontogenic Carcinosarcoma
202 Essentials of Pediatric Oral Pathology

CURRENT CLASSIFICATION OF ODONTOGENIC TUMORS (JULY 2003)

A. NEOPLASMS AND TUMOR-LIKE LESIONS ARISING FROM ODONTOGENIC APPARATUS

BENIGN
1. Odontogenic epithelium with mature, fibrous stroma: odontogenic ectomesenchyme not present
• Ameloblastomas
– Solid/multicystic
– Extraosseous/peripheral
– Desmoplastic
– Unicystic
• Adenomatoid odontogenic tumor
• Calcifying epithelial odontogenic tumor
• Squamous odontogenic tumor
• Keratinizing cystic odontogenic tumor
2. Odontogenic epithelium with odontogenic ectomesenchyme with or without dental hard tissue formation
• Ameloblastic fibroma
• Ameloblastic fibrodentinoma
• Ameloblastic fibro-odontoma
• Complex odontoma
• Compound odontoma
• Odontoameloblastoma
• Calcifying cystic odontogenic tumor
• Dentinogenic ghost cell tumor
3. Mesenchyme and/or odontogenic ectomesenchyme with or without included odontogenic epithelium
• Odontogenic fibromas (epithelium poor and epithelium rich)
• Odontogenic myxoma/fibromyxoma
• Cementoblastoma
MALIGNANT
1. Odontogenic carcinomas
• Metastasizing malignant ameloblastoma
• Ameloblastic carcinoma
– Primary
– Secondary (dedifferentiated), intraosseous
– Secondary (dedifferentiated), extraosseous
• Primary intraosseous squamous cell carcinoma (PIOSCC)
– PIOSCC solid type
– PIOSCC derived from odontogenic cysts
– PIOSCC derived from keratinizing cystic odontogenic tumor
• Clear cell odontogenic carcinoma
• Ghost cell odontogenic carcinoma
2. Odontogenic sarcomas
• Ameloblastic fibrosarcoma
• Ameloblastic fibrodentinosarcoma
B. NEOPLASMS AND OTHER LESIONS IN THE MAXILLOFACIAL SKELETON
OSSEOUS NEOPLASMS
Ossifying fibroma
NON-NEOPLASTIC LESIONS
• Fibrous dysplasia
• Osseous dysplasia
• Central giant cell lesion
• Cherubism
• Aneurysmal bone cyst
• Simple bone cyst
Odontogenic Tumors in Children 203

• Dietary deficiency • Expansion and erosion of cortical plates (egg shell

• Virus. crackling) may be evident
• Ameloblastomas in children differ from adults, with a higher
Pathogenesis percentage of unicystic tumors. African children appear to
• Most solid/multicystic ameloblastomas occur as growths resemble the adult pattern.6
arising from remnants of odontogenic epithelium, more
specifically rests of the dental lamina RADIOGRAPHIC FEATURES
• As early as 1885, Malassez suggested that intraosseous • Multilocular radiolucent appearance.
ameloblastomas may originate from “les debris épithéliaux” • May be unilocular (Fig. 7.2).
(cell rests of Malassez)3
• May arise as a result of neoplastic changes in the lining Various Terminologies Radiographically Used
of odontogenic cysts (OKC, dentigerous cyst)
• May arise on odontomas • Soap bubble: Consists of several circular compartments that
• Residues of dental lamina outside the bone may also give vary in size and usually appear to overlap somewhat (Fig. 7.3)
rise to ameloblastoma. • Honeycomb: Lesions whose compartments are small and
tend to be quite uniform in size (Fig. 7.4)
Types • Tennis racket: Lesions composed of angular rather than
rounded compartments that result from the development
Basically of four types:
of more or less straight septae (Fig. 7.5).
1. Solid/Multicystic
2. Extraosseous/Peripheral
3. Desmoplastic
4. Unicystic.

SOLID/MULTICYSTIC
CLI NICAL FEATU RES
• Occurs in the age range of 4 to 92 years.
• Seen more often in males as compared to females.
• Occurrence in posterior mandibular area is common (Fig. 7.1).
• Painless slow growing lesion.
FIGURE 7.2: Radiographic picture of solid/multicystic ameloblastoma
• Migration, loosening and root resorption may be seen. showing a unilocular radiolucent expansile lesion in the lower right
• Paresthesia may be present. posterior region of the jaw

FIGURE 7.1: Solid/multicystic ameloblastoma showing a swelling FIGURE 7.3: Diagrammatic representation of radiographic
in the lower right posterior region of the jaw picture of soap bubble appearance
204 Essentials of Pediatric Oral Pathology

FIGURE 7.4: Diagrammatic representation of radiographic

picture of honeycomb appearance

FIGURES 7.6A and B: Histopathologic picture of follicular

ameloblastoma showing islands with central mass of polyhedral
cells

Histopathological types of ameloblastomas

• Follicular
• Plexiform
• Acanthomatous
FIGURE 7.5: Diagrammatic representation of radiographic picture • Granular cell
of tennis racket appearance • Desmoplastic
• Unicystic
HISTOPATHOLOGIC FEATURES
• Basal cell
Definition • Clear cell
• Keratoameloblastoma
Ameloblastoma may be defined as a polymorphic neoplasm
• Hemangiomatous.
consisting of proliferating odontogenic epithelium usually
occuring in two main patterns. In follicular type of growth, tumor Follicular ameloblastoma (Figs 7.6A and B)
consists of enamel organ-like islands or follicles of epithelial cells, • Islands with central mass of polyhedral cells
while in the plexiform type, the epithelium forms continuous • Polyhedral cells resemble stellate reticulum
anastomosing strands. In both types, epithelial components are • Stellate reticulum surrounded by layer of cuboidal or
embedded in mature connective tissue stroma. columnar cells resembling preameloblasts.
Odontogenic Tumors in Children 205

FIGURES 7.7A and B: Histopathologic picture of plexiform ameloblastoma showing tumor epithelium arranged as a network

FIGURES 7.8A and B: Histopathologic picture of acanthomatous ameloblastoma showing tumor islands with extensive squamous metaplasia

Plexiform ameloblastoma (Figs 7.7A and B) • Acidophilic granules are usually lysosomal aggregates
• Central mass of polyhedral cells resembles stellate reticulum • Immunohistochemical studies indicate that granularity may
• Tumor epithelium is arranged as a network be due to increased apoptosis and associated phagocytosis
• Network is bound by a layer of cuboidal or columnar cells by neighbouring neoplastic cells.
resembling preameloblasts.
Basal cell ameloblastoma (Fig. 7.10)
Acanthomatous ameloblastoma (Figs 7.8A and B) • Basal cell ameloblastoma is rarely seen
• It is a variant of ameloblastoma • Most actively proliferating
• Tumor islands consist of extensive squamous metaplasia • Most immature
• Sometimes keratin formation is seen within the islands • Predominantly basaloid pattern of cells is seen.
• Keratin pearls may become calcified. Clear cell ameloblastoma
Granular cell ameloblastoma (Fig. 7.9) May contain periodic-acid-Schiff (PAS) positive cells localized
• It is similar to the follicular type; shows extensive granular in stellate reticulum like cells.
transformation of stellate reticulum like cells Keratoameloblastoma and Papilliferous Keratoameloblastoma
• Granular cells may be cuboidal, columnar or rounded with • Pindborg, 1970, first stated that an ameloblastoma
cytoplasm showing acidophilic granules consisting partly of keratinizing cysts and partly of tumor
206 Essentials of Pediatric Oral Pathology

— Collision tumor: A stromal cyst in plexiform amelo-

blastoma, thus resulting in a secondary change.

Management
Radical surgical intervention.

PERIPHERAL AMELOBLASTOMA
Peripheral ameloblastomas occur mostly in the soft tissues
overlying the tooth-bearing areas and do not invade the
underlying bone. Stanley and Korgh, 1959, were the first to
report a completely documented case of this lesion.10

Pathogenesis
Peripheral ameloblastoma arises from two major sources:
FIGURE 7.9: Histopathologic picture of granular cell ameloblastoma 1. Remnants of dental lamina located in the soft tissues
showing extensive granular transformation of stellate reticulum like overlying the tooth bearing areas of the jawbones.
cells 2. Surface epithelium.

Clinical Features
• Painless, sessile, firm, exophytic growth
• Surface may be smooth or granular, normal in color or
slightly red
• Occurs in the age range of 9 to 92 years
• Males are more commonly affected than females
• Mandibular premolar areas are most often affected than
maxilla, and in maxilla, tuberosity area is most often
involved.

Radiographic Features
Since it is a superficial lesion, there is no involvement of bone
but, there may be cupping or saucerization of the bone due to
pressure resorption.

FIGURE 7.10: Histopathologic picture of basal cell ameloblastoma Histopathologic Features

showing basaloid pattern of cells • Similar features as compared to solid/multicystic amelo-
blastoma but the tumor is seen peripherally.
islands with papilliferous appearance be called Papilliferous • Palisading, cuboidal/columnar peripheral cells.
Keratoameloblastoma.7 • Central polyhedral cells resemble stellate reticulum.
• Altini et al, 1991, noted a similar tumor without papilli- • Stroma is of mature connective tissue.
ferous epithelium and extensive necrosis in the middle of
the follicle.8 Management
Hemangiomatous ameloblastoma 1. Peripheral ameloblastomas require conservative
• Kuhn, 1932, called hemangiomatous ameloblastoma as an supraperiosteal surgical excision.
ameloblastoma in which, part of the tumor contains spaces 2. Good results can be achieved in the treatment of
filled with blood or large endothelium lined capillaries.9 ameloblastoma in children using conservative
• Origin of vascular component: surgery. In the event of recurrence, a second surgery
— Angiogenesis during tumor development and trauma can be successful. Patient compliance and careful
from tooth extraction. follow-up are important.11
Odontogenic Tumors in Children 207

FIGURE 7.11: Histopathologic picture of desmoplastic ameloblas- FIGURE 7.12: Unicystic ameloblastoma showing an ulcerated
toma showing bizarrely shaped islands and cords of odontogenic lesion in the lower left posterior region of the jaw
epithelium of varying sizes embedded in a desmoplastic connective
tissue stroma

DESMOPLASTIC AMELOB LASTOMA • A possible “transitional” form of desmoplastic amelo-

blastoma, showing microscopic features of the
Eversole and coworkers, 1984, are usually credited for the
first English language publication on the desmoplastic desmoplastic variant together with areas typical of
ameloblastoma.12 This tumor is characterized by an unusual “classic” follicular or plexiform ameloblastoma, has been
histomorphology, including extensive stromal collagenization called a “hybrid” lesion of ameloblastoma.
or desmoplasia, leading to the proposed term ameloblastoma
with pronounced desmoplasia or desmoplastic ameloblastoma. UNICYSTIC AMELOBLASTOMA

Pathogenesis Robinson and Martinez, 1977, introduced the term unicystic

ameloblastoma.13 The term unicystic is derived from the macro-
Presence of oxytalan fibers in the stromal tissue indicates that
and micro-scopic appearance, the lesion being essentially a
the tumor is derived from epithelial rests of Malassez in
well-defined, often large monocystic cavity with a lining, focally
periodontal membrane.
but rarely entirely composed of odontogenic (ameloblasto-
Clinical Features matous) epithelium.
• Variant of solid/multicystic ameloblastoma
Types
• Occurs in age range of 17 to 72 years
• Males are affected more as compared to females Basically of two types:
• Occurs with equal distribution in maxilla and mandible 1. Those associated with unerupted tooth (dentigerous variant)
• Presents as a painless swelling. 2. Those lacking association with unerupted tooth (non-
dentigerous variant).
Radiographic Features
Unilocular or multilocular radiolucency with ill-defined borders. Clinical Features (Fig. 7.12)
• Dentigerous variant occurs in younger age group. Almost
Histopathologic Features (Fig. 7.11)
78.3% of cases occur in the first two decades as compared
• Benign invasive variant of solid/multicystic ameloblastoma to non-dentigerous variant in which, 29.4% of cases occur
consisting of proliferating, irregular, bizarre shaped islands in the first two decades
and cords of odontogenic epithelium of varying sizes • Males are affected more as compared to females
embedded in a desmoplastic connective tissue stroma. • Occurrence in mandible is more as compared to females.
208 Essentials of Pediatric Oral Pathology

• WHO classification, 1992: (histopathological classification)

Subgroup Interpretation

1 Luminal

1.2 Luminal and Intraluminal

1.2.3 Luminal, intraluminal, intramural

1.3 Luminal and intramural

Unicystic ameloblastoma group 1.2 (luminal and intraluminal)
is sometimes referred to as plexiform unicystic ameloblastoma.

Management
1. Conservative treatment for group 1 and 1.2
2. Aggressive management for group 1.2.3 and 1.3

FIGURE 7.13: Radiographic picture of unicystic ameloblastoma ADENOMATOID ODONTOGENIC TUMOR

showing a unilocular radiolucent lesion with resorption of root
Unal and coworkers quoted that, Steensland, 1905, in his
report for the first time coined the term epithelioma
adamantinum for this lesion.14 Finally, Philipsen and Birn, 1969,
termed this lesion as adenomatoid odontogenic tumor.15 Few
consider it to be a hamartomatous lesion, while few perceive
it as a benign embryonal neoplasm. The current classification
groups it into odontogenic tumor with odontogenic epithelium
with mature stroma without ectomesenchyme.

Classification
Two Types
1. Intraosseous
• Follicular
• Extrafollicular
2. Extraosseous.

Pathogenesis
FIGURE 7.14: Histopathologic picture of unicystic ameloblastoma May arise from:
showing a large monocystic cavity with a lining composed of • Rests of dental lamina
odontogenic (ameloblastomatous) epithelium • Enamel organ.

Clinical Features
Radiographic Features (Fig. 7.13)
• Occurs in the age range of 3 to 87 years
• Either unilocular or multilocular radiolucency • More commonly seen in females as compared to males
• Unilocular pattern is commonly seen in dentigerous variant, • Follicular variant is three times more frequently seen than
and non-dentigerous variant shows either unilocular or the extrafollicular variant
multilocular pattern. • Occurrence in maxilla is more common than mandible
• Maxillary canine region accounts for 40% of adenomatoid
Histopathologic Features odontogenic tumors (Fig. 7.15)
• Extraosseous variant is common in maxillary anterior gingival
• Well-defined, large monocystic cavity with a lining region
composed of odontogenic (ameloblastomatous) epithelium • Usually it is roughly spherical with well-defined fibrous
(Fig. 7.14). capsule
Odontogenic Tumors in Children 209

FIGURE 7.15: Adenomatoid odontogenic tumor presenting as a

swelling in upper right anterior region of the jaw

FIGURE 7.17: Radiographic picture of adenomatoid odontogenic

tumor showing a unilocular radiolucency associated with the crown
of canine

• Characteristic feature is duct-like arrangement of ameloblast

like cells (Fig. 7.18)
• Nuclei of cells face away from the center
• Central space may contain eosinophilic material (eosinophilic
coagulum)
• Foci of calcification may be scattered throughout the tumor
• Connective tissue is scanty.
May be associated with the following lesions:
• Dentigerous cyst
• Calcifying epithelial odontogenic tumor
• Odontoma
FIGURE 7.16: Specimen of adenomatoid odontogenic tumor showing • Ameloblastoma
a solid tumor mass with large cystic spaces containing yellowish
• Calcifying odontogenic cyst.
semisolid material

• Cut surface shows a solid tumor mass with large cystic Management
spaces containing yellowish semisolid material (Fig. 7.16) 1. Surgical enucleation is the treatment of choice.
• Peripheral variant shows epulis like growth attached to 2. Curettage.
palatal or labial gingiva.

Radiographic Features CALCIFYING EPITHELIAL ODONTOGENIC

TUMOR
• Follicular: Well-defined unilocular radiolucency associated
with crown or part of root of the tooth (Fig. 7.17) Initially was called as calcifying ameloblastoma, malignant
• Extrafollicular: Not associated with tooth. Well-defined, odontoma, etc. Then Pindborg, 1955, coined the term 'Calcifying
unilocular radiolucency between, above or superimposed epithelial odontogenic tumor'. 16 Hence, also referred to
on tooth roots as Pindborg's tumor. This term has also been accepted by
• Peripheral variant: Saucerization of bone. WHO.

Histopathologic Features Pathogenesis

• Ameloblast like cells are arranged in sheets or strands or • May arise from reduced enamel epithelium of unerupted
form a rosette pattern tooth
210 Essentials of Pediatric Oral Pathology

FIGURES 7.18A and B: Histopathologic picture of adenomatoid odontogenic tumor showing a duct-like arrangement of ameloblast like cells

FIGURE 7.20: Radiographic picture of calcifying epithelial odonto-

genic tumor showing a multilocular radiolucent area containing
FIGURE 7.19: Calcifying epithelial odontogenic tumor presenting as radiopaque masses of varying sizes and opacities
a swelling in the lower left posterior region of the jaw

• Peripheral variant may arise from: Radiographic Features

— Rests of dental lamina
Irregular unilocular or multilocular radiolucent area
— Basal cells of oral epithelium.
containing radiopaque masses of varying sizes and opacities
(Fig. 7.20).
Clinical features
• It is a benign neoplasm located intraosseously and extra- Histopathologic Features
osseously
• Occurs in the age range of 8 to 92 years • Tumor composed of sheets or islands of polyhedral
• Males are affected more commonly than females odontogenic epithelial cells.
• Occurs more commonly in mandibular premolar-molar area • Cells have a prominent cellular outline and intercellular bridges.
as compared to maxilla (Fig. 7.19) • Nuclei show variations in shape and size.
• Intraosseously, it appears as a painless mass with slow growth • Epithelial component encloses homogenous, eosinophilic
• In the maxilla, nasal congestion, epistaxis and headache amyloid-like material.
are common symptoms • Calcified concentric rings may be seen intraepithelially
• Extraosseously, it appears as a painless, firm mass on called as “Liesegang rings” (Fig. 7.21).
gingival tissue. • Connective tissue is fibrous in nature.
Odontogenic Tumors in Children 211

Pathogenesis
• Controversial
• May arise from epithelial rests of Malassez in the periodontal
ligament
• May be hamartomatous
• The peripheral variant may arise from gingival surface
epithelium.

Clinical Features
• Occurs in the age range of 8 to 74 years
• Incidence in females is more as compared to males
• Occurrence in mandibular posterior region is more common
as compared to maxilla
• The most common variant is the central type, peripheral
FIGURE 7.21: Histopathologic picture of calcifying epithelial odontogenic
variant is rare
tumor showing sheets or islands of polyhedral odontogenic epithelial • Swelling in alveolar process, mobility of teeth and pain are
cells. Presence of "Liesegang rings" can be appreciated within the common signs and symptoms.
epithelium
Radiographic Features
• Central variant shows well-defined radiolucency between
roots of teeth
• May be unilocular or multilocular
• Peripheral variant shows saucerization of bone.

Histopathologic Features
• Islands of squamous epithelium are seen within mature
connective tissue stroma (Fig. 7.23)

FIGURE 7.22: Squamous odontogenic tumor presenting as an

incisor-canine gingival mucosal ulceration and labial infiltration

Management
1. Small intrabony lesions with well defined borders are
treated with enucleation or curettage followed by
removal of a thin layer of bone adjacent to the tumor.
2. Recurrent tumors require segmental resections or
hemimandibulectomy or hemimaxillectomy.

SQUAMOUS ODONTOGENIC TUMOR

(FIG. 7.22) FIGURE 7.23: Histopathologic picture of squamous odontogenic tumor
showing numerous islands of benign, well-differentiated, stratified,
Pullon et al, 1975, first coined the term squamous odontogenic squamous epithelium in an abundant, mature, connective tissue
tumor for this lesion located in the periodontium.17 It is a benign stroma. Epithelial cells are without cytologic atypia and surrounded by
but locally infiltrative odontogenic neoplasm. fibrous tissue
212 Essentials of Pediatric Oral Pathology

FIGURE 7.24: Ameloblastic fibroma presenting as a FIGURE 7.25: Histopathologic picture of ameloblastic fibroma show-
swelling in the upper anterior region of the jaw ing islands of odontogenic epithelium within the ectomesenchyme

• Peripheral layer of cells are low cuboidal as compared to Pathogenesis

tall columnar cells seen in solid/multicystic ameloblastoma
• Ameloblastic fibroma is a neoplasm of odontogenic origin
• Few islands may show degeneration of spinous cells.
with epithelial and ectomesenchymal components
Management • Morphologically, ameloblastic fibroma is similar to the
normal tooth anlage before hard tissue formation has
1. Conservative surgical intervention started
2. Curettage
• Pathogenetically, the epithelial components—the amelo-
3. Local excision
4. Aggressive lesions are treated by en bloc excision. blast-like cells are too primitive to induce the cells of the
ectomesenchyme for odontoblast differentiation. The step
of induction of odontoblastic differentiation is lacking in
ODONTOGENIC EPITHELIUM WITH
ameloblastic fibroma.
ODONTOGENIC ECTOMESENCHYME WITH
OR WITHOUT DENTAL HARD TISSUE
FORMATION Clinical Features

• Ameloblastic fibroma • Occurs in first and second decade of life.

• Ameloblastic fibrodentinoma • Painless, slow growing, expansile lesion.
• Ameloblastic fibroodontoma • Males are more commonly affected as compared to females.
• Complex odontoma • Posterior mandible is affected more often than maxilla.
• Compound odontoma
• Odontoameloblastoma
Radiographic Features
• Calcifying cystic odontogenic tumor
• Dentinogenic ghost cell tumor. Well-defined uni- or multilocular radiolucency with a
radiopaque border.
AMELOBLASTIC FIBROMA (FIG. 7.24)
Histopathologic Features (Fig. 7.25)
Kruse, 1891, first coined the term ameloblastic fibroma.18
It is considered to be a true mixed tumor as both epithelial • Irregular strands, islands or cords of odontogenic
and ectomesenchymal components are neoplastic. Only in epithelium
com parison with ameloblast ic fibrodent inoma and • Peripheral row of tall columnar cells resembling ameloblasts.
ameloblastic fibroodontoma, dental hard tissue formation is • Center of ameloblast-like cells show cells resembling
not seen. stellate reticulum
Odontogenic Tumors in Children 213

• Ectomesenchymal cells show loose, delicate collagen fibers

• Sometimes myxomatous area may be seen within the
ectomesenchyme.

Management
1. Surgical resection.
2. Mosby et al, 1998, suggest the conservative removal
of ameloblastic fibroma and modified block resection
to prevent any recurrence.19

AMELOBLASTIC FIBRODENTINOMA
Straith, 1936, first coined the term ameloblastic fibrodenti-
noma.20 He defined ameloblastic fibrodentinoma as a very rare
neoplasm composed of odontogenic epithelium and immature
connective tissue and characterized by the formation of
dysplastic dentin. FIGURE 7.26: Histopathologic picture of ameloblastic fibrodentinoma
showing an island of ameloblast-like cells embedded within a primitive
Pathogenesis dental papilla-like area
• Ameloblastic fibrodentinoma is of odontogenic origin with
epithelial and ectomesenchymal components
Pathogenesis
• Considered to be one of the intermediate stages between
ameloblastic fibroma and ameloblastic fibrodentinoma. • Ameloblastic fibro-odontoma is a member of the family of
mixed odontogenic tumors. It is of odontogenic origin with
Clinical Features epithelial and ectomesenchymal components
• Occurs in first and second decade of life. Most of the cases • The inductive changes that lead to formation of enamel in
are diagnosed before the age of 20 years addition to dentin are more advanced as compared to
• Painless, slow growing tumor ameloblastic fibroma and ameloblastic fibrodentinoma.
• May be associated with unerupted teeth.
Clinical Features
Radiographic Features
• It is basically described as the tumor of childhood and
Well-defined uni- or multilocular radiolucency with adolescence
radiopacities.
• Occurs in the age range 1 to 22 years
• Males are affected more as compared to females
Histopathologic Features (Fig. 7.26)
• Occurrence in mandibular posterior area is common
• Strands and islands of odontogenic epithelium in cell-rich • Painless, slow growing, expansile lesion.
primitive ectomesenchyme resembling dental papilla
• There are some inductive changes that lead to the Radiographic Features (Fig. 7.27)
formation of dentinoid.
Well-defined uni- or multilocular radiolucency with a
radiopaque border.
Management
Surgical resection. Histopathologic Features (Fig. 7.28)
• Irregular strands and islands of odontogenic epithelium
AMELOBLASTIC FIBRO-ODONTOMA
within the ectomesenchymal tissue
Hooker, 1967, first used the term 'ameloblastic odontoma' for • Center of the islands consists of enamel matrix, surrounded
ameloblastic fibro-odontoma.21 Later on various cases were by dentin and cementum
reported in the literature and finally the term ameloblastic fibro- • Epithelium appears ameloblast-like with few stellate
odontoma was agreed upon. reticulum cells.
214 Essentials of Pediatric Oral Pathology

Etiology
• Local trauma
• Infection
• Family history
• Genetic mutation.

Pathogenesis
It is a self-limiting developmental anomaly or hamartomatous
malformation.

Two Types
1. Complex odontoma
2. Compound odontoma.
FIGURE 7.27: Radiographic picture of ameloblastic fibro-odontoma This is an arbitrary distinction because it is based on the
showing a multilocular radiolucency with radiopaque border appearance of tooth-like structures (compound) or mass of
disorganized odontogenic tissues (complex).

Complex odontoma
Clinical features
• Occurs in the age range of 2 to 74 years
• Males are more commonly affected as compared to females
• Occurrence in the posterior mandible is most common
followed by anterior maxilla
• It is a slow growing painless mass, expanding in nature.
Radiographic Features
Radiographically, it shows three stages based on the degree
of mineralization:
1. First stage: Radiolucency due to lack of calcification
is seen ("weiches odontom" = soft odontoma).
2. Intermediate stage: Partial calcification is seen.
FIGURE 7.28: Histopathologic picture of ameloblastic fibro-odontoma 3. Third (Final) stage: Radiopacity surrounded by a thin
showing irregular strands and islands of odontogenic epithelium within radiolucent line is seen (Fig. 7.29).
the ectomesenchymal tissue. Center of the islands consists of enamel
matrix Histopathologic features
• Cementum or cementum-like material admixed with
dentinoid substance, small spaces with pulp tissue, enamel
Management matrix and epithelial remnants are generally observed
1. Depends on the nature of the lesion. (Fig. 7.30)
2. Conservative approach if lesion is not extensive. • In 16% of the cases, ghost cells are seen
3. Surgical resection if lesion is extensive. • Ghost cells are eosinophilic altered epithelial cells
characterized by the loss of nuclei with preservation of
ODONTOMA basic cellular outline
• It either represents coagulative necrosis or may be an
Odontoma means a tumor of odontogenic origin. Yet, it comes aberrant keratinization of odontogenic epithelium.
under the category of mixed odontogenic tumors as it consists
of both epithelial and ectomesenchymal components. Both Management
epithelial and ectomesenchymal components appear normal
morphologically but there is a deficit in structural arrangement. 1. Conservative enucleation is recommended as the
Thus few opine on it being a hamartomatous lesion than a treatment of choice for complex odontomas.
2. Blinder et al, 1993, suggested an intraoral lingual
true neoplasm. It is called as a composite odontoma as it is
approach for mandibular odontomas.22
composed of more than one type of tissue.
Odontogenic Tumors in Children 215

FIGURE 7.31: Radiographic picture of compound odontoma showing

small radiopaque masses preventing eruption of the central incisor

FIGURE 7.29: Radiographic picture of complex odontoma (arrow)

showing the odontoma surrounded by a radiolucent periphery. There
is retention of the deciduous canine

FIGURE 7.32: Histopathologic picture of compound odontoma

showing denticles embedded in fibrous stroma

Radiographic features
Radiopaque mass of varying size composed of a disorderly
pattern of tooth-like structure (Fig. 7.31).
Histopathologic features
FIGURE 7.30: Histopathologic picture of complex odontoma • Consists of dental tissues like enamel, dentin, cementum,
showing enamel matrix and odontogenic epithelium pulp tissue with more or less regular arrangement (Fig. 7.32)
• Three percent of the cases consist of ghost cells.
Compound odontoma
Pathogenesis Management
It is odontogenic in origin and may develop in the later stages 1. Conservative enucleation of the odontoma is the
of ameloblastic fibroma if left untreated. treatment of choice with care taken to see that all
denticles have been removed, because some may
Clinical features be easily overlooked.
• 75% of cases occur before the age of 20 years 2. Unerupted neighboring teeth may be saved when
• Males are affected more frequently than females the prognosis for tooth eruption is good.
• Anterior maxilla is the most frequent location.
216 Essentials of Pediatric Oral Pathology

FIGURE 7.33: Odontoameloblastoma presenting as a swelling in FIGURE 7.34: Histopathologic picture of odontoameloblastoma
the alveolar bone of lower right anterior region showing odontogenic epithelium within the ectomesenchymal tissue.
Areas of dentinoid are evident

ODONTOAMELOBLASTOMA • Due to the presence of odontogenic ectomesenchyme,

inductive changes take place forming dysplastic dentin and
Hooker, 1967,21 used the term ameloblastic odontoma for this
enamel that are arranged haphazardly
lesion. Then Thoma, 1970, coined the term 'Odontoameloblas-
• Ghost cells may be present.
toma'.23
Management
Pathogenesis
Since these lesions are considered to be as aggressive
Although clearly of odontogenic origin, its pathogenesis is as ameloblastoma, radical surgical intervention is the
not known. treatment of choice.

Clinical features
Note: Calcifying cystic odontogenic tumor and dentinogenic
• Occurs in the age range of 3 to 50 years ghost cell tumor are generally not found in the pediatric age
• Males are affected more as compared to females group, hence, are outside the scope of this book.
• Occurrence in posterior mandible is more common as
compared to maxilla MESENCHYME AND/OR ODONTOGENIC
• It is an expansile, destructive lesion ECTOMESENCHYME WITH OR WITHOUT
• Presents with a swelling in alveolar bone (Fig. 7.33) INCLUDED ODONTOGENIC EPITHELIUM
• Dull pain may be associated with the lesion
• Delayed eruption of teeth may be seen. • Odontogenic fibromas (epithelium poor and epithelium
rich)
Radiographic features • Odontogenic myxoma/fibromyxoma
• Cementoblastoma.
Well-defined unilocular or multilocular radiolucency containing
radiopaque masses. ODONTOGENIC FIBROMA

Histopathologic features The odontogenic fibroma is an elusive and controversial

tumor, elusive because of its rarity and controversial because
• Odontoameloblastoma was defined by WHO, 1992, as a of the uncertainty as to the number of distinct types that
very rare neoplasm that includes odontogenic ectomesen- exist.
chyme, in addition to odontogenic epithelium that
resembles an ameloblastoma in both structure and behavior
Two types
(Fig. 7.34)
• Irregular strands and islands of odontogenic epithelium 1. Central
may be seen within the ectomesenchymal tissue 2. Peripheral.
Odontogenic Tumors in Children 217

Central odontogenic fibroma

The central odontogenic fibroma (COF), as the name implies,
is a benign odontogenic neoplasm occurring within the jaws.
Pathogenesis
Based on its anatomic distribution and histological appearance,
this neoplasm is considered to be a tumor of the mesenchymal
components of the odontogenic apparatus—the periodontal
ligament, dental papilla or dental follicle.
Clinical features
• Very uncommon tumor
• Occurs in the age range of 4 to 80 years
• Females are more commonly affected as compared to males
• Occurrence in maxilla is more common than in mandible
• Usually occurs as a painless swelling with slow,
progressive growth, frequently resulting in cortical FIGURE 7.35: Histopathologic picture of central odontogenic fibroma
showing thick collagen bundles with few inactive looking odontogenic
expansion
islands
• Mobility of teeth may be seen.
Radiographic features
• Well-defined unilocular or multilocular radiolucent lesions Clinical features (Fig. 7.36)
often associated with teeth • Occurs in the age range of 5 to 65 years
• Root resorption is a common finding • No gender predilection is seen
• 12 percent of the cases have radiopaque flakes. • Occurrence in mandible is more as compared to maxilla
• Presents as a slow growing, firm gingival mass.
Histopathologic features
They show considerable diversity. Radiographic features
• Peripheral variant does not have any radiographic features
Types • May show saucerization of bone due to pressure effect.
They are of two types: Histopathologic features (Fig. 7.37)
1. Simple odontogenic fibroma: Composed of stellate • Mature, cellular fibrous connective tissue stroma is seen
fibroblasts, often arranged in whorled pattern with fine • Strands of odontogenic epithelium surrounding a small
collagen fibrils. amount of calcified material is present
• Small foci of odontogenic islands may or may not be • Calcified mass may resemble bone, dentin-like or cementum-
present like material.
• Occasional foci of dystrophic calcification may be present.
2. WHO type (Fig. 7.35): Management
• More complex pattern
Surgical excision with vigorous curettage is the treatment
• Fibrous connective tissue with collagen fibers in an
of choice.
interlacing pattern
• Odontogenic epithelium in the form of strands or cords
ODONTOGENIC MYXOMA
• Calcifications may be present.
Thoma and Goldman, 1947, introduced myxomas of the jaws
Management to the society for the first time.24 In the 1992 WHO classifi-
Enucleation and vigorous curettage is the treatment of cation, the term myxoma is used along with odontogenic
choice. myxoma and myxofibroma as alternative terms.

Peripheral odontogenic fibroma Pathogenesis

It is usually considered as the soft tissue counterpart of the • Adekeye et al, 1984, stated that this lesion represents a
central lesion. degenerative form of odontogenic fibroma25
218 Essentials of Pediatric Oral Pathology

FIGURE 7.36: Peripheral odontogenic fibroma presenting as a FIGURE 7.38: Histopathologic picture of odontogenic myxoma
swelling in the upper and lower anterior regions of the jaws showing a hypocellular tumor with prominent myxoid stroma

Radiographic features
• Unilocular or multilocular radiolucency causing root
resorption.
• Margins of the radiolucency are scalloped.
• Large myxomas may show soap bubble pattern.

Histopathologic features (Fig. 7.38)

• Haphazardly arranged stellate, spindle shaped and round
cells in an abundant loose myxoid stroma that contains
few collagen fibers.
• Small islands of odontogenic epithelium may or may not
be present.

Management
FIGURE 7.37: Histopathologic picture of peripheral odontogenic 1. Small lesions may be treated by curettage.
fibroma showing inactive looking odontogenic epithelium amongst 2. Larger lesions require extensive resection.
scattered pleomorphic fibroblasts
Note: Cementoblastoma is generally not seen in the pediatric
• The classification of the odontogenic myxoma as an age group, hence is not within the scope of this book.
odontogenic tumor has been justified by its frequent
occurrence in adolescence, its association with missing or MALIGNANT
unerupted teeth and the sporadic presence of odontogenic
epithelium within the neoplastic, myxomatous tissue. ODONTOGENIC CARCINOMAS
• Metastasizing malignant ameloblastoma
Clinical features
• Occurs mainly between the age ranges of 1 to 73 years. • Ameloblastic carcinoma
• No sex predilection is seen. – Primary
• Occurrence in mandible is more common as compared to – Secondary (dedifferentiated), intraosseous
the maxilla. – Secondary (dedifferentiated), extraosseous.
• Smaller lesions are discovered during routine radiography. • Primary Intraosseous Squamous Cell Carcinoma (PIOSCC)
• Larger lesions are associated with painless expansion of – PIOSCC solid type
bone. – PIOSCC derived from odontogenic cysts
Odontogenic Tumors in Children 219

– PIOSCC derived from keratinizing cystic odontogenic AMELOB LASTIC CAR CINOMA
tumor.
Ameloblastic carcinoma is a subtype of primary intraosseous
• Clear cell odontogenic carcinoma carcinomas exhibiting the features of both ameloblastoma and
• Ghost cell odontogenic carcinoma carcinoma. It is a rarely reported disorder in children and adults.
Corio et al, 1987, classified this tumor as any ameloblastoma
ODONTOGENIC SARCOMAS in which there is histologic evidence of malignancy in the primary
or recurrent tumor, regardless of whether it has metastasized or
• Ameloblastic fibrosarcoma
not.29
• Ameloblastic fibrodentinosarcoma
Recently, a case report of an extremely rare occurrence of
ameloblastic carcinoma located in the maxilla of a pediatric
METASTASIZING AMELOBLASTOMA
patient was presented by Nalan Yazici et al in 2006.30
Emura, 1923, first reported the metastasis of solid/multicystic
ameloblastoma to local lymph nodes.26 Vorzimer and Perla, Types
1932, were the first to report metastasis of ameloblastoma to • Central variant
the right lung.27 • Peripheral variant.

Pathogenesis Pathogenesis
• Controversial in nature Both the variants may arise either from their respective benign
• Authors predict that metastasis may be a result of multiple counterparts or may arise de novo.
recurrences or either by implantation of the tumor into
lymphatics and blood vessels. Clinical features
• Swelling, pain, paresthesia are the common presenting
Clinical features features
• Occurs in the age range of 5 to 74 years • Rapid growth is a characteristic feature
• Males are more commonly affected as compared to females • Dysphonia may be present.
• Occurrence in mandible is more common as compared to
Radiographic features
maxilla
• Swelling, pain, delayed tooth eruption, ulcerations were the • Ill-defined radiolucencies are seen
signs and symptoms reported by Reichart et al, 199528 • Foci of radiopacities due to dystrophic calcification may
• Lung is the most common site for the tumor to metastasize. also be seen within the radiolucent lesion.

Radiographic features Histopathologic features (Fig. 7.39)

• Malignant ameloblastomas cannot be distinguished • Irregular islands and cords of odontogenic epithelium
radi ograph ically from t heir n on-met astasi zing within the ectomesenchyme
counterparts • Nuclear hyperchromatism, increased nuclear cytoplasmic
• Computed tomography (CT) and Magnetic resonance ratio, abnormal mitotic activity may be seen
imaging (MRI) are required for diagnostic purposes. • Stellate reticulum cells show less orderly pattern
• Few areas show clear cells.
Histopathologic features
Management
• Neoplasm is characterized by irregular islands of Radical surgery with neck dissection is the treatment of
odontogenic epithelium within the ectomesenchyme choice.
• These components lack the features of malignancy.
PRIMARY INTRAOSSEOUS SQUAMOUS CELL
Management CARCINOMA (PIOSCC) SOLID TYPE
1. Radical resection with primary reconstruction is Morrison and Deeley stated that Loos in the year 1913, first
recommended. described central squamous cell carcinoma of the jaw. 31
2. Chemotherapy and radiotherapy as a palliative Pindborg, 1971, suggested the term primary intraosseous
treatment along with aggressive surgical intervention.
squamous cell carcinoma.32
220 Essentials of Pediatric Oral Pathology

FIGURES 7.39A and B: Histopathologic picture of ameloblastic carcinoma showing irregular islands and cords of
dysplastic odontogenic epithelium within the ectomesenchyme

Pathogenesis PRIMARY INTRAOSSEOUS SQUAMOUS CELL

CARCINOMA (PIOSCC) DERIVED FROM
May originate from:
ODONTOGENIC CYSTS
• Odontogenic epithelial cells consisting of reduced enamel
epithelium Primary intraosseous squamous cell carcinomas (PIOSCCs) are
• Rests of Malassez in periodontal ligament uncommon jaw malignancies derived from odontogenic
• Remnants of dental lamina in gingival tissue. epithelial remnants. The World Health Organization's
classification of primary intraosseous carcinomas distinguishes
Clinical features among solid-type carcinomas, carcinomas originating from
keratocystic odontogenic tumors (odontogenic keratocyst), and
• Occurs in the age range of 4 to 81 years carcinomas derived from odontogenic cysts other than
• Males are more commonly affected as compared to females keratocystic odontogenic tumors. Most PIOSCCs associated
• Occurrence in posterior mandible is more common as with cysts are well-differentiated squamous cell carcinomas
compared to maxilla arising from residual periapical cysts and dentigerous cysts;
• Pain and swelling are common presenting signs PIOSCCs originating from odontogenic keratocysts and
• Sensory disturbances may be present. orthokeratinized odontogenic cysts are less common.

Pathogenesis
Radiographic features
• Pathogenesis is unknown
Irregular diffuse radiolucencies are seen. • Long standing chronic inflammatory changes may be
possible predisposing factors of malignant transformation
Histopathologic features of the epithelial lining of the cyst
• Keratinization of cyst epithelium may be associated with
• Irregular odontogenic islands with basal type of cells
higher risk of malignant transformation.
showing palisading
• Squamous metaplasia and keratinization may be seen Clinical features
• Since histopathological diagnosis is difficult, examination
of serial sections is advised. • Occurs in the age range of 4 to 90 years
• Females are more commonly affected as compared to
Management males
• Occurrence in mandible is more as compared to maxilla
Radical surgery with neck dissection is the treatment of
• Recently, in 2008, an unusual case of a tumor resembling a
choice.
primary intraosseous carcinoma arising from an
Odontogenic Tumors in Children 221

FIGURE 7.40: Radiographic picture of primary intraosseous squamous cell carcinoma (PIOSCC) derived from odontogenic cysts showing
a lesion involving the developing tooth bud of the mandibular right first premolar with expansion of the contents of the crypt. Advanced root
resorption of both the first and second deciduous molars is evident

FIGURES 7.41A and B: Histopathologic picture of primary intraosseous squamous cell carcinoma (PIOSCC) derived from odontogenic
cysts showing islands and infiltrative cords of carcinoma cells with cohesive round cells and early squamoid differentiation

odontogenic cyst in a 5-year-old girl, with 7 years of Management

follow-up after treatment was reported by Makepeace
Charles et al.33 1. Surgical enucleation is the line of treatment for
smaller lesions.
2. Radical surgery with neck dissection is the treatment
Radiographic features (Fig. 7.40) of choice for larger lesions.
Radiolucency surrounded by well-defined radiopaque
PIOSCC derived from keratinizing cystic odontogenic
border.
tumor, clear cell odontogenic carcinoma and ghost cell
odontogenic carcinoma are generally not seen in the
Histopathologic features (Fig. 7.41) pediatric population and hence are outside the domain of
• Transition between normal cyst epithelium and squamous this book.
cell carcinoma is seen
• Epithelium may show nuclear hyperchromatism, increased AMELOBLASTIC FIBROSAR COMA
nuclear cytoplasmic ratio and abnormal mitosis Heath, 1887, first described this lesion as a spindle cell
• Thickened fibrous capsule of the cyst is seen as a result sarcoma of the mandible.34 The ameloblastic fibrosarcoma is a
of chronic inflammation. rare malignant neoplasm composed of benign odontogenic,
222 Essentials of Pediatric Oral Pathology

• Ectomesenchyme shows fibroblast-like cells that are

pleomorphic, rounded or fusiform
• These cells display atypical and increased mitosis.

Management
Radical surgery with neck dissection is the treatment of
choice.

R EFER EN CES
1. Broca PP. Recherches sur un nouveau groupe de tumeurs
designees sous le nom d'odontomes. Gaz Hebd Sci Med 1868;
5:70-84.
2. Malassez L. Note sur la pathogenie des kystes dentaires dites
periostiques. J Conn Med Prat (Paris) 1884;7:98-9;106-7;
115-6.
3. Malassez L. Sur le role des debris epitheliaux paradentaires.
FIGURE 7.42: Histopathologic picture of ameloblastic fibrosarcoma
Arch Physiol Norm Pathol 1885;5:309-40 and 6:379-449.
showing islands of well-differentiated ameloblastic epithelium
4. Churchill HR. Histological differentiation between certain
separated by a neoplastic stroma showing marked pleomorphism
dentigerous cysts and ameloblastomata. Dent Cosmos 1934;76:
and mitotic activity
1173.
5. Robinson HBG. Proceedings of the Fifth Annual Meeting of
the American Academy of Oral Pathology. Oral Surg 1952;5:
ameloblastomatous epithelium and malignant ectomesenchyme 177-8.
which resembles a fibrosarcoma. It is generally considered to 6. Ord RA, Blanchaert Jr RH, Nikitakis NG, Sauk JJ.
be the malignant form of the ameloblastic fibroma in which the Ameloblastoma in children 2002;60(7):762-70.
ectomesenchymal cells have retained their embryonic 7. Pindborg JJ. Odontogenic Tumors. In: Pathology of the Dental
appearance and develop malignant characteristics. Hard Tissues. Copenhagen: Munksgaard 1970;367-428.
8. Altini M, Slabbert HD, Johnston T. Papilliferous kerato-
Pathogenesis ameloblastoma. J Oral Pathol Med 1991;20:46-8.
9. Kuhn A. Über eine Kombination von Adamantinom mit
Gradual transformation of ameloblastic fibroma to ameloblastic Hämangiom als zentrale Kietergeschwulst. Dtsch Mschr Z
fibrosarcoma has been documented in a number of cases. 1932;50:49-56.
10. Stanley HR, Krogh HW. Peripheral ameloblastoma. Report of
Clinical features a case. Oral Surg Oral Med Oral Pathol 1959;12:760-5.
11. Huang I-Yueh, Lai Sheng-Tsung, Chen Chung-Ho, Chen Chun-
• Pain and swelling are the most constant findings
Ming, Wu Chung, Shen Yee-Hsiung. Surgical management of
• Ulceration, bleeding, paresthesia of the lip have also been ameloblastoma in children. Oral Surgery, Oral Medicine, Oral
reported Pathology, Oral Radiology, and Endodontics October 2007;
• Mobility of teeth may also be seen 104(4):478-85.
• Cervical lymphadenopathy and involvement of the 12. Eversole LR, Leider AS, Hansen LS. Ameloblastomas with
submandibular lymph nodes is uncommon. pronounced desmoplasia. J Oral Maxillofac Surg 1984;42:735-40.
13. Robinson L, Martinez MG. Unicystic ameloblastoma. A
Radiographic features prognostically distinct entity. Cancer 1977;40:2278-85.
14. Unal T, Cetingul E, Gunbay T. Peripheral adenomatoid
• Irregular radiolucencies with indistinct margins are odontogenic tumor: Birth of a term. J Clin Pediatr Dent 1995;
characteristic 19:139-42.
• Large radiolucencies with a multilocular appearance and 15. Phillipsen HP, Birn H. The adenomatoid odontogenic tumor,
gross expansion and thinning of the cortical bone may be ameloblastic adenomatoid tumor or adeno-ameloblastoma. Acta
seen. Pathol Microbial Scand 1969;75:375-98.
16. Pindborg JJ. A Calcifying epithelial odontogenic tumor. Cancer
Histopathologic features (Fig. 7.42) 1958;11:838-43.
17. Pullon PA, Shafer W, Elzay RP, Kerr DA, Corio RL. Squamous
• Irregular islands and strands of odontogenic epithelium odontogenic tumor. Oral Surg Oral Med Oral Pathol 1975;40:
similar to that seen in solid/multicystic ameloblastoma 616-30.
Odontogenic Tumors in Children 223

18. Kruse A. Über die Entwicklung cystischer Geschwülste im 28. Reichart PA, Phillipsen HP, Sonner S. Ameloblastoma:
Unterkiefer. Arch Pathol Anat 1981;124:137-89. Biological profile of 3677 cases. Eur J Cancer B Oral Oncol
19. Mosby EL, Russell D, Noren S, Barker BF. Ameloblastic 1995;31B:86-99.
fibroma in a 7-week-old infant: a case report and review of the 29. Corio RL, Goldblatt LI, Edwards PA, Hartmann KS.
literature. J Oral Maxillofac Surg 1998;0368-72. Ameloblastic carcinoma: A clinicopathologic study and
20. Straith FE. Odontoma: A rare type. Dent Dig 1936;42:196-9. assessment of eight cases. Oral Surg Oral Med Oral Pathol 1987;
21. Hooker SP. Ameloblastic odontoma: an analysis of twenty-six 64:570-6.
cases. Oral Surg Oral Med Oral Pathol 1967;24:375-6. 30. Nalan Yaz C , Begül Karagöz, Ali Varan, Taner Y lmaz, Arzu
22. Blinder D, Peleg M, Taicher S. Surgical consideration in cases Öztürk, Alp Usubütün, Münevver Büyükpamukçu. Pediatr
of large mandibular angle. Int J Oral Maxillofac Surg 1993; Blood Cancer 2008;50:175-6.
22:163-5. 31. Morrison R, Deeley TJ. Intra-alveolar carcinoma of the jaw:
23. Thoma KH. Oral Pathology, 6th ed. St Louis: Mosby, Treatment by supervoltage radiotherapy. Br J Radiol
1970;497-9. 1964;35:321-6.
24. Thoma KH, Goldman HM. Central myxoma of the jaw. J Oral 32. Pindborg JJ, Kramer IRH. Histologic Typing of odontogenic
Surg Orthod 1947;33:532. tumors, jaw cysts and allied lesions. Berlin: Springer-Verlag
25. Adekeye EO, Avery BS, Williams HK, Edwards MB. Advanced 1971.
central myxoma of the jaws in Nigeria. Clinical features, 33. Makepeace Charles, Torin Barr, Iona Leong, Bo Yee Ngan,
treatment and pathogenesis. J Oral Surg 1984;13:177-86. Vito Forte, George KB. Sándor. Primary intraosseous
26. Emura M. A case of metastatic ameloblastoma. Jpn J Surg 1923; malignancy originating in an odontogenic cyst in a young
24:760-4. child. Journal of Oral and Maxillofacial Surgery
27. Vorzimer J, Perla D. An instance of adamantinoma of the jaw 2008;66(4):813-9.
with metastasis of the right lung. Am J Pathol 1932;8:445-53. 34. Heath C. Certain diseases of the jaws. Br Med J 1887;2:5-13.
8
224 Essentials of Pediatric Oral Pathology

Epithelial Pathology
in Children
Mayur Chaudhary, Shweta Dixit Chaudhary

CHAPTER OVERVIEW
Introduction Ephelis
Squamous papilloma Lentigo simplex
Verruca vulgaris (Common wart) Pigmented nevi
Condyloma acuminatum Oral submucous fibrosis
Focal epithelial hyperplasia (Heck’s disease)

INTRODUCTION
Epithelium is a tissue composed of cells that line the cavities
and surfaces of structures throughout the body. There are four
main types of tissues: epithelial, connective, muscular, and
nervous. All of these tissues are found in our bodies, but
epithelial tissue has a special function—it must cover all the
surfaces of the body. Therefore, it is found in our skin, and it
is also found covering all the surfaces of the openings (each
one is called a lumen) within our bodies. The bottom edge of
the epithelial tissue abuts the basement membrane; this bottom
edge is called the basal surface. The edge of the epithelial tissue
that faces the lumen (or the outside world) is called the apical
surface. Cells within this tissue readily divide to make more
cells. This helps this tissue recover after any sort of abrasions
occur. FIGURE 8.1: Squamous papilloma showing a pedunculated,
This tissue does not have any vasculature. The cells painless, exophytic mass with papillary projections
within this tissue are firmly attached to each other. Cell to
cell junctions with each other are called tight junctions. SQUAMOUS PAPILLOMA (FIG. 8.1)
Functions of epithelial cells include secretion, selective It is a benign neoplasm of epithelial tissue. It is thought to be
absorption, protection, transcellular transport and detection induced by human papilloma virus (HPV) mainly the subtypes
of sensation. As a result, they commonly present extensive 6 and 11. The lesions are less virulent as compared to other
apical-basolateral polarity (e.g. different membrane proteins lesions produced by HPV.
expressed) and specialization. Any structural or functional
deviation from the normal may give rise to a variety of CLINICAL FEATURES
pathological entities of the epithelium termed as 'Epithelial • No sex predilection
Pathologies.' In this chapter, we shall discuss the epithelial • Greer and Goldman, 1974, in their study on 110 cases
pathologies commonly encountered in the pediatric reported that this lesion is most often seen in children, but
population. may occur at any age1
Epithelial Pathology in Children 225

FIGURE 8.2: Histopathologic picture of squamous papilloma in FIGURE 8.3: Verruca vulgaris showing
which epithelium appears to be arranged in a papillary fashion keratin horn on the index finger

• Sites most commonly affected are tongue, lips and soft palate CLINICAL FEATURES
• Occurs as a soft, pendunculated, painless, exophytic mass • Mostly seen in children
with papillary projections • Pink, yellow or white, painless pedunculated or sessile
• Due to the papillary projections, it is referred to as papules with papillary projections mostly seen on the skin
‘cauliflower-like’ of hands
• It may appear normal, whitish or reddish in color • Most common sites in the oral cavity are vermillion border
depending on the amount of keratinization of lip, labial mucosa and anterior tongue
• A rare form of disease termed as laryngeal papillomatosis • In some cases, cutaneous horn or keratin horns are seen
is seen in both children and adults on the skin surface that are produced as a result of
• In children, hoarseness of voice may occur and blockage continuous accumulation of keratin on the surface of skin
of the airways is the potential complication. that becomes hard (Fig. 8.3).

HISTOPATHOLOGIC FEATURES (FIG. 8.2) HISTOPATHOLOGIC FEATURES (FIG. 8.4)

• Proliferation of keratinized stratified squamous epithelium • Epithelium is keratinized stratified squamous with
in the form of projections acanthosis in the spinous cell layer
• The underlying connective tissue is fibrocellular, with • Club shaped rete ridges are seen that are at the normal level
chronic inflammatory cell infiltrate as that of the normal adjacent tissue
• Sometimes numerous virus altered cells termed koilocytes • Sometimes numerous virus altered cells termed koilocytes
are seen in the spinous layer.
are seen in the spinous layer
• Sometimes, epithelial cells show altered nucleus showing
Management
mitotic figures termed as mitosoid cells.
1. Conservative surgical excision.
2. In case of laryngeal papillomatosis in children, radical Management
surgical debulking is done as a treatment modality for
The following modes of management may be employed:
relieving obstruction.
1. Liquid nitrogen cryotherapy
2. Conservative surgical excision.
VERRUCA VULGARIS (COMMON WART) 3. Curettage.
4. Topical application of keratinolytic agents mostly
It is a contagious disease causing focal hyperplasia of stratified containing salicylic and lactic acid.
squamous epithelium and is mostly associated with HPV 5. Use of lasers or electrosurgery.
subtypes 2, 4, 6 and 40.
226 Essentials of Pediatric Oral Pathology

FIGURE 8.4: Histopathologic picture of verruca vulgaris showing FIGURE 8.5: Condyloma acuminatum appears as a sessile, pink,
keratinized stratified squamous epithelium with acanthosis in the well-demarcated lesion
spinous cell layer and club shaped rete ridges

CONDYLOMA ACUMINATUM (FIG. 8.5)

Condyloma acuminatum is one of the most common sexually
transmitted diseases in the world. It is a venereal wart induced by
human papilloma virus (HPV) of any of the following types 2, 6,
11, 53 and 54. In anogenital lesions, high risk type 16 and 18 are
also found. It is usually considered to be a sexually transmitted disease.
The incubation period of the virus averages two to three months.

CLINICAL FEATURES
• It occurs as a fleshy exophytic, sessile, well-demarcated,
painless lesion of anogenital region
• It may be seen in the oral cavity due to autoinoculation or
orogenital sexual practice. Knapp and Uohara, 1967, were
the first to report such a case in the oral cavity2
FIGURE 8.6: Histopathologic picture of condyloma acuminatum
• In the oral cavity, it occurs as a sessile, pink, well- showing vacuolization of granular cells and pyknotic nuclei in prickle
demarcated lesion on labial mucosa, soft palate and lingual cell layer surrounded by clear zone termed as ‘koilocytes’
frenum
• It usually occurs in children and young adults, is uncommon
in young children with a peak from 12 to 16 years that correlates with presence of HPV in lesions in which
• In children, its occurrence may raise the issue of sexual morphology is suggestive, but not diagnostic of condyloma.4
abuse.
Management
HISTOPATHOLOGIC FEATURES (FIG. 8.6) 1. Conservative surgical excision.
• Lesional tissue is covered by stratified squamous 2. Laser ablation, but it caries the risk of airborne spread
of HPV through aerosolized microdroplets.
epithelium with marked acanthosis
• Mild form of papillomatosis and hyperkeratosis may be seen
• Vacuolization of granular cells and pyknotic nuclei in FOCAL EPITHELIAL HYPERPLASIA
(HECK’S DISEASE) (FIG. 8.7)
prickle cell layer are observed. Such cells with pyknotic
nuclei surrounded by clear zone are termed as koilocytes3 Focal epithelial hyperplasia is essentially an oral infection with
• Special immunochemical staining used is MIB-1 the wart-producing papilloma virus type 13 and possibly type
immunostaining in nuclei of upper two-thirds of epidermis 32. It was first described in 1965 in Native Americans.5
Epithelial Pathology in Children 227

FIGURE 8.7: Focal epithelial hyperplasia showing slightly FIGURE 8.8: Ephelis showing multiple, light tan colored macules
elevated and well-demarcated plaques

CLINICAL FEATURES • Virus-like particles have been noted ultrastructurally within

both cytoplasm and nuclei of cells within the spinous layer,
• Heck’s disease primarily occurs in children, but lesions
and this layer is positive for HPV antigen with in situ
may occur in young and middle-aged adults
hybridization.
• There is no gender predilection
• Sites of greatest involvement include the labial, buccal and
Management
lingual mucosa, but gingival and tonsillar lesions have also
been reported Conservative surgical excision is the treatment of choice.
• Individual lesions are broad-based or slightly elevated so
as to present as well-demarcated plaques EPHELIS (FIG. 8.8)
• They may frequently appear papillary in nature, but Ephelides are sun-induced freckles which are most common
relatively smooth-surfaced, flat-topped lesions are more in fair-skinned individuals especially those with red or auburn
commonly seen hair.
• Papules and plaques are usually the color of normal
mucosa, but may be pale or, rarely, white CLINICAL FEATURES
• Hyperplastic lesions are small (0.3–1.0 cm), discrete and
well-demarcated, but they frequently cluster so closely • Ephelis usually appear as hyperpigmented macules and
together that the entire mucosal area takes on a cobblestone represent a region of increased melanin production
or fissured appearance. • They are more common in children and are less frequent
with increasing age
HISTOPATHOLOGIC FEATURES • They occur as autosomal dominant trait
• No sex predilection
• Lesional tissue shows stratified squamous epithelium
• They are usually uniform, multiple, light tan in color and
showing hyperplasia, sometimes with considerable focal
less than 3 mm with regularly defined borders. They are
acanthosis.
transient and related to sun exposure
• The thickened mucosa extends upwards, not down into
• They often appear on vermillion border of lips, with greater
underlying connective tissues, hence, the lesional rete ridges
frequency on lower lip. Because ephelides require sun
are at the same depth as the adjacent normal rete ridges.
exposure they do not occur intraorally.
• The ridges themselves are widened, often confluent and
sometimes club-shaped. Some superficial keratinocytes
show a koilocytic change similar to that seen in other HPV HISTOPATHOLOGIC FEATURES (FIG. 8.9)
infections; while occasional others demonstrate a collapsed Histologically there is increased pigmentation of basal cell
nucleus which resembles a mitotic figure (mitosoid cell). layer without increase in melanocytes or elongation of rete
These presumably result from viral alteration of the cells. pegs. The epidermis is normal.
228 Essentials of Pediatric Oral Pathology

FIGURE 8.9: Histopathologic picture of ephelis showing

increased pigmentation of basal cell layer

FIGURE 8.11: Histopathologic picture of lentigo simplex showing

hyperplasia of the epidermis and increased pigmentation of the
basal layer with variable number of melanocytes

• Clinically, the lesions are round or oval asymptomatic

macules that are 3 to 15 mm in diameter
• Their margins can be either jagged or smooth
• Pigmentation is evenly distributed, with a color ranging
from brown to black
• The lesions are few in number and may occur anywhere
on the skin or mucous membranes
• The lesions usually appear first in early childhood, but they
can also be present at birth or develop later.
FIGURE 8.10: Lentigo simplex showing brown
to black round macules HISTOPATHOLOGIC FEATURES (FIG. 8.11)
• Histopathologic findings may include hyperplasia of the
Management epidermis and increased pigmentation of the basal layer
1. No specific treatment.
• A variable number of melanocytes are present; these
2. Use of sunscreens can prevent darkening of existing melanocytes may be increased in number, but they do not
lesions. form nests.

LENTIGO SIMPLEX (FIG. 8.10) Management

1. No specific treatment.
A lentigo [lentigines (plural)], is a small, sharply circumscribed,
2. Conservative surgical excision for esthetic purpose.
pigmented macule surrounded by normal-appearing skin.
Lentigines may evolve slowly over years, or they may be eruptive
and appear rather suddenly. Pigmentation may be homogeneous PIGMENTED NEVI
or variegated, with a color ranging from brown to black. They Nevus is a general term that may refer to any congenital lesion
are thought to represent the early stages of melanocytic nevus.
of various cell types or tissue types such as epidermis, vessels
and pigmented cells.6
CLINICAL FEATURES
Nevus here refers to the pigmented lesion composed of
• Lentigo simplex (also called simple lentigo, juvenile nevus cells. These cells lack a dendritic process but otherwise
lentigo) is the most common form of lentigo are similar to melanocytes. They have the same enzyme,
• Lentigo simplex is not induced by sun exposure and it is tyrosinase and this enzyme is responsible for conversion of
not associated with systemic disease tyrosine to melanin in the melanosome organelle.
Epithelial Pathology in Children 229

FIGURE 8.12: Pigmented nevus seen on the palate as a round FIGURE 8.13: Histopathologic picture of pigmented nevus
bluish-black area showing fibroblast-like spindle cells with fasciculation

These cells are found in the epithelium and connective Lesions are elevated, smooth-surfaced papules or plaques that
tissue but the origin is not clear. It has been postulated that are gray-blue to bluish black in color. Lesions are usually
they are derived from pigment cells that migrate from neural solitary and found on the buttocks, the sacral region and
crest to the epithelium and submucosa, or that may develop occasionally on the dorsal aspects of the hands and the feet.
from altered resident melanocytes. • Blue color of the nevi is explained by Tyndall effect. This
The lesion they cause is not a true neoplasm but represents relates to the interaction of light with particles in a colloidal
a developmental malformation. suspension.
• In case of blue nevus, melanin particles are deep to the
CLINICAL FEATURES (FIG. 8.12) surface, so that the light reflected back has to pass through
the overlying tissue. Color with long wavelength (red and
• Pigmented nevi are rarely seen in the oral mucosa and if
yellow) tends to be more readily absorbed by the tissues;
present are located on the lower lip and less commonly on
shorter wavelength blue light is more likely to be reflected
the tongue, gingiva, hard palate and buccal mucosa.
back to the observer’s eye.
• They may appear at birth, puberty or in early adulthood
• Compound nevus is a lesion composed of two elements:
and are more common in females.
an intradermal nevus and overlying junctional nevus.
• They are raised, well-demarcated discrete lesions varying
• White, non-pigmented nevi of the oral mucosa have also
from light brown to blue-black lesions and they do not
been reported and these non-pigmented nevi appear as
blanch on pressure.
raised pink to white plaques.
• In the oral cavity, the intramucosal nevus is more common
• These pigmented nevi have a potential for malignant
and is seen in 55 percent of the cases, followed by blue
transformation. An increase in size, inflammation or
nevi seen in 36 percent of the cases.
increase in intensity of color of pigmented nevi specially
• The pigmented nevi are of 4 types:
Junctional and Compound type are danger signs and
1. Junctional
require immediate attention. Blue nevus may also undergo
2. Compound
malignant transformation but the prognosis is better.
3. Intramucosal and
4. Blue nevi.
HISTOPATHOLOGIC FEATURES (FIG. 8.13)
• The blue nevus occurs as a single, smooth, firm, round or
oval bluish-black or blue area less than 1 cm in size and is • The essential component of the Junctional, Compound and
commonly seen on the palate. Intramucosal type is the nevus cell, the location of which
• Andres Pinto et al, 2003, reported the first documented case helps in distinction of the three types.8
of epitheloid blue cell nevus involving the oral mucosa.7 • The nevus cell is a small, round or polyhedral cell with a
• The cellular blue nevus is a less common lesion but often distinctly outlined homogeneous or clear cytoplasm and a
clinically similar to the common blue nevus. These lesions centrally located nucleus. The cells are usually found in
tend to be large, usually measuring 1 to 3 cm in diameter. clusters or groups and they contain melanin.
230 Essentials of Pediatric Oral Pathology

• In junctional nevus, the nevus cells are limited to the lower

layers of the epithelium at the connective tissue junction
and there are long epithelial ridges.
• In intramucosal nevus, the epithelium is normal and nevus
cells are seen only in the connective tissue.
• In compound nevus, the nevus cells are seen both in the
lower layers of epithelium and connective tissue.
• The blue nevus is composed of fusiform cells or fibroblast-
like spindle cells with fasciculation that contain melanin
and these cells are in masses. Sometimes they resemble
Schwann-cells. A cellular variant of blue nevus called
cellular blue nevus is composed of large plump melano-
blasts and can be mistaken for melanoma. However this is
a benign lesion but can metastasize into the lymph nodes.
• In common blue nevus, a vaguely nodular collection of
poorly melanized spindle melanocytes and deeply FIGURE 8.14: Oral submucous fibrosis showing thick fibrous
pigmented dendritic melanocytes within thickened collagen bands on the buccal mucosa in a 14-year-old boy
bundles are seen. Scattered melanophages are usually
noted. No mitoses are present. in five Indian women from Kenya.9 Joshi subsequently coined
• In cellular blue nevus, a well-demarcated nodule formed the termed oral submucous fibrosis (OSF) for the condition
by fascicles and nests of tightly packed, moderately sized, in 1953.10 The condition was reported first by Paymaster for
spindle to oval melanocytes with scattered melanophages its precancerous potential. It is particularly associated with
is seen. areca nut chewing, the main component of betel quid.11
• The lesion is centered in the reticular dermis; blunt-ended, Betel quid is a combination of the areca nut (fruit of the
bulbous extensions that extend into the subcutaneous fat Areca catechu palm tree, erroneously termed betel nut) and
may be noted. Occasional mitoses may be present, but betel leaf (from the Piper betel, a pepper shrub), tobacco,
significant cytologic atypia and areas of necrosis are slaked lime (calcium hydroxide) and catechu (extract of the
absent. Often, a component of common blue nevus is seen Acacia catechu tree). Lime acts to keep the active ingredient
within these lesions. in its freebase or alkaline form, enabling it to enter the
• A number of variants of blue nevi with corresponding bloodstream via sublingual absorption.
histologic changes have been described, including epithe-
loid blue nevus, atypical blue nevus, deep penetrating blue ETIOPATHOGENESIS
nevus, sclerosing blue nevus and amelanotic blue nevus.
• The pathogenesis of the disease is not well-established, but
• The term malignant blue nevus is synonymous with
the cause of oral submucous fibrosis is believed to be
malignant melanoma arising in association with a cellular
multifactorial. A number of factors trigger the disease
blue nevus or growing in a histologic pattern similar to
process by causing a juxtaepithelial inflammatory reaction
that of a cellular blue nevus.
in the oral mucosa. Factors include areca nut chewing,
• These lesions typically have a pronounced cytologic atypia,
ingestion of chillies, genetic and immunologic processes,
hyperchromasia, necrosis, an increased mitotic rate, and
nutritional deficiencies and other factors.
an infiltrative growth pattern.
• Arecoline, an active alkaloid found in betel nuts, stimulates
Management
fibroblasts to increase production of collagen by 150
percent.12
1. No medicinal therapy is available. • Keratinocyte growth factor-1, insulin like growth factor-
2. Biopsy should be performed on any pigmented lesion 1, and interleukin 6 expression, which have all been
that changes with time. implicated in tissue fibrogenesis, were also significantly
3. For a solitary lesion, simple excision is usually
up-regulated in persons with oral submucous fibrosis due
curative.
to areca quid chewing and arecoline may be responsible
for their enhanced expression.13-15
ORAL SUBMUCOUS FIBROSIS (FIG. 8.14)
• Further studies have shown that arecoline is an inhibitor
In 1952, Schwartz coined the term atrophica idiopathica of metalloproteinases (particularly metalloproteinase-2)
mucosa oris to describe an oral fibrosing disease he discovered and a stimulator of tissue inhibitor of metalloproteinases,
Epithelial Pathology in Children 231

thus decreasing the overall breakdown of tissue collagen.16 given by Pindborg et al in 1966 and Utsunomiya H et al
Flavanoid, catechin and tannin in betel nuts cause collagen in 2005; classification systems based on clinical and
fibers to cross-link, making them less susceptible to histopathological features by Khanna JN et al in 1995.
collagenase degradation.17 • Khanna JN and Andrade NN, 1995, developed a group of
• This results in increased fibrosis by causing both increased classification system for the surgical management of
collagen production and decreased collagen breakdown. OSF.23
Oral submucous fibrosis remains active even after
cessation of the chewing habit, suggesting that components Group I: Very Early Cases
of the areca nut initiate oral submucous fibrosis and then • Common symptom is burning sensation in the mouth
affect gene expression in the fibroblasts, which then • Acute ulceration and recurrent stomatitis
produce greater amounts of normal collagen. • Not associated with mouth opening limitation.
• Areca nuts have also been shown to have a high copper
content and chewing areca nuts for 5 to 30 minutes signifi- Histopathologic features
cantly increases soluble copper levels in oral fluids. This • Fine fibrillar collagen network interspersed with marked
increased level of soluble copper supports the hypothesis edema
that copper acts as an initiating factor in persons with oral • Blood vessels dilated and congested
submucous fibrosis by stimulating fibrogenesis through up- • Large aggregate of plump, young fibroblasts present with
regulation of copper-dependent lysyl oxidase activity.18 abundant cytoplasm
• The role of chilli ingestion in the pathogenesis of oral • Inflammatory cells mainly consists of polymorphonuclear
submucous fibrosis is controversial. A hypersensitivity leukocytes with few eosinophils
reaction to chillies is believed to contribute to oral • Epithelium normal.
submucous fibrosis.
Group II: Early Cases
• A genetic component is assumed to be involved in oral
submucous fibrosis. Patients with oral submucous fibrosis • Buccal mucosa appears mottled and marble-like
have been found to have an increased frequency of HLA- • Widespread sheets of fibrosis palpable
A10, HLA-B7 and HLA-DR3.19 • Patients with an interincisal distance of 26 to 35 mm.
• Some authors have demonstrated increased levels of Histopathologic features
proinflammatory cytokines and reduced antifibrotic • Juxtaepithelial hyalinization present
interferon gamma (IFN-gamma) in patients with oral • Collagen present as thickened but separate bundles
submucous fibrosis, which may be central to the • Blood vessels dilated and congested
pathogenesis of oral submucous fibrosis.20 • Young fibroblasts seen in moderate number
• Iron deficiency anemia, vitamin B complex deficiency and • Inflammatory cells mainly consists of polymorphonuclear
malnutrition are promoting factors that derange the repair leukocytes with few eosinophils and occasional plasma cells
of the inflamed oral mucosa, leading to defective healing • Flattening or shortening of epithelial rete pegs evident with
and resultant scarring. The resulting atrophic oral mucosa varying degree of keratinization.
is more susceptible to the effects of chillies and betel nuts.
• Some authors have found a high frequency of mutations in Group III: Moderately Advanced Cases
the APC gene and low expression of the wild-type TP53
tumor suppressor gene product in patients with oral • Trismus evident, with an interincisal distance of 15 to 25 mm
submucous fibrosis, providing some explanation for the • Buccal mucosa appears pale and firmly attached to
increased risk of oral squamous cell carcinoma development underlying tissues
in patients with oral submucous fibrosis.21 Other studies • Atrophy of vermillion border
have suggested that altered expression of retinoic acid • Vertical fibrous bands palpable at soft palate, pterygo-
receptor-beta may be related to the disease pathogenesis.22 mandibular raphe and anterior faucial pillars.
Histopathologic features
CLASSIFICATION • Juxtaepithelial hyalinizination present
• There are several classification systems based on different • Thickened collagen bundles faintly discernible, separated
aspects of OSF, e.g. classification systems based on clinical by very slight residual edema
features given by Pindborg JJ in 1989, Lai DR in 1995, • Blood vessels, mostly constricted
Ranganathan K et al in 2001 and Rajendran R in 2003; • Mature fibroblasts with scanty cytoplasm and spindle-
classification systems based on histopathological features shaped nuclei
232 Essentials of Pediatric Oral Pathology

• Inflammatory exudates consists mainly of lymphocytes and

plasma cells
• Epithelium markedly atrophic with loss of rete pegs
• Muscle fibers seen interspersed with thickened and dense
collagen fibers.

Group IVA: Advanced Cases

• Trismus is severe with interincisal distance of less than
15 mm
• The faucet is thickened, shortened and firm to palpation
• Uvula is shrunken and appears as small, fibrous bud
• Tongue movements limited
• On palpation of lips, circular band felt around entire mouth.

Group IVB: Advanced Cases with Premalignant and

Malignant Changes
FIGURE 8.15: Oral submucous fibrosis showing diffuse hyalinization
Hyperkeratosis, leukoplakia, or squamous cell carcinoma can of subepithelial stroma with few, small fibroblastic nuclei and with
be seen. pigment incontinence from the overlying epithelial melanin

Histopathologic features
HISTOPATHOLOGIC FEATURES (FIG. 8.15)
• Collagen hyalinized as smooth sheet
• Extensive fibrosis obliterated the mucosal blood vessels • Epithelium in early lesions shows sub-epithelial vesicles
and eliminated the melanocytes whereas hyperkeratosis with atrophy is seen in advanced
• Fibroblasts were markedly absent within the hyalinized cases
zones • Connective tissue shows dense collagen fibers, sometimes
• Total loss of epithelial rete pegs compressing the blood vessels that lead to deprivation of
• Mild to moderate atypia present nutrition to the epithelium causing epithelial atrophy
• Extensive degeneration of muscle fibers evident. • Chronic inflammatory cell infiltrate is evident in the
connective tissue
CLINICAL FEATURES • Epithelial dysplasia is found in 10 to 15 percent of all cases.
• Oral submucous fibrosis is a chronic debilitating disease
of the oral cavity characterized by inflammation and Management
progressive fibrosis of the submucosal tissues (lamina The treatment of patients with oral submucous fibrosis
propria and deeper connective tissues). depends on the degree of clinical involvement.
• It mostly occurs in adults and very few cases have been
seen in children.24 Treatment strategies include the following:
1. Steroids: In patients with moderate oral submucous
• The buccal mucosa is the most commonly involved site,
fibrosis, weekly submucosal intralesional injections or
but any part of the oral cavity can be involved, even the topical application of steroids may help prevent further
pharynx. damage.
• Progressive inability to open the mouth (trismus) due to 2. Placental extracts: The rationale for using placental
oral fibrosis and scarring. extract in patients with oral submucous fibrosis derives
• Oral pain and a burning sensation upon consumption of from its proposed anti-inflammatory effect, hence,
spicy foodstuffs. preventing or inhibiting mucosal damage. Cessation
• Increased salivation. of areca nut chewing and submucosal administration
• Change of gustatory sensation. of aqueous extract of healthy human placental extract
• Hearing loss due to stenosis of the eustachian tubes. (Placentrex) has shown marked improvement of the
• Dryness of the mouth. condition.25
3. Hyaluronidase: The use of topical hyaluronidase has
• Nasal tonality to the voice.
been shown to improve symptoms more quickly than
• Dysphagia to solids (if the esophagus is involved). steroids alone. Hyaluronidase can also be added to
• Impaired mouth movements (e.g. eating, whistling, intralesional steroid preparations. The combination of
blowing, sucking).
Epithelial Pathology in Children 233

steroids and topical hyaluronidase shows better long- REFERENCES

term results than either agent used alone.26
1. Greer RO, Goldman HM. Oral papillomas. Clinicopathologic
4. IFN-gamma: This plays a role in the treatment of
evaluation and retrospective examination for dyskeratosis in 110
patients with oral submucous fibrosis because of its
lesions. Oral Surg 1974;38:435.
immunoregulatory effect. IFN-gamma is a known
antifibrotic cytokine. IFN-gamma, through its effect of 2. Knapp MJ, Uohara GI. Oral condyloma acuminatum. Oral Surg
altering collagen synthesis, appears to be a key factor 1967;23:538.
in the treatment of patients with oral submucous fibrosis 3. Von Krogh G. Condyloma acuminata 1983: an updated review.
and intralesional injections of the cytokine may have a Semin Dermatol 1983;2:109-29.
significant therapeutic effect on oral submucous 4. Pirog EC, Chen Y-T, Isacson C. MIB-1 immunostaining is a
fibrosis.27 beneficial adjunct test for accurate diagnosis of vulvar
5. Lycopene: Newer studies highlight the benefit of this condyloma acuminatum. Am J Surg Pathol 2000;24:1393-9.
oral nutritional supplement at a daily dose of 16 mg. 5. Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and
Mouth opening in 2 treatment arms (40 patients total) maxillofacial pathology. Philadelphia: WB Saunders 1995;
was statistically improved in patients with oral 320.
submucous fibrosis. This effect was slightly enhanced
6. Rajendran R, Shivapathasundharam B. Shafer’s textbook of
with the injection of intralesional betamethasone (two
Oral Pathology. Elsevier: a division of Reed Elsevier India Pvt.
1-ml ampules of 4 mg each) twice weekly, but the
Ltd. 5th edition 2006;117-21.
onset of effect was slightly delayed.28
6. Pentoxifylline: In a pilot study, 14 test subjects with 7. Andres P, Raghavendra S, Richard L, Scott D, Faizan A.
advanced oral submucous fibrosis given Epithelioid blue nevus of the oral mucosa: A rare histologic
pentoxifylline at 400 mg 3 times daily were compared variant. Oral surgery, oral medicine, oral pathology, oral
to 15 age- and sex-matched diseased control radiology, and endodontics 2003;96:429-36.
subjects. Statistical improvement was noted in all 8. Bjorlin, Gunnar and Bergman, Frank. Pigmented mole in oral
measures of objective (mouth opening, tongue mucosa. Odont Revy 1959;10:241-4.
protrusion, and relief from fibrotic bands) and 9. Schwartz J. Atrophia idiopathia tropica mucosaeoris.
subjective (intolerance to spices, burning sensation Demonstrated at the Eleventh International Dental Congress,
of mouth, tinnitus, difficulty in swallowing, and London, 1952.
difficulty in speech) symptoms over a 7-month
10. Joshi SG. Fibrosis of the palate and pillars. Indian J Otolaryngol
period.29 Further studies are needed, but this could
1953;4:1.
be used in conjunction with other therapies.
7. The role of these treatments is still evolving. The US 11. Paymaster JC. Cancer of buccal mucosa. Cancer 1956;9:
Food and Drug Administration has not yet approved 431-5.
these drugs for the treatment of oral submucous 12. Canniff JP, Harvey W. The aetiology of oral submucous fibrosis:
fibrosis. the stimulation of collagen synthesis by extracts of areca nut.
8. Surgical treatment is indicated in patients with severe Int J Oral Surg 1981;10:163-7.
trismus and/or biopsy results revealing dysplastic or 13. Tsai CH, Yang SF, Chen YJ, Chou MY, Chang YC. Raised
neoplastic changes. Surgical modalities that have keratinocyte growth factor-1 expression in oral submucous fibrosis
been used include the following: in vivo and upregulated by arecoline in human buccal mucosal
a. Simple excision of the fibrous bands: Excision can fibroblasts in vitro. J Oral Pathol Med 2005;34(2):100-5.
result in contracture of the tissue and exacerbation 14. Tsai CH, Yang SF, Chen YJ, Chu SC, Hsieh YS, Chang YC.
of the condition. Regulation of interleukin-6 expression by arecoline in human
b. Split-thickness skin grafting following bilateral buccal mucosal fibroblasts is related to intracellular glutathione
temporalis myotomy or coronoidectomy: Trismus
levels. Oral Dis 2004;10(6):360-4.
associated with oral submucous fibrosis may be
15. Tsai CH, Yang SF, Chen YJ, Chou MY, Chang YC. The
due to changes in the temporalis tendon
secondary to oral submucous fibrosis; therefore, upregulation of insulin-like growth factor-1 in oral submucous
skin grafts may relieve symptoms. fibrosis. Oral Oncol 2005;41(9):940-6.
c. Nasolabial flaps and lingual pedicle flaps: Surgery 16. Chang YC, Yang SF, Tai KW, Chou MY, Hsieh YS. Increased
to create flaps is performed only in patients with tissue inhibitor of metalloproteinase-1 expression and inhibition
oral submucous fibrosis in whom the tongue is not of gelatinase A activity in buccal mucosal fibroblasts by
involved. arecoline as possible mechanisms for oral submucous fibrosis.
d. Use of a KTP-532 laser release procedure was Oral Oncol 2002;38(2):195-200.
found to increase mouth opening range in 9 17. Harvey W, Scutt A, Meghji S, Canniff JP. Stimulation of human
patients over a 12-month follow-up period in one buccal mucosa fibroblasts in vitro by betel-nut alkaloids. Arch
study.30 Oral Biol 1986;31(1):45-9.
234 Essentials of Pediatric Oral Pathology

18. Trivedy CR, Warnakulasuriya KA, Peters TJ, Senkus R, 24. Hazarey VK, Erlewad DM, Mundhe KA, Ughade SN. Oral
Hazarey VK, Johnson NW. Raised tissue copper levels in oral submucous fibrosis: study of 1000 cases from central India.
submucous fibrosis. J Oral Pathol Med 2000;29(6):241-8. Journal of Oral Pathology and Medicine 2006;36(1):12-7.
19. Aziz SR. Oral submucous fibrosis: an unusual disease. JNJ Dent 25. Anil S, Beena VT. Oral submucous fibrosis in a 12-year-old
Assoc Spring 1997;68(2):17-9. girl: Case report. Pediatr Dent 1993;15(2):120-2.
20. Haque MF, Meghji S, Khitab U, Harris M. Oral submucous 26. Kakar PK, Puri RK, Venkatachalam VP. Oral submucous fibrosis—
fibrosis patients have altered levels of cytokine production. J treatment with hyalase. J Laryngol Otol 1985;99(1): 57-9.
Oral Pathol Med 2000;29(3):123-8. 27. Haque MF, Meghji S, Nazir R, Harris M. Interferon gamma
21. Liao PH, Lee TL, Yang LC, Yang SH, Chen SL, Chou MY. (IFN-gamma) may reverse oral submucous fibrosis. J Oral
Adenomatous polyposis coli gene mutation and decreased wild- Pathol Med 2001;30(1):12-21.
type p53 protein expression in oral submucous fibrosis: a 28. Kumar A, Bagewadi A, Keluskar V, Singh M. Efficacy of lycopene
preliminary investigation. Oral Surg Oral Med Oral Pathol Oral in the management of oral submucous fibrosis. Oral Surg Oral Med
Radiol Endod 2001;92(2):202-7. Oral Pathol Oral Radiol Endod 2007;103(2): 207-13.
22. Kaur J, Chakravarti N, Mathur M, Srivastava A, Ralhan R. 29. Rajendran R, Rani V, Shaikh S. Pentoxifylline therapy: A new
Alterations in expression of retinoid receptor beta and p53 in adjunct in the treatment of oral submucous fibrosis. Indian J
oral submucous fibrosis. Oral Dis 2004;10(4):201-6. Dent Res 2006;17(4):190-8.
23. Khanna JN, Andrade NN. Oral submucous fibrosis: a new 30. Nayak DR, Mahesh SG, Aggarwal D, Pavithran P, Pujary K,
concept in surgical management:Report of 100 cases. Int J Oral Pillai S. Role of KTP-532 laser in management of oral
Maxillofac Surg 1995;24(6):433-9. submucous fibrosis. J Laryngol Otol 2008;1-4.
9 Connective Tissue Pathology in Children 235

Connective Tissue
Pathology in Children
Mayur Chaudhary, Shweta Dixit Chaudhary

CHAPTER OVERVIEW
Introduction Lymphangioma
Myofibroma Rhabdomyoma
Pyogenic granuloma Fibrosarcoma
Peripheral ossifying fibroma Osteosarcoma
Melanotic neuroectodermal tumor of infancy Rhabdomyosarcoma
Congenital epulis of newborn
Synovial sarcoma
Hemangiomas
Alveolar soft part sarcoma
Nasopharyngeal angiofibroma

INTRODUCTION fatal within days or weeks of birth, usually as a result of

pulmonary or gastrointestinal involvement.3
Connective tissue is a tissue that fills the interstices between
more specialized elements and serves to hold them together CLINICAL FEATURES
and support them. It develops from mesenchyme, an embryonic
• Incidence of occurrence in first four decades with mean
type of tissue. It anchors, binds and supports various cells,
age of 27 years.
tissues and organs in the body. The matrix of connective tissue
• Solitary nodules and diffuse myofibromatosis usually occur
consists of fibers, ground substance and tissue fluid. Any form
in the head and neck.
of mutation in the components of connective tissue either
genetic or environmental or due to some other conditions leads • Most commonly affects males.
to connective tissue pathologies. This chapter focuses on the • Superficial tumors usually present as palpable, rubbery, firm
connective tissue pathologies that are seen in children. nodules that are freely mobile, while deeper lesions are
typically fixed.
• In the oral cavity, most commonly seen on lips, cheek and
MYOFIBROMA
tongue.
Myofibromas are mesenchymal tumors that are commonly
found in the dermis or subcutaneous tissue and are rarely found RADIOGRAPHIC FEATURES
in muscle or bone. They were first described by Stout in 19541
and further classified by Chung and Enzinger in 1981.2 Well defined and multilocular radiolucent appearance.
There are three types of infantile myofibromas: solitary
HISTOPATHOLOGIC FEATURES
myofibroma, multicentric fibromatosis without visceral
involvement, and multicentric fibromatosis with visceral • They are characterized by presence of myofibroblastic
involvement. The prognosis is good for patients with a solitary spindle cells arranged in bundles or fascicles, often with
myofibroma or multicentric myofibromatosis without visceral areas of extensive hyalinization (Fig. 9.1).
involvement because these lesions often regress spontaneously. • Tumor cells are positive for smooth muscle actin and
Conversely, myofibromatosis with visceral involvement can be muscle specific actin.
236 Essentials of Pediatric Oral Pathology

• Occurs more commonly on maxillary gingiva as compared

to mandibular gingiva.
• Anterior areas of jaws are more commonly affected as
compared to posterior areas.

RADIOGRAPHIC FEATURES
• Usually resorption of the underlying bone is evident. This
may be due to the pressure phenomenon caused by the
lesion on adjacent bone.

HISTOPATHOLOGIC FEATURES (FIG. 9.3)

• Lesional tissue shows highly vascular proliferative areas
resembling granulation tissue with numerous small and
large endothelium lined vascular channels engorged with
FIGURE 9.1: Histopathologic picture of myofibroma showing red blood cells.
spindle cells arranged in fascicles

Management

1. Surgical excision.
2. In cases where surgery might cause major morbidity,
chemotherapy is an option for reducing the size of
the lesion and alleviating associated symptoms.4

PYOGENIC GRANULOMA
Pyogenic granulomas are benign vascular lesions that occur
most commonly on the acral skin and also on oral mucous
membranes of children. The term pyogenic granuloma is a
misnomer. It is now referred to as telangiectic granuloma.
Originally, these lesions were thought to be caused by bacterial
infection; however, the etiology has not been determined. They
are thought to be reactive in nature caused by exuberant tissue FIGURE 9.2: Pyogenic granuloma showing
response to local irritation or trauma. a sessile, lobulated mass

CLINICAL FEATURES
• Incidence of occurrence in children and young adults with
male predilection in children and female predilection in
young adults.
• Usually occurs as a pedunculated or sessile, smooth and
lobulated mass (Fig. 9.2).
• Surface is characteristically ulcerated and color ranges from
pink to red to purple.
• Young lesions appear reddish due to high vascularity as
compared to older lesions that appear more pink due to
presence of collagenized tissue.
• Bleeds easily due to extreme vascularity.
• In the oral cavity, it occurs most commonly on the gingiva,
where gingival inflammation and irritation resulting from
poor oral hygiene act as precipitating factors.
• Other common sites in the oral cavity are lip, tongue and FIGURE 9.3: Histopathologic picture of pyogenic granuloma showing
buccal mucosa. highly vascular proliferative areas resembling granulation tissue
Connective Tissue Pathology in Children 237

• Chronic inflammatory cell infiltrate consisting of plasma associated with an orthodontic appliance was detected in 3.8
cells and lymphocytes is evident. Neutrophils if present are percent of cases described by Buchner and Hansen7 and seven
mostly seen near the ulcerated surface. percent of pediatric cases described by Cuisia and Brannon.8
• Older lesions appear more fibrous. Thus it is suggested that Inflammatory hyperplasia originating in the superficial
gingival fibromas of the oral cavity represent pyogenic periodontal ligament is considered to be a factor in the
granulomas that have undergone fibrous maturation. histogenesis of peripheral ossifying fibroma.9

Management CLINICAL FEATURES (FIG. 9.4)

1. Conservative surgical excision. The excision should • Peripheral ossifying fibroma presents as a painless,
extend down to periosteum and adjacent teeth. hemorrhagic and often lobulated mass of gingiva or alveolar
2. Thorough scaling should be done to remove any mucosa
source of irritation and to prevent recurrence. • A lesion may vary somewhat in size over time, depending
3. Therapy with the pulsed-dye laser at vascular-specific on the amount of superficial inflammation and edema.
585 nm is very selective, usually requires no • Color of the lesion ranges from red to pink with ulcerated
anesthesia and produces excellent cosmetic results.
or non-ulcerated surface.
The pulsed-dye laser works quite well for intraoral
pyogenic granulomas. • They may often be mistaken for pyogenic granuloma.
4. Cryotherapy or silver nitrate therapy may be effective • Mostly occurs in teenagers and young adults.
for very small lesions; however, treatment failure rates • Females are more commonly affected than males.
are high. • Most common site of occurrence is maxillary anterior
5. In pediatric cases, a eutectic mixture of local anesthetics region of the jaw.
(EMLA) applied to the lesion and surrounding skin • Rarely migration or loosening of teeth is visible due to the
under an occlusive dressing for 1 to 2 hours prior to pressure phenomenon of the lesion on the underlying bone.
additional intralesional anesthesia may be of
significant value. RADIOGRAPHIC FEATURES
Radiographs may show irregular, scattered radiopacities in the
PERIPHERAL OSSIFYING FIBROMA
lesion.
Peripheral ossifying fibroma (POF) is a reactive, relatively
common gingival growth. Shepherd first reported this entity as HISTOPATHOLOGIC FEATURES (FIG. 9.5)
alveolar exostosis in 1844.5 The main etiological factors of POF • An aggregated submucosal proliferation of primitive oval
are trauma and chronic irritation, particularly from subgingival and bipolar mesenchymal cells is the hallmark of peripheral
plaque and calculus.6 Moreover, the occurrence of this lesion ossifying fibroma.

FIGURE 9.4: Peripheral ossifying fibroma presenting as a FIGURE 9.5: Histopathologic picture of peripheral ossifying fibroma
painless lobulated mass showing islands and trabeculae of woven or lamellar bone
surrounded by cellular stroma
238 Essentials of Pediatric Oral Pathology

• The lesion may be very cellular or may be somewhat melanotic neuroectodermal tumor of infancy and that
fibrotic, but scattered throughout are islands and trabeculae melanocytic cell population is the proliferative component of
of woven or lamellar bone, usually with abundant tumor.12
osteoblastic rimming. Metaplastic bone may also be seen.
• The calcified tissues may have the dark-staining, acellular, CLINICAL FEATURES
rounded appearance of cementum, in which case the term
peripheral cementifying fibroma has traditionally been • More than 90 percent cases present within the first year of
used. Many examples show an admixture of bone and life, usually from age one month to six months. Mean age
cementum, i.e. peripheral ossifying/cementifying fibroma, of patients with MNTI is 4.3 months.
and early lesions may contain only small ovoid areas of • Male to female ratio is 6:7. More than 90 percent of cases
dystrophic calcification. occur in the head and neck region with most occurring on
• Few cases show presence of multinucleated giant cells. the anterior part of maxillary ridge. Other common sites
include skull, mandible, epididymis and brain.
Management • The lesion appears as a sessile or slightly pedunculated,
1. Local surgical excision. lobulated, firm mass which typically has a deep blue or
2. The excision should extend down to periosteum and black surface discoloration.
adjacent teeth. • It is often rapidly growing, non-ulcerated and darkly
3. Thorough scaling should be done to remove any pigmented.
source of irritation and to prevent recurrence. • Lesion can destroy the developing deciduous and
permanent dentition.
MELANOTIC NEUROECTODERMAL
TUMOR OF INFANCY (FIG. 9.6) RADIOGRAPHIC FEATURES
Melanotic neuroectodermal tumor of infancy (MNTI) is a It presents as an unilocular or rarely as a multilocular
relatively uncommon tumor of infancy that primarily affects radiolucency.
jaws of newborn infants. It was initially reported by
Krompecker in 1918 as congenital melanocarcinoma.10 HISTOPATHOLOGIC FEATURES
In 1966, Borello and Gorlin reported a case with high
urinary excretion of vanillylmandelic acid (VMA), suggesting • Lesional tissue shows non-encapsulated, infiltrating tumor
a neural crest origin, and they proposed the term melanotic mass of cells arranged in pattern of alveolus like spaces,
neuroectodermal tumor of infancy.11 lined by cuboidal or large polygonal cells.
Paulo Eduardo Alencer et al 1999, selected three cases • These cells have pale abundant cytoplasm and nuclei with
of melanotic neuroectodermal tumor of infancy and stated that finely dispersed chromatin which may contain melanin
MDM-2 expression may be important for development of pigment (Fig. 9.7).

FIGURE 9.6: Melanotic neuroectodermal tumor of infancy FIGURE 9.7: Histopathologic picture of melanotic neuroectodermal
showing a non-ulcerated, sessile, lobulated, firm mass tumor of infancy showing cells arranged in pattern of alveolus like
spaces and containing melanin
Connective Tissue Pathology in Children 239

• Melanosomes are present in the cytoplasm and the cells

are immunoreactive for cytokeratin and melanoma-
associated antigen (HMB-45). Some lesional cells react for
neuron specific enolase (NSE), synaptophysin and Leu-7
as well.

Management
1. Wide surgical excision of the lesion is performed. This
treatment can usually be accomplished with a partial
maxillectomy by using a Weber-Fergusson incision
and a facial degloving approach. Teeth, developing
teeth and the adjacent bone must be sacrificed when
they lie near the borders of MNTI.
2. In instances of inoperable recurrence or where clear
margins are impossible to obtain, radiation therapy
and/or chemotherapy have been used, but too few
examples exist for preferences to be established.

CONGENITAL EPULIS OF NEWBORN FIGURE 9.8: Congenital epulis of newborn appearing as a

smooth-surfaced, pedunculated and lobulated tumor
The word epulis is derived from the Greek word, meaning “on
the gum” or “gumboil” and has unfortunately been used for a
variety of benign tumors and tumor-like conditions having
dissimilar structures and histogenesis.13
Congenital epulis is a rare benign soft-tissue tumor that is
also called “gingival granular cell tumor of the newborn” and
was described first by Neumann in 1871. 14 Although
congenital epulis is composed of granular cells and is similar
to the true granular cell tumor (granular cell myoblastoma),
the histology and epidemiology of these two lesions differ.
Granular cell tumors are less vascular and often have a
component of pseudoepitheliomatous hyperplasia. They contain
more conspicuous nerve bundles than do congenital epulides.
Congenital epulides only occur in the gum pads of infants,
whereas granular cell tumors usually occur in adults (between
20 and 60 years of age) and may involve multiple organs.15
The histogenesis of the lesion is uncertain, and proposed
cells of origin include odontogenic epithelium, undifferentiated
mesenchymal cells, pericytes, fibroblasts, smooth muscle cells,
nerve related cells and histiocytes.16,17 FIGURE 9.9: Histopathologic picture of congenital epulis of newborn
showing large, rounded and polyhedral cells with eosinophilic
CLINICAL FEATURES granular cytoplasm and small, round or ovoid nuclei
• Appears as smooth-surfaced, pedunculated and sometimes
lobulated tumor arising from the alveolar crest of newborn • The cells appear large, rounded and polyhedral with
infants (Fig. 9.8). eosinophilic granular cytoplasm and small, round or ovoid
• The lesion is most common in females, with a female-to- nuclei (Fig. 9.9).
male ratio of 8:1, and is three times more common in the • Blood vessels and collagen fiber bundles of various sizes
maxilla than the mandible.18 are evident among the granular cell nests.
• Older lesions show elongated cells separated by fibrous
HISTOPATHOLOGIC FEATURES connective tissue.
• Lesional tissue shows a covering of stratified squamous • Immunohistochemical examination reveals negativity of the
epithelium. granular cells for S-100 protein.
240 Essentials of Pediatric Oral Pathology

Management 6. Metastasizing hemangioma

7. Nevus vinosus
Surgical excision of the lesion is the most preferred
8. Hereditary hemorrhagic telangiectasis.
treatment.

HEMANGIOMA (FIG. 9.10)

The term hemangioma was originally used to describe any

vascular tumour like structure, both present around birth or
appearing later in life. In 1982, Mulliken and Glowacki
separated these conditions into a family of self-involuting
tumors (growing lesions that eventually disappear) from the
family of malformations (enlarged or abnormal vessels present
at birth and essentially permanent) (Table 9.1).19
A simple classification proposed by Watson and McCarthy
based on 1,308 blood vessel tumors is as follows:20
1. Capillary hemangioma
2. Cavernous hemangioma
3. Angioblastic or hypertrophic hemangioma
4 Racemose hemangioma FIGURE 9.10: Hemangioma appearing as a red colored,
5. Diffuse systemic hemangioma well-demarcated and flat lesion

TABLE 9.1: Classification of vasoformative tumors19

Vasoformative tumor New nomenclature Old nomenclature

Hemangiomas Capillary hemangioma Strawberry hemangioma

Juvenile hemangioma

Cavernous hemangioma

Mixed hemangioma Parotid hemangioma

Vascular malformations Venous malformation Cavernous hemangioma

Hemangiomatosis

Intramuscular venous malformation Intramuscular hemangioma

Capillary malformation Capillary hemangioma

Port-wine stain

Arteriovenous malformation Arteriovenous hemangioma

Arterial angioma

Arteriovenous aneurysm

Cirsoid angioma

Red angioma

Serpentine aneurysm

Lymphatic malformation Capillary lymphangioma

Cavernous lymphangioma

Lymphangioma

Cystic hygroma
Connective Tissue Pathology in Children 241

ETIOPATHOGENESIS and the University of Arkansas25 that maternal placenta embolizes

The cause of hemangioma is currently unknown; however, to the fetal dermis during gestation resulting in hemangio-
several studies have suggested the importance of estrogen magenesis; yet Duke researchers26 conducted genetic analyses of
signaling in hemangioma proliferation. small nucleotide polymorphisms in hemangioma tissue compared
Vascular malformations need to be understood in terms of to the mother’s DNA that contradicted this notion.
their embryology and development. The classic sequence of
events usually falls into three stages: CLINICAL FEATURES
1. The undifferentiated capillary network stage, in which the • Hemangiomas are approximately three to five times more
primitive mesenchyme is nourished by an interlacing system common in females than in males.
of blood spaces without distinguishable arterial and venous • Mainly occur in infants and children. The incidence of
channels. The more common capillary hemangioma hemangiomas increases to 23 percent in premature infants
represents an arrest in the development of the mesenchyme with a birthweight of less than 1000 g.
primordia in the undifferentiated capillary network stage. • It is a lesion of unknown etiology. One hypothesis postulates
As differentiation progresses, primitive vessels penetrate that placental cells, such as the trophoblast, may be the cell
deeper into the subcutaneous layer, the muscle, or the bone of origin for hemangiomas. Therefore, hemangiomas may
tissue and give rise to capillary hemangiomas. arise secondary to some event in utero. However, conflicting
2. The retiform developmental stage, that begins at about 48 evidence supports this hypothesis.
days of embryonic development and in which separate • Capillary hemangiomas are usually not present at birth but
venous and arterial stems appear on either side of the are antedated by a pale, well-demarcated, flat area, most
capillary network. Termination of development in the visible with agitation. Gene map loci for capillary
retiform developmental stage may produce venous, arterial, hemangiomas are 5q35.3, 5q31-q33, 4q12.
or capillary malformations because this stage is • Cavernous hemangiomas are composed of large, irregular,
characterized by an established venous, arterial and deep dermal and subcutaneous blood-filled channels that
capillary system. impart a purplish discoloration to the overlying skin. They
3. The final developmental stage that begins at two months’ are typically soft, poorly defined and readily blanch with
development and involves the gradual replacement of the compression.
immature plexiform network by the mature vascular • Mostly involved facial bones are mandible, maxilla and the
channels. In the final developmental stage, the maturation nasal bones.
of the venous and lymphatic systems predominates. • Central jaw lesions can show hypermobility of the teeth
Aberrations in this mature stage of development result in and distortion of the arch form.
venous malformations and lymphangiomas. • Severe hemorrhage following dental extraction is not an
Takahashi, 1994, hypothesized that during the third uncommon presentation of central hemangiomas of the
trimester of fetal development, immature endothelial cells maxilla and the mandible.
coexist with immature pericytes, which maintain their • Common clinical findings in central hemangiomas of the
proliferative capacity for a limited period during postnatal jaws include gingival bleeding, postextraction bleeding,
life.21 Angiogenic peptides, such as beta-fibroblast growth swelling, pain, mobility of the teeth and bony expansion.
factor, vascular endothelial growth factor, and proliferating cell • Root resorption of the teeth has been reported in 30 percent
nuclear antigen, induce proliferation of these immature cells, of cases, but the vitality of the teeth is usually not affected.
resulting in the development of the hemangioma. As the • Intramuscular hemangiomas in the oral region are most
endothelial cells differentiate, an influx of mast cells, various commonly seen in the masseter muscle.
myeloid cells, and tissue inhibitors of metalloproteinases
(TIMPs) occurs.22 TIMPs, along with interferon and trans- RADIOGRAPHIC FEATURES
forming growth factor produced by the mast cells, terminate • Some of them show a “honeycomb” appearance on
the endothelial cell proliferation and passively induce radiograph while sometimes it appears as a “sunburst”
involution by senescence of endothelial cells. appearance as seen in osteosarcoma.
In 2007, a paper from the Stanford Children’s Surgical • Angiography is considered to be the most definitive for
Laboratory revealed that localized soft tissue hypoxia coupled with identification of a vascular lesion.
increased circulating estrogen after birth may be the stimulus.23 • Contrast enhanced MRI can be used to differentiate
There is also a hypothesis presented by researchers at Harvard24 hemangioma from lymphangioma.
242 Essentials of Pediatric Oral Pathology

HISTOPATHOLOGIC FEATURES • Hemangiomas in their involuting phase show less mitotic

activity, foci of fibrofatty infiltration and normal mast cell
• The usual hemangioma is composed of many small
count.
capillaries lined by a single layer of endothelial cells
• Hemangiomas are associated with the following syndromes:
supported by connective tissue stroma of varying density
— Rendu-Osler-Weber syndrome: Autosomal dominant
(Fig. 9.11).
inheritance, multiple telangiectasis, occasional GI tract
• Cavernous form of hemangioma consists of large dilated
involvement, occasional CNS involvement.
blood sinuses with thin walls each showing an endothelial
lining. The sinusoidal spaces are usually filled with blood, — Sturge-Weber-Dimitri syndrome: Noninherited and
although an admixture with occasional lymphatic vessels nonfamilial, portwine stain, leptomeningeal angiomas.
occurs in some instances (Fig. 9.12). — Kasabach-Meritt syndrome: Thrombocytopenic
• Hemangiomas in their proliferative stages show endothelial purpura associated with hemangioma, consumptive
cell hyperplasia forming syncytial masses, thickened coagulopathy, microangiopathic hemolysis, intra-
endothelial basement membrane, and presence of large lesional fibrinolysis.
number of mast cells.
Management
1. Spontaneous regression occurs at an early age.
2. The two primary medical treatments are steroids and
interferon therapy. Steroids have become a mainstay
in the treatment of proliferating hemangiomas in
infants and children. High doses of systemic or
intralesional steroids are the first-line treatment and
a dramatic response is observed in 30 percent of
patients.27
3. Fost and Esterly first reported the use of systemic
steroids in the treatment of hemangiomas. Prednisone
at a dose of 20 to 30 mg/d was given for two weeks to
four months. Both of the patients with capillary
hemangiomas had a definite response, and three of
the four patients with mixed hemangiomas had a
definite response. Fost proposed that therapy be
discontinued if no response occurred after two weeks
because of the multiple adverse effects of systemic
FIGURE 9.11: Histopathologic picture of capillary hemangioma steroids in infants.28
showing numerous capillaries lined by a single layer of endothelial 4. Pope et al demonstrated in a randomized controlled
cells trial that oral corticosteroids offered more clinical and
biological benefit than pulse steroids, with a higher
risk of adverse effects noted in 20 patients with
problematic hemangiomas.29
5. Frequent monitoring of blood pressure should be
performed using the appropriately sized blood
pressure cuff during the administration of systemic
corticosteroid therapy.
6. Greinwald et al described a prospective randomized
trial of interferon alfa-2a involving 24 patients with
massive or life-threatening hemangiomas of the head
and the neck. They were given daily subcutaneous
injections for four months. Of those patients, 58
percent had a greater than 50 percent reduction in
the size of the tumor and 42 percent had a complete
response. Response rates were greater than those
for corticosteroids (58% v/s 30%). Another
investigation found that interferon alfa-2b was
FIGURE 9.12: Histopathologic picture of cavernous hemangioma
showing large dilated blood sinuses with thin walls each showing effective in reducing the size of the tumor in more than
an endothelial lining two-thirds of patients.30
Connective Tissue Pathology in Children 243

7. However, some concern exists regarding the toxicity — Nonparticulate agents

of interferon alfa, especially in children. The most i. Stainless steel coils
serious adverse effects include neurologic effects ii. Platinum coils
(e.g. spastic paresis, seizures, coma), hematologic iii. Silk streamers
effects (e.g. neutropenia, thrombocytopenia) and iv. Plastic brushes
hepatic toxicity. v. Detachable balloons
8. Complete surgical excision of these lesions offers the 10. In the treatment of vasoformative tumors, the
best chance of cure, but, often, because of the extent resorbable materials are not particularly useful in the
of these benign lesions, significant sacrifice of tissue long term, except when they precede a surgical
is necessary. treatment and only short-term occlusion is required.
9. Embolotherapy is one of the more commonly used Nonresorbable materials comprise the mainstay of
adjunctive procedures in the treatment of vascular embolotherapy for vasoformative tumors. Polyvinyl
tumors. Embolization literally means the occlusion of alcohol sponges (Ivalon) are obtained by reticulation
a vessel by the introduction of a foreign body. In a of polyvinyl alcohol with formaldehyde. The sponge
broader definition, it also means any other occlusion has the property of being compressible when wet
that is obtained with a proliferating reaction of the and reexpanding to its original shape and size when
vessel wall. In 1904, Dawbain, Lussenhop and a dried piece is placed in an aqueous solution, such
Spence described the preoperative injection of melted as blood. These properties make Ivalon particularly
paraffin-petrolatum into the external carotid arteries well suited for large vessels, in which it produces a
of patients with head and neck tumors. In 1930, permanent occlusion.
Brooks introduced particulate embolization when he 11. Isobutyl-2-cyanoacrylate (IBCA) is a rapidly
described the occlusion of a traumatic carotid- hardening plastic adhesive similar to superglue. The
cavernous fistula by injecting a fragment of muscle liquid plastic is readily injectable, even through very
attached to a silver clip into the internal carotid artery. small catheters, and it polymerizes almost instantly
Agents for embolotherapy can be broadly divided into upon contact with ionic fluids, such as blood or
two groups: absorbable materials and nonabsorbable vascular endothelium. This polymerization leaves
materials (see the list below). The nonabsorbable the plastic solid.
materials can be further subdivided into particulate, 12. Absolute ethanol is used as a sclerosing agent. Its
liquid, sclerosing, and nonparticulate agents. presumed mechanism of action is a direct toxic
effect on the vascular endothelium that activates the
Embolotherapy agents:
coagulation system on the dehydrated endothelium.
• Absorbable materials
Thus, the vascular occlusion is not achieved
— Autologous blood clot
instantly but rather in days to weeks. The toxic effect
— Modified blood clot
extends to the perivascular tissue, and the use of
— Gelfoam
absolute ethanol has led to perivascular necrosis.
— Oxycel
Injection of alcohol into oral lesions is followed by
• Nonabsorbable materials
marked swelling 6 to 8 hours later. By using small
— Particulate agents
volumes and carefully avoiding direct deposition into
i. Acrylic spheres
the overlying mucosa, necrosis of the mucosa can
ii. Autologous fat of muscle usually be avoided. Other agents used for sclerosis
iii. Ferromagnetic microspheres of oral vascular tumors include sodium morrhuate,
iv. Methylmethacrylate spheres sodium tetradecyl sulfate (STS), and hydroxy-
v. Polyvinyl alcohol (Ivalon) polyethoxydodecan (an agent that is a double
vi. Silastic spheres hydrophilic and hydrophobic chain).
vii. Stainless steel pellets 13. Use of laser therapy for the treatment of heman-
— Injectable (fluids) giomas has gained popularity. Lasers have evolved
i. Amino acid occlusion gel (Ethibloc) to where more selective photothermolysis can be
ii. Isobutyl-2-cyanoacrylate attained rather than nonselective tissue destruction.
iii. Microfibrillar collagen (Avitene) 14. The yellow light lasers (578 to 585 nm) are
iv. Silicone rubber selectively absorbed by hemoglobin.
— Sclerosing agents 15. Oral mucosa may be amenable to these lasers
i. Absolute ethanol because little melanin is present in the mucosa. Little
ii. Boiling contrast medium to no damage to the mucosa or the epithelium has
iii. Polidocanol been reported. In the macular stage of development,
iv. Sodium morrhuate a 585 nm pulsed dye laser has been used to treat
v. Sodium tetradecyl sulfate (Sotradecol) a capillary hemangioma.31 The tunable dye laser
244 Essentials of Pediatric Oral Pathology

can ablate superficial ecstatic blood vessels without • Nasal obstruction is the most frequent symptom, especially
significant epidermal damage or scarring. However, in initial stages.
the 585 nm pulsed dye laser has limited penetration • Epistaxis is mostly unilateral and recurrent.
(1 – 2 mm). • Headache is seen especially if paranasal sinuses are blocked
16. Apfelberg reported using a neodymium:yttrium- • Facial swelling may be evident.
aluminum-garnet (Nd:YAG) laser to treat massive • Other symptoms—Unilateral rhinorrhea, anosmia,
hemangiomas and vascular malformations in the
hyposmia, rhinolalia, deafness, otalgia, swelling of the
head and the neck via intralesional laser photo-
coagulation.32 The laser is theorized to institute an
palate, proptosis, deformity of the cheek.
initial thrombogenesis in many areas of the • Lesions in the oral cavity are rare.
hemangioma or the vascular malformation, and this
event initiates involution by normal body processes. RADIOGRAPHIC FEATURES
The Nd:YAG laser emits beams in the near infrared
Characteristic features of anterior bowing of posterior wall of
region of the spectrum (1064 nm). This laser has
deep penetration (1 cm) and an excellent hemostatic
maxillary sinus may be evident on CT scan and MRI.
capability that makes it more suitable for thicker,
larger, more developed hemangiomas. HISTOPATHOLOGIC FEATURES (FIG. 9.13)
17. Cryosurgery for cutaneous lesions has been asso- Lesional area shows a dense fibrous connective tissue
ciated with scarring, but it may have a role in the
containing numerous dilated thin-walled blood vessels of
treatment of oral mucosal lesions. Several authors
variable size.
have used cryosurgery for treating oral vascular
tumors, although this technique has fallen into
disfavor in recent years. Management
18. Surgery of intrabony lesions of the jaws is usually 1. The testosterone receptor blocker flutamide was
completed in combination with other procedures reported to reduce stage I and II tumors to 44 percent.
(e.g. embolization, sclerotherapy) to reduce blood
2. Stereotactic radiotherapy (i.e. gamma knife) delivers
loss.
a lower dose of radiation to surrounding tissues.
However, most authorities reserve radiotherapy for
NASOPHARYNGEAL ANGIOFIBROMA intracranial disease or recurrent cases.
Hippocrates described this tumor in the 5th century BC, but 3. Three-dimensional conformal radiotherapy in
Friedberg first used the term angiofibroma in 1940. It is a rare, extensive juvenile nasopharyngeal angiofibroma
vascular and fibrous tumor-like lesion that occurs only in the (JNA) or intracranial extension provides a good
nasopharynx. alternative to conventional radiotherapy regarding
disease control and radiation morbidity.
ETIOPATHOGENESIS 4. A lateral rhinotomy, transpalatal, transmaxillary, or
sphenoethmoidal route is used for small tumors.
• Although the etiology remains unknown, a hormonal theory 5. The infratemporal fossa approach is used when the
has been suggested because of the lesion’s occurrence in tumor has a large lateral extension. The midfacial
adolescent males. degloving approach, with or without a LeFort
• Other theories include a desmoplastic response of the osteotomy, improves posterior access to the tumor.
nasopharyngeal periosteum or the embryonic fibrocartilage 6. The facial translocation approach is combined with
between the basiocciput and the basisphenoid. Weber-Ferguson incision and coronal extension for
• Etiology from nonchromaffin paraganglionic cells of the a frontotemporal craniotomy with midface osteotomies
terminal branches of the maxillary artery has also been for access.
suggested. Comparative genomic hybridization analysis of 7. An extended anterior subcranial approach facilitates
these tumors revealed deletions of chromosome 17, en bloc tumor removal, optic nerve decompression
including regions for the tumor suppressor gene p53 as well and exposure of the cavernous sinus.
as the Her-2/neu oncogene. 8. Intranasal endoscopic surgery is reserved for tumors
limited to the nasal cavity and paranasal sinuses.
CLINICAL FEATURES Some authors advocate its use for lesions with limited
extension to the infratemporal fossa. Image-guided,
• Most commonly seen in males.
endoscopic, laser-assisted removal has also recently
• Onset is most commonly in the second decade; the range
been used.
is 7 to19 years.
Connective Tissue Pathology in Children 245

The different types of lymphangiomas are classified as

follows:
• Lymphangioma circumscriptum is a simplex superficial red
macular or vesicular lesion of mucous membranes or skin
• Lymphangioma capillary type is a simplex lesion of dilated
capillary-like channels.
• Lymphangioma cavernosa is a simplex lesion of dilated
lymphatic channels with deep extension and without cyst
formation.
• Lymphangioma cystica (i.e. cystic hygroma) is composed
of large lymphatic cysts that expand into adjacent soft tissue
planes and are well defined, circumscribed or lobulated.
• Lymphangioma complex is composed of multiloculated
FIGURE 9.13: Histopathologic picture of nasopharyngeal angio- poorly defined cysts extending to more than one anatomic
fibroma showing numerous dilated blood vessels surrounded by area, tissue plane or organ system.
connective tissue stroma
CLINICAL FEATURES
LYMPHANGIOMA • Children or, less commonly, adults usually present with a
Lymphatic malformations were first described by Redenbacher mass in the head and neck area.
in 1828. Many of the early researchers believed that lymphatic • Common head and neck sites in childhood are the cervical
malformations were neoplasms. Currently, most researchers agree area, floor of the mouth and the tongue.
that lymphatic malformations are not neoplastic and have • Orofacial manifestations include mandibular or maxillary
adopted the term “lymphatic malformation” to emphasize this deformation, rotation, and malocclusion (i.e. crossbite).
fact. • In a study by Orvidas et al, the most common location
involved was the submandibular region, followed by the
ETIOPATHOGENESIS parotid and cheek.36
Two major theories of development of the lymphatic system • Most of the cases are reported at birth.
have been proposed to explain the origin of lymphatic • Occur as soft, flaccid, fluctuant mass with a multilobulated
malformations. Sabin proposed that the lymphatic system consistency.
develops from five primitive sacs which sprout from the venous • Most common oral site is anterior two-third of the tongue,
system. In the head and neck, endothelial outbuddings from resulting in macroglossia.
the jugular sac spread centrifugally to form the lymphatic • May be seen bilaterally on the mandibular ridge.
system. 33 McClure and Huntington proposed that the
lymphatic system develops from mesenchymal clefts in the RADIOGRAPHIC FEATURES
venous plexus reticulum and spread centripetally towards the
jugular sac. Lymphatic malformations develop from • Radiographic findings are not so characteristic for this
sequestration or congenital blockage of the primitive lymphatic lesion.
anlage. • CT and MRI are most useful in determining proximity to
A role of vascular endothelial growth factor (VEGF) and vital structures and mediastinal involvement preoperatively.
pigment epithelium-derived factor (PEDF) had been shown in
their studies by Sidle et al.34 HISTOPATHOLOGIC FEATURES
CLASSIFICATION • Lesional tissue in case of cavernous lymphangioma is
composed of lymphatic vessels that may show marked
The most popular classification system was proposed by
dilatation (Fig. 9.14)
Landing and Farber in 1956. Lymphangioma simplex is
• Cystic hygroma may show macroscopic cyst-like spaces.
composed of three walled lymphatic channels. Cavernous
lymphangioma consists of dilated lymphatic spaces with Spaces contain proteinaceous fluid and occasionally
increased fibrous tissue. They also tend to invade surrounding lymphocytes.
tissue. Cystic lymphangioma is made up of endothelial lined • Capillary lymphangioma contains numerous endothelium
cysts varying in size from mm to several cm.35 lined, blood filled channels.
246 Essentials of Pediatric Oral Pathology

striated muscle development. Drugs or environmental factors

have not been identified as causes of this neoplasm.

CLINICAL FEATURES
• Fetal rhabdomyoma occurs between birth and age three years.
• Lesions in fetal rhabdomyoma are usually found in the
subcutaneous tissues of the head and neck (Fig. 9.15).
• Adult rhabdomyoma occurs in patients older than 40 years.
• Patients with adult rhabdomyoma might experience some
hoarseness, difficulty in breathing, difficulty in swallowing
or a combination. The lesions most commonly occur as round
or polypoid mass in the region of the neck and mostly affect
the oropharynx, the larynx, and the muscles of the neck.
• Genital rhabdomyoma is observed in young and middle-
aged women.
• Patients with genital rhabdomyoma are young or middle-
FIGURE 9.14: Histopathologic picture of cavernous lymphangioma
showing lymphatic vessels containing fluid and lined by endothelial
aged women presenting with vaginal masses.
cells • Cardiac rhabdomyomas occur chiefly, but not exclusively,
in the pediatric age group.
Management • Patients with cardiac rhabdomyoma may present with a
history of shortness of breath, heart murmurs and sometimes
1. Surgical excision is the treatment of choice. The associated with signs and symptoms suggestive of cerebral
primary intention is to accomplish total resections. palsy (suggesting the possibility of associated tuberous
2. Radiation therapy has been effective but abandoned sclerosis).
because of later malignant transformation or • Rhabdomyomatous mesenchymal hamartoma of the skin
retardation of growth sites. is observed in newborns and infants.
3. Carbon dioxide laser therapy has been effective in
managing upper airway lesions and superficial
HISTOPATHOLOGIC FEATURES
mucosal microcystic lesions.
4. Intralesional sclerotherapy with group A Streptococcus • Lesional tissue in adult type is composed of well-
pyogenes of human origin (OK-432) has had some differentiated large cells that resemble striated muscle cells.
success controlling lymphangiomas. The mechanism Cross-striation has been demonstrated by phosphotungstic
suggested is the stimulation of increased permeability acid hematoxylin (PTAH), muscle specific actin, desmin,
of the endothelium, accelerating lymphatic fluid and myoglobin while dystrophin is shown to be expressed
drainage and size reduction of the lymphangioma. in the cell membranes. The cells are deeply eosinophilic
5. Somnoplasty shows promise for reduction of tongue
lymphatic malformations.
6. Occasional reports have described the use of
triamcinolone, cyclophosphamide, bleomycin, fibrin
glue, and alcohol (Ethibloc). Results have been
inconsistent, and success is limited.

RHABDOMYOMA
Rhabdomyoma is an exceedingly rare tumor of striated muscle.

CLASSIFICATION
The two types of rhabdomyoma are neoplastic and hamartoma.
1. The neoplastic variety is subclassified into adult, fetal, and
genital types.
2. Hamartomas are divided into cardiac rhabdomyoma and
rhabdomyomatous mesenchymal hamartomas of the skin. FIGURE 9.15: Rhabdomyoma presenting as
Rhabdomyoma probably represents a genetic variant of a discrete nodular mass
Connective Tissue Pathology in Children 247

FIBROSARCOMA
Fibrosarcomas are relatively uncommon tumors and account
for 12 to 19 percent of soft tissue sarcomas. More than half
of all tumors arise in the lower extremities; approximately
10 percent occur in the head and neck, most commonly in
the sinonasal tract and neck.

ETIOLOGY
Fibrosarcomas arise from fibroblasts. The development of
fibrosarcoma is associated with previous radiation therapy or
burn injury; tumors are reported to arise in irradiated sites or
burn scars. They are of two varieties, viz. adult and infantile.

CLINICAL FEATURES
FIGURE 9.16: Histopathologic picture of fetal rhabdomyoma • Fibrosarcomas may arise in patients of any age; a slight
showing a mixture of spindle-shaped cells with indistinct cytoplasm male predominance exists. Most cases occur in those aged
and muscle fibers
30 to 60 years.
polygonal cells with small peripherally placed nuclei and • An infantile variant that occurs in patients younger than five
occasional intracellular vacuoles. years appears to represent a distinct subtype and is associated
• Lesional tissue in fetal type is composed of a mixture of with a better prognosis. An association with trisomy of
spindle-shaped cells with indistinct cytoplasm and muscle chromosomes 8, 11, 17 and 20 has been reported.
fibers, which resemble striated muscle tissue (Fig. 9.16). • They most commonly manifest as painless, gradually
• Lesional tissue in genital rhabdomyoma is composed of a enlarging masses.
mixture of fibroblast-like cells with clusters of mature cells • They are homogeneous and nonenhancing on CT scan and
containing distinct cross-striations and a matrix containing they may cause bone remodeling.
varying amounts of collagen and mucoid material.
• Lesional tissue in rhabdomyomatous mesenchymal HISTOPATHOLOGIC FEATURES
hamartoma is composed of poorly oriented or perpendi- • Fibrosarcomas are divided into well-differentiated and
cular bundles of well-differentiated skeletal muscle with poorly differentiated subtypes based on the degree of
islands of fat, fibrous tissue and occasionally proliferating cellular uniformity, collagen production, and mitotic bodies.
nerves. • Well-differentiated or low-grade tumors have a uniform
spindle-cell appearance, eosinophilic cytoplasm, tapered
Management nuclei arranged in an interlocking fascicular or herring bone
1. Proper medical assistance is required for these pattern and substantial collagen production (Fig. 9.17).
patients as they may experience difficulties in • Poorly differentiated or high-grade lesions have greater
breathing and swallowing. In such instances, nasal cellular variability, with hyperchromatism, an increased
oxygen may help patients with breathing difficulties. number of mitotic figures, scant collagen production and a
In circumstances in which swallowing is extremely greater degree of necrosis and hemorrhage. The infantile
difficult, supplemental intravenous fluids may be variant resembles the adult variant.
administered until surgery is performed.
2. Local surgical excision is the treatment of choice most Management
of the time.
Surgical excision including a wide margin of adjacent
3. Fetal rhabdomyomas are usually located in the
normal tissue.
subcutaneous tissues. In most instances, they can be
excised from various parts of the body without much
difficulty. OSTEOSARCOMA
4. Local excision is the treatment of choice for genital
rhabdomyomas.
Osteosarcoma is an ancient disease that is still incompletely
5. Open heart surgery may be necessary for the understood. The term “sarcoma” was introduced by the English
treatment of cardiac rhabdomyomas surgeon John Abernathy in 1804 and was derived from Greek
words meaning “fleshy excrescence”. In 1805, the French
248 Essentials of Pediatric Oral Pathology

FIGURE 9.17: Histopathologic picture of well-differentiated FIGURE 9.18: Radiographic picture of osteosarcoma
fibrosarcoma showing uniform spindle-cell appearance, eosinophilic showing ‘sun-ray appearance’
cytoplasm, tapered nuclei arranged in an interlocking fascicular or
herring bone pattern

surgeon Alexis Boyer (personal surgeon to Napoleon) first used RADIOGRAPHIC FEATURES
the term “osteosarcoma”. Boyer realized that osteosarcoma is
a distinct entity from other bone lesions, such as osteo- • Osteosarcoma shows varied radiographic appearance
chondromas (exostoses).37,38 ranging from osteolytic to mixed to osteogenic pattern of
Osteosarcoma of jaws is uncommon and constitutes bone. If the tumor invades the periosteum, many thin
approximately 15 percent of all primary bone tumors confirmed irregular spicules of new bone may develop outwards and
at biopsy.39 They affect most rapidly growing parts of the perpendicular to the surface of the lesion producing the so
skeleton; metaphyseal growth plates in femur, tibia and humerus called ‘sun ray appearance’ (Fig. 9.18).
being the commonest sites. Etiology of the primary type is
unknown; it may be due to genetic influence or other HISTOPATHOLOGIC FEATURES
environmental factors. Secondary craniofacial osteogenic • Depending upon the predominant type of extracellular
sarcomas occur in patients of skeletal Paget’s disease, fibrous matrix present, osteosarcomas are categorized histopatho-
dysplasia of bone and as a late sequela to craniofacial logically into osteoblastic, chondroblastic and fibroblastic
irradiation. Majority of craniofacial osteosarcomas occur in subtypes.
skeletally mature patients in contrast to those that affect the • The osteoblastic variety consists of tumor osteoid
appendicular skeleton. surrounded by bizarrely arranged fibroblast-like cells.
• In chondroblastic osteosarcoma, tumor cells lie in the
CLINICAL FEATURES lacunae and form lobules. The center of lobule has bony
• Osteosarcoma of long bones presents as pain during activity trabeculae producing a feathery appearance, and towards
as compared to osteosarcoma of jaw bones where swelling the periphery, the tumor becomes hypercellular (Fig. 9.19).
rather than pain is the commonest finding. • Fibroblastic osteosarcoma is the least common variant where
• In a study by Forteza et al on 81 cases of osteosarcoma, the tumor cells are spindle shaped and characteristically
maxillary osteosarcomas occurred in females with the ratio arranged in herring bone pattern typically resembling
of 4:1 whereas mandibular lesions occurred only in males.39 fibrosarcoma. The formation of tumor osteoid differentiates
Few reports state even distribution of the lesion between this variant of osteosarcoma from fibrosarcoma.40
maxilla and mandible.
• May occur in both children and adults. Management
• A palpable mass may or may not be present. The mass may 1. Wide radical resection is the treatment of choice for
be tender and warm, although these signs are indistinguishable osteosarcoma of jaws. Surgery and adjuvant
from osteomyelitis. Increased skin vascularity over the mass chemotherapy and radiotherapy may be required
may be discernible. Pulsations or a bruit may be detectable. sometimes.
• Decreased range of motion may be evident. 2. The presence of micro metastases decides the need
of adjuvant therapy.
• Pathologic fractures may occur.
Connective Tissue Pathology in Children 249

FIGURE 9.19: His topathologic pic ture of c hondroblas tic FIGURE 9.20: Rhabdomyosarcoma presenting as
osteosarcoma showing areas of atypical chondroid tissue seen with a painless and infiltrative mass on left eye
large chondrocytes surrounded by osteoid

RHABDOMYOSARCOMA (FIG. 9.20) • Most commonly seen in children, teenagers and young adults.
• Two age peaks tend to be associated with different
Rhabdomyosarcoma (RMS) is the most common soft tissue
locations. Patients aged 2 to 6 years tend to have head and
sarcoma in children. The name is derived from the Greek
neck or genitourinary tract primary tumors, whereas
words rhabdo, which means rod shape, and myo, which means
adolescents aged 14 to 18 years tend to have primary
muscle. Although Weber first described rhabdomyosarcoma
tumors in extremities, truncal or paratesticular locations.
in 1854, a clear histologic definition was not available until • Embryonal variety is most common in the first decade of
1946, when Stout recognized the distinct morphology of life.
rhabdomyoblasts.41 • Alveolar type is common in 11 to 26 years of age.
The cause of rhabdomyosarcoma is unknown. Although it • Pleomorphic type occurs in adults over 40 years of age.
is hypothesized that alveolar variety occurs due to chromosomal • Most common head and neck lesions are of embryonal and
translocations, namely, t (2;13) or t (1;13). The embryonal alveolar variety.
subtype usually has a loss of heterozygosity at band 11p15.5.
• Lesion occurs as a rapidly growing, painless and infiltrative
This suggests a role of genetic alterations in formation of these
mass.
tumors. They are basically of three varieties, viz. alveolar,
• Common symptoms seen in patients are proptosis or
embryonal and pleomorphic.
dysconjugate gaze, painless scrotal mass, bladder or bowel
difficulties, menorrhagia or metrorrhagia, protruding polypoid
CLINICAL FEATURES
mass occurring in vagina also termed ‘botryoid’, meaning a
• Although the tumor is believed to arise from primitive grapelike cluster, upper respiratory symptoms or pain.
muscle cells, tumors can occur anywhere in the body except • Palate is the most frequent intraoral site.
bone. The most common sites are head and neck, • Several genetic syndromes and environmental factors are
extremities, genitourinary tract. associated with increased prevalence of rhabdomyosarcoma.
• Other notable sites include the trunk, orbit and retro- • Genetic syndromes include the following:
peritoneum. — Neurofibromatosis (4–5% risk of any one of numerous
• Rhabdomyosarcoma occurs at other sites in less than three malignancies)
percent of patients. The botryoid variant of embryonal variety — Li-Fraumeni syndrome (germline mutation of the tumor
arises in mucosal cavities, such as the bladder, vagina, suppressor gene TP53)
nasopharynx and middle ear. — Rubinstein-Taybi syndrome
• Lesions in the extremities are most likely to have an — Gorlin basal cell nevus syndrome
alveolar type of histology. — Beckwith-Wiedemann syndrome
• The male-to-female ratio is 1.2 to 1.4:1. — Costello syndrome
250 Essentials of Pediatric Oral Pathology

• Environmental factors appear to influence the development ETIOPATHOGENESIS

of rhabdomyosarcoma, as follows:
They do not arise from synovial tissue; rather, they originate
— Parental use of marijuana and cocaine
from pluripotential mesenchymal cells and rarely occur within
— Intrauterine exposure to X-rays joint spaces. A reciprocal translocation, t(X;18)(p11.2;q11.2)
— Previous exposure to alkylating agents. has been identified in monophasic and biphasic synovial
sarcoma.
HISTOPATHOLOGIC FEATURES
CLINICAL FEATURES
• Lesional tissue in the well differentiated embryonic variety
is composed of round to ovoid rhabdomyoblasts with • Incidence of occurrence in teenagers and young adults is
eosinophilic cytoplasm and fibrillar material around the most common.
nucleus. Stout described rhabdomyoblasts as appearing in • Most commonly seen in males.
round, strap, racquet and spider forms. Rhabdomyoblasts • The hypopharynx and retropharynx are the most common
sometimes have discernible muscle striations. In case of sites of involvement in the head and neck.
poorly-differentiated variety, cells are round or oval with • The most common presentation is that of a painless mass;
nuclear hyperchromatism and indistinct cytoplasm. Botyroid various associated symptoms may be present, depending
variety of this type shows myxoid stroma (Fig. 9.21). on the location of the tumor.
• Lesional tissue in alveolar variety is composed of round to • Calcifications are present in more than 50 percent of tumors
ovoid rhabdomyoblasts with lack of cohesion and atypical and may be noted on radiography.
mitosis. Numerous multinucleated giant cells are also HISTOPATHOLOGIC FEATURES
evident.
• Three subtypes of synovial sarcoma are described: biphasic,
• Lesional tissue in pleomorphic variety is composed of
monophasic, and poorly differentiated.
haphazardly arranged cluster of cells with cellular
• Biphasic synovial sarcomas are composed of epitheloid and
pleomorphism. spindle cells (Fig. 9.22).
• Usually, the spindle cell component predominates.
Management • Mast cells, mitoses, areas of calcification and scant collagen
Treatment in patients with rhabdomyosarcoma (RMS) production are typical of biphasic synovial sarcoma.
involves a combination of surgery, chemotherapy, and • The epitheloid cells form pseudoglandular cavities filled
radiation therapy. with mucin.
• Monophasic synovial sarcoma is composed of a single
cellular type and may be derived from epitheloid or spindle
SYNOVIAL SARCOMA
cells. In either of the cases, spindle cells predominate.
Synovial sarcomas represent 6 to 10 percent of all soft tissue • A rare poorly differentiated subtype has been described.
sarcomas. Only 3 to 10 percent of synovial sarcomas arise in These tumors may consist predominantly of epithelioid cells,
the head and neck. spindle cells or a small cell variant that forms rosettes.

FIGURE 9.21: Histopathologic picture of rhabdomyosarcoma showing FIGURE 9.22: Histopathologic picture of biphasic synovial
round to ovoid rhabdomyoblasts with eosinophilic cytoplasm sarcoma composed of epitheloid and spindle cells
Connective Tissue Pathology in Children 251

Management
1. Surgical excision combined with postoperative
radiation therapy is the primary treatment for synovial
sarcoma.
2. Chemotherapy with ifosfamide compounds appears
to be of benefit in the treatment of distant metastases.

ALVEOLAR SOFT PART SARCOMA

Alveolar soft part sarcoma (ASPS) is a rare tumor, accounting
for less than one percent of sarcomas. Head and neck
involvement occurs in 27 percent of cases.

ETIOPATHOGENESIS
The origin of these tumors is unclear. Some authors suggest a
neuroendocrine origin, citing evidence of myelinated axon FIGURE 9.23: Histopathologic picture of alveolar soft part sarcoma
formation within the lesion. Others support the idea of a showing grouped polygonal tumor cells with granular eosinophilic
myogenous origin for ASPS because of the presence of MyoD1, cytoplasm
myogen, and desmin in many lesions. Mutation at the 17q25
site has been reported in ASPS, although the significance of Management
this finding is unclear. 1. Surgical excision is the treatment of choice; elective
neck dissection is not indicated because of the low
CLINICAL FEATURES incidence of cervical metastases.
2. Adjuvant radiation therapy or chemotherapy has not
• Incidence of occurrence in young adults and children is
been shown to provide any improvement in disease
more common.
control or survival.
• The most common sites for alveolar soft part sarcoma in
the head and neck are the orbit and tongue. REFERENCES
• In comparison to older patients where the lesion occurs in
extremities, the lesion is seen more frequently involving 1. Stout AP. Juvenile fibromatosis. Cancer 1954;7:953-78.
the head and neck region in children. 2. Chung EB, Enzinger FM. Infantile myofibromatosis. Cancer
• Most commonly occurs in females in case of young 1981;48:1807-18.
patients. 3. Enzinger FM, Weiss SW, Eds. Fibrous tumors of infancy and
• It occurs as a slow growing painless mass. childhood. In: Soft tissue tumors. 3rd edn St. Louis: Mosby,
• Alveolar soft part sarcomas tend to be highly vascular and 1995;357-63.
a bruit may be auscultated on examination. 4. Beck JC, Devaney KO, Weatherly RA, et al. Pediatric
myofibromatosis of the head and neck. Arch Otolaryngol Head
HISTOPATHOLOGIC FEATURES Neck Surg 1999;125:39-44.
5. Shepherd SM. Alveolar exostosis. Am J Dent Sc 1844;4:43-4.
• The name alveolar soft part sarcoma is derived from its 6. Eversole LR, Rovin S. Reactive lesions of the gingival. J Oral
characteristic appearance at light microscopy, which is Pathol 1972;1:30-8.
described as groups of epitheloid tumor cells in a highly 7. Buchner A, Hansen LS. The histomorphologic spectrum of
vascular matrix. peripheral ossifying fibroma. Oral Surg Oral Med Oral Pathol
• Grouped polygonal tumor cells with granular eosinophilic 1987;63:452-61.
cytoplasm are arranged in an organoid configuration and 8. Cuisa ZE, Brannon RB. Peripheral ossifying fibroma: A clinical
separated by thin fibrovascular septa (Fig. 9.23). evaluation of 134 pediatric cases. Pediatr Dent 2001;23:245-8.
• Central areas within these nests of cells become necrotic and 9. Miller CS, Henry RG, Damm DD. Proliferative mass found in
the loss of architecture produces an alveolar appearance. the gingiva. J Am Dent Assoc 1990;121:559-60.
• Mitotic bodies are uncommon. Rhomboid and rod-shaped 10. Krompecker E. Zur Histogenese und Morphologie der
crystals are arranged in a sheaflike orientation in the cytoplasm Adamantinome und sonstiger Kiefergeschwuelste. Beitr Pathol
of 75 percent of tumors. Anat 1918;64:169-97.
252 Essentials of Pediatric Oral Pathology

11. Borello ED, Gorlin RJ. Melanotic neuroectodermal tumor of molecular genetic investigation”. J Invest Dermatol 2006;126
infancy: a neoplasm of neural crest origin. Cancer 1966;19:196- (11):2533-8.
203. 27. Mulliken JB, Boon LM, Takahashi K, et al. Pharmacologic
12. Alencer PE, Merly F, Maria D, Henriques W, et al. Cell cycle therapy for endangering hemangiomas. Curr Opin
associated protein in melanotic neuroectodermal tumor of Dermatol 1995;109-13.
infancy. J Oral Surg Pathol Radio Endo 1999;88:466-8. 28. Fost NC, Esterly NB. Successful treatment of juvenile
13. Lack EE, Worsham GF, Gallihan MD. Gingival granular cell tumors hemangiomas with prednisone. J Pediatr 1968;72(3):351-7.
of the newborn (congenital “epulis”): A clinical and pathologic 29. Pope E, Krafchik BR, Macarthur C, Stempak D, Stephens D,
study of 21 patients. Am J Surg Pathol, 1981; 5:37-46. Weinstein M, et al. Oral versus high-dose pulse corticosteroids
14. Neumann E. Ein fall vin congenitale epulis. Arch Heilkd 1871; for problematic infantile hemangiomas: A randomized,
12:189-90. controlled trial. Pediatrics (Epub) 2007;119(6):e1239-47.
15. Damm DD, Cibull ML, Geissler RH, et al. Investigation into 30. Greinwald JH, Burke DK, Bonthius DJ, Bauman NM, Smith
histogenesis of congenital epulis of the newborn. Oral Surg Oral RJ. An update on the treatment of hemangiomas in children with
Med Oral Pathol 1993;76:205-12. interferon alfa-2a. Arch Otolaryngol Head Neck Surg 1999;
16. Lack EE, Perez-Atayde AR, McGill TJ, Vawter GF. Gingival 125(1):21-7.
granular cell tumor of the newborn (congenital “epulis”): 31. Glassberg E, Lask G, Rabinowitz LG, Tunnessen WW
ultrastructural observations relating to histogenesis. Hum Pathol Jr. Capillary hemangiomas: Case study of a novel laser treatment
1982;13:686-9. and a review of therapeutic options. J Dermatol Surg
17. Tucker MC, Rusnock EJ, Azumi N, Hoy GR, Lack EE. Gingival Oncol 1989;15(11):1214-23.
granular cell tumors of the newborn: an ultrastructural and 32. Apfelberg DB. Intralesional laser photocoagulation-steroids as an
immunohistochemical study. Arch Pathol Lab Med 1990;114: adjunct to surgery for massive hemangiomas and vascular
895-8. malformations. Ann Plast Surg 1995;35(2):144-8; discussion 149.
18. Fuhr AH, Krogh PHJ. Congenital epulis of the newborn: 33. Sabin FR. The lymphatic system in human embryos, with a
centennial view of the literature. J Oral Surg 1972;30:30-35. consideration of the morphology of the system as a whole. Am
19. Mulliken JB, Glowacki J. Hemangiomas and vascular J Anat 1909;9:43-91.
malformations in infants and children: A classification based 34. Sidle DM, Maddalozzo J, Meier JD, et al. Altered pigment
on endothelial characteristics. Plast Reconstr Surg 1982;69(3): epithelium-derived factor and vascular endothelial growth factor
412-22. levels in lymphangioma pathogenesis and clinical recur-
20. Watson WL, McCarthy WD. Blood and lymph vessel tumors: rence. Arch Otolaryngol Head Neck Surg 2005;131(11): 990-5.
a report of 1,056 cases. Surg Gynecol Obstet 1940;71:569. 35. Landing BH, Farber S. Tumors of the cardiovascular system. In:
21. Takahashi K, Mulliken JB, Kozakewich HP, et al. Cellular Atlas of Tumor Pathology. Washington, DC: Armed Forces
markers that distinguish the phases of hemangioma during Institute of Pathology; 1956;124-38.
infancy and childhood. J Clin Invest 1994;93(6):2357-64. 36. Orvidas LJ, Kasperbauer JL. Pediatric lymphangiomas of the
22. Ritter MR, Reinisch J, Friedlander SF, Friedlander M. Myeloid head and neck. Ann Otol Rhinol Laryngol 2000;109(4):411-21.
cells in infantile hemangioma. Am J Pathol 2006;168(2): 37. Peltier LF. Tumors of bone and soft tissues. Orthopedics: A
621-8. history and iconography. San Francisco, Calif: Norman
23. Kleinman ME, Greives MR, Churgin SS, et al. “Hypoxia- Publishing; 1993;264-91.
induced mediators of stem/progenitor cell trafficking are 38. Rutkow IM. The nineteenth century. Surgery: An illusrated
increased in children with hemangioma”. Arterioscler Thromb history. St Louis, Mo: Mosby-Year Book; 1993;321-504.
Vasc Biol 2007;27(12):2664-70. 39. Forteza G, Colmenero B, Lopez-Barea F. Osteogenic sarcoma
24. Barnés CM, Huang S, Kaipainen A, et al. “Evidence by of maxilla and mandible. Oral Surg Oral Med Oral Pathol 1986;
molecular profiling for a placental origin of infantile 62:179-84.
hemangioma”. Proc Natl Acad Sci, USA 2005;102(52):19097- 40. Neville BW, Damm DD, Allen CM, Bouquot JE. Bone
102. pathology. In: Neville BW, Damm DD, Allen CM, Bouquot JE.
25. North PE, Waner M, Brodsky MC. “Are infantile hemangiomas Oral and maxillofacial Pathology. Philadelphia: Saunders, an
of placental origin?” Ophthalmology 2002;109(4):633-4. imprint of Elsevier; 2002. p. 533-87.
26. Pittman KM, Losken HW, Kleinman ME, et al. “No evidence 41. Stout AP. Rhabdomyosarcoma of the skeletal muscles. Ann
for maternal-fetal microchimerism in infantile hemangioma: a Surg 1946;123:447-72.
10

Bone Pathology
in Children
Mayur Chaudhary, Shweta Dixit Chaudhary, Manasi Dixit

CHAPTER OVERVIEW
Introduction Pierre Robbin syndrome
Osteogenesis imperfecta (OI) Apert syndrome
Osteopetrosis Thanatophoric dysplasia
Cleidocranial dysplasia Achondroplasia
Cherubism Robinow syndrome
Fibrous dysplasia Hyperostosis corticalis generalisata
Chondromyxoid fibroma (CMF) Chondroectodermal dysplasia
Familial gigantiform cementoma Trichodento-osseous syndrome
Juvenile ossifying fibroma (JOF) Down’s syndrome
Marfan syndrome Infantile cortical hyperostosis
Achondrogenesis Massive osteolysis
Chondrodysplasia punctata
Cementoblastoma
Pycnodysostosis
TMJ abnormalities:
Mucopolysaccharidosis
Aplasia of mandibular condyle
Rickets
Hyperparathyroidism Hypoplasia of mandibular condyle
Hypoparathyroidism Hyperplasia of mandibular condyle
Craniosynostosis syndromes Ankylosis
Craniofacial dysostosis – Crouzon syndrome Langerhans cell histiocytosis
Mandibulofacial dysostosis – Treacher collins syndrome Hand-Schüller-Christian disease
Franceschetti syndrome Eosinophilic granuloma

INTRODUCTION from mutation on genes COL1A1 on chromosome 17 and

COL1A2 on chromosome 7 that guide formation of collagen
Bone is a specialized connective tissue and consists of cells,
type 1. Type I collagen fibers are found in the bones, organ
fibers and extracellular matrix. This matrix gets ossified and
forms hard structure i.e. bone. Bone formation takes place by capsules, fascia, cornea, sclera, tendons, meninges, and dermis.
endochondral and intramembranous ossification. Its main Type I collagen, which constitutes approximately 30 percent
functions are protection of internal structures, hemopoiesis and of the human body by weight, is the defective protein in OI.
it also serves as a reservoir for calcium, phosphate and other This results in bone with a thin cortex, fine trabeculation and
minerals. Any impairment in the structure and function of bone diffuse osteoporosis.
which may be due to one or other cause leads to pathological
conditions of bone. This chapter focuses on some of the bone CLINICAL FEATURES
pathologies pertaining to children. • Affects 1 in 20,000 individuals.
• Occurs as autosomal dominant and recessive hereditary
OSTEOGENESIS IMPERFECTA patterns.
Osteogenesis imperfecta (OI) comprises of hereditary disorders • The age when symptoms (i.e. fractures) begin widely varies.
characterized by impairment of collagen maturation and arises Patients with mild forms may not have fractures until
254 Essentials of Pediatric Oral Pathology

TABLE 10.1: Conditions in which blue sclera is seen

Blue sclera may also be seen in other conditions like:

• Progeria
• Cleidocranial dysplasia
• Menkes syndrome
• Cutis laxa
• Cheney syndrome
• Pyknodysostosis.

adulthood or patients may present with fractures in infancy.

Patients with severe forms present with fractures in utero
also.
• Common symptoms of blue sclera, altered teeth, hearing
FIGURE 10.1: Osteogenesis imperfecta showing blue sclera
loss, and long bone and spine deformities are seen in
affected individuals.
• Patients most commonly present with fractures after minor Type III—Severe
trauma. • Patients may have joint hyperlaxity, muscle weakness,
• Patients may bruise easily. chronic unremitting bone pain, and skull deformities (e.g.
• Other conditions in which blue sclera is seen have been
posterior flattening) due to bone fragility during infancy.
mentioned in Table 10.1.
• Deformities of upper limbs may compromise function and
• Four major types of osteogenesis imperfecta have been
mobility.
reported:
• The presence of dentinogenesis imperfecta is independent
of the severity of OI.
Type I OI— Mild Forms
• The sclera has variable hues.
• Patients have no long-bone deformity. • In utero fractures are common.
• The sclera can be blue or white. • Limb shortening and progressive deformities can occur.
• Dentinogenesis imperfecta may be present. • Patients may have a triangular face with frontal bossing.
• Over a lifetime, the number of fractures can range from • Basilar invagination is an uncommon but potentially fatal
1 to 60. occurrence in OI.
• Height is usually normal in individuals with mild forms of OI. • Vertigo is common in patients with severe OI.
• People with OI have a high tolerance for pain. Old fractures • The incidence of congenital malformations of the heart in
can be discovered in infants when radiographs are obtained children with OI is probably similar to that of the healthy
for reasons other than an assessment of OI, and they can population.
occur without any signs of pain.
• Hypercalciuria may be present in about 36 percent of
• Exercise tolerance and muscle strength are significantly
patients with OI but does not appear to affect renal function.
reduced in patients with OI, even in the mild forms.
• Respiratory complications secondary to kyphoscoliosis are
• Fractures are most common during infancy but may occur
common in individuals with severe OI.
at any age.
• Constipation and hernias are also common in people with
• Other possible findings include kyphoscoliosis, hearing
loss, premature arcus senilis, and easy bruising. OI.

Type II—Extremely Severe Type IV—Undefined

• Type II is often lethal. • This type of OI is not clearly defined.
• Blue sclera may be present (Fig. 10.1). • Whether patient has normal height or whether scleral hue
• Patients may have a small nose, micrognathia, or both. defines the type has not been established in consensus.
• All patients have in utero fractures, which may involve the • Dentinogenesis imperfecta may be present. Some have
skull, long bones, and/or vertebrae. suggested that this sign can be used to divide type IV OI
• The ribs are beaded and the long bones are severely deformed. into subtypes a and b.
• Causes of death include extreme fragility of the ribs, • Fractures usually begin in infancy, but in utero fractures
pulmonary hypoplasia, and malformations or hemorrhages may occur. The long bones are usually bowed (Figs 10.2
of the CNS. and 10.3).
Bone Pathology in Children 255

Management
1. Since osteogenesis imperfecta (OI) is a genetic
condition, it has no cure.
2. Cyclic administration of intravenous pamidronate
reduces the incidence of fracture and increases bone
mineral density, while reducing pain and increasing
energy levels.1 Doses vary from 4.5 to 9 mg/kg/y,
depending on the protocol used.
3. Current evidence does not support the use of oral
bisphosphonates in patients with OI.
4. Nutritional evaluation and intervention are paramount
to ensure appropriate intake of calcium and vitamin
D. Caloric management is important, particularly in
adolescents and adults with severe forms of OI.
5. Orthopedic surgery is one of the pillars of treatment
for patients with OI. Surgical interventions include
FIGURE 10.2: Osteogenesis imperfecta showing bowing of leg intramedullary rod placement, surgery to manage
basilar impression, and correction of scoliosis.
6. Surgery for basilar impression is reserved for cases
with neurologic deficiencies, especially those caused
by compression of brainstem and high cervical cord.
A team of orthopedic surgeons and neurosurgeons
is required.
7. Correction of scoliosis may be difficult because of
bone fragility. Spinal fusion may be helpful.
Pretreatment with pamidronate appears to improve
the surgical outcome.

OSTEOPETROSIS
Osteopetrosis was first described by a German radiologist,
Albers-Schönberg in 1904. 2 It is a clinical syndrome
characterized by the failure of osteoclasts to resorb bone.
Osteoclasts are derived from the monocyte/macrophage
lineage. Osteoclasts can tightly attach to the bone matrix by
integrin receptors to form a sealing zone, within which a
sequestered compartment is acidified. Acidification promotes
solubilization of the bone mineral in the sealing zone, and
FIGURE 10.3: Osteogenesis imperfecta showing bowing of
legs due to formation of immature bone
various proteases, notably cathepsin K, catalyze degradation
of the matrix proteins.
Bone modeling and remodeling differ in that, modeling
RADIOGRAPHIC FEATURES implies a change in the shape of the overall bone and is
prominent during childhood and adolescence. Modeling is
• Deformity and multiple fractures of long bones along with
the process by which the marrow cavity expands as the bone
the formation of wormian bones (immature bones arranged
grows in length and diameter. Failure of modeling is the
haphazardly) in the skull.
basis of hematopoietic failure in osteopetrosis. Remodeling,
• Premature pulpal obliteration is seen in both primary and
in contrast, involves the degradation of bone tissue from a
permanent dentitions.
preexisting bony structure and replacement of the degraded
• On occasion, may show mixed radiolucencies.
bone by newly synthesized bone. Failure of remodeling is
the basis of the persistence of primary spongiosa and woven
HISTOPATHOLOGIC FEATURES
bone.
• Reduction in bone matrix production The defect in bone turnover characteristically results in
• Failure of woven bone to become lamellar bone skeletal fragility despite increased bone mass, and it may also
256 Essentials of Pediatric Oral Pathology

TABLE 10.2: Clinical classification of human osteopetrosis

Characteristic Adult onset Infantile Intermediate

Inheritance Autosomal dominant Autosomal recessive Autosomal recessive

Bone marrow failure None Severe None

Prognosis Good Poor Poor

Diagnosis Often diagnosed Usually diagnosed Not applicable

incidentally before age 1 year

cause hematopoietic insufficiency, disturbed tooth eruption,

nerve entrapment syndromes and growth impairment.

CLINICAL FEATURES
• Three variants of the disease are diagnosed in infancy,
childhood (intermediate), or adulthood (Table 10.2).
• Infantile osteopetrosis (also called malignant osteopetrosis)
is diagnosed early in life (Fig. 10.4).
• Failure to thrive and growth retardation are symptoms.
• Bony defects occur. Nasal stuffiness due to mastoid and
paranasal sinus malformation is often the presenting feature
of infantile osteopetrosis. Neuropathies related to cranial
nerve entrapment occur due to failure of the foramina in the
skull to widen completely. Manifestations include deafness,
proptosis, and hydrocephalus. Dentition might be delayed
(Fig. 10.5). Osteomyelitis of the mandible is common due
to an abnormal blood supply. Bones are fragile and can
fracture easily.
• Defective osseous tissue tends to replace bone marrow,
which can cause bone marrow failure with resultant FIGURE 10.4: Infantile osteopetrosis
pancytopenia. Patients might have anemia, easy bruising
and bleeding (due to thrombocytopenia), and recurrent
infections (due to inherent defects in the immune system).
Extramedullary hematopoiesis might occur with resultant
hepatosplenomegaly, hypersplenism, and hemolysis.
• Other manifestations include sleep apnea and blindness due
to retinal degeneration.
• Adult osteopetrosis (also called benign osteopetrosis) is
diagnosed in late adolescence or adulthood.
• Two distinct types have been described, type I and type II,
on the basis of radiographic, biochemical, and clinical
features.3 (Table 10.3)
• Recent work has demonstrated that the clinical syndrome
of adult type I osteopetrosis is not true osteopetrosis, but
rather, increased bone mass due to activating mutations of
LRP5.4
• Some cases of type II osteopetrosis result from mutations FIGURE 10.5: Infantile osteopetrosis showing
of CLCN7, the type 7 chloride channel.5 absence of teeth due to delayed eruption
Bone Pathology in Children 257

TABLE 10.3: Characteristic differences between Type I and 3. Treatment with gamma interferon has produced long-
Type II osteopetrosis term benefits. It improves WBC function, tremen-
Characteristic Type I Type II dously decreasing the incidence of new infections.
With treatment, trabecular bone volume substantially
Skull sclerosis Marked sclerosis Sclerosis mainly of decreases, and bone-marrow volume increases. This
mainly of the vault the base effects increase in hemoglobin, platelet counts, and
Spine Does not show much Shows the “rugger- survival rates. Combination therapy with calcitriol is
sclerosis jersey appearance” clearly superior to calcitriol alone.
4. Erythropoietin can be used to correct anemia.
Pelvis No endobones Shows endobones 5. Corticosteroids have been used to stimulate bone
in the pelvis resorption and treat anemia. Prednisone 1–2 mg/kg/
Transverse banding Absent May or may not be day is usually administered for months to years.
of metaphysis present Steroids are not the preferred treatment option.
6. Adult osteopetrosis requires no treatment by itself,
Risk of fracture Low High though complications of the disease might require
Serum acid Normal Very high
intervention. No specific medical treatment exists for
phosphatase the adult type.
7. Bone marrow transplant (BMT) markedly improves
• Many patients have bone pains. Bony defects are common some cases of infantile osteopetrosis.
and include neuropathies due to cranial nerve entrapment 8. In pediatric osteopetrosis, surgical treatment is
(e.g.: with deafness, with facial palsy), carpal tunnel sometimes necessary because of fractures.
9. In adult osteopetrosis, surgical treatment may be
syndrome, and osteoarthritis. Bones are fragile and might
needed for aesthetic reasons (e.g. in patients with
fracture easily. Approximately 40 percent of patients have notable facial deformity) or for functional reasons (e.g.
recurrent fractures. Osteomyelitis of the mandible occurs in patients with multiple fractures, deformity, and loss
in 10 percent of patients. of function). Severe, related degenerative joint
• Bone marrow function is not compromised. disease may warrant surgical intervention as well.
• Other manifestations include visual impairment due to
retinal degeneration and psychomotor retardation. CLEIDOCRANIAL DYSPLASIA

RADIOGRAPHIC FEATURES It is basically a disorder that primarily affects the development

of the bones and teeth. It is caused by a defect in CBFA 1 gene
Patients generally show osteosclerosis. Sclerotic areas may be of chromosome 6p21 that normally guides osteoblastic
noted at the ends of long bones (Figs 10.6A and B). Thickening differentiation and appropriate bone formation. It usually
of skull at the base is seen. Due to brittleness of bones, affects membranous bone (clavicle, skull and flat bones).
sometimes fractures are noted; density of jaw may be reduced Recently it has been shown to affect endochondral bone. It
such that the roots of the teeth are visible. shows autosomal dominant pattern.
HISTOPATHOLOGIC FEATURES
• Lesional area is comprised of irregular, lamellar trabeculae
replacing the cancellous portion of the bone.
• Globular amorphous bone deposition in the marrow spaces.
• Osteophytic bone formation.

Management
1. Infantile osteopetrosis warrants treatment because of
the adverse outcome associated with the disease.
2. Vitamin D (calcitriol) appears to help by stimulating
dormant osteoclasts and thus stimulate bone
resorption. Large doses of calcitriol, along with
restricted calcium intake, sometimes improve
osteopetrosis dramatically.6 It usually produces only A B
modest clinical improvement, which is not sustained
after therapy is discontinued. FIGURES 10.6 A and B: Infantile osteopetrosis showing
sclerosis at the end of long bones
258 Essentials of Pediatric Oral Pathology

CLINICAL FEATURES
• Patients suffering from this disorder show short, tapered
fingers and broad thumbs.
• Short forearms, flat feet, knock knees.
• An abnormal curvature of the spine (scoliosis).
• A wide, short skull (brachycephaly) and a prominent
forehead.
• Wide-set eyes (hypertelorism).
• A flat nose and a small upper jaw.
• Delayed loss of the primary teeth.
• Delayed appearance of the permanent (adult) teeth. FIGURE 10.7: Cleidocranial dysplasia showing numerous
• Unusually shaped, peg-like teeth. unerupted and supernumerary teeth
• Misalignment of the teeth and jaws (malocclusion).
• Supernumerary teeth, sometimes accompanied by cysts in
the gingiva (Fig. 10.7).
• Hearing loss, and sinus and ear infections.
• Some young children with this condition are mildly delayed
in the development of motor skills such as crawling and
walking, but intelligence is unaffected.
• Hypermobility of shoulders is seen in some children
(Fig. 10.8).

RADIOGRAPHIC FEATURES
• Skull radiographs show delayed closure of sutures and
fontanelles may remain open throughout patient’s life.
• Mandible shows area of increased density, narrow
ascending rami and slender pointed coronoid process.
• Maxilla shows thin zygomatic arch and small or absent
maxillary sinuses.
• Chest radiographs may show absence of clavicles (Fig. 10.9).
FIGURE 10.8: Cleidocranial dysplasia showing
hypermobility of shoulder
HISTOPATHOLOGIC FEATURES
Microscopic examination of unerupted permanent teeth reveals
lack of secondary cementum.

Management
1. One or more of the treatment modalities may be
followed depending upon the extent of malformation.
2. Extensive orthodontic treatment for functional
alignment of the teeth.
3. Full mouth extractions followed by placement of
dentures.
4. Autotransplant of selected impacted teeth followed by
prosthetic restoration.
5. Removal of primary and supernumerary teeth
followed by exposure of permanent teeth.

CENTRAL GIANT CELL GRANULOMA

Reactive condition thought to be due to organization of slow, FIGURE 10.9: Cleidocranial dysplasia showing complete
minute, recurrent hemorrhages, characterized by osteoclast-like absence of clavicles
Bone Pathology in Children 259

multinucleated giant cells with vascular stroma and new bone

formation. The World Health Organization has defined central
giant cell granuloma as an intraosseous lesion consisting of
cellular fibrous tissue that contains multiple foci of hemorrhage,
aggregations of multinucleated giant cells and occasionally
trabeculae of woven bone.7

CLINICAL FEATURES
• Incidence of occurrence at 2 to 70 years is most common.
• Occurs most commonly in females.
• Most commonly seen in the anterior region of mandible,
most often crossing the midline.
• They are classified on the basis of radiographic and clinical
features as:
— Nonaggressive lesions: Slow growing, asymptomatic and
do not show cortical perforation or root resorption of FIGURE 10.10: Central giant cell granuloma showing large
associated teeth.8 unilocular radiolucent area in posterior mandible
— Aggressive lesions: Found in young patients and
characterized by rapid growth, pain, cortical perforation
and root resorption and marked tendency to recur.9

RADIOGRAPHIC FEATURES
Lesion appears as a well demarcated, unilocular or multi-
locular radiolucent defect (Fig. 10.10).

HISTOPATHOLOGIC FEATURES
• Lesional tissue shows proliferating endothelial cells,
numerous small capillaries, multinucleated giant cells and
active fibroblasts and myofibroblasts embedded in fibrous
stroma (Fig. 10.11).
• Few studies suggest that multinucleated giant cells represent
osteoclasts. FIGURE 10.11: Histopathologic picture of central giant cell granu-
• Areas of hemorrhage are evident. loma showing multinucleated giant cells and active fibroblasts
• Younger lesions mature over a period of time and become
fibrous.
“cherubism”, to describe the round appearance of the cheeks,
Management
typical of cherubs, resulting from jaw hypertrophy.10 Mutations
in the SH3BP2 gene have been identified in about 80 percent
1. Thorough curettage. of people with cherubism. The gene responsible for cherubism
2. Aggressive tumors are treated by either administration was recently mapped to the chromosome 4p16.3.
of corticosteroids, calcitonin and interferon alpha-2a.
3. Injections of triamcinolone acetonide for six weeks into
CLINICAL FEATURES
the tumor have been used successfully.
• Cherubism is a disease of childhood that usually presents
before the age of five, most often between 12 and 36 months.
CHERUBISM
• Males are affected more commonly than females.
Cherubism is a benign disease with a characteristic symmetrical • Wide rim of exposed sclera is noted below the iris, therefore
involvement of the maxilla and mandible. It was first described called ‘eye to heaven’ appearance (Figs 10.12A and B).
by Jones in 1933 as a “familial multilocular disease of the jaws” • Mandibular lesions appear as painless, bilateral expansion
in three siblings who appeared as though they were “looking of the posterior mandible that tends to involve angles and
towards heaven”. This inspired him to call the condition ascending rami.
260 Essentials of Pediatric Oral Pathology

B
B FIGURES 10.13A and B: Cherubism showing bilateral
involvement of mandible
FIGURES 10.12A and B: Cherubism showing eye to
heaven appearance

• Maxillary involvement is rare but if it occurs, it mainly

affects the tuberosity area.
• Displacement or failure of eruption of tooth, speech
difficulty, loss of normal vision or hearing.

RADIOGRAPHIC FEATURES
• Unilocular or multilocular radiolucencies of the jaw.
• Most of the cases show bilateral radiolucencies (Figs
10.13A and B).
CT scans showing expansile remodeling, cortical thinning,
and multilocular contour with coarse trabecular pattern.
Maxillary disease resulting in dental derangement is also seen.

HISTOPATHOLOGIC FEATURES
• Lesional tissue shows vascular fibrous connective tissue FIGURE 10.14: Histopathologic picture of cherubism showing
with variable number of multinucleated giant cells and cellular fibrous mass with interspersed multinucleated giant cells
spindle shaped cells (Fig. 10.14).
• Eosinophilic, cufflike deposits surrounding small blood • Older lesions appear more fibrous and there is a decrease
vessels are the characteristic features of this lesion. in the number of giant cells.
Bone Pathology in Children 261

Management
1. Early surgical intervention with curettage of the
lesions. But rapid regrowth of the lesions is a potential
complication.
2. Use of calcitonin in severe cases has been suggested.
3. Radiotherapy is contraindicated because of risk of
development of post irradiation sarcoma.

FIBROUS DYSPLASIA
The term fibrous dysplasia was first mentioned by Lichtenstein
in 1938.11 It is a rare localised disease often associated with bony
deformities caused by the abnormal proliferation of fibrous tissue
interspersed with normal or immature bone because of poorly
differentiated, mutated osteoblasts. Some authors suggest that
greater resorption of bone in affected areas is because of the
activation of Gs 1 and increased synthesis of IL6, a cytokine FIGURE 10.15: Craniofacial fibrous dysplasia
showing distortion of the face
involved in the differentiation of osteoclasts.12
Two types of fibrous dysplasia are as follows:
1. Monostotic: When only one bone is involved.
2. Polyostotic: When multiple bones are involved. Occurs
along with cutaneous and endocrine abnormalities.

MONOSTOTIC FIBROUS DYSPLASIA

• Occurs in childhood.
• Higher incidence of occurrence in males.
• Occurrence in maxilla is more common than mandible.
• There occurs painless bulging of the jaw.
• Tipping or displacement of teeth is evident.
• When occurs in maxilla along with other bones, it is termed
as ‘craniofacial fibrous dysplasia’. This form is most
common in children (Fig. 10.15).
FIGURE 10.16: Craniofacial fibrous dysplasia showing ground
glass appearance of the bony trabeculae
RADIOGRAPHIC FEATURES (FIG. 10.16)
Ground glass appearance due to collection of poorly calcified • The bony trabeculae often assume curvilinear shape termed
bony trabeculae arranged in disorganized pattern. as “Chinese letter pattern” (Fig. 10.17).
• These trabeculae are lined by osteoblasts.
POLYOSTOTIC FIBROUS DYSPLASIA
Management
• It is usually less common.
• Mostly occurs unilaterally. 1. Resection cures fibrous dysplasia in bones such as
• When combined with cutaneous abnormalities (café au ribs.
lait=coffee with milk) pigmentation—termed as Jaffe 2. Curettage is adequate in long bones such as tibia.
3. Partial removal in maxilla which may resolve some
Lichtenstein syndrome.
of the deformity.
• Polyostotic fibrous dysplasia, when occurs alongwith café
4. Pamidronate 60 mg/day given intravenously on three
au lait spots and endocrine abnormalities, is termed as successive days to reduce osteoclastic activity has
McCune-Albright syndrome. been given every 6 months for 18 months. It resulted
in a decreased intensity of bony pain, reduced bony
HISTOPATHOLOGIC FEATURES resorption, and improved radiological features such as
filling of lytic lesions in about half the patients. But such
• Lesional tissue shows irregular shaped trabeculae of
treatment modality had not been tried yet in children.13
immature bone in cellular, loosely arranged fibrous stroma.
262 Essentials of Pediatric Oral Pathology

FIGURE 10.17: Histopathologic picture of craniofacial fibrous FIGURE 10.18: Chondromyxoid fibroma showing well
dysplasia showing “Chinese letter pattern” circumscribed radiolucent lesion

CHONDROMYXOID FIBROMA
Chondromyxoid fibroma (CMF) is a rare, slow-growing bone
tumor of chondroblastic derivation. Jaffe and Lichtenstein first
described the condition in 1943.14 In a study of four patients
with CMF, Granter and colleagues found that all of the subjects
had a clonal rearrangement of chromosome 6. Each of these
rearrangements involved band 6q13, which has not been
associated with other bone tumors.15
CLINICAL FEATURES
• Males and females are affected equally.
• Incidence of occurrence is common in the age range of
3 to 87 years.
• Pain and swelling are common symptoms.
• The proximal tibia is the most common location, followed
by the distal femur, pelvis, and foot. Long bones are
FIGURE 10.19: Histopathologic picture of chondromyxoid fibroma
involved much more frequently than are other bones,
showing vague lobularity caused by alternating highly cellular and
especially in younger patients.16 less cellular areas
• Rarely is an involvement of the jaw seen.
RADIOGRAPHIC FEATURES (FIG. 10.18)
Well circumscribed radiolucent lesion with scalloped margins
is seen. Sometimes there is presence of radiopacities within
the lesion.
HISTOPATHOLOGIC FEATURES
(FIGS 10.19 AND 10.20)
• Lesional tissue shows lobules of spindle shaped cells
surrounded by myxoid stroma.
• Sometimes multi-nucleated giant cells are visible within the
stroma.
• Low power view shows a moderately cellular chondro-
myxoid tissue with the following two characteristic features:
1. Vague lobularity caused by alternating highly cellular FIGURE 10.20: Histopathologic picture of chondromyxoid fibroma
and less cellular areas; showing mildly pleomorphic, angular and stellate cells set in bluish-
2. Increased cellularity at the periphery of the lobules. pink chondromyxoid stroma
Bone Pathology in Children 263

Higher magnification view of the lobule shows mildly JUVENILE OSSIFYING FIBROMA
pleomorphic, angular and stellate cells set in bluish-pink
chondromyxoid stroma. Note that the tumor lacks true hyaline Juvenile ossifying fibroma (JOF) is a rare fibro-osseous
cartilage matrix seen in chondromas and chondrosarcomas. neoplasm that arises within the craniofacial bones in individuals
Another important feature is lack of mitotic activity. under 15 years of age.

Management
CLINICAL FEATURES

1. Nonsteroidal anti-inflammatory agents or analgesics • They occur in two forms, viz. trabecular and psammoma-
may be beneficial for pain control. toid, as seen radiographically.
2. CMFs are treated with intralesional curettage or en • JOF is often seen in a very young child. In reviews
bloc excision.17 published by Hamner et al20 and Slootweg et al21, the mean
age of onset was 11.5 and 11.8 years old respectively, for
the trabecular variety. The psammomatoid variety occurs
FAMILIAL GIGANTIFORM CEMENTOMA at almost twice the age that of the trabecular variety.
Gigantiform cementoma (GC) was first reported in 1930 by • Lesion shows no sex predilection.
Norberg to describe a condition characterized by diffuse • The first clinical manifestation is a swelling of the maxilla.
radiopaque masses scattered throughout the jaws.18 These • When the orbital bone and paranasal sinuses are involved
masses frequently caused expansion. In 1953, Agazzi and as seen most of the times in psammomatoid variety, the
Belloni described an Italian family in which several members patients may develop exophthalmos, bulbar displacement
were affected, and this was designated Familial gigantiform and nasal obstruction.
cementoma (FGC) or familial multiple cementomas.19
RADIOGRAPHIC FEATURES
CLINICAL FEATURES
• The radiographic features are variable and depend on the
• It occurs as an autosomal dominant disorder. tumor’s location and the amount of calcified tissue
• Shows no sex predilection. produced by the tumor.
• Incidence of occurrence is common during first decade of • Thus the lesion will show varying degrees of radiolucency.
life.
• Both maxilla and mandible are equally involved. HISTOPATHOLOGIC FEATURES
• It occurs as an expansile lesion which results in facial
• Lesional tissue is basically composed of loose fibrous
deformity and malocclusion.
connective tissue stroma interspersed with numerous bony
trabeculae.
RADIOGRAPHIC FEATURES • Trabecular variety shows trabeculae of immature bone
surrounded by fibrocellular connective tissue stroma.
Radiographic features are variable from radiolucent to mixed Trabeculae are lined by osteoblasts and osteoclasts and
features to radiopaque as the lesion matures. contain osteocytes.
• Psammomatoid variety shows numerous spherical ossicles
HISTOPATHOLOGIC FEATURES with basophilic centers and peripheral eosinophilic rims
surrounded by fibrocellular connective tissue stroma
• Lesional tissue is composed of mature connective tissue
(Fig. 10.21).
stroma showing fibroblasts and collagen fibers.
• These components surround bony trabeculae and mostly
Management
cementum-like material.
• Numerous blood vessels are also evident. 1. Complete local excision.
• As the lesion matures, connective tissue component 2. Thorough curettage.
3. Wide local excision for rapidly growing tumors.
decreases and cemento-osseous component dominates.

Management MARFAN SYNDROME (FIGS 10.22A AND B)

• Wide surgical resection of the lesional area and Marfan syndrome is a heritable condition that affects the
reconstruction of facial skeleton and associated connective tissue. The primary purpose of connective tissue is
structures has been recommended. to hold the body together and provide a framework for growth
264 Essentials of Pediatric Oral Pathology

parents have only a one in 10,000 chance of having a child

with Marfan syndrome. Possibly 25 percent of cases are due
to a spontaneous mutation at the time of conception.

CLINICAL FEATURES
Some people affected with Marfan syndrome have only mild
symptoms, while others are more severely affected. In most
cases, the symptoms progress as the person ages. The body
systems most often affected by Marfan syndrome are:
• Oral manifestations—Baden and Spirgi, 1965, reviewed
oral manifestations of Marfan syndrome and reported that
a high arched palatal vault was the most common finding.
Other oral manifestations are bifid uvula, malocclusion,
multiple odontogenic cysts of maxilla and mandible and
FIGURE 10.21: Histopathologic picture of juvenile ossifying temporomandibular joint dysarthrosis.22
fibroma showing spherical ossicles with basophilic centers • Skeleton—People with Marfan syndrome are typically very
tall, slender, and loose jointed. Since Marfan syndrome
affects the long bones of the skeleton, arms, legs, fingers,
and toes may be disproportionately long in relation to the
rest of the body. A person with Marfan syndrome often has
a long, narrow face, and the roof of the mouth may be
arched, causing the teeth to be crowded. Other skeletal
abnormalities include a sternum (breastbone) that is either
protruding or indented, curvature of the spine (scoliosis),
and flat feet.
• Eyes—More than half of all people with Marfan syndrome
experience dislocation of one or both lenses of the eye. The
lens may be slightly higher or lower than normal and may
be shifted off to one side. The dislocation may be minimal,
or it may be pronounced and obvious. Retinal detachment
is a possible serious complication of this disorder. Many
people with Marfan syndrome are also myopic, and some
can develop early glaucoma or cataracts.
• Cardiovascular system—Most people with Marfan
FIGURES 10.22A and B: Marfan syndrome showing arachnodactyly syndrome have abnormalities associated with the heart and
(a) positive thumb sign: entire thumbnail protrudes beyond ulnar
border of hand. (b) Positive wrist sign: thumb and fifth finger overlap
blood vessels. Because of faulty connective tissue, the wall
when encircling the wrist of the aorta may be weakened and stretched, a process
called aortic dilatation. Aortic dilatation increases the risk
and development. Because connective tissue is found of aortic dissection or rupture, causing serious heart
throughout the body, Marfan syndrome can affect many body problems or sometimes sudden death. Sometimes, defects
systems, including the skeleton, eyes, heart and blood vessels, in heart valves can also cause problems creating ‘murmurs’.
nervous system, skin, and lungs. Small leaks may not result in any symptoms, but larger ones
Marfan syndrome is caused by a mutation in the gene FBN1 may cause shortness of breath, fatigue, and palpitations.
on chromosome 15, bands q15 to q23, that determines the • Nervous system—The brain and spinal cord are
structure of fibrillin, a protein that is an important part of surrounded by fluid contained in the dura, which is
connective tissue. A person with Marfan syndrome is born with composed of connective tissue. As people with Marfan
the disorder, even though it may not be diagnosed until later syndrome get older, the dura often weakens and stretches,
in life. Although everyone with Marfan syndrome has a defect then begins to weigh on the vertebrae in the lower spine
in the same gene, variable expression is seen, meaning that the and wear away the bone surrounding the spinal cord. This
defective gene expresses itself in different ways in different is called dural ectasia. These changes may cause only mild
people. The child of a person who has Marfan syndrome has a discomfort or may lead to radiating pain in the abdomen
50 percent chance of inheriting the disease, but two unaffected or pain, numbness, or weakness of the legs.
Bone Pathology in Children 265

• Skin—Many people with Marfan syndrome develop stretch ACHONDROGENESIS (FIG. 10.23)
marks on their skin, even without any weight change. These
stretch marks can occur at any age and pose no health risk. Marco Fraccardo first described achondrogenesis in 1952.23
However, people with Marfan syndrome are also at increased Achondrogenesis is a group of severe disorders that affect
risk for developing an abdominal or inguinal hernia. cartilage and bone development. These conditions are
• Lungs—Although connective tissue abnormalities make the characterized by a small body, short limbs, and other skeletal
pulmonary alveoli less elastic, people with Marfan syndrome abnormalities. As a result of serious health problems, infants
generally do not experience noticeable problems with their with achondrogenesis usually die before birth, are stillborn, or
lungs. However, the risk of lung collapse may be present. die soon after birth from respiratory failure. Some infants,
Rarely, people with Marfan syndrome may have sleep-related however, have lived for a short time with intensive medical
breathing disorders such as snoring or sleep apnea. support.
Researchers have described at least three forms of
DIAGNOSIS achondrogenesis, designated as type 1A, type 1B, and type 2.
The types are distinguished by their signs and symptoms,
There is no specific laboratory test, such as a blood test or skin
inheritance pattern, and genetic cause; however, types 1A and
biopsy, to diagnose Marfan syndrome. The doctor and/or
1B are often hard to tell apart without genetic testing.
geneticist relies on observation and a complete medical history,
Achondrogenesis type 1A, which has also been called the
including:
• Information about any family members who may have the Houston-Harris type, is the least well understood of the three
disorder or who had an early, unexplained heart-related forms. Affected infants have extremely short limbs, a narrow
death. chest, short ribs that fracture easily, and soft skull bones. They
• A thorough physical examination, including an evaluation also lack normal bone formation (ossification) in the spine and
of the skeletal frame for the ratio of arm/leg size to trunk pelvis.
size. Achondrogenesis type 1B, also known as the Parenti-
• An eye examination, including a “slit lamp” evaluation. Fraccaro type, is characterized by extremely short limbs, a
• Heart tests such as an echocardiogram. narrow chest, and a prominent, rounded abdomen. The fingers
and toes are short and the feet may be rotated inward. Affected
Management infants frequently have a soft out-pouching around the belly-
1. There is no cure for Marfan syndrome. Scientists are button (an umbilical hernia) or near the groin (an inguinal
trying to identify and change the specific gene hernia).
responsible for the disorder before birth. Infants with achondrogenesis type 2, which is sometimes
2. Orthodontic treatment for malocclusion, surgical called the Langer-Saldino type, have short arms and legs, a
enucleation of cysts and treatment of TMJ dysarthro-
sis may be necessary for management of oral defects.
3. Annual evaluations are important to detect any
changes in the spine or sternum. This is particularly
important in times of rapid growth, such as
adolescence. In some cases, an orthopedic brace or
surgery may be recommended to limit damage and
disfigurement.
4. In most cases, eyeglasses or contact lenses can
correct ocular problems, although surgery may be
necessary in some cases.
5. Those with heart problems are encouraged to wear
a medical alert bracelet and to go to the emergency
room if they experience chest, back, or abdominal
pain. Some heart valve problems can be managed
with drugs such as beta-blockers, which may help
decrease stress on the aorta. In other cases, surgery
to replace a valve or repair the aorta may be
necessary. Surgery should be performed before the
aorta reaches a size that puts it at high risk for tear
or rupture.
6. If dural ectasia develops, medication may help FIGURE 10.23: Achondrogenesis showing
minimize any associated pain. short limbs and protuberant abdomen
266 Essentials of Pediatric Oral Pathology

narrow chest with short ribs, and underdeveloped lungs. This Management
condition is also associated with a lack of ossification in the
1. Supportive medical care is the only option.
spine and pelvis. Distinctive facial features include a prominent
2. No specific treatment for the underlying disorder.
forehead, a small chin, and, in some cases, an opening in the
roof of the mouth (a cleft palate). The abdomen is enlarged,
CHONDRODYSPLASIA PUNCTATA
and affected infants often have a condition called hydrops
fetalis in which excess fluid builds up in the body before birth. Chondrodysplasia punctata is a clinically and genetically
Achondrogenesis types 1A and 1B are rare genetic dis- diverse group of rare diseases, first described by Conradi, that
orders; their incidence is unknown. Combined, achondrogenesis share the features of stippled epiphyses and skeletal changes.
type 2 and hypochondrogenesis (a similar skeletal disorder)
occur in one in 40,000 to 60,000 newborns. TYPES
Mutations in the SLC26A2 and COL2A1 genes cause Rhizomelic Chondrodysplasia Punctata
achondrogenesis types 1B and 2, respectively.24 The genetic
cause of achondrogenesis type 1A is unknown. Rhizomelic chondrodysplasia punctata type 1 (RCDP1) classic
type, a peroxisome biogenesis disorder (PBD), is characterized
CLINICAL FEATURES by proximal shortening of the humerus and to a lesser degree
the femur (rhizomelia), punctate calcifications in cartilage with
• Males and females are equally affected. epiphyseal and metaphyseal abnormalities (chondrodysplasia
• Achondrogenesis is detected prenatally or at birth because punctata, or CDP), coronal clefts of the vertebral bodies, and
of typical clinical, radiological, histological, and molecular cataracts that are usually present at birth or appear in the first
findings. few months of life. Birth weight, length, and head circumference
• Achondrogenesis type I are often at the lower range of normal; postnatal growth
— Growth—Lethal neonatal dwarfism, mean birth weight deficiency is profound. Mental deficiency is severe, and the
of 1200 g majority of children develop seizures. Most affected children
— Craniofacial—Disproportionately large head; soft skull; do not survive the first decade of life; a proportion die in the
sloping forehead; convex facial plane; flat nasal bridge, neonatal period. A milder phenotype in which all affected
occasionally associated with a deep horizontal groove; individuals have congenital cataracts and chondrodysplasia is
small nose, often with anteverted nostrils; long now recognized; some do not have rhizomelia, and some have
philtrum; retrognathia; increased distance between less severe mental and growth deficiency.25
lower lip and lower edge of chin; double chin
appearance (often). X-Linked Recessive Chondrodysplasia Punctata
— Neck—Extremely short.
It is caused by defects in arysulfatase E (ARSE), a vitamin K-
— Thorax—Short and barrel-shaped thorax, lung
dependent enzyme. Affected males have hypoplasia of the distal
hypoplasia.
phalanges without limb shortening or cataracts. The diagnosis
— Heart—Patent ductus arteriosus, atrial septal defect,
is confirmed by molecular genetic testing. Contiguous gene
ventricular septal defect.
deletions involving ARSE result in more complex phenotypes,
— Abdomen—Protuberant.
including ichthyosis and corneal opacities resulting from steroid
— Limbs—Extremely short (micromelia), much shorter
sulfatase deficiency.
than type II; flipper-like appendages.
• Achondrogenesis type II
Conradi-Hünermann Syndrome
— Growth—Lethal neonatal dwarfism, mean birth weight
of 2100 g. It is usually lethal in males. It is caused by defects in sterol-
— Craniofacial—Disproportionately large head, large and 8-isomerase, which catalyzes an intermediate step in the
prominent forehead, flat facial plane, flat nasal bridge, conversion of lanosterol to cholesterol. Lyonization in females
small nose with severely anteverted nostrils, normal results in phenotypic variability and asymmetric findings.
philtrum (often), micrognathia. Cataracts are sectorial and limb shortening is rhizomesomelic
— Neck—Extremely short. and usually asymmetric. Severely affected infants have bilateral
— Thorax—Short and flared thorax, bell-shaped cage, findings resembling those of RCDP1. The diagnosis is
lung hypoplasia. confirmed by measuring the plasma concentration of sterols,
— Abdomen—Protuberant. which show accumulation of the precursors 8(9)-cholestenol
— Limbs—Extremely short (micromelia). and 8-dehydrocholesterol.
Bone Pathology in Children 267

Autosomal Dominant Chondrodysplasia Punctata CLINICAL FEATURES

• They are most commonly found. Pycnodysostosis causes abnormalities of head and face, teeth,
• It occurs as an autosomal dominant form with the male: collar bones, skin, and nails. The front and back of the head
female ratio of 3:1. are prominent. Within the open sutures of the skull, there may
• Clinically patients with this disorder show flat nasal bridge, be many small bones (called wormian bones).
high arched palate, hypertelorism, cataract, glaucoma, The midface is less full than usual. The nose is prominent.
microphthalamus, ichthyosis, hyperkeratosis, bowing of The jaw can be small. The palate is narrow and grooved.
legs, scoliosis. There occurs delayed eruption of deciduous teeth.
• Radiographic findings show shortening of long bones, calcific The permanent teeth are commonly irregular and teeth may
deposits in infantile cartilaginous skeleton and scoliosis. be missing (hypodontia). The collar bones are often under-
developed and malformed. The skin over the back of the fingers
Management is much wrinkled. The nails are flat and grooved.
Vertebral defects may permit the spine to curve laterally
1. Management is supportive and limited by the multiple (resulting in scoliosis).
handicaps present at birth and poor outcome.
2. Surveillance includes monitoring of growth and
development and regular assessments for seizure
RADIOGRAPHIC FEATURES
control, vision, hearing, contractures, and orthopedic Radiographically, generalized osteosclerosis is evident. There
complications. is a tendency for bone to fracture.
3. Genetic counseling is also recommended. Skull radiographs reveal “Harlequin appearance” or
“Raccoon mask” sign, open fontanelles and cranial sutures,
PYCNODYSOSTOSIS absence of facial sinuses (Fig. 10.24).
Pycnodysostosis is a condition which is characterized by a Panoramic radiograph may reveal acute mandibular angle
hereditary syndrome of short stature, osteoporosis, and skeletal and malpositioned teeth (Fig. 10.25)
abnormalities.
Management
The name for this disease was coined by the French
physicians Maroteaux and Lamy in 1962.26 They described 1. Bone fractures are a big problem for patients with
the disorder in a report entitled “La pycnodysostose.” (They pycnodysostosis. It is important that the disease be
were not the only discoverers of the disease. Andren and diagnosed and the tendency to fractures be
colleagues independently described the condition in 1962.) recognized so that fractures can be minimized, if not
entirely prevented.
Maroteaux and Lamy put “pyknos” from the Greek meaning 2. Replacement treatment with growth hormone was
“dense” together with the compound word “dysostosis” then tested and found effective to increase linear
meaning abnormal bone formation. The name “pycnody- growth of bone.28
sostosis” was designed to convey the abnormally dense bone
that is a hallmark of the disease. MUCOPOLYSACCHARIDOSIS
In 1995, the gene for pycnodysostosis was first charted by
Mucopolysaccharides (MPS) consist of glycosaminoglycans
Gelb and associates.27 It was found to travel preferentially with
attached to a link protein with a hyaluronic acid core. Lysosomal
gene markers known to be in chromosome region 1q21.
enzymes degrade these macromolecules into smaller
Pycnodysostosis is now clearly recognized as being due to
components. Heparan sulfate, dermatan sulfate, and keratan
cathepsin K deficiency. sulfate are by-products of an incomplete degradation process.
Cathepsin K is an enzyme (a catalyst for a reaction of body The accumulation of these compounds interferes with cell
metabolism) of the type called a cysteine protease. This function.
protease is important in cells of normal bone (osteoclasts) that Defective activity of the lysosomal enzymes blocks the
are responsible for bone reabsorption. It is thought that degradation process of mucopolysaccharides, leading to
osteoclasts in patients with pycnodysostosis are hampered by abnormal accumulation of heparan sulfate, dermatan sulfate,
a lack of cathepsin K and cannot adequately reabsorb that and keratan sulfate. These degradation by-products are then
component of bone called the organic matrix. Because of this secreted and detected in the urine. Mucopolysaccharidosis
inadequate resorption, the bones in pycnodysostosis are (MPS) can be subclassified according to the type and amount
abnormally dense and brittle. of substance that accumulates, as follows: Hurler syndrome
268 Essentials of Pediatric Oral Pathology

CLINICAL FEATURES
• MPS IH (Hurler syndrome): Infants born with Hurler
syndrome appear healthy at birth. Diagnosis is usually made
in infants aged 6 to 24 months. Inguinal and umbilical
hernias are commonly seen at birth. On physical
examination, these patients are observed to have corneal
clouding, hepatosplenomegaly, skeletal deformities
(dysostosis multiplex), coarse facial features, large tongue,
prominent forehead, joint stiffness, and short stature. They
also have upper airway obstruction, recurrent ear infections,
noisy breathing, and persistent nasal discharge. Other
features include hirsutism, hearing loss, hydrocephalus, and
mental retardation. Death usually occurs by age 10 years.
• MPS I-H/S (Hurler-Scheie syndrome): This is an
intermediate form of Hurler syndrome with milder features.
FIGURE 10.24: Postero-anterior skull radiography revealing Onset is seen in children aged 3 to 8 years. These patients
generalized sclerosis of the skeleton, more pronounced in the have normal intelligence and micrognathia, which gives
periorbital region (“Harlequin appearance” or “Raccoon mask” sign), them a characteristic facies. Corneal clouding, joint
open fontanelles and cranial sutures, absence of facial sinuses stiffness, and heart disease develop by the early to mid-
teens. Patients survive well into the third decade of life.
• MPS IS (Scheie syndrome): Onset occurs in patients older
than five years. These patients have aortic valve disease,
corneal clouding, and joint stiffness with broad short claw
hands. They have normal intelligence and stature and a
normal life span.
• MPS II (Hunter syndrome): Mild and severe forms exist,
both of which have the same enzyme deficiency. This form
of MPS is characterized by pebbly ivory skin lesions on
the back, arms, and thighs. The extent of the skin lesions
does not correlate with severity of the disease.
— MPS II, severe form: Onset of disease occurs in
children aged 2 to 4 years, with severe progressive
somatic and neurologic involvement. Coarse facial
FIGURE 10.25: Panoramic radiography revealing obtuse
mandible angle, acute caries and malpositioned teeth
features, skeletal deformities (such as claw hand) (Figs
10.26A and B), and joint stiffness are present. These
(MPS IH), Hurler-Scheie (MPS I-H/S), Scheie syndrome (MPS patients also have retinal degeneration with clear cornea
IS), Hunter syndrome (MPS II), Sanfilippo syndrome (MPS and hydrocephalus, mental retardation, and aggressive
III), Morquio syndrome (MP IV), Maroteaux-Lamy syndrome behavior. Death occurs in patients aged 10 to 15 years.
(MPS VI), and Sly syndrome (MPS VII).29-31 — MPS II, mild form: These patients have similar features
The prevalence of all types of MPS is 1 case in 16,000 to to the severe form but a much slower rate of
30,000 births. MPS III accounts for 80 percent of cases. These progression. They have normal intelligence and no
syndromes are found in persons of all ethnic groups, but hydrocephalus. Hearing impairment and loss of hand
prevalence is increased in Israeli Jews and French Canadians. function secondary to joint stiffness and deformities are
All mucopolysaccharidoses are inherited as autosomal common in the mild form of Hunter. These patients
recessive disorders with the exception of Hunter syndrome survive into the sixth and seventh decades of life.
(MPS II), which is inherited as sex-linked recessive condition. • MPS III (Sanfilippo syndrome): This appears to be the most
Thus, all patients with Hunter syndrome are males. common of the MPS disorders. Four subtypes of this disease
MPS features mostly present in the first few months of life. exist, based on the lysosomal enzyme deficiency (types A,
However, Morquio syndrome usually presents in children aged B, C, and D). However, these subtypes are not distinguishable
2 to 4 years, and MPS IS and MPS VI can present late in clinically. Onset of the disease usually occurs in children aged
childhood. 3 to 6 years. These patients have severe central nervous
Bone Pathology in Children 269

MPS VII can be detected in the neonatal period associated

with hydrops fetalis and hepatosplenomegaly, with death
occurring within the first few months of life. Patients with the
mild form survive into adolescence. The phenotype is similar
to that of Hurler syndrome. Physical findings include corneal
clouding, coarse facies, macrocephaly, metatarsus adductus,
prominent sternum, pelvic hypoplasia, hepatosplenomegaly,
and hernias.

Management
1. Specific treatment or cure is limited for MPS.
Management has been limited to supportive care and
A B experimental treatment modalities.
2. Laronidase (Aldurazyme) is a polymorphic variant of
FIGURES 10.26A and B: Mucopolysaccharidosis showing the human enzyme alpha-L-iduronidase produced by
characteristic skeletal deformity of the hands recombinant DNA technology. It is indicated to treat
system involvement and only minimal somatic involvement. MPS type I (Hurler and Hurler-Scheie forms). It
They commonly present with hyperactivity, mental increases catabolism of glycosaminoglycans (GAGs),
which accumulate with MPS I. Laronidase therapy has
deterioration, and developmental delay. Physical findings
shown to improve walking capacity and pulmonary
include coarse hair, hirsutism, mild hepatosplenomegaly, and function.
enlarged head. Occasionally, mild dysostosis multiplex and 3. Idursulfase (Elaprase) is a purified form of human
joint stiffness are seen. By age 8 to 10 years, these patients iduronate-2-sulfatase, a lysosomal enzyme. It hydro-
are profoundly retarded with severely disturbed social lyzes 2-sulfate esters of terminal iduronate sulfate
behavior (e.g. uncontrollable hyperactivity, destructive residues from the GAGs dermatan sulfate and heparan
physical aggression). These patients usually survive into the sulfate in the lysosomes of various cell types. It is used
second or third decade of life. to replace insufficient levels of the lysosomal enzyme
• MPS IV (Morquio syndrome): Deficiencies of two different iduronate-2-sulfatase in MPS II.32
enzymes leading to a severe form (MPS IV A) and a mild 4. Bone marrow transplantation (BMT) has been
form (MPS IV B) are recognized. Orthopedic involvement successful in the treatment of MPS conditions,
especially Hurler syndrome.33
is the primary finding in these patients, with preservation
5. Surgical management for specific conditions like
of intelligence and varying degrees of skeletal involvement. hydrocephalus, cardiovascular disease, obstructive
Spondyloepiphyseal dysplasia is the hallmark of this airway disease, orthopedic conditions is recommended.
disease. Physical findings include genu valgum, short
stature, spinal curvature, odontoid hypoplasia, and
RICKETS
ligamentous laxity. Atlantoaxial instability is common in
Morquio syndrome and can lead to severe myelopathy, Rickets is a disease of growing bone that is unique to children
paralysis, and death. Patients with the severe form do not and adolescents. It is caused by a failure of osteoid to calcify in
survive beyond the third or fourth decade of life. Patients a growing person. Failure of osteoid to calcify in adults is called
with the mild form have much slower progression of osteomalacia. Vitamin D deficiency rickets occurs when the
skeletal dysplasia and a normal life span. metabolites of vitamin D are deficient. Less commonly, a dietary
• MPS VI (Maroteaux-Lamy syndrome): Onset occurs in deficiency of calcium or phosphorus may also produce rickets.
patients aged 1 to 3 years. Mild, intermediate, and severe Vitamin D-3 (cholecalciferol) is formed in the skin from a
types have been identified, all with the same enzyme derivative of cholesterol under the stimulus of ultraviolet-B light.
deficiency. Features are very similar to Hurler syndrome, Natural nutritional sources of vitamin D are limited
including corneal clouding, coarse facies, joint stiffness, primarily to fatty, ocean-going fish. In the United States, dairy
skeletal deformities, and heart valvular disease. milk is fortified with vitamin D (400 IU/L). Human milk
Intelligence, however, is normal. These patients may contains little vitamin D, generally less than 20 to 40 IU/L.
survive into the third decade of life. Most die from Therefore, infants who are breastfed are at risk for rickets,
cardiopulmonary complications. especially those who receive no oral supplementation and those
• MPS VII (Sly syndrome): This is a very rare condition. Mild who have darkly pigmented skin, which blocks penetration of
and severe forms have been identified. The severe form of ultraviolet light.
270 Essentials of Pediatric Oral Pathology

In the vitamin D deficiency state, hypocalcemia develops,

which stimulates excess parathyroid hormone, which stimulates
renal phosphorus loss, further reducing deposition of calcium
in the bone. Excess parathyroid hormone also produces changes
in the bone similar to those occurring in hyperparathyroidism.
Early in the course of rickets, the calcium concentration in the
serum decreases. After the parathyroid response, the calcium
concentration usually returns to the reference range, though
phosphorus levels remain low. Alkaline phosphatase, which is
produced by overactive osteoblast cells, leaks to the extra-
cellular fluids so that its concentration rises to anywhere from
moderate elevation to very high levels.
Intestinal malabsorption of fat and diseases of the liver or
kidney may produce the clinical and secondary biochemical
picture of nutritional rickets. Anticonvulsant drugs (e.g.
phenobarbital, phenytoin) accelerate metabolism of calcidiol, FIGURE 10.27: Rickets showing bowed legs in a child
which may lead to insufficiency and rickets, particularly in
children who are kept indoors in institutions.
Calcium and vitamin D intakes are low in infants who are
fed vegan diets, particularly lactovegans, and monitoring of
their vitamin D status is essential.
Recent studies have noted that disorders of increased
fibroblast growth factor 23 (FGF-23) functions are associated
with rickets.34

CLINICAL AND RADIOGRAPHIC FEATURES

• No sexual predilection is noted.
• It is observed only in growing children, although the effects
may be observed later in life.
• Generalized muscular hypotonia of an unknown mechanism
is observed in most patients with clinical (as opposed to
biochemical and radiographic) signs of rickets. FIGURE 10.28: Rickets showing bowed hands in a child
• Craniotabes manifests early in infants with vitamin D
deficiency, although this feature may be normal in infants,
especially for those born prematurely.
• If rickets occurs at a later age, thickening of the skull
develops. This produces frontal bossing and delays the
closure of the anterior fontanelle. In the long bones, laying
down of uncalcified osteoid at the metaphyses leads to
spreading of those areas, producing knobby deformity,
which is visualized on radiography as cupping and flaring
of the metaphyses.
• Weight bearing produces deformities such as bowlegs and
knock-knees (Figs 10.27 to 10.30).
• In the chest, knobby deformities results in the rachitic
rosary along the costochondral junctions. The weakened
ribs pulled by muscles also produce flaring over the
diaphragm, which is known as Harrison groove. The
sternum may be pulled into a pigeon-breast deformity. A B
• In more severe instances in children older than two years, FIGURES 10.29A and B: Rickets showing bowing of bones of
vertebral softening leads to kyphoscoliosis. The ends of the the legs leading to a typical knock knee appearance in a child
Bone Pathology in Children 271

FIGURE 10.30: Rickets showing deformity of bones of the FIGURE 10.31: Rickets showing poorly defined lamina dura
forearms seen in rickets around the teeth

long bones demonstrate that same knobby thickening. At

5. Infants weighing less than 1500 g need special
the ankle, palpation of the tibial malleolus gives the
supplementation (i.e. vitamin D, calcium, phosphorus)
impression of a double epiphysis (Marfan sign). Because if breast milk is their primary dietary source.
the softened long bones may bend, they may fracture one 6. Recommending a vitamin D supplement from the first
side of the cortex (i.e. greenstick fracture). Panoramic week of life for susceptible infants who are breastfed
radiograph may reveal poorly defined lamina dura around is safe and effective and, therefore, should be
the teeth (Fig. 10.31). considered.

HISTOPATHOLOGIC FEATURES HYPERPARATHYROIDISM

Lesional areas when observed show uncalcified cartilage matrix There are four parathyroid glands located below the thyroids.
covering the shaft of long bones separated by the capillaries. These glands produce parathormone and maintain plasma
Osteoid is formed on the cartilage but it does not calcify. ionized calcium levels. Any deviation from the normal activity
of secretion from these glands either leads to hypo or
Management hypersecretion from these glands which manifests in the form
of symptoms of hypo or hyperparathyroidism respectively. It
1. Treatment for rickets may be gradually administered may be primary or secondary.
over several months or in a single-day dose of 15,000 Primary hyperparathyroidism is a disease which occurs as
mcg (600,000 U) of vitamin D. If the gradual method
a result of excessive secretion of parathyroid hormones. It may
is chosen, 125-250 mcg (5000-10,000 U) is given
occur due to adenoma of the glands, hyperplasia of parathyroid
daily for 2-3 months until healing is well established
and the alkaline phosphatase concentration is
tissue or due to carcinoma of the gland. Secondary hyperpara-
approaching the reference range. thyroidism occurs when parathormone is secreted continuously
2. If the vitamin D dose is administered in a single day, as a result of low levels of serum parathormone due to some
it is usually divided into four or six oral doses. An kind of renal disease.Primary hyperparathyroidism occurs
intramuscular injection is also available. Vitamin D is during childhood and is therefore considered here.
well stored in the body and is gradually released over
many weeks. Because both calcitriol and calcidiol CLINICAL FEATURES
have short half-lives, they are unsuitable; they would
bypass the natural physiologic controls of vitamin D • It occurrs most commonly in women.
synthesis. • Incidence of occurrence is most common in middle aged
3. If severe deformities have occurred, orthopedic persons but may also be seen in children.
correction may be required after healing. Most of the • Bone and joint pains, and pathologic fractures are most
deformities correct with growth. common findings.
4. Human milk contains little vitamin D and contains too • Sometimes there occurs giant cell tumor in the bones.
little phosphorus for babies who weigh less than Schour and Massler, 1943,35 reported that malocclusion
1500 g. due to drifting of teeth may be the first sign of this disease.
272 Essentials of Pediatric Oral Pathology

• There is elevated level of serum calcium above the normal Hypoparathyroidism results in loss of the direct and indirect
level of 9 to 12 mg/dl and serum parathormone. effects of PTH on bone, the kidney, and the gut. Calcium and
phosphate release from bone is impaired, calcium absorption
RADIOGRAPHIC FEATURES from the gut is limited, calciuria develops despite hypocal-
cemia, and retention of phosphate from the urine causes
The lesion appears radiolucent and has been described as increased plasma phosphate levels.
‘ground glass’ appearance when it occurs in jaws. Sometimes Hypoparathyroidism may be transient, genetically inherited,
there is partial loss of lamina dura around the teeth. or acquired.
Genetically inherited forms arise from defects of
HISTOPATHOLOGIC FEATURES parathyroid gland development, defects in the parathyroid
• Lesional area does not show any pathognomonic changes hormone (PTH) gene, defects in the calcium-sensing receptor
gene, defects in PTH action, defects in the autoimmune
for this lesion but shows osteoclastic resorption of the bony
regulator gene, and genetic syndromes.
trabeculae.
Acquired hypoparathyroidism may be due to an autoimmune
• Osteoblastic rimming is seen around the osteoid.
process or may occur after neck irradiation or surgery.
• Fibrosis is seen replacing resorbed bone.
Transient hypoparathyroidism occurs during the neonatal
• Presence of hemosiderin pigments and giant cells is evident.
period. Preterm infants are at increased risk, and as many as
50 percent of very low birth weight infants may have a deficient
Management
surge in PTH that results in hypocalcemia.
Surgical excision of the parathyroid tissue is performed • Hypocalcemia is noted in 10 to 20 percent of infants of
to reduce the levels of parathormone to normal. diabetic mothers. These infants may be born prematurely,
which is a risk factor for insufficient PTH response. They
HYPOPARATHYROIDISM may have hypomagnesemia from maternal magnesuria
complicating glucosuria. Low serum magnesium can impair
Hypoparathyroidism results from defective synthesis or
PTH release and action.
secretion of Parathyroid hormone (PTH), end-organ resistance,
• DiGeorge syndrome (i.e. hypoparathyroidism, T-cell
or inappropriate regulations that result from the activated or
abnormalities, cardiac anomalies) is associated with
antibody-stimulated Calcium-sensing receptor (CaSR). These
abnormal development of the third and fourth pharyngeal
defects can be inherited or acquired. PTH secretion by the
pouches from which the parathyroids derive embryologi-
parathyroid glands (prime regulators of serum calcium
cally and represents an example of a defect in parathyroid
concentration) maintains serum calcium within a strict range.
gland development. DiGeorge syndrome and velocardio-
Biochemical hallmarks of hypoparathyroidism include
facial syndrome are variants of the chromosome arm 22q11
hypocalcemia and hyperphosphatemia. Severe hypocalcemia
microdeletion syndrome.
presents with seizures, stridor and tetany.
• Hypocalcemia associated with a 22q11 microdeletion may
Mature PTH is an 84-amino acid protein. Production and
be transiently present in infancy but recur later in life,
secretion of PTH are regulated by a G protein-coupled calcium-
particularly during periods of stress.
sensing receptor. Unlike other protein hormones, its production
• Familial cases of hypoparathyroidism due to mutations of
and secretion are stimulated by decreased intracellular calcium
the PTH gene located on chromosome arm 11p15 have
concentrations, which reflect serum calcium concentrations.
been identified. These mutations have been both
PTH exerts its action through the PTH receptor, which is
dominantly and recessively inherited.
another member of the G protein-linked receptor family.
The net effects of PTH activity are an increase in serum
CLINICAL FEATURES
calcium and a decrease in serum phosphate. PTH acts directly on
bone to stimulate bone resorption and cause calcium and phosphate • Hypoparathyroidism is equally prevalent in males and
release. PTH acts directly on the kidney to decrease calcium females.
clearance and to inhibit phosphate reabsorption. By stimulating • Age of onset depends on the etiology of hypoparathy-
renal 1-alpha-hydroxylase activity, PTH increases serum roidism. Newborns may present with hypoparathyroidism;
concentrations of 1,25-dihydroxyvitamin D, the active form of however, it can manifest at almost any age.
vitamin D and, thus, indirectly stimulates calcium and phosphate • Hyper-reflexia due to hypocalcemia is common.
absorption by the gut through the actions of vitamin D. The • Trousseau sign is a carpopedal spasm that occurs after a
phosphaturic effect of PTH offsets the increases of serum blood pressure cuff around the arm is inflated to the systolic
phosphate driven by increased bone resorption and GI absorption. blood pressure for several minutes.
Bone Pathology in Children 273

• Chvostek sign (i.e. twitching of facial muscles with tapping calcemic symptoms. Severe hypocalcemia can be
on the facial nerve in front of the ear) is a manifestation of treated with a continuous calcium infusion; a transition
neuromuscular irritability. Chvostek sign is present in 25 to the oral form can be made when the serum calcium
percent of healthy adults and in even higher rates in concentration is within a safe range. Tailoring of calcium
children. Thus, its presence or absence should be dosing to each patient’s needs is essential. In fact, once
documented prior to thyroidectomy. adequate amounts of active vitamin D are present,
• Nasal speech can occur from a cleft palate or velopharyn- some patients can absorb all the calcium they need
geal insufficiency. through the diet and oral calcium preparations can be
discontinued.
• Bulbous nasal tip, micrognathia, ear anomalies, and short
philtrum are typical facial features but may not be evident
in nonwhite children. CRANIOSYNOSTOSIS SYNDROMES
• A heart murmur may signify a conotruncal heart defect. Craniosynostosis consists of premature fusion of one or more
• Short stature may be a feature of the genetic syndrome, but cranial sutures, often resulting in an abnormal head shape. It
in some cases, it is due to hypopituitarism. may result from a primary defect of ossification (primary
craniosynostosis) or, more commonly, from a failure of brain
Management
growth (secondary craniosynostosis). Simple craniosynostosis
1. Symptomatic hypocalcemia (e.g. seizure, tetany, is a term used when only 1 suture fuses prematurely. Complex
laryngospasm) in patients with hypoparathyroidism or compound craniosynostosis is used to describe premature
requires intravenous calcium and continuous fusion of multiple sutures. When children with craniosynostosis,
monitoring for cardiac arrhythmias. usually complex, also display other body deformities, this is
2. Oral calcium and vitamin D should be initiated as soon
termed syndromic craniosynostosis.
as possible (e.g., when the patient is tolerating oral
feeds).
When one or more sutures fuse prematurely, skull growth
3. Once serum calcium concentrations are in a safe can be restricted perpendicular to the suture. If multiple sutures
range (>7.5 mg/dL), intravenous calcium can be fuse while the brain is still increasing in size, intracranial
stopped. However, rebound hypocalcemia can occur pressure can increase.
and requires that a patient be monitored for • Scaphocephaly – Early fusion of the sagittal suture
therapeutic success on oral agents for at least 24 • Anterior plagiocephaly – Early fusion of 1 coronal suture
hours after intravenous calcium is withdrawn. • Brachycephaly – Early bilateral coronal suture fusion
4. The active form of vitamin D, 1,25-dihydroxyvitamin • Posterior plagiocephaly – Early closure of 1 lambdoid
D, is preferred in the treatment of hypoparathyroidism suture
because both the parathyroid hormone (PTH) • Trigonocephaly – Early fusion of the metopic suture.
deficiency/resistance and the hyperphosphatemia
More frequent than the primary type, secondary
impair the activation of 25-hydroxyvitamin D by
1-alpha-hydroxylase. craniosynostosis can result from early fusion of sutures due to
5. No special diet is required, but adequate calcium and primary failure of brain growth. Since brain growth drives the
vitamin D intake is recommended. bony plates apart at the sutures, a primary lack of brain growth
6. Calcium and vitamin D are the mainstays of treatment allows premature fusion of all the sutures.
for hypoparathyroidism and pseudohypoparathyroidism Intracranial pressure is usually normal, and surgery is
(PHP). To relieve immediate severe symptoms of seldom needed. Typically, failure of brain growth results in
hypocalcemia, an intravenous bolus of 9-15 mg microcephaly. Premature suture closure does not compromise
elemental calcium/kg (1 g calcium gluconate = 90 mg
brain growth and does not require surgery to open sutures.
elemental calcium = 4.5 mEq elemental calcium) is
administered over 10-30 min. Then, either intermittent
Intrauterine space constraints may play a role in the
boluses or a continuous IV infusion is initiated (£ 60 premature fusion of sutures in the fetal skull. This has been
mg elemental calcium/kg/d). Oral calcium is initiated demonstrated in coronal craniosynostosis. Other secondary
for a total of 100 mg elemental calcium/kg/d divided 4 causes of craniosynostosis include systemic disorders that affect
times daily. Once serum calcium concentrations range bone metabolism such as rickets and hypercalcemia.
from 8 to 9 mg/dL, the calcium dose is weaned to the • Multiple theories have been proposed for the etiology of
minimum dose necessary to maintain a low-normal primary craniosynostosis, but the most widely accepted is
serum calcium concentration. a primary defect in the mesenchymal layer ossification in
7. Numerous calcium preparations are available. An
the cranial bones.
intravenous dose quickly but transiently corrects the
serum calcium concentration and relieves hypo-
• Secondary craniosynostosis typically results from systemic
disorders such as the following:
274 Essentials of Pediatric Oral Pathology

— Endocrine–Hyperthyroidism, hypophosphatemia, • Posterior plagiocephaly

vitamin D deficiency, renal osteodystrophy, hyper- — The two predominant causes of posterior plagiocephaly
calcemia, and rickets are craniosynostosis of the lambdoid suture (<2%) or
— Hematologic disorders that cause bone marrow positional molding (vast majority).
hyperplasia (e.g. sickle cell disease, thalassemia) — Since the American Academy of Pediatrics recom-
— Inadequate brain growth, including microcephaly and mended that infants sleep on their backs to reduce
its causes and shunted hydrocephalus. sudden infant death syndrome (SIDS) incidence,
• The syndromic causes appear to result from genetic positional molding has been seen with increased
mutations responsible for fibroblast growth factor receptors frequency.
two and three. A gene locus for single suture craniosy- — Torticollis is frequently associated with positional
nostosis has not been identified. molding.
• Other important factors to consider — Viewed from above, the head shape in positional
— Differentiating plagiocephaly that results from molding resembles a parallelogram, whereas that in
positional molding (which does not require surgery and lambdoid craniosynostosis is trapezoid shaped.
is seen frequently) from lambdoid suture fusion is — In positional molding, ear position is more anterior on
extremely important. the side of flattening; in lambdoid synostosis, ear
— The presence of multiple suture fusions strongly position is more posterior.
suggests a craniofacial syndrome, which frequently — Frontal bossing is observed ipsilateral to the flattening
requires the diagnostic expertise of a pediatric in positional molding and contralateral in lambdoid
geneticist. synostosis.
• Trigonocephaly
CLINICAL FEATURES — Premature fusion of the metopic suture frequently
results in pointed forehead (i.e. triangular shaped head).
Craniosynostosis is equally distributed in both boys and girls.
The abnormality is usually mild and requires no surgical
• Neonatal period: Craniosynostosis is evident at birth when
intervention. Surgery is performed if the abnormality
associated with other craniofacial abnormalities.
is persistent and severe.
• Infancy (0-18 mo): Secondary or primary craniosynostosis
— Oxycephaly (i.e. turricephaly) is fusion of all skull
becomes evident as the child grows.
sutures and the sutures at the base of the skull.
• Microcephaly usually suggests a secondary cranio-
• Craniosynostosis sometimes is associated with sporadic
synostosis.
craniofacial syndromes such as Crouzon, Apert, Chotzen,
• Scaphocephaly
Pfeiffer, or Carpenter syndromes. In this context, facial
— Premature fusion of the sagittal suture is the most
features, typically craniofacial abnormalities, suture ridging,
common craniosynostosis, constituting more than half
and early closure of fontanelles, suggest the diagnosis.
of all cases. It occurs frequently in premature infants.
• Kleeblattschãdel (i.e. cloverleaf skull) results from fusion
— The head typically is elongated in the anterior-posterior
of all sutures except the metopic and squamosal sutures,
diameter and shortened in the biparietal diameter.
giving the head a cloverleaf appearance.
Ridging of the sagittal suture is palpable.
• Intracranial pressure may be elevated in primary multiple
• Anterior plagiocephaly – Premature fusion of one coronal
suture craniosynostosis, such as cloverleaf skull and the
suture.
syndromic synostoses. Signs include sun-setting eyes,
• Brachycephaly
papilledema, vomiting, and lethargy.
— Premature fusion of both coronal sutures results in
increased biparietal diameter. This anomaly is often
Management
syndromic. The skull is shorter in the anterior-posterior
diameter. 1. Currently, surgery is usually cosmetic for infants with
— Because the coronal suture develops in conjunction with fusion of 1 to 2 sutures that result in a misshapen
the sutures at the base of the skull, unilateral or bilateral head. For infants with microcephaly (i.e. secondary
craniosynostosis), surgery usually is not required.
mid and upper face hypoplasia may occur. Orbits may
2. Surgery typically is indicated for increased intracranial
be elliptical (i.e. Harlequin features), and the supraorbital
pressure or for cosmetic reasons.
ridge may not be formed well. 3. Consideration for cosmetic surgery is dependent on
— Consider these features when planning surgery for the age of presentation and on which sutures have
brachycephaly.
Bone Pathology in Children 275

fused prematurely. Cosmetic surgery is performed in • Premature synostosis of the coronal, the sagittal, and,
infants aged 3 to 6 months. occasionally, the lambdoidal sutures begins in the first year
4. Infants with a defined syndrome causing cranio- of life and is completed by the second or third year. The
synostosis should be evaluated early for surgery. order and rate of suture fusion determine the degree of
Results are best when surgery is performed in infants deformity and disability. Once a suture becomes fused,
younger than six months. Patients with associated growth perpendicular to that suture becomes restricted, and
facial deformities may need a staged surgical the fused bones act as a single bony structure. Compen-
approach. satory growth occurs at the remaining open sutures to allow
5. No surgery is indicated for posterior plagiocephaly continued brain growth. However, multiple sutural
secondary to positional molding. The vast majority of synostoses frequently extend to premature fusion of the
infants improve with repositioning maneuvers and
skull base sutures, causing midfacial hypoplasia, shallow
physical therapy for torticollis.
orbits, a foreshortened nasal dorsum, maxillary hypoplasia,
and occasional upper airway obstruction.
CRANIOFACIAL DYSOSTOSIS – CROUZON • The phenotypic spectrum of the FGFR3 P250R mutation,
SYNDROME (FIG. 10.32) called Muenke craniosynostosis or FGFR3-associated
• In 1912, Crouzon described the hereditary syndrome of coronal synostosis, is so widely variable that patients with
craniofacial dysostosis in a mother and son. He described this specific mutation had been previously diagnosed as
the triad of calvarial deformities, facial anomalies, and having Crouzon syndrome, Pfeiffer syndrome, Saethre-
exophthalmos.36 Chotzen syndrome, Jackson-Weiss syndrome, and
• Crouzon syndrome is an autosomal dominant disorder with nonsyndromic craniosynostosis.37
complete penetrance and variable expressivity. It is • A newly identified mutation in the tyrosine kinase I domain
characterized by premature closure of calvarial and cranial of the FGFR2 gene (1576A>G, encoding the missense
base sutures as well as those of the orbit and maxillary substitution Lys526Glu) is associated with variable
complex (craniosynostosis). expressivity of Crouzon syndrome, including clinical
• Other clinical features include hypertelorism, exophthal- nonpenetrance.
mos, strabismus, beaked nose, short upper lip, hypoplastic
maxilla, and relative mandibular prognathism. Unlike some CLINICAL FEATURES
other forms of autosomal dominant craniosynostosis, no
digital abnormalities are present. • Crouzon syndrome has no known sex predilection.
Crouzon syndrome is caused by mutations in the • The condition is detected in the newborn or infant period
fibroblast growth factor receptor-2 (FGFR2) gene but because of dysmorphic features.
exhibits locus heterogeneity with causal mutations in • Craniosynostosis: Craniosynostosis commonly begins during
FGFR2 (Crouzon syndrome) and FGFR3 (Crouzon the first year of life and usually completes by the second or
syndrome with acanthosis nigricans) in different affected third year. Coronal and sagittal sutures are most commonly
individuals. involved, resulting in acrocephaly, brachycephaly,
turricephaly, oxycephaly, flat occiput, and high prominent
forehead with or without frontal bossing. Ridging of the skull
is usually palpable. Cloverleaf skull is rare (only 7%) and
occurs in the most severely affected individuals.
• Flattened sphenoid bone
• Shallow orbits
• Hydrocephalus (progressive in 30%)
• Midface (maxillary) hypoplasia may be present.
• Exophthalmos (proptosis) secondary to shallow orbits
resulting in frequent exposure conjunctivitis or keratitis
• Ocular hypertelorism
• Divergent strabismus
• Rare occurrence of nystagmus, iris coloboma, aniridia,
anisocoria, microcornea, megalocornea, cataract, ectopia
lentis, blue sclera, glaucoma, luxation of the eye globes,
FIGURE 10.32: Crouzon syndrome in a patient showing papilledema, and optic atrophy from raised intracranial
hypertelorism pressure leading to blindness.
276 Essentials of Pediatric Oral Pathology

• Beaked appearance 5. Early craniectomy with frontal bone advancement is

• Compressed nasal passage most often indicated to prevent or treat increased
• Choanal atresia or stenosis intracranial pressure because newborns with Crouzon
• Deviated nasal septum syndrome develop multiple suture synostoses and
• Mandibular prognathism fused synchondroses.
• Overcrowding of upper teeth, malocclusions, and V-shaped 6. Fronto-orbital and midfacial advancements help in the
maxillary dental arch cosmetic reconstruction of facial dysmorphisms.
• Narrow, high, or cleft palate and bifid uvula 7. A new technique, craniofacial disjunction, followed by
• Occasional oligodontia, macrodontia, peg-shaped, and gradual bone distraction (Ilizarov procedure) has been
reported to produce complete correction of
widely spaced teeth
exophthalmos and improvement in the functional and
• Narrow or absent ear canals
aesthetic aspects of the middle third of the face
• Deformed middle ears without the need for bone graft in patients aged 6 to
• Cervical fusion (18%), C2 to C3, C3 to C4, and C5 to C6 11 years.
• Block fusions involving multiple vertebrae
• Subluxation of the radial heads MANDIBULOFACIAL DYSOSTOSIS – TREACHER
• Ankylosis of the elbows COLLINS FRANCESCHETTI SYNDROME (FIG. 10.33)
• Approximately 5 percent of patients have acanthosis
nigricans, which is detectable after infancy. The hallmark Mandibulofacial dysostosis, also known as Treacher Collins
of these lesions is a darkened thickened skin with syndrome was named after the eminent British ophthalmologist
accentuated markings and a velvety feel. Edward Treacher Collins (1862-1932), who described the
• Approximately 73 percent of patients have chronic tonsillar essential features of this syndrome in a paper in 1900. 39
herniation (47 percent have progressive hydrocephalus). However, some features of this syndrome were probably first
• Syringomyelia may be present. described by Thomson and Toynbee40,41 in 1846 to 1847 and
• Postnatal subtype of Crouzon syndrome (in patients at later by Berry (1889),42 who is usually given credit for its
risk, such as family members of patients with Crouzon discovery. It is an inherited developmental disorder with a
syndrome, or those with some degree of exorbitism at birth) prevalence estimated to ranges between one in 40,000 to one
from birth to at least age three years. in 70,000 of live births. Growth of craniofacial structures
• Development of digital impressions and/or ossification of derived from the first and second pharyngeal arch, groove, and
sutures starting at the occipital region of the skull. pouch is diminished symmetrically and bilaterally.
• Development of a prominent bregma. Inheritance of Treacher Collins syndrome is autosomal
• Development of “spontaneous” intracranial hypertension. dominant, with complete penetrance and variable expressivity.
• Progressive characteristic crouzonoid features such Nonpenetrance is rare. Approximately 60 percent of cases
as progressive exorbitism. represent fresh mutations.
Management
1. Early detection of eye problems to reduce amblyopia
by correction of refractory errors and timely treatment
of strabismus and patching is indicated. Optic atrophy
remains an important cause of visual impairment
before decompressive craniectomy.38
2. To relieve airway obstruction, a nasal continuous
positive airway pressure device may be needed.
3. Close otologic and audiologic follow-up is indicated
to detect sensorineural hearing loss. Management of
speech may be necessary.
4. Surgical treatment varies according to the variable
expressivity of the disease and usually begins during
a child’s first year with fronto-orbital advancement with
cranial decompression. Subsequent development of
midfacial hypoplasia needs correction. Procedures for
this purpose include the Le Fort III osteotomy or its
segmental variants, monobloc frontofacial advance- FIGURE 10.33: Treacher Collins syndrome showing downward
ment, or bipartition osteotomy. displacement of mouth and recession of chin
Bone Pathology in Children 277

Treacher Collins syndrome is caused by mutations in the 4. Operative repair of Treacher Collins syndrome is
TCOF1 gene. TCOF1 was mapped to chromosome bands based upon the anatomic deformity and timing of
5q31.3-33.3. The TCOF1 gene codes for the treacle protein, correction is done according to physiologic need and
which may be involved in nucleolar trafficking and is required development.
for normal craniofacial development. Single mutations in the 5. A new management technique has been performed
gene result in the premature termination of the protein product.43 in selected cases. Distraction osteogenesis, an
orthopedic method of lengthening bone, has been
CLINICAL FEATURES used to lengthen the neonatal mandible. The infant
is intubated at birth, and, within a few days, a cut is
• Males and females are equally affected.
made on both sides of the jaw and distraction
• In the vast majority of cases, Treacher Collins syndrome
hardware is placed. The jaw is stretched at 1 to 2 mm/
is clearly diagnosed at birth. d, and extubation is usually performed when 10 mm
• The face of an individual with Treacher Collins syndrome is of lengthening is achieved.
characteristic. Abnormalities are usually present bilaterally and 6. For more minor obstructions that can be corrected
symmetrically. The nose has a normal size; however, it appears with positioning, a tongue-lip adhesion is considered.
large because of hypoplastic supraorbital rims and hypoplastic Surgical adhesion is performed between the tongue,
zygomas. The palpebral fissures are downward-sloping, the lip, and anterior mandible. This pulls the tongue
pinnae are malformed with widely varying severity, and the forward, correcting the base of tongue obstruction,
chin recedes with a large, down-turned mouth. and pulls the tongue out of the nasopharynx in the
• A cleft palate is found in one third of patients with Treacher presence of cleft palate.
Collins syndrome, and congenital palatopharyngeal incompe- 7. The lateral coloboma of the lower eyelid has
tence (foreshortened, immobile, or absent soft palate; traditionally been corrected with a skin-muscle flap
submucous cleft palate) is found in an additional one-third of from the upper lid or brow. This adds vertical height
patients. The parotid glands are missing or hypoplastic. to the lateral lid, correcting the notch and down-turned
Pharyngeal hypoplasia is a constant finding. lateral palpebral fissure.
8. Macrostomia, if present, can be repaired at the same
time with Z-plasty or straight-line skin repair; however,
RADIOGRAPHIC FEATURES
restoration of continuity in the oral musculature is
The malar bones, zygomatic process of frontal bone, lateral important, as it restores the function of the oral
pterygoid plates, paranasal sinuses, and mandibular condyles sphincter and limits scar contracture.
are hypoplastic. The mastoids are not pneumatized. The lateral 9. Cleft palate, present in one third of cases, is repaired
margins of the orbits may be defective, and the orbits are at approximately age 10 to 12 months but can be
hyperteloric. The cranial base is progressively kyphotic. The delayed if airway concerns exist. Extra time before
calvaria are essentially normal. The mandibular angle is more cleft palate repair allows for some mandibular growth
obtuse than normal and the ramus is deficient. The coronoid to occur prior to the surgical narrowing of the airway
and condyloid processes are flat or aplastic. There is hypoplasia with repair of the palate.
10. Microtia is addressed at age 5 to 7 years, which is
and a retropositioned tongue. There occur difficulties with
when the external ear is approximately 80-90 percent
swallowing and feeding (caused by musculoskeletal
of adult size and rib cartilage is of sufficient volume
underdevelopment and a cleft palate).
to use as graft material. The ear is constructed in 3
stages; the first stage is the most involved.
Management Autologous costochondral grafts are usually
1. In patients with severe manifestations in which airway harvested from the fifth, sixth, and seventh rib.
inadequacy is the prominent feature after birth, a 11. Osteotomies and bone grafts address the long
tracheostomy is performed. To relieve airway midface, short mandible, and lateral facial clefts.
obstruction, a nasal continuous positive airway 12. The Le Fort osteotomy II is performed to derotate
pressure device may be needed. the middle face with the nasofrontal junction as the
2. In patients with severe swallowing difficulty, introduce fulcrum. The maxilla and nasal bones are disjoined
feeding by gavage or even through a gastrostomy from the cranium to shorten the anterior midface
tube to ensure adequate caloric intake and hydration. while lengthening the posterior facial height.
3. Fit hearing aids shortly after birth if the patient has 13. A compensatory mandibular osteotomy is performed
substantial conductive hearing loss. Hearing aids are to both vertically and horizontally lengthen the jaw
important for the development of the infant’s and equilibrate the dental occlusion. Bone grafts fill
communication skills and for the normal bonding in the congenital defects at the lateral orbital rim,
process within the family. zygoma, and malar prominence. This stage of
278 Essentials of Pediatric Oral Pathology

reconstruction is the most invasive and physically as has a delay in hypoglossal nerve conduction. The
taxing on the patient. Additional procedures such as spontaneous correction of the majority of cases with age
rhinoplasty and genioplasty (chin advancement) can supports this theory.
be performed after the major osteotomies. 3. The rhombencephalic dysneurulation theory: In this theory,
the motor and regulatory organization of the rhombence-
PIERRE ROBBIN SYNDROME (FIG. 10.34) phalus is related to a major problem of ontogenesis.

Lannelongue and Menard first described Pierre Robin CLINICAL FEATURES

syndrome in 1891 in a report on two patients with micrognathia,
cleft palate, and retroglossoptosis.44 In 1923, Pierre Robin • Micrognathia is reported in the majority of cases (91.7%).
published the case of an infant with the complete syndrome. It is characterized by retraction of the inferior dental arch
Until 1974, the triad was known as Pierre Robin syndrome; 10 to 12 mm behind the superior arch. The mandible has a
however, the term syndrome is now reserved for those errors small body, obtuse gonial angle, and a posteriorly located
of morphogenesis with the simultaneous presence of multiple condyle. The growth of the mandible catches up during the
anomalies caused by a single etiology. The term sequence has first year; however, mandibular hypoplasia resolves and the
been introduced to include any condition that includes a series child attains a normal profile by approximately age 5 to 6
of anomalies caused by a cascade of events initiated by a single years.
malformation. • Glossoptosis is noted in 70 to 85 percent of reported cases.
Three pathophysiological theories exist to explain the Macroglossia and ankyloglossia are relatively rare findings,
occurrence of Pierre Robin sequence. noted in 10 to 15 percent of reported cases.
1. The mechanical theory: This theory is the most accepted. • The combination of micrognathia and glossoptosis may
The initial event, mandibular hypoplasia, occurs between cause severe respiratory and feeding difficulty in the
the 7th and 11th week of gestation. This keeps the tongue newborn. Obstructive sleep apnea may also occur.
high in the oral cavity, causing a cleft in the palate by • The most common otic anomaly is otitis media, occurring
preventing the closure of the palatal shelves. This theory 80 percent of the time, followed by auricular anomalies in
explains the classic inverted U-shaped cleft and the absence 75 percent of cases. Hearing loss, mostly conductive, occurs
of an associated cleft lip. Oligohydramnios could play a in 60 percent of patients, while external auditory canal
role in the etiology since the lack of amniotic fluid could atresia occurs in only 5 percent of patients.
cause deformation of the chin and subsequent impaction • Nasal deformities are infrequent and consist mostly of
of the tongue between the palatal shelves. anomalies of the nasal root. Dental and philtral malforma-
2. The neurological maturation theory: A delay in neurological tions occur in one-third of cases.
maturation has been noted on electromyography of the • Speech defects occur frequently in patients with Pierre
tongue musculature, the pharyngeal pillars, and the palate, Robin sequence. Velopharyngeal insufficiency is usually
more pronounced in these patients than in those with
isolated cleft palate.
• Cardiovascular findings such as benign murmurs,
pulmonary stenosis, patent ductus arteriosus, patent
foramen ovale, atrial septal defect, and pulmonary
hypertension have all been documented.
• Anomalies involving the musculoskeletal system include
syndactyly, dysplastic phalanges, polydactyly, clinodactyly,
hyperextensible joints, and oligodactyly in the upper limbs.
In the lower extremities, foot anomalies (clubfeet,
metatarsus adductus), femoral malformations (coxa varus
or valgus, short femur), hip anomalies (flexure contractures,
congenital dislocation), anomalies of the knee (genu valgus,
synchondrosis), and tibial abnormalities have been
reported. Vertebral column deformities include scoliosis,
kyphosis, lordosis, vertebral dysplasia, sacral agenesis, and
coccygeal sinus.
• Central nervous system (CNS) defects such as language
FIGURE 10.34: Pierre Robin syndrome showing delay, epilepsy, neurodevelopmental delay, hypotonia, and
severe mandibular retrognathism hydrocephalus may occur.
Bone Pathology in Children 279

• Genitourinary defects may include undescended testes has a gaping defect, extending from the glabellar area to
(25%), hydronephrosis (15%), and hydrocele (10%). the posterior fontanelle via the metopic suture area, anterior
• Associated syndromes and conditions include Stickler fontanelle, and sagittal suture area. The skull with a gaping
syndrome, trisomy 11q syndrome, trisomy 18 syndrome, midline defect appears to permit adequate accommodation
velocardiofacial (Shprintzen) syndrome, deletion 4q of the growing brain. The lambdoidal sutures appear normal
syndrome, rheumatoid arthropathy, hypochondroplasia, in all cases.
Möbius syndrome, and CHARGE association. • During the first 2 to 4 years of life, the midline defect is
obliterated by coalescence of the enlarging bony islands
Management without evidence of any proper formation of sutures. An
1. Children with severe micrognathia may have extreme short squama and orbital part of the frontal bone
significant respiratory obstruction at birth, requiring a together with the posterior convexity of the coronal bone
nasopharyngeal airway or intubation. condensation line suggest that growth inhibition in the
2. For most newborns, the earliest physical problem sphenofrontal and coronal suture area has its onset very
involves feeding. The cleft hampers the generation early in fetal life.
of enough negative pressure to nurse. The milk or • Unique fibroblast growth factor receptor 2 (FGFR2)
formula has to be delivered through a bottle with a mutations lead to an increase in the number of precursor
nipple that has a large hole cut into the top to make cells that enter the osteogenic pathway. Ultimately, this
the delivery effortless. leads to increased subperiosteal bone matrix formation and
3. The cleft palate team includes pediatricians,
premature calvaria ossification during fetal development.
otolaryngologists, plastic surgeons, pedodontists,
The order and rate of suture fusion determine the degree
orthodontists, nurses, speech therapists, audiologists,
and social workers. The most common procedure is
of deformity and disability. Once a suture becomes fused,
the single-stage palate (hard and soft) closure, growth perpendicular to that suture becomes restricted, and
performed when the child is aged 6 to 18 months. the fused bones act as a single bony structure. Compen-
4. Although many different surgical procedures have satory growth occurs at the remaining open sutures to allow
been described, tracheostomy remains the most continued brain growth; however, complex, multiple sutural
widely used technique. Other surgical procedures, synostosis frequently extends to premature fusion of the
such as subperiosteal release of the floor of the sutures at the base of the skull, causing midfacial
mouth, and different types of glossopexy, such as the hypoplasia, shallow orbits, a foreshortened nasal dorsum,
Routledge procedure or other forms of tongue-lip maxillary hypoplasia, and occasional upper airway
adhesions, can be used. Any glossopexy should be
obstruction (Fig. 10.39).
released before significant dentition develops (age 9-
12 mo). Mandibular lengthening by gradual distraction
CLINICAL FEATURES
may be used for severe mandibular hypoplasia that
causes obstructive apnea.45 • Apert syndrome has no sex predilection.
5. As the therapy of choice to correct the conductive • Apert syndrome is detected in the newborn period due to
hearing loss and prevent middle ear complications, craniosynostosis and associated findings of syndactyly in
tympanostomy tubes are usually inserted when the the hands and feet.
palatoplasty is performed. • Craniostenosis is present. Coronal sutures most commonly
are involved, resulting in acrocephaly, brachycephaly,
APERT SYNDROME turribrachycephaly, flat occiput, and high prominent
• Apert syndrome is named after the French physician who forehead.
described the syndrome acrocephalosyndactylia in 1906.46 • Large late-closing fontanels are observed.
• Apert syndrome is a rare autosomal dominant disorder • A gaping midline defect is present.
characterized by craniosynostosis, craniofacial anomalies, • A rare cloverleaf skull anomaly is present in approximately
and severe symmetrical syndactyly (cutaneous and bony 4 percent of infants.
fusion) of the hands and feet. • Common facial features during infancy include horizontal
• During early infancy, the coronal suture area is prematurely grooves above the supraorbital ridges that disappear with
closed. A bony condensation line beginning at the cranial age, a break in the continuity of the eyebrows, and a
base and extending upward with a characteristic posterior trapezoid-shaped mouth at rest.
convexity represents this occurrence. Anterior and posterior • A flattened, often asymmetric face is observed.
fontanelles are widely patent. The midline of the calvaria • Maxillary hypoplasia with retruded midface is present.
280 Essentials of Pediatric Oral Pathology

• Patients have apparent low-set ears with occasional

conductive hearing loss and congenital fixation of stapedial
footplate.
• Eyes exhibit down-slanting palpebral fissures,
hypertelorism, shallow orbits, proptosis, exophthalmos,
strabismus, (Fig. 10.35) amblyopia, optic atrophy, and,
rarely, luxation of the eye globes, keratoconus, ectopic
lentis, congenital glaucoma, lack of pigment in the fundi
with occasional papilledema, and preventable visual loss
or blindness.
• The nose has a markedly depressed nasal bridge. It is short
and wide with a bulbous tip, parrot-beak appearance, and
choanal stenosis or atresia (Fig. 10.36).
• The mouth area has a prominent mandible, down-turned
corners, high arched palate, bifid uvula, and cleft palate.
• Orthodontic problems include crowded upper teeth,
malocclusion, delayed dentition, ectopic eruption, shovel-
shaped incisors, supernumerary teeth, V-shaped maxillary FIGURE 10.35: Extraoral photograph of Apert syndrome
dental arch, bulging alveolar ridges, dentitio tarda, some showing strabismus and hypertelorism
impaction, partial eruption, idiopathic root resorption,
transposition or other aberrations in the position of the tooth
germs, and severe crowding (Figs 10.37 and 10.38).
• The upper limbs are more severely affected than lower
limbs.
• Syndactyly involves the hands and feet with partial-to-
complete fusion of the digits, often involving second, third,
and fourth digits. These are often termed mitten hands and
sock feet. In severe cases, all digits are fused, with the palm
deeply concave and cup-shaped and the sole supinated (Figs
10.39 and 10.40).
• Hitchhiker posture or radial deviation of short or broad
thumbs results from abnormal proximal phalanx.
• Common CNS malformations include megalencephaly,
agenesis of the corpus callosum, malformed limbic
structures, variable ventriculomegaly, encephalocele, gyral
abnormalities, hypoplastic cerebral white matter, pyramidal
tract abnormalities, and heterotopic gray matter. Progressive
hydrocephalus is uncommon. FIGURE 10.36: Extraoral photograph of Apert syndrome showing
midface hypoplasia with a relative mandibular prognathism and
• Papilledema and optic atrophy with loss of vision may be
depressed nasal bridge
present in cases of subtle increased intracranial pressure.
• Congenital cervical spinal fusion (68%), especially C5-C6 • Hyperkeratosis in the plantar surface
(Figs 10.41A and B) • Paronychial infections (more common in feet than hands
• Aplasia or ankylosis of shoulder, elbow, and hip joints and in patients who are institutionalized)
• Tracheal cartilage anomalies • Excessive skin wrinkling of forehead
• Rhizomelia • Skin dimples at knuckles, shoulders, and elbows
• Hyperhidrosis (common) • Atrial septal defect
• Synonychia • Patent ductus arteriosus
• Brittle nails • Ventricular septal defect
• Acneiform lesions (frequent after adolescence) • Pulmonary stenosis
• Interruption of the eyebrows • Overriding aorta
• Hypopigmentation • Coarctation of aorta
Bone Pathology in Children 281

A B

FIGURES 10.37A and B: Intraoral photograph of Apert syndrome showing high arched palate with pseudocleft, ectopic teeth
eruption, crowding of teeth and malformed molars, macrodontia of premolars, crowding of teeth and Class III malocclusion

A B
FIGURES 10.38A and B: Panoramic radiograph of Apert syndrome showing over retained teeth, impacted teeth, macrodontia of premolars
and prominent gonial angle with vertical ramus of the mandible and three-dimensional computerized tomography showing craniostenosis

A B
FIGURES 10.39A and B: Both hands of Apert syndrome showing syndactyly of 2nd, 3rd and 4th digits and both feet showing
syndactyly of all digits
282 Essentials of Pediatric Oral Pathology

A B
FIGURES 10.40A and B: Hands X-ray (left) of Apert syndrome showing syndactyly of 2nd, 3rd and
4th digits and feet X-ray (right) showing syndactyly of all the toes

A B
FIGURES 10.41A and B: Skull radiograph on left of Apert syndrome revealing brachycephaly with characteristic beaten metal
appearance and cervical spine X-ray on right showing congenital fusion of neural arches of C3-C4 and C5-C6-C7

• Dextrocardia • Partial biliary atresia with agenesis of gall bladder

• Tetralogy of Fallot • Anomalous tracheal cartilage
• Endocardial fibroelastosis • Tracheoesophageal fistula
• Polycystic kidneys • Pulmonary aplasia
• Duplication of renal pelvis • Absent right middle lobe of lung
• Hydronephrosis • Absent interlobular lung fissures.
• Stenosis of bladder neck
• Bicornuate uterus Management
• Vaginal atresia
1. Protection of the cornea.
• Protuberant labia majora 2. Instill lubricating bland ointments in the eyes at
• Clitoromegaly bedtime to protect corneas from desiccation.
• Cryptorchidism 3. Artificial teardrops during the day.
• Pyloric stenosis 4. Remove excessive nasal secretions.
• Esophageal atresia and tracheoesophageal fistula 5. Treat upper airway infection.
• Ectopic or imperforate anus 6. Humidification with added oxygen.
Bone Pathology in Children 283

7. Judicious use of topic nasal decongestants.

8. Sleep apnea
9. Polysomography (a sleep recording of multiple
physiologic variables), currently the most reliable
method for determining the presence of sleep apnea.
10. Continuous positive pressure.
11. Chronic middle ear effusion associated with bilateral
conductive hearing deficit - Antimicrobial therapy.
12. Psychological and social challenges confronted by
individuals with Apert syndrome.
13. Emotional adjustment.
14. Cranial surgery.
15. Facial cosmetic reconstruction.
16. A new technique of craniofacial disjunction, followed
by gradual bone distraction (Ilizarov procedure), has FIGURE 10.42: Frontal view of the arches during orthodontic
been reported to produce complete correction of treatment
exophthalmos and improvement in the functional and
aesthetic aspects of the middle third of the face without
the need for bone graft in patients aged 6-11 years.
17. Surgical separation of digits (mitten-glove syndactyly)
provides relatively little functional improvement
Shunting procedure reduces intracranial pressure.
18. For orthodontic treatment, most patients require 2-jaw
surgery (bilateral sagittal split osteotomy with
mandibular setback and distraction in the maxilla).
19. Alternatively, orthodontic treatment may comprise of
alignment and levelling of the arches using 0.014
CuNiTi followed by 0.016 NiTi and other suitable
management of local malpositioning of teeth (Fig.
10.42). FIGURE 10.43: Patient holding the custom made toothbrush for
improvement of manual dexterity
Custom made toothbrushes may be provided to improve
the manual dexterity of the patient (Fig. 10.43).
• TD1: Amino acid substitutions in the extracellular domain
THANATOPHORIC DYSPLASIA of FGFR3 have resulted in TD1. The most common
Thanatophoric dysplasia (TD) is the most common form of mutation in TD1 is arg248cys, which is present in
skeletal dysplasia that is lethal in the neonatal period. The term approximately 50 percent of patients.
thanatophoric derives from the Greek word thanatophorus, • TD2: In patients with TD2, lys650blu is the only reported
which means “death bringing”. Characteristics of thanatophoric gene mutation.
dysplasia include severe shortening of the limbs, a narrow Patients with TD2 have a single recurrent mutation (A-to-
thorax, macrocephaly, and a normal trunk length. G) in the tyrosine kinase domain of FGFR3, but patients with
Thanatophoric dysplasia is divided into two clinically defined TD1 have different mutations that affect either the extracellular
subtypes. Thanatophoric dysplasia type 1 (TD1), the most or intracellular domains of FGFR3. The most common TD1
mutation is a C-to-T transition, which results in a change of
common subtype, is characterized by a normal-shaped skull
arginine to cysteine in the extracellular domain of FGFR3.
and curved long bones (shaped like a telephone receiver); the
femurs are most affected. Thanatophoric dysplasia type 2 (TD2)
CLINICAL FEATURES
is associated with a cloverleaf-shaped skull and straight femurs.
Some clinical overlap exists between the two subtypes. • Males and females are equally affected.
Autosomal dominant mutations in the fibroblast growth factor • Thanatophoric dysplasia is lethal in neonates; however,
receptor three gene (FGFR3), which has been mapped to long-term survival has been reported.
chromosome band 4p16.3, results in both subtypes. The vast • Severe growth deficiency with an average length of 40 cm
majority of cases are due to de novo mutations. at term.
The following mutations that affect distinct domains of • A macrocephalic head with a frontal bossing, a flattened
FGFR3 cause the thanatophoric dysplasia subtypes: nasal bridge, and proptotic eyes.
284 Essentials of Pediatric Oral Pathology

• In TD2, a cloverleaf-shaped skull due to premature closure Achondroplasia is caused by mutations in the fibroblast
of the cranial sutures. growth factor receptor-3 (FGFR3) gene. This gene has been
• Narrow thorax with small ribs. mapped to chromosome 4, band p16.3 (4p16.3).
• Micromelic limbs with brachydactyly.
• Protuberant abdomen. CLINICAL FEATURES
• Hydrocephalus and other cerebral parenchymal
• It is usually seen in children.
abnormalities.
• Males and females are equally affected.
• Hypotonia in infancy and early childhood.
Management
• Delayed motor milestones.
1. Inpatient care is necessary. Intubation may be • Normal intelligence with possible minor deficit in visual-
performed as aggressive treatment for respiratory spatial tasks.
distress. • Large calvarial bones in contrast to the small cranial base
2. If aggressive treatment is deferred, palliative treatment and facial bones.
consists of keeping the infant warm, comfortable, and
• True megalencephaly (large head) with frontal bossing
nourished.
• Midface hypoplasia.
• Dental malocclusion and crowding.
ACHONDROPLASIA
• Disproportionate short stature.
Achondroplasia, a nonlethal form of chondrodysplasia, is the • Normal trunk length that appears long and narrow, small
most common form of short-limb dwarfism. It is inherited as a thoracic cage.
Mendelian autosomal dominant trait with complete penetrance. • Rhizomelic shortening of the proximal limbs with
Approximately 80 percent of cases are due to new or de novo redundant skin folds.
dominant mutations with a mutation rate estimated to be • Brachydactyly and trident hand configuration: A marked
0.000014 per gamete per generation. Salient phenotypic separation between the ring and middle fingers giving the
features include disproportionate short stature, megalencephaly, hand a 3-pronged appearance.
a prominent forehead (frontal bossing), midface hypoplasia, • Thoracolumbar gibbus (lumbar kyphosis) in infancy, which
rhizomelic shortening of the arms and legs, a normal trunk is replaced by an exaggerated lumbar lordosis once
length, prominent lumbar lordosis, genu varum, and a trident ambulation begins.
hand configuration (Fig. 10.44). • Hyperextensibility of most joints, especially the knees.
• Limited elbow extension and rotation.
• Genu varum (bow legs).

RADIOGRAPHIC FEATURES
• Contracted skull base.
• Progressive interpedicular narrowing in the lumbar spine
region.
• Short pedicles which can cause spinal stenosis.
• Short femoral neck and metaphyseal flaring.
• Dysplastic ilium, narrow sacroiliac groove, flat-roofed
acetabula.

Management
1. Closely monitor growth in patients with achondro-
plasia.
2. Perform careful neurologic examinations.
3. Manage frequent middle ear infections and address
any concerns for hearing loss.
4. Orthodontic treatment is recommended for dental
malocclusion and crowding.
5. Control weight to prevent obesity and associated co-
FIGURE 10.44: Achondroplasia showing rhizomelic
morbidities.
shortening of the arms and legs
Bone Pathology in Children 285

6. Therapy is performed using growth hormone (GH) to • Other craniofacial and oral features include hypertelorism
foster linear growth. (wide apart eyes), short upturned nose, broad nasal bridge
7. Anti-inflammatory agents, such as nonsteroidal anti- (wide, flat nose), anteverted mares (upturned nose),
inflammatory drugs (NSAIDs), are useful to alleviate triangular mouth (bottom corners face downward), frontal
pain and inflammation for patients with degenerative
bossing (boxlike forehead), long/short philtrum (upper lip),
joint disease.
8. Leg-lengthening procedures using distraction
micrognathia (small chin), wide palpebral fissures (wide
osteogenesis have been performed successfully. eye openings), down slanting palpebral fissures (slanted
Successful height increase is reported to be 12 to 14 eyes), ear abnormality (small and lower set), facial nevus
inches. Better outcomes are reported with the use of (birthmark on face), normal intelligence, dental
the Orthofix Garches lengthening device along with abnormalities/malaligned teeth (crowded, crooked, missing
tenotomy of the Achilles tendon and syndesmosis, teeth), gingival hyperplasia (thick gums), abnormal uvula
which provide the fewest complications with healing
(heart shaped), cleft lip/palate (non-midline) and shortened
indices similar to those of other operative protocols.
Another procedure associated with fewer
tongue/some with midline indentation.
complications in children and adolescents younger • Though the eyes do not protrude, abnormalities in the lower
than 14 years is tibia, rather than femur, lengthening. eyelid may give that impression. Surgery may be necessary
9. In children with signs of craniomedullary compression, if the eyes cannot close fully. In addition, the ears may be
surgical treatment to release the compression can set low on the head or have a deformed pinna.
improve neurologic, cognitive, and respiratory • Patients suffer from dwarfism, short lower arms, small feet,
functions. Indications for the possible need for and small hands. Fingers and toes may also be abnormally
suboccipital decompression include lower limb
short and laterally or medially bent. The thumb may be
hyperreflexia or clonus on examination, central
displaced and some patients, notably in Turkey, experience
hypopnea demonstrated by polysomnography, and
foramen magnum measurements lower than the mean. ectrodactyly. All patients often suffer from vertebral
10. Lumbar laminectomy can be performed for spinal segmentation abnormalities. Those with the dominant variant
stenosis, which is a condition that usually manifests have, at most, a single butterfly vertebra. Those with the
in early adulthood. recessive form, however, may suffer from hemivertebrae,
vertebral fusion, and rib anomalies. Some cases resemble
ROBINOW SYNDROME Jarcho-Levin syndrome or spondylocostal dysostosis.
• Genital defects characteristically seen in males include a
Robinow syndrome is an extremely rare genetic disorder
characterized by short-limbed dwarfism, abnormalities in the micropenis with a normally developed scrotum and testes.
head, face, and external genitalia, as well as vertebral Sometimes, testicles may be undescended, or the patient
segmentation. The disorder was first described in 1969 by may suffer from hypospadias. Female genital defects may
human geneticist Meinhard Robinow, along with physicians include a reduced size clitoris and underdeveloped labia
Frederic N Silverman and Hugo D Smith, in the American minora. Infrequently, the labia majora may also be
Journal of Diseases of Children.47 underdeveloped. Some research has shown that females
Two forms of the disorder exist, dominant and recessive, may experience vaginal atresia or hematocolpos.
of which the former is more common. Patients with the • The autosomal recessive form of the disorder tends to be
dominant version often suffer moderately from the aforemen- much more severe. Examples of differences are summarized
tioned symptoms. Recessive cases, on the other hand, are
in the Table 10.4.48
usually more physically marked, and individuals may exhibit
more skeletal abnormalities. TABLE 10.4: Difference between autosomal dominant and
Genetic studies have linked the autosomal recessive form recessive Robinow syndrome
of the disorder to the ROR2 gene on position nine of the long
Characteristic Autosomal dominant Autosomal recessive
arm of chromosome nine. The gene is responsible for aspects
of bone and cartilage growth. This same gene is involved in Stature More than 2 SD shorter Short or normal
causing autosomal dominant brachydactyly.
Arms Very short Slightly short

CLINICAL FEATURES Elbow Radial head dislocation No dislocation

• Robinow noted the resemblance of affected patients’ faces Upper lip Very broad Tented
to that of a fetus, using the term “fetal facies” to describe Mortality rate Normal 10%
the appearance of a small face and widely spaced eyes.
286 Essentials of Pediatric Oral Pathology

Management Bony changes in Van Buchem disease are consistent with

increased bone formation, but clear evidence of increased bone
There is no specific treatment for Robinow syndrome, but formation has not been obtained.
for ROR2-related Robinow syndrome, specific treatment
modalities are as follows: Management
• Orofacial assessment to determine the need for
plastic surgery. There is no specific treatment for this disorder.
• Dental assessment for misaligned, crowded teeth.
• Radiographic documentation of the forearm CHONDROECTODERMAL DYSPLASIA
shortening and hand anomalies.
• In 1940, Ellis and van Creveld (Ellis and van Creveld,
• Micropenis assessment for reconstructive surgery.
1940) formally described the syndrome that would bear their
• Corrective surgery may be necessary for the eyes if
names, although they termed it chondroectodermal dysplasia.52
they cannot close fully.
• Pathophysiology of this syndrome is unknown; however,
• Several corrective surgeries are usually required for the
limb and spine defects and facial abnormalities.
recent identification of the EVC gene should lead to a better
• In individuals with growth hormone deficiency, understanding.53
response to growth hormone therapy is good and near
normal height can be attained. CLINICAL FEATURES
• Injection of human chorionic gonadotropin to improve • Frequency of Ellis-van Creveld syndrome is equal in males
penile length and testicular volume is recommended. and females.
Hormone therapy should be monitored by a pediatric • In patients with Ellis-van Creveld syndrome, physical
endocrinologist. findings, such as disproportionate extremities, small stature,
• Orthodontic treatment is usually required. polydactyly, cardiac defects, and minor dysmorphic
features, are seen at birth.
HYPEROSTOSIS CORTICALIS GENERALISATA • A clinical tetrad of Ellis-van Creveld syndrome consists of
chondrodystrophy, polydactyly, ectodermal dysplasia, and
First documented in 1955 by Van Buchem, the skeletal cardiac anomalies.
condition that carries his name was categorized as hyperostosis • Chondrodystrophy (the most common feature affecting the
corticalis generalisata.49 Van Buchem disease is an autosomal tubular bones):
recessive bone dysplasia linked to a genetic locus on — Disproportionate dwarfism (small stature of prenatal
chromosome 17q12–21.50 onset; average adult height, 109–155 cm).
— Progressive distal limb shortening, symmetrically
CLINICAL FEATURES affecting the forearms and lower legs.
This disease is characterized by a symmetrically increased • Polydactyly (Fig. 10.45):
— Bilateral and postaxial.
thickness of bones, most frequently found as an enlarged
— Polydactyly, observed in the hands in most cases but
jawbone, but also an enlargement of the skull, ribs, diaphysis
in the feet in 10% of cases.
of long bones, as well as tubular bones of hands and feet,
resulting in increased cortical bone density.
The clinical consequences of increased thickness of the
skull include facial nerve palsy causing hearing loss, visual
problems, neurological pain, and, very rarely, blindness as a
consequence of optic atrophy.
The most common radiological features are massive
hyperostosis of the calvarium and mandible, resulting in
increased weight of skull and sclerosis of the diaphyses of the
long bones, clavicles, ribs, and pelvis with disruption of bone
contours resulting in a very rough bone surface.51
Bone anomalies are symmetric, appearing in the first decade
of life and becoming more prominent among the oldest patients, FIGURE 10.45: Ellis-van Creveld syndrome
suggesting progression of disease with aging. showing polydactyly
Bone Pathology in Children 287

• Hidrotic ectodermal dysplasia (observed in as many as 93% Variable chondrocyte disorganization was seen in the central
of cases): physeal growth zone of the vertebrae.
— Nails are hypoplastic, dystrophic, and friable. Nails can
be completely absent in some cases. Management
— Tooth involvement may include neonatal teeth, partial
1. The management of Ellis-van Creveld (EVC)
anodontia, small teeth, and delayed eruption. Enamel syndrome is multidisciplinary.
hypoplasia may result in abnormally shaped teeth with 2. Care for respiratory distress, recurrent respiratory
frequent malocclusion. infections, and cardiac failure is supportive.
— Hair may occasionally be sparse. 3. Dental care in childhood includes the following:
• Congenital cardiac anomalies: – Neonatal teeth should be removed because they may
— Heart defects occur in 50 to 60% of patients; the most impair feeding.
common anomaly is a common atrium (40%). – Prevention of caries includes dietary counseling,
— Other cardiac anomalies include atrioventricular canal, plaque control, and oral hygiene instruction.
ventricular septal defect, atrial septal defect, and patent – Crown or composite build-ups for microdonts may be
ductus arteriosus. indicated.
— The cardiac anomaly is the major cause of shortened – Partial dentures can maintain space and improve
life expectancy. mastication, esthetics, and speech due to
• Other anomalies may also be present. congenitally missing teeth.
– For dental care during adulthood, implants and
• Musculoskeletal anomalies include low-set shoulders, a
prosthetic rehabilitation are required to replace
narrow thorax frequently leading to respiratory difficulties,
congenitally missing teeth.
knock knees, lumbar lordosis, broad hands and feet, and 4. In cases of short stature considered as a result of
sausage-shaped fingers. chondrodysplasia of the legs, a possible treatment with
• Oral lesions include the following: growth hormone is considered ineffective.
— A fusion of the anterior portion of the upper lip to the 5. Orthopedic procedures correct polydactyly and other
maxillary gingival margin, resulting in an absence of orthopedic malformations.
mucobuccal fold and the upper lip to present a slight 6. Cardiac surgery may be needed to correct cardiac
V-notch in the middle. anomalies.
— Short upper lip, bound by frenula to alveolar ridge (lip 7. Thoracic expansion has been attempted in some
tie). patients.
8. Dental care is usually necessary.
— Often serrated lower alveolar ridge.
9. Urologic surgery is required if epispadias, cryptor-
— Teeth may be prematurely erupted at birth or exfoliate
chidism, or both are present.
prematurely. 10. Perioperative morbidity may result from difficulties with
• Occasional genitourinary anomalies include hypospadias, airway management and pulmonary abnormalities.
epispadias, hypoplastic penis, cryptorchidism, vulvar
atresia, focal renal tubular dilation in medullary region,
TRICHODENTO-OSSEOUS SYNDROME
nephrocalcinosis, renal agenesis, and megaureters.
• Occasionally, CNS anomalies or mental retardation are A rare genetic disorder characterized by kinky hair, tooth enamel
present. and bone abnormalities. There are two different subtypes with
• Clinical manifestations in heterozygous carriers: type I being distinguished from type II by the presence of a small
— Polydactyly has been reported in relatives of four head and increased density in the long bones.
unrelated Ellis-van Creveld syndrome families.
— A father of a child with Ellis-van Creveld syndrome who CLINICAL FEATURES
had finger and teeth abnormalities has been reported, as
• Kinky hair
have several other reports of symptomatic heterozygous
• Small teeth
manifestations.
• Widely spaced teeth (Fig. 10.46)
• Pitted teeth (Fig. 10.46)
HISTOPATHOLOGIC FEATURES
• Poor tooth enamel
Histopathologic examination of fetuses with Ellis-van Creveld • Increased tooth pulp chamber size (Fig. 10.47)
syndrome revealed that the cartilage of long bones showed • Frontal bossing
chondrocyte disorganization in the physeal growth zone. • Long head
288 Essentials of Pediatric Oral Pathology

FIGURE 10.46: Trichodento-Osseous syndrome showing pitted

and widely spaced teeth

FIGURE 10.48: Down syndrome showing mongoloid facie

DOWN SYNDROME (FIG. 10.48)

• In 1866, Down described clinical characteristics of the
syndrome that now bears his name.54
• In 1959, Lejeune and Jacobs et al independently determined
that trisomy 21 is the cause.55
• Down syndrome is by far the most common and best known
FIGURE 10.47: Trichodento-Osseous syndrome showing large chromosomal disorder in humans and the most common
pulp chambers cause of intellectual disability.
• Mental retardation, dysmorphic facial features, and other
• Squarish jaw distinctive phenotypic traits characterize the syndrome.
• Increased bone density • The extra chromosome 21 affects almost every organ
• Delayed bone age system and results in a wide spectrum of phenotypic
• Round face consequences. These include life-threatening complications,
• Brittle nails clinically significant alteration of life course (e.g. mental
• Superficial nail peeling retardation), and dysmorphic physical features. Down
• Dense long bones syndrome decreases prenatal viability and increases
• Thin nails prenatal and postnatal morbidity. Affected children have
• Premature nails delays in physical growth, maturation, bone development,
• Thickened top portion of skull and dental eruption.
• Small head • The extra copy of the proximal part of 21q22.3 appears to
• Poorly pneumatized mastoids result in the typical physical phenotype: mental retardation,
• Obliterated frontal sinuses characteristic facial features, hand anomalies, and
• Undertubulated clavicles. congenital heart defects. Molecular analysis reveals that the
21q22.1-q22.3 region, or Down syndrome critical region
RADIOGRAPHIC FEATURES (DSCR), appears to contain the gene or genes responsible
for the congenital heart disease observed in Down
Dental radiographs of this disorder show large pulp chambers
syndrome. A new gene, DSCR1, identified in region
and sometimes numerous unerupted teeth are evident.
21q22.1-q22.2, is highly expressed in the brain and the
heart and is a candidate for involvement in the pathogenesis
Management
of Down syndrome, particularly, in mental retardation and/
1. There is no specific treatment for this disorder. or cardiac defects.56
2. Treatment is aimed at cosmetic reconstruction. • Abnormal physiologic functioning affects thyroid
metabolism and intestinal malabsorption. Frequent infections
Bone Pathology in Children 289

are presumably due to impaired immune responses, and the • Brachycephaly, microcephaly, a sloping forehead, a flat
incidence of autoimmunity, including hypothyroidism and occiput, large fontanels with late closure, a patent metopic
rare Hashimoto thyroiditis, is increased. suture, absent frontal and sphenoid sinuses, and hypoplasia
• Patients with Down syndrome have decreased buffering of of the maxillary sinuses occur.
physiologic reactions, resulting in hypersensitivity to • Up-slanting palpebral fissures, bilateral epicanthal folds,
pilocarpine and abnormal responses on sensory-evoked Brushfield spots (speckled iris), refractive errors (50%),
electroencephalographic tracings. Children with leukemic strabismus (44%), nystagmus (20%), blepharitis (33%),
Down syndrome also have hyperreactivity to methotrexate. conjunctivitis, tearing from stenotic nasolacrimal ducts,
Decreased buffering of metabolic processes results in a congenital cataracts (3%), pseudopapilledema, spasm
predisposition to hyperuricemia and increased insulin nutans, acquired lens opacity (30-60%), and keratoconus
resistance. Diabetes mellitus develops in many affected in adults are observed.
patients. Premature senescence causes cataracts and • Hypoplastic nasal bone and flat nasal bridge are typical
Alzheimer disease. Leukemoid reactions of infancy and an characteristics.
increased risk of acute leukemia indicate bone-marrow • An open mouth with a tendency of tongue protrusion, a
dysfunction. fissured and furrowed tongue, mouth breathing with
• Children with Down syndrome are predisposed to drooling, a chapped lower lip, angular cheilitis, partial
developing leukemia, particularly transient myeloprolife- anodontia (50%), tooth agenesis, malformed teeth, delayed
rative disorder and acute megakaryocytic leukemia. Nearly tooth eruption, microdontia (35-50%) in both the primary
all children with Down syndrome who develop these types and secondary dentition, hypoplastic and hypocalcified
of leukemia have mutations in the hematopoietic teeth, malocclusion, taurodontism (0.54-5.6%), and
transcription factor gene, GATA1. Leukemia in children increased periodontal destruction are noted.
with Down syndrome requires at least three cooperating • The ears are small with an overfolded helix. Chronic otitis
events: trisomy 21, a GATA1 mutation, and a third media and hearing loss are common.
undefined genetic alteration. • Atlantoaxial instability (14%) can result from laxity of
transverse ligaments that ordinarily hold the odontoid
CLINICAL FEATURES
process close to the anterior arch of the atlas. Laxity can
• The male-to-female ratio is increased (approximately cause backward displacement of the odontoid process,
1.15:1) in newborns with Down syndrome. This effect is leading to spinal cord compression in about two percent
restricted to free trisomy 21. of children with Down syndrome.
• Down syndrome can be diagnosed prenatally with • Congenital heart defects are common (40-50%); they are
amniocentesis, percutaneous umbilical blood sampling frequently observed in patients with Down syndrome who
(PUBS), chorionic villus sampling (CVS), and extraction are hospitalized (62%) and are a common cause of death
of fetal cells from the maternal circulation. in this aneuploidy in the first two years of life. The most
• Shortly after birth, Down syndrome is diagnosed by common congenital heart defects are endocardial cushion
recognizing dysmorphic features and the distinctive defect (43%), ventricular septal defect (32%), secundum
phenotype. atrial septal defect (10%), tetralogy of Fallot (6%), and
• Short stature and obesity occurs during adolescence.
isolated patent ductus arteriosus (4%). About 30% of
• Moderate-to-severe mental retardation occurs, with an
patients have several cardiac defects. The most common
intelligence quotient (IQ) of 20 to 85 (mean, approximately
lesions are patent ductus arteriosus (16%) and pulmonic
50). Hypotonia improves with age.
stenosis (9%). About 70% of all endocardial cushion
• Natural spontaneity, genuine warmth, cheerfulness,
gentleness, patience, and tolerance are characteristics. A few defects are associated with Down syndrome.
patients exhibit anxiety and stubbornness. • Diastasis recti and umbilical hernia occur.
• Infantile spasms are the most common seizures observed • Duodenal atresia or stenosis, Hirschsprung disease (<1%),
in infancy, whereas tonic-clonic seizures are most common Meckel diverticulum, imperforate anus, and omphalocele
in older patients. are observed. Celiac disease occurs in genetically
• Decreased skin tone, early graying or loss of hair, susceptible individuals, specifically those who have the
hypogonadism, cataracts, hearing loss, age-related increase human leukocyte antigen (HLA) heterodimers DQ2
in hypothyroidism, seizures, neoplasms, degenerative (observed in 86 to 100% of individuals with celiac
vascular disease, loss of adaptive abilities, and increased disease) and DQ8. These are strong linkages with high
risk of senile dementia of Alzheimer type are observed. sensitivity and poor specificity.
290 Essentials of Pediatric Oral Pathology

• Renal malformations, hypospadias, micropenis, and Infantile cortical hyperostosis is an inflammatory process
cryptorchidism occur. of unclear etiology. In the early stages of this condition,
• Short and broad hands, clinodactyly of the fifth fingers with inflammation of the periosteum and adjacent soft tissues is
a single flexion crease (20%), hyperextensible finger joints, observed. As this resolves, the periosteum remains thickened,
increased space between the great toe and the second toe, and subperiosteal immature lamellar bone is noted. The bone
and acquired hip dislocation (6%) are typical presentations. marrow spaces contain vascular fibrous tissue. Mature
— Hashimoto thyroiditis that causes hypothyroidism is by specimens show hyperplasia of the lamellar cortical bone
far the most common acquired thyroid disorder in without inflammation or subperiosteal changes.
patients with Down syndrome. The onset is usually from CLINICAL FEATURES
school age onwards, but onset in infancy is reported.
— Diabetes and decreased fertility can occur. • No sex predilection has been established.
• Patients have about a 12-fold increased risk of infectious • The disease may be present at birth or shortly thereafter. The
diseases, especially pneumonia, because of impaired familial form tends to have an earlier onset and is present at
birth in 24 percent of cases, with an average age at onset of
cellular immunity.
6.8 weeks.58 The average age at onset for the sporadic form
• Xerosis, localized hyperkeratotic lesions, elastosis
of infantile cortical hyperostosis is 9 to 11 weeks.
serpiginosa, alopecia areata (<10%), vitiligo, folliculitis,
• The classic presentation of infantile cortical hyperostosis
abscess formation, and recurrent skin infections are observed. includes a triad of irritability, swelling, and bone lesions.59
• Distal axial triradius in the palms, transverse palmar The swelling appears suddenly, is deep and firm, and may
creases, a single flexion crease in the fifth finger, ulnar be tender. Fever may occur.
loops (often 10), a pattern in hypothenar, and interdigital • Infantile cortical hyperostosis is often multifocal and
III regions are observed. asymmetric. The disease has been described in many bones,
• Most children with Down syndrome do not have a coexisting including the mandible, tibia, ulna, clavicle, scapula, ribs,
psychiatric or behavioral disorder. The disorders include humerus, femur, fibula, skull, scapula, ilium, and metatarsal.
attention deficit hyperactivity disorder, oppositional defiant
disorder, nonspecific disruptive disorder, autism spectrum RADIOGRAPHIC FEATURES
disorders, and stereotypical movement disorder in Dental radiographs show an ill-defined increase in bone density
prepubertal children with Down syndrome and depressive with marked cortical thickening and a wavy contour. During the
illness, obsessive-compulsive disorder, and psychotic-like healing phase, a laminated periosteal reaction may be present.
disorder in adolescents and adults with Down syndrome.
• Trisomy 21 mosaicism HISTOPATHOLOGIC FEATURES
— Trisomy 21 mosaicism can present with absent or
Lesional area shows an acute inflammatory reaction in the
minimal manifestations of Down syndrome and may be periosteum along with infiltration of the connective tissues by
underdiagnosed as a cause of early-onset Alzheimer
polymorphonuclear leukocytes. The process then extends to the
disease. adjacent soft tissues and muscles, resulting in collagen
— The phenotype of persons having mosaicism for trisomy
hyperplasia and fibrinoid degeneration. Osteoid trabeculae are
21 and Down syndrome reflects the percentage of formed and, during a subacute phase, the periosteum re-forms
trisomic cells present in different tissues.
around both the old and new bone posteriorly, and it calcifies
and appears subperiosteally. During the final stage, remodeling,
Management
the peripheral hyperostotic bone disappears by resorption.
1. Genetic counseling.
2. Vaccination and medication is recommended to Management
prevent against secondary infections.
1. No specific treatment exists for infantile cortical
3. Cosmetic surgical treatment if required.
hyperostosis. The disease is self-limited and usually
resolves without sequelae. Some periods of
INFANTILE CORTICAL HYPEROSTOSIS exacerbation and remission may occur during the
In 1945, Caffey first described infantile cortical hyperostosis, course of this condition.
also known as Caffey disease, a self-limited disorder that affects 2. Corticosteroids may be helpful in alleviating symptoms
in severe cases, but these agents do not have any
infants and causes bone changes, soft-tissue swelling, and
effect on the bone lesions. Nonsteroidal anti-inflam-
irritability.57
matory drugs (NSAIDs) may also be used to
Although the etiology of this condition is not completely treat symptoms.
understood, familial and sporadic forms appear to exist.
Bone Pathology in Children 291

MASSIVE OSTEOLYSIS (GORHAM’S DISEASE) CEMENTOBLASTOMA

Gorham’s Disease (GD) is an extremely rare bone disorder; Cementoblastoma, as distinguished from cementoma, is a true
fewer than 200 cases are reported in the medical literature. The neoplasm of cementum. It is a result of interruptions in or
etiology of Gorham disease is unknown but is thought to be reactivation of tissues involved in the normal sequence of
related to increased vascularity of the affected bones. The odontogenesis.
resultant hyperemia has been hypothesized to uncouple the
balance between osteoblasts and osteoclasts, leading to bone CLINICAL FEATURES
resorption at a far greater rate than bone formation.
This benign neoplasm is rare and is usually observed in patients
CLINICAL FEATURES younger than 25 years. It is most often found in association
with the apex of the mandibular first molars (50% of lesions),
• Gorham disease can present with dull, aching pain or and it is never found in association with the anterior dentition.
insidious weakness, and rarely is suspected prior to The lesion is usually asymptomatic, although occasionally the
radiographic evaluation. associated tooth may be slightly sensitive to percussion.
• Patients usually are younger than 40 years.
• It is also characterized by bone loss (osteolysis) often
RADIOGRAPHIC FEATURES
associated with swelling or abnormal blood vessel growth
(angiomatous proliferation). A round opaque sunburst mass attached to the apex of a tooth
• Although the disease may strike any of the bones of the that is well-demarcated and surrounded by a thin radiolucent
body, it is more often recognized earlier when the bones at rim is observed (Fig. 10.50). The lesion obscures the lamina
the top of the head (calvarium) and/or the mandibles are dura. Students sometimes confuse it with condensing osteitis,
involved. a common lesion resulting from low-grade periapical irritation
that stimulates bone growth. Although the most usual location
HISTOPATHOLOGIC FEATURES (FIG. 10.49) for the two lesions is the same, condensing osteitis does not
Lesional area shows that the normal bone is replaced by obscure the periodontal ligament (PDL) space and tends to be
connective tissue containing numerous blood vessels lined by more irregular in outline. The mature cementoma, also known
endothelial cells. as periapical cemental dysplasia, is another common lesion that
students may confuse with cementoblastoma. However,
Management cementoma is usually located in the mandibular anterior region
and does not obscure the PDL space. Cementomas actually
Radiation may help to control the progression and
have three developmental stages: osteolytic (at which point the
symptoms of Gorham disease, and steroids have been
used with variable success. lesion appears as a radiolucency), cementoblastic (mixed
radiolucent/radiopaque), and mature (radiopaque).

FIGURE 10.49: Histopathologic picture of massive osteolysis FIGURE 10.50: Cementoblastoma showing radiopaque mass
showing replacement of bone by connective tissue attached to the root apex of tooth
292 Essentials of Pediatric Oral Pathology

Management involves cartilage or bone transplants and

unilateral or bilateral sliding osteotomy.

HYPERPLASIA OF MANDIBULAR CONDYLE

It is a rare local, unilateral enlargement of mandibular condyle.
Mostly occur due to inflammation in condylar area.
Clinically, there occurs deviation of chin away from the
affected side. Severe malocclusion is common finding.
Management involves resection of the mandible.

ANKYLOSIS OF TEMPOROMANDIBULAR JOINT

Bony ankylosis of the TMJ is a disabling disease that is not
confined to the first two decades, but can occur later in life.
FIGURE 10.51: Histopathologic picture of cementoblastoma
Different causes have been attributed to the condition, such as
showing cemental trabeculae and fibrocellular connective tissue condylar fracture with involvement of the articular surface,
advanced arthritis and trauma from obstetric forceps. Ankylosis
HISTOPATHOLOGIC FEATURES (FIG. 10.51) can be classified into true (intra-articular) and false (extra-
articular).60 TMJ ankylosis could also be classified according
Plump cementoblasts separated by cemental partitions form the
to type of tissue involved, bony, fibrous or fibro-osseous, or
histology of this encapsulated lesion. Bone may show reversal
lines. Connective tissue is fibrocellular. Cemental trabeculae according to location, intra- or extra-capsular. If the cause is
at places show cementoblasts and cementoclasts. trauma, it is hypothesized that the extravasation of blood into
the joint, along with the disruption of fibrocartilage integrity,
Management permits the ingrowth of fibrous connective tissue into the joint,
which subsequently results in ossification, leading to the fusion
Removal of attached tooth and tumor is the method of of the mandibular condyle to the articular surface of the
treatment. No recurrences are reported.
temporal bone.61
The classic sign is the limitation of opening movement, as
TMJ ABNORMALITIES
these patients presented. In general, patients related a history
APLASIA OF MANDIBULAR CONDYLE of progressive restriction of opening movement up to an
unacceptable level of limitation. In some cases, the patient is
It is failure of development of mandible. It may be unilateral hampered by a complete lack of mandibular movement. Once
or bilateral. the movements of the TMJ are restricted; pain is not a
It mostly occurs in association with other defects such as characteristic symptom of ankylosis. Early ankylosis of the
absence of external ear, macrostomia and underdeveloped TMJ in children can be a deterrent to normal mandibular
mandibular ramus. growth, resulting in a mandibular hypoplasia, especially if there
Mandible is shifted towards the affected side. is a bilateral problem. The clinical and radiographic findings
Management of these conditions requires osetoplasty and are in agreement with those of Sawhney.62 Long-standing, early-
correction of malocclusion. onset ankylosis in childhood results in marked facial
asymmetry, whereas the bony changes are minimal when the
HYPOPLASIA OF MANDIBULAR CONDYLE
problem occurs during adolescence or the patient has early
Mandibular hypoplasia is a frequently encountered craniofacial treatment. Osseous ankylosis presents characteristic
difference and can be classified into three groups: congenital, radiographic features, which facilitate the diagnosis. In general,
developmental, and acquired. it is observed that the condyle is bridged with a temporal bone.
Congenital form is idiopathic and may be unilateral or It could be a small piece of bone or even a huge bone mass
bilateral. that could involve the condyle of the mandible, temporal bone
The acquired form occurs due to either forceps deliveries, and zygomatic process.
external trauma to the condylar area, X-ray radiation, infections Management is based upon various treatment modalities
of dental origin and endocrine and vitamin derangements. such as forceful opening of jaws, condylectomy, gap arthro-
There occurs facial asymmetry, mandibular midline shift plasty and interposition of material for reconstruction of dead
during opening and closing. Malocclusion is the common finding. space.
Bone Pathology in Children 293

LANGERHANS CELL HISTIOCYTOSIS proteins, and FADD, FLICE, and FLIP proteins in the Fas
signaling pathway may be involved in the pathogenesis of
Langerhans cell histiocytosis (LCH) is a group of idiopathic
Langerhans cell histiocytosis.68
disorders characterized by the proliferation of specialized, bone
marrow–derived Langerhans cells (LCs) and mature
CLINICAL FEATURES
eosinophils.
In 1868, Paul Langerhans discovered the epidermal • Langerhans cell histiocytosis affects patients from neonates
dendritic cells that now bear his name.63 The term Langerhans to adults, although it appears to be more common in
cell histiocytosis is generally preferred to the older term, children aged 0 to 15 years.
histiocytosis X. This newer name emphasizes the histogenesis • Letterer-Siwe disease occurs predominantly in children
of the condition by specifying the type of lesional cell and younger than two years.
removes the connotation of the unknown (X) because its • The chronic multifocal form, including Hand-Schüller-
cellular basis has now been clarified.64 Christian syndrome, has a peak of onset in children aged 2
The working group of the Histiocyte Society has divided to 10 years.
histocytic disorders into three different groups: (1) dendritic • Localized eosinophilic granuloma occurs most frequently
cell histiocytosis, (2) erythrophagocytic macrophage disorders, in children aged 5 to 15 years.
and (3) malignant histiocytosis. Langerhans cell histiocytosis • Signs of Langerhans cell histiocytosis (LCH) depend on the
belongs in group 1 and encompasses a number of diseases. The localization and the extent of the disease. Chronic unifocal
clinical spectrum includes on one end an acute, fulminant, Langerhans cell histiocytosis (eosinophilic granuloma of
disseminated disease called Letterer-Siwe disease; and, on the bone) classically presents as a solitary calvarial lesion in
young adults; other frequent sites of involvement include a
other end, solitary or few, indolent and chronic lesions of bone
vertebra, rib, mandible, femur, ilium, and scapula.
or other organs called eosinophilic granulomas. The
• Lesions are usually asymptomatic, but bone pain and a soft
intermediate clinical form called Hand-Schüller-Christian
tissue mass may occur.
disease is characterized by multifocal, chronic involvement and
• Bony lesions may cause otitis media by destruction of the
classically presents as the triad of diabetes insipidus, proptosis,
temporal and mastoid bones, proptosis secondary to orbital
and lytic bone lesions. masses, loose teeth from infiltration of the mandibles, or
The pathogenesis of Langerhans cell histiocytosis (LCH) pituitary dysfunction due to involvement of the sella turcica.
is unknown. An ongoing debate exists over whether this is a • Spontaneous fractures can result from osteolytic lesions of
reactive or neoplastic process. Arguments supporting the the long bones; vertebral collapse with spinal cord
reactive nature of this disorder include the occurrence of compression has been described.
spontaneous remissions, the extensive elaboration of multiple • Solitary cutaneous disease presents with nodulo-ulcerative
cytokines by dendritic cells (DCs) and T cells (i.e. the so-called lesions in the oral, perineal, perivulvar, or retroauricular
cytokine storm) in Langerhans cell histiocytosis lesions, and regions.
the good survival rate in patients without organ dysfunction.65 • The classic multifocal form of Langerhans cell histiocytosis
Furthermore, a rigorous investigation of potential chromosomal (Hand-Schüller-Christian disease) includes diabetes
aberrations in Langerhans cell histiocytosis via analysis of insipidus, exophthalmos, and bony defects, particularly of
ploidy, karyotype, single-nucleotide polymorphism arrays, and the cranium. Lesions may affect a variety of systems,
array-based comparative genomic hybridization did not reveal including liver (20%), spleen (30%), and lymph nodes
genetic abnormalities; these findings strongly support the idea (50%). Pulmonary involvement may occur.
of Langerhans cell histiocytosis as a reactive process.66 Osteolytic lesions of the long bones can lead to spontaneous
On the other hand, the infiltration of organs by a fractures.
monoclonal population of aberrant cells, the possibility of lethal • One-third of patients have mucocutaneous lesions, most
evolution, and the cancer-based modalities of successful frequently infiltrated nodules and ulcerated plaques,
treatment are all consistent with a neoplastic process.67 In especially in the mouth, axillae, and anogenital region.
addition, the demonstration of Langerhans cell histiocytosis as Other cutaneous manifestations include extensive
a monoclonal proliferation by X chromosome-linked DNA coalescing, scaling, or crusted papules.
probes supports a neoplastic origin for this proliferation; • Patients with acute disseminated Langerhans cell
however, the presence of this finding in distinct subtypes with histiocytosis (multiorgan involvement) present with fever,
different evolutions demands further investigations to elucidate anemia, thrombocytopenia, pulmonary infiltrates, skin
its significance. lesions, and enlargement of lymph nodes, spleen, and liver.
In addition, some studies indicate that expression of • The eruption may be extensive, involving the scalp, face, trunk,
vascular endothelial growth factor (VEGF), Bcl-2 family buttocks, and intertriginous areas. Lesions consist of closely
294 Essentials of Pediatric Oral Pathology

FIGURE 10.52: Langerhans cell histiocytosis showing crusted FIGURE 10.53: Histopathologic picture of Langerhans cell
eruption mimicking seborrheic dermatitis histiocytosis showing sheets of proliferating histiocytes

set petechiae and yellow-brown papules topped with scale and node involvement). In addition, the presence or the
crust. The papules may coalesce to form an erythematous, absence of organ dysfunction is used to stratify
weeping or crusted eruption mimicking seborrheic dermatitis patients with multisystemic disease; the presence of
(Fig. 10.52). any organ dysfunction portends a poorer prognosis.
• Osteolytic lesions are not common in the disseminated form Treatment modalities for single-system disease
of Langerhans cell histiocytosis, but the mastoid can be includes the following:
affected, resulting in a clinical picture of otitis media, which • Solitary bone lesions are treated locally with
may be the presenting complaint. Aural discharge, curettage or excision.
conductive hearing loss, and postauricular swelling have • Painful bone lesions may require intralesional
been described. steroid injection (triamcinolone acetonide).
• Congenital self-healing histiocytosis presents at birth or • Polyostotic bone lesions are best treated with
indomethacin or a short course of systemic
during the early neonatal period with firm, red-brown,
steroids.
painless papulonodules (1–10 mm in diameter) or vesicles • Bisphosphonates such as zoledronic acid can
and crusts scattered over the scalp, face, and, to a lesser also be used to reverse bone destruction and
extent, trunk and the extremities. Lesions may ulcerate. mitigate the pain of bony lesions.
Solitary lesions may occur. Lesions may be followed by • Early treatment with vinblastine and prednisolone
residual hypopigmented or hyperpigmented macules. has been suggested for bony lesions at vital
anatomic locations requiring prompt resolution.
HISTOPATHOLOGIC FEATURES (FIG. 10.53) • Rarely, lesions that are unusually large and
painful occur in inaccessible sites or involve vital
Lesional area shows a characteristic proliferation of histiocytes structures and require radiation therapy (3-6 Gy
in the form of sheets spread diffusely throughout the [300-600 rad]).
microscopic field. • Localized skin disease is best treated with
moderate-to-potent topical steroids (e.g.
Management mometasone furoate [Elocon] cream 0.1%,
triamcinolone [Kenalog] cream 0.1%, fluocinolone
1. The choice of therapeutic regimen is based on
[Synalar] ointment 0.025%) or superpotent topical
disease severity. The International LCH Study of the
steroids (e.g. clobetasol propionate 0.05%).
Histiocyte Society proposes the stratification of
• In cases of severe cutaneous involvement, topical
Langerhans cell histiocytosis (LCH) cases by the
nitrogen mustard (20% solution) may be used.
number of systems involved. They further categorize • Psoralen plus ultraviolet A (PUVA) is another
those cases with single-system involvement by the effective modality for cutaneous-only Langerhans
number of sites within that system (e.g. monostotic cell histiocytosis or for cutaneous involvement in
vs polyostotic bone disease, solitary vs multiple lymph multisystemic disease. PUVA consists of
Bone Pathology in Children 295

photosensitizing psoralens (8-methoxypsoralen or histiocytosis, but it is associated with considerable

5-methoxypsoralen) either applied topically or bone marrow toxicity.
ingested systemically 2 hr prior to treatment with • Resistant Langerhans cell histiocytosis may also
long-wave ultraviolet A (320-400 nm). The be treated with a combination of cyclosporin A,
purpose of this treatment is to induce remission antithymocyte globulin, and prednisolone if
of skin diseases by repeated and controlled patients do not have a matched donor for bone
phototoxic reaction. The photoconjugation of marrow transplantation.
psoralens with DNA produces an antiproliferative • Other potential treatments include monoclonal
reaction in the skin, generates programmed cell antibody targeting with indium In–labeled anti-
death (apoptosis), and induces down-regulation CD1a, cytokine inhibitors, alemtuzumab, and all-
of the cutaneous immune system. trans retinoic acid.
• Ultraviolet B excimer laser has been found, in at • Diabetes insipidus is treated symptomatically with
least one case report, to offer effective adjuvant desmopressin acetate (DDAVP).
therapy in the management of cutaneous
Langerhans cell histiocytosis, and it may HAND-SCHÜLLER-CHRISTIAN DISEASE
be particularly useful for patients with comorbidities
who cannot tolerate more aggressive treatment. In Hand-Schüller-Christian disease, abnormal scavenger cells
• For single lymph node infiltration, excision is the called histiocytes and eosinophils (another immune system cell
treatment of choice. type) grow uncontrollably, proliferating usually in the lungs and
• Regional lymph node enlargement can be treated bone, often forming scars that interfere with normal functioning.
with a short course of systemic steroids. These cells constitute malignant or benign macrophages in the
• Treatment-resistant nodes with sinus tracts to the tissues, or Langerhans’ cells in the bone marrow.
skin may require systemic chemotherapy. This syndrome is characterized by three, simultaneous
• Single-agent chemotherapy with cladribine (2- conditions:
CDA) may be a promising treatment for single- • Diabetes insipidus
system pulmonary Langerhans cell histiocytosis. • Exopthalmus
2. Treatment modalities for multisystem disease includes
• Destructive Bone Lesions.
the following:
Diabetes insipidus is a condition in which the body fails to
• Systemic chemotherapy is indicated for
produce the hormone vasopressin (anti-diuretic hormone), or the
multisystem disease and cases of single-system
disease not responsive to other treatment.
kidneys fail to use vasopressin properly. The result is loss of large
• The combination of cytotoxic drugs and systemic volumes of water through excretion (urination). Exophthalmos
steroids is generally effective. Low-to-moderate means protrusion of the globe of the eye (i.e. the “eyeball”
doses of methotrexate, prednisone, and protrudes). It is caused by damage to the pituitary gland.
vinblastine are used. Radiographic findings show severe bilateral loss of bone
• Thalidomide has also been proposed as an agent in maxilla and mandible (Fig. 10.54). ‘Scooped out’ lesions of
for treating refractory/relapsing multisystem bones are seen. Sometimes, teeth appear floating in air.
disease, but its efficacy appears to be limited to
low-risk patients with only skin or bone
involvement. Its use also is associated with
significant toxicities, including pancytopenia
and pulmonary failure.
3. Other treatment options:
• In Langerhans cell histiocytosis patients with a
very poor prognosis (rapid disease progression,
refractory to conventional treatment, or with
disseminated risk organ involvement), bone
marrow transplantation or reduced-intensity
condition stem cell transplantation has shown
promise as effective salvage therapy.
• The combination of 2-chlorodeoxyadenosine
(2-CDA) and cytosine arabinoside (Ara-C) has
also shown promise as an effective combination FIGURE 10.54: Hand-Schüller-Christian disease showing severe
therapy for refractory multisystem Langerhans cell bone loss occurring bilaterally in maxilla and mandible
296 Essentials of Pediatric Oral Pathology

FIGURE 10.55: Histopathological picture of Hand-Schüller-Christian FIGURE 10.56: Histopathologic picture of eosinophilic
disease showing diffuse infiltrate of pale staining histiocytes granuloma showing diffuse sheet-like collection of histiocytes
interspersed within lymphocytes, plasma cells

HISTOPATHOLOGIC FEATURES HISTOPATHOLOGIC FEATURES (FIG. 10.56)

• Lesional areas show diffuse infiltrate of pale staining • Lesional areas show diffuse sheet-like collection of
histiocytes interspersed within lymphocytes, plasma cells histiocytes interspersed with lymphocytes, plasma cells and
and often eosinophils (Fig. 10.55). eosinophils.
• Hand-Schuller-Christian syndrome often occurs with • Eosinophils decrease in number as the lesion matures.
lymphoma, carcinoma, and multifocal eosinophilic
granuloma (it occurs in 25% of all cases of multifocal Management
eosinophilic granuloma).
• Treatment involves treating the underlying cause, such as 1. Curettage and X-ray therapy are curative.
lymphoma, and manifestations, such as diabetes 2. Symptoms subside within two weeks after treatment.
insipidus.
REFERENCES
EOSINOPHILIC GRANULOMA 1. Rauch F, Munns C, Land C, Glorieux FH. Pamidronate in
children and adolescents with osteogenesis imperfecta: effect
Lichtenstein and Jaffe, 1940, introduced the term Eosinophilic
of treatment discontinuation. J Clin Endocrinol Metab 2006;
granuloma.69
91:1268-74.
2. Albers-Schonberg H. Roentgenbilder einer seltenen
CLINICAL FEATURES Knochennerkrankung. Munch Med Wochenschr 1904;51:365.
• Clinically no signs and symptoms are seen except fever and 3. el-Tawil T, Stoker DJ. Benign osteopetrosis: a review of 42 cases
malaise. showing two different patterns. Skeletal Radiol 1993;22(8): 587-
• There may be localized pain and swelling. 93.
• Lesion occurs in skull and jaws. Other sites involved are 4. Van Wesenbeeck L, Cleiren E, Gram J, et al. Six novel missense
long bones, ribs, humerus etc. mutations in the LDL receptor-related protein 5 (LRP5) gene
• It appears brownish to grayish in color. in different conditions with an increased bone density. Am J
Hum Genet 2003;72(3):763-71.
RADIOLOGICAL FEATURES 5. Cleiren E, Benichou O, Van Hul E, et al. Albers-Schönberg
disease (autosomal dominant osteopetrosis, type II) results from
Radiographically, the lesion appears localized well- mutations in the ClCN7 chloride channel gene. Hum Mol
circumscribed, irregular and radiolucent area. Genet 2001;10(25):2861-7.
Bone Pathology in Children 297

6. Key L, Carnes D, Cole S, et al. Treatment of congenital 25. Rapini Ronald P, Bolognia Jean L, Jorizzo Joseph L.
osteopetrosis with high-dose calcitriol. N Engl J Med 1984; Dermatology: 2-Volume, 2007 Set. St. Louis: Mosby.
310(7):409-15. 26. Maroteaux P, Lamy M. Pyknodysostosis. Presse Med
7. Krameri R, Pindborg JJ, Shear M. Histologic typing of 1962;70:999-1002.
odontogenic tumors. 2nd ed. Berlin: Springer Verlag, 1991;31. 27. Gelb BD, Edelson JG, Desnick RJ. Linkage of pycnodysostosis
8. Chuong R, Kaban LB, Kozakewich H. Central giant cell lesions: to chromosome 1q21 by homozygosity mapping. Nature Genet
a clinico-pathologic study. J Oral Maxillofacial Surg 1986;44: 1995;11:235-7.
708-13. 28. Soliman AT, Ramadan MA, Sherif A, Aziz Bedair ES, Rizk MM.
9. Eisenbud L, Stern M, Rothberg M, Sachs. Central giant cell Pycnodysostosis: clinical, radiologic, and endocrine evaluation
granuloma of the jaws: experience in management of thirty and linear growth after growth hormone therapy. Metabolism
seven cases. J Oral Maxillofacial Surg 1988;46:376-84. 2001;50(8):905-11.
10. Jones WA. Familial multilocular cysts of the jaws. Am J Cancer 29. Bassyouni HT, Afifi HH, el-Awadi MK. Mucopolysaccharidosis
1933;17:946-50. type I: clinical and biochemical study. East Mediterr Health
11. Lichtenstein L. Polyostotic fibrous dysplasia. Arch Surg 1938; J 2000;6(2-3):359-66.
36:874-98. 30. Jones KL. Storage disorders. In: Smith’s recognizable patterns
12. Weinstein LS. G(s) alpha mutations in fibrous dysplasia and of human malformation. Philadelphia, Pa: WB Saunders Co;
McCune-Albright syndrome. J Bone Miner Res 2006;21(Suppl 1997;456-71.
2):120-4. 31. Muenzer J. Mucopolysaccharidoses. Adv Pediatr 1986;33:269-
13. Chapurlat RD. Medical therapy in adults with fibrous dysplasia 302.
of bone. J Bone Miner Res 2006;21(Suppl 2):114-9. 32. Dupont C, Hachem CE, Harchaoui S, Ribault V, Amiour M,
14. Jaffe HL, Lichtenstein L. Chondromyxoid fibroma of bone: a Guillot M. Hurler syndrome: Early diagnosis and successful
distinctive benign tumor likely to be mistaken especially for enzyme replacement therapy: A new therapeutic approach. Case
chondrosarcoma. Arch Path 1943;19:541-51. report. Arch Pediatr 2008;15(1):45-9.
15. Granter SR, Renshaw AA, Kozakewich HP, et al. The 33. Guffon N, Souillet G, Maire I, et al. Follow-up of nine patients
pericentromeric inversion, inv (6) (p25q13), is a novel with Hurler syndrome after bone marrow transplantation. J
diagnostic marker in chondromyxoid fibroma. Mod Pediatr 1998;133(1):119-25.
Pathol 1998;11(11):1071-4. 34. McKay CP, Portale A. Emerging topics in pediatric bone and
16. Wu KK. Chondromyxoid fibroma of the foot bones. J Foot mineral disorders 2008. Semin Nephrol 2009;29(4):370-8.
Ankle Surg 1995;34(5):513-9. 35. Schour I, Massler M. Endocrines and dentistry, JADA 1943;
17. Durr HR, Lienemann A, Nerlich A, et al. Chondromyxoid fibroma 30:595, 753, 943.
of bone. Arch Orthop Trauma Surg 2000;120(1-2):42-7. 36. Crouzon O. Dysostose cranio-faciale hereditaire. Bull Mem Soc
18. Norberg O. Zur kenntnis der dysontogenetischen geschwulste Med Hop. Paris 1912;33:545-55.
der kieferknochen. Vrtljsschr Zahn 1930;46:321-55. 37. Muenke M, Gripp KW, McDonald-McGinn DM, et al. A unique
19. Agazzi C, Belloni L. Gli odontomi duri dei mascellari: point mutation in the fibroblast growth factor receptor 3 gene
contribute clinico-rontgenologico e anatomo-microscopico con (FGFR3) defines a new craniosynostosis syndrome. Am J Hum
particolare riguardo alle formle ad ampia estensione e alla Genet 1997;60(3):555-64.
comparsa familiare. Arch Ital Otol 1953;64(suppl 16):3-102. 38. Gray TL, Casey T, Selva D, Anderson PJ, David DJ. Ophthalmic
20. Hamner JE, Gamble JW, Gallegos GJ. Odontogenic fibroma. sequelae of Crouzon syndrome. Ophthalmology 2005;
Report of two cases. Oral Surg Oral Med Oral Pathol 1966; 112(6):1129-34.
21:113-9. 39. Treacher Collins E. Case with symmetrical congenital notches
21. Slootweg PJ, Panders AK, Koopmans R, Nikkels PG. Juvenile in the outer part of each lid and defective development of the
ossifying fibroma. An analysis of 33 cases with emphasis on malar bones. Trans Ophthalmol Soc UK 1900;20:190-2.
histopathological aspects. J Oral Pathol Med 1994;23:385-8. 40. Thomson A. Notice of several cases of malformation of the
22. Baden E, Spirgi M. Oral manifestations of Marfan’s syndrome. external ear, together with experiments on the state of hearing
Oral Surgery, Oral Medicine, Oral Pathology 1965;19(6):757- in such persons. Monthly J Med Sci 1846;7:420.
70. 41. Toynbee J. Description of a congenital malformation in the ears
23. Fraccardo M. Contributo allo studio delle malattie del of a child. Monthly J Med Sci 1847;1:738-9.
mesenchima osteopoietico: l achondrogenesi. Folia Hered 42. Berry GA. Note on a congenital defect (coloboma?) of the lower
Path 1952;1:190-208. lid. Royal London Ophthalmological Hospital Report 1889;12:
24. Mortier GR, Weis M, Nuytinck L, et al. Report of five novel 255-77.
and one recurrent COL2A1 mutations with analysis of 43. The Treacher Collins Syndrome Collaborative Group. Positional
genotype-phenotype correlation in patients with lethal type II cloning of a gene involved in the pathogenesis of Treacher
collagen disorder. J Med Genet 2000;37:263-71. Collins syndrome. Nat Genet 1996;12(2):130-6.
298 Essentials of Pediatric Oral Pathology

44. Lannelongue V, Menard M. Affection’s congenitales. Paris: 58. Bernstein RM, Zaleske DJ. Familial aspects of Caffey’s
Hasselin and Houzeau, 1891;1:423. disease. Am J Orthop 1995;24(10):777-81.
45. Robin P. La chute de la base de la langue considérée comme 59. Saul RA, Lee WH, Stevenson RE. Caffey’s disease revisited.
une nouvelle cause de gans la respiration naso-pharyngienne. Further evidence for autosomal dominant inheritance with
Bull Acad Natl Med (Paris) 1923;89:37-41. incomplete penetrance. Am J Dis Child 1982;136(1):55-60.
46. Apert ME. De racrocephalosyndatylie. Bull Soc Med Hop 60. I E el-hakim and SA Metwalli. Imaging of temporo-mandibular
(Paris) 1906;23:1310-30. joint ankylosis—A new radiographic classification.
Dentomaxillofacial Radiology 2002;31:19-23.
47. Meinhard Robinow, Frederic N Silverman, Hugo D Smith. Am
J Dis Child 1969;117(6):645-51. 61. De Bont LG, van der Kuijl B, Stegenga B, Vencken LM, Boering
G. Computed Tomography in differential diagnosis of Temporo
48. Robinow M. “The Robinow (fetal face) syndrome: a continuing
mandibular joint disorders. Int J Oral Maxillofac Surg 1993;
puzzle”. Clin Dysmorphol 1993;2(3):189-98.
22:200-9.
49. Van Buchem FSP, Hadders HN, Ubbens R. An uncommon 62. Sawhney CP. Bony ankylosis of the temporomandibular joint:
familial systemic disease of the skeleton: hyperostosis corticalis Follow-up of 70 patients treated with Arthroplasty and acrylic
generalisata familiaris. Acta Radiol (Stockh), 1955;44:109-120. spacer interposition. Plast Reconst Surg 1986;77:29-38.
50. Van Hul W, Balemans W, Van Hul E, Dikkers FG, Obee H, 63. Langerhans P. Uber die Nerven der menschlichen Haut.
Stokroos RJ, et al. Van Buchem disease (hyperostosis corticalis Virchows Arch [Pathol Anat], 1868;44:325-37.
generalisata) maps to chromosome 17q12–q21. Am J Hum 64. Egeler RM, Favara BE, van Meurs M, Laman JD, Claassen
Genet 1998;62:391-9. E. Differential in situ cytokine profiles of Langerhans-like cells
51. Beighton G, Barnard A, Hamersma H, Van der Wouden A. The and T cells in Langerhans cell histiocytosis: abundant expression
syndromic status of sclerosteosis and van Buchem disease. Clin of cytokines relevant to disease and treatment. Blood 1999;
Genet 1984;25:175-81. 94(12):4195-201.
52. Ellis RWB, van Creveld S. A syndrome characterized by 65. da Costa CE, Szuhai K, van Eijk R, et al. No genomic
ectodermal dysplasia, polydactyly, chondro-dysplasia and aberrations in Langerhans cell histiocytosis as assessed by
congenital morbus cordis: report of three cases. Arch Dis Child diverse molecular technologies. Genes Chromosomes
1940;15:65-84. Cancer 2009;48(3):239-49.
53. Tompson SW, Ruiz-Perez VL, Blair HJ, et al. Sequencing EVC 66. Willman CL. Detection of clonal histiocytes in Langerhans cell
and EVC2 identifies mutations in two-thirds of Ellis-van histiocytosis: biology and clinical significance. Br J Cancer
Creveld syndrome patients. Hum Genet 2007;120(5):663-70. Suppl 1994;23:S29-33.
54. Down JL. Observations on ethnic classification of idiots. Ment 67. Willman CL, Busque L, Griffith BB, et al. Langerhans’ -cell
histiocytosis (histiocytosis X)—a clonal proliferative disease. N
Retard 1866;33(1):54-6.
Engl J Med 1994;331(3):154-60.
55. Lejeune J. Le mongolisme. Premier example d’aberration
68. Dina A, Zahava V, Iness M. The role of vascular endothelial
autosomique humaine. Ann Genet 1959;1:41-9.
growth factor in Langerhans, cell histiocytosis. J Pediatr
56. Jacobs PA, Baikie AG, Court Brown WM, Strong JA. The Hematol Oncol 2005;27(2):62-6.
somatic chromosomes in mongolism. Lancet 1959;1(7075):710. 69. Lichtenstein L, Jaffe HL. Eosinophilic granuloma of bone, with
57. Caffey J. Infantile cortical hyperostoses. J Pediatr 1946;29:541-59. report of a case. Am J Path 1940;16:595.
11

Salivary Gland Lesions

in Children
Mayur Chaudhary, Shweta Dixit Chaudhary

CHAPTER OVERVIEW
Introduction Adenomatoid hyperplasia of mucous glands
Classification Polycystic (dysgenetic) disease of the parotid glands
Developmental disturbances of salivary glands in children: Sialadenitis
Aplasia Sarcoidosis
Xerostomia Sialolithiasis
Hyperplasia of palatal salivary glands
Benign neoplasms:
Atresia
Pleomorphic adenoma
Diverticuli
Aberrancy Warthin’s tumor
Developmental lingual salivary gland depression Malignant neoplasms:
Heterotropic salivary glands Mucoepidermoid carcinoma
Accessory parotid glands Acinic cell adenocarcinoma

INTRODUCTION CLASSIFICATION
Salivary glands are divided into major and minor salivary Salivary Gland Lesions (Fig. 11.1)
gland categories. The major salivary glands are the parotid,
Developmental disturbances
the submandibular and the sublingual glands. The minor • Aplasia
glands are dispersed throughout the upper aerodigestive • Xerostomia
submucosa (i.e. palate, lip, pharynx, nasopharynx, larynx,
• Hyperplasia of parotid glands
parapharyngeal space).
• Atresia
Salivary gland diseases represent a disparate group of
• Abberancy
disorders affecting both the major and minor salivary glands.
• Developmental lingual mandibular salivary gland depression
These conditions range from inflammatory disorders of
• Heterotropic salivary glands
infectious, granulomatous, or autoimmune etiology to
• Accessory parotid glands
obstructive, developmental and idiopathic disorders and
• Adenomatoid hyperplasia of mucous glands
certain neoplasms (either benign or malignant). The major
• Polycystic disease of parotid gland.
salivary glands are most often involved and many of these
salivary gland disorders are associated with the presence of Reactive lesions
other systemic illnesses. A thorough history and physical • Mucous extravasation phenomenon
examination is typically adequate to recognize and • Mucous retention cyst
differentiate this group of conditions and an elaborate • Pseudocyst (mucous retention cyst)
diagnostic evaluation is usually not required to manage these • Necrotizing sialometaplasia
illnesses. • Adenomatoid hyperplasia
300 Essentials of Pediatric Oral Pathology

FIGURE 11.1: Classification of salivary gland lesions1 FIGURE 11.2: Classification of non-neoplastic salivary gland
diseases2
Infectious diseases • Abberancy
• Mumps • Developmental lingual salivary gland depression
• Cytomegalovirus sialadenitis • Heterotropic salivary glands
• Bacterial sialadenitis • Accessory parotid glands
• Sarcoidosis • Adenomatoid hyperplasia of mucous glands
• Metabolic conditions • Polycystic disease of parotid gland.
• Sjögren’s syndrome
• Salivary lymphoepithelial lesion Obstructive lesions
Sjögren’s syndrome is now considered under autoimmune • Mucous escape reaction
disorders. • Mucous retention reaction
• Sialolithiasis.
Benign neoplasms
• Mixed tumor (Pleomorphic adenoma) Infectious diseases
• Monomorphic adenoma • Tuberculosis
• Myoepithelioma • Cat scratch disease
• Ductal papillomas • Salivary gland cyst as manifestation of AIDS
• Warthin’s tumor (papillary cystadenoma lymphomatosum). • Cytomegaloviral infection
• Mumps
Malignant neoplasms • Sarcoidosis.
• Mucoepidermoid carcinoma
• Adenoid cystic carcinoma Idiopathic
• Acinic cell carcinoma • Necrotizing sialometaplasia
• Polymorphous low grade carcinoma • Benign cysts of parotid glands
• Clear cell carcinoma — Lymphoepithelial cyst
• Adenocarcinoma not otherwise specified. — Salivary duct cyst
• Angiolymphoid hyperplasia with eosinophilia and Kimura’s
Rare tumors
disease
• Epimyoepithelial carcinoma
• Salivary duct carcinoma • Cheilitis glandularis
• Basal cell adenocarcinoma • Benign lymphoepithelial lesion and Sjögren’s syndrome.
• Squamous cell carcinoma.
DEVELOPMENTAL DISTURBANCES OF SALIVARY
Non-neoplastic Salivary Gland Diseases (Fig. 11.2) GLANDS IN CHILDREN

Ellis, Auclair and Gnepp 2 have classified non neoplastic APLASIA

salivary gland diseases as follows:
Aplasia is also called as agenesis. In this condition, any one or
Developmental disturbances
a group of salivary glands may be congenitally absent,
• Aplasia
• Xerostomia unilaterally or bilaterally. It was first described by Gruber, 1885.
• Hyperplasia of palatal salivary glands It may occur in association with other developmental abnor-
• Atresia malities such as atresia of lachrymal puncta and congenital
Salivary Gland Lesions in Children 301

malformation of the temporomandibular component. It may — D–dentally, peg shaped teeth, hypodontia and enamel
also occur in conjunction with other developmental defects such hypoplasia may be seen.
as hemifacial microsomia, Lachrymal Auricular Dental Digital — D–digital deformities may present as deviation of the
syndrome (LADD syndrome), mandibulofacial dysostosis fingers medially or laterally (clinodactyly).
(Treacher Collins syndrome). • Mandibulofacial dysostosis:
— Usually familial in origin following an irregular form
ETIOLOGY of dominant transmission.
• Local disturbance in early fetal development. — Rare; incidence being 1 in 25,000 to 1 in 50,000.
• Mc Donald et al, 1986, suggested an ectodermal origin for — Symmetric notching of the lower eyelids with eyes
this anomaly.3 slanting downwards at the lateral borders (anti-
mongoloid palpebral fissures).
CLINICAL FEATURES — Hypoplasia of facial bones, especially, malar bones and
• Initial manifestation may be development of xerostomia and mandible.
its sequelae. — Malformation of the external ear.
• CT scan or MRI help in the diagnosis by indicating absence — Macrostomia, high palate, malocclusion of teeth.
of the gland and its replacement by fat and fibrous tissue. — Salivary aplasia.
• Scintiscanning with a radioisotope may be used for — Characteristic facies referred to as bird-like or fish-like
confirmation of the diagnosis. in nature.
• Absence of the salivary duct orifice/papilla may be one of Management
the indicators of aplasia.
1. Treatment is primarily supportive in nature.
• Patient may present with increased caries, burning 2. Primary aim is to relieve xerostomia by the use of
sensation, oral infections and taste aberrations due to salivary substitutes, frequent mouthwashes, compre-
absence of saliva. hensive dental care, fluoride therapy and good oral
• Xerostomia may also necessitate constant sipping of water hygiene.
throughout the day and cause difficulty in swallowing at
meal times. XEROSTOMIA
• Oral mucosa appears dry, smooth or sometimes pebbly and
The term xerostomia was first described by Bartley in 1868
shows a tendency for accumulation of debris.
and stands for the Greek terminology of xeros means dry and
• Cracking of lips and fissuring of the corners of the mouth
stoma means mouth.4
may be seen. Xerostomia is defined as dry mouth resulting from reduced
• Although hypoplasia of salivary glands is rare, hypoplasia or absent salivary flow. Xerostomia is not a disease, but it may
of parotid gland has been reported to be present with be a symptom of various medical conditions. Although usually
Melkerson-Rosenthal syndrome. said to be common in almost 20 percent of the geriatric
• Hemifacial microsomia: population, it is important to bear in mind that xerostomia can
— Incidence is 1 in 3500 births. occur in any age group. Its occurrence is not related as much
— Asymmetric, mild to severe underdevelopment of the to age as the causative factor, disease, radiation or medication
craniofacial skeleton, the external ear and facial soft taken by a particular individual. However, it is not very
tissues including the parotid gland is seen. common in the pediatric age group.
— Usually involves structures derived from the first and
second branchial arch. NORMAL SALIVARY FUNCTION
— Usually sporadic in nature. Normal salivary function is mediated by the muscarinic M3
• LADD syndrome: receptor. Stimulation of this receptor results in increased watery
— Follows the autosomal dominant mode of inheritance. flow of salivary secretions. When the oral mucosal surface is
— L–lachrymal apparatus may show occlusion of the stimulated, afferent nerve signals travel to the salivatory nuclei
lachrymal puncta, nasolachrymal duct obstruction with in the medulla. The medullary signal may also be affected by
overflow of tears (epiphora), lachrymal sac inflamma- cortical inputs resulting from stimuli such as taste, smell,
tion (dacrocystitis) and lachrymal gland aplasia. anxiety or depression. Efferent nerve signals, mediated by
— A–auricles are deformed with the ear having a cup acetylcholine, also stimulate salivary glandular epithelial cells
shaped appearance alongwith some hearing loss. and increase salivary secretions.5
302 Essentials of Pediatric Oral Pathology

ETIOLOGY • The acid produced after eating or drinking sugary foods

leads to further mineral loss from the teeth, causing even
Developmental conditions such as aplasia, agenesis of the
more tooth decay
salivary glands or a deficiency of the M3 receptors or chronic
• There is an increased risk of cavities because the mouth is
administration of anticholinergic or parasympatholytic drugs
less able to control bacteria
may lead to signs and symptoms of xerostomia.
• Plaque becomes thicker and heavier because of the patient’s
Radiotherapy in cancer patients is one of the most common
difficulty in maintaining good oral hygiene.
causes of xerostomia. However, since radiotherapy can prevent
the normal growth and development of bones, muscles and
other tissues, most cancer specialists avoid radiotherapy or use DIAGNOSIS AND EVALUATION OF XEROSTOMIA
the lowest possible doses of radiation when caring for children Diagnosis of xerostomia may be based on evidence obtained
with carcinomas. from the patient’s history, an examination of the oral cavity
A number of medications can cause xerostomia, including and/or sialometry, a simple office procedure that measures the
many drugs used in the management of cancer or cancer flow rate of saliva. Xerostomia should be considered if the
treatment side effects. Some of these are: atropine (used with patient complains of dry mouth, particularly at night, or of
caution in children), carbamazepine (used with caution in
difficulty eating dry foods such as crackers.6, 7 When the mouth
children), diphenhydramine (used with caution in children),
is examined, a tongue depressor may stick to the buccal
haloperidol, loperamide (used with caution in children), and
mucosa.8 When wearing lipstick, the “lipstick sign,” where
scopolamine (used with caution in children), among several
lipstick adheres to the front teeth, may be a useful indicator of
others.
Xerostomia may develop in children with HIV infection, xerostomia.
as the virus often damages the salivary glands. The oral mucosa may be dry and sticky, or it may appear
Diseases like sarcoidosis, rheumatoid arthritis, thyroid erythematous due to an overgrowth of Candida albicans. The
dysfunction may also result in sialadenitis and reduction in red patches often affect the hard and soft palate and dorsal
salivary flow. surface of the tongue. Occasionally, pseudomembranous
candidiasis may be present, appearing as removable white
CLINICAL FEATURES plaques on any mucosal surface. There may be little or no
pooled saliva in the floor of the mouth and the tongue may
Xerostomia can cause a derangement in the nutritional, dental
as well as psychological health of the affected individual. The appear dry with decreased numbers of papillae. The saliva may
following features may be present: appear stringy, ropy or foamy. Dental caries may be found at
• Dryness of the mouth the cervical margin or neck of the teeth, the incisal margins or
• Cracked lips, cuts, or cracks at the corners of the mouth. the tips of the teeth.6
• Taste changes (dysgeusia) Several office tests and techniques can be utilized to
• A burning sensation of the tongue, painful tongue (glossodynia) ascertain the function of salivary glands. In sialometry, or
• Changes in the surface of the tongue salivary flow measurement, collection devices are placed
• Constant sore throat over the parotid gland or the submandibular/sublingual
• Hoarseness and/ or dry nasal passages gland duct orifices and saliva is stimulated with citric acid.
• Difficulty wearing dental appliances (like space main- The normal salivary flow rate for unstimulated saliva from
tainers, myofunctional appliances) the parotid gland is 0.4 to 1.5 ml/min/gland. The normal flow
• Difficulty swallowing fluids accompanied by an increase rate for unstimulated, “resting” whole saliva is 0.3 to 0.5
in thirst ml/min; for stimulated saliva, 1 to 2 ml/min. Values less than
• Difficulty in eating especially dry, crumby foods 0.1 ml/min are typically considered xerostomic, although
• Development of gum disease and cavities reduced flow may not always be associated with complaints
• Development of candidiasis, sialadenitis of dryness. 7
• Development of halitosis. Sialography is an imaging technique that may be useful in
Xerostomia causes the following mouth changes that can identifying salivary gland stones and masses. It involves the
contribute to discomfort for the patient and an increased risk injection of radio-opaque media into the salivary glands.
for oral lesions: Salivary scintigraphy can be useful in assessing salivary gland
• Saliva becomes thick and is less able to lubricate the mouth. function. Technetium-99m sodium pertechnate is intravenously
• Acids in the mouth cannot be neutralized, leading to mineral injected to ascertain the rate and density of uptake and the time
loss from the teeth of excretion in the mouth.9
Salivary Gland Lesions in Children 303

Management adrenergic antagonist), human interferon alfa (IFN-a) or

sugarless candies and chewing gum which can
Identification of the underlying cause is the first step when
stimulate saliva secretion from the remaining salivary
it comes to management of xerostomia. Xerostomia usually
glands. Eating foods such as carrots or celery may also
cannot be reversed when the cause is a developmental
help patients with residual salivary gland function. Saliva
disorder like aplasia or agenesis or the destruction of the
stimulants like Natrol dry mouth relief (anhydrous
salivary glands by radiation treatment. It may be reversible
crystalline maltose) may help in stimulating saliva
if related to a medication. All of the treatment measures
production.
serve to increase the level of comfort, decrease the chance
11. Applying a prescription-strength fluoride gel daily at
for oral lesions and reduce the occurrence of gum disease
bedtime to clean the teeth.
and cavities.
12. A cold air humidifier may aid mouth breathers who
1. Cleaning the mouth well at least four times per day (after
typically have their worst symptoms at night.
every meal and at bedtime).
13. Addition of flavor enhancers such as herbs, condiments
2. Rinsing the mouth immediately after every meal.
and fruit extracts may make food more palatable to
3. Sipping water frequently.
patients complaining of their food tasting bland, papery,
4. Rinsing the mouth with a salt and baking soda solution
salty or otherwise unpleasant.
four to six times per day (1/2 tsp salt, 1/2 tsp baking
14. Using low abrasive fluoride toothpaste to brush the teeth.
soda, and 8 oz of water). Biotene® and oralbalance® dentifrices are available
5. Chlorhexidine rinses may also be useful in preventing over-the-counter from Laclede, Inc. These are
caries by reducing lactobacillus counts in the mouth. antixerostomia dentifrices that contain three salivary
6. Avoiding foods and liquids containing large amounts of enzymes, lactoperoxidase, glucose oxidase and
sugar as well as irritating foods that are dry, spicy, lysozyme, specifically formulated to activate intra-oral
astringent or excessively hot or cold. bacterial systems.
7. Avoiding mouthwashes containing alcohol. 15. Symptoms of xerostomia are often worse between
8. Using moisturizer and lubricants such as orajel® or meals, at night and in the morning. Therefore, consider
vaseline® and glycerin swabs on the lips. modifying drug schedules to achieve maximum plasma
9. Using saliva substitutes which offer a replacement levels when the patient is awake. Consider easy-to-take
therapy to help relieve discomfort. Commercially formulations, such as liquids and avoid sublingual
available products come in a variety of formulations dosage forms if possible.
including solutions, sprays, gels and lozenges. In
general, they contain an agent to increase viscosity,
HYPERPLASIA OF PALATAL SALIVARY GLANDS
such as carboxymethylcellulose or hydroxyethyl-
cellulose, minerals such as calcium and phosphate ions Giansanti et al, 1971, reported unusual localized hyperplasia
and fluoride, preservatives such as methyl- or propyl- or hypertrophy of minor accessory salivary glands in the palate.10
paraben, and flavoring and related agents.
Carboxymethyl or hydroxycellulose solutions: ETIOLOGY
• Entertainer’s secret® (KLI Corp), spray
Unknown, but the following have been reported to result in
• Glandosane ® (Kenwood/ Bradley) spray
salivary gland enlargement:
• Moi-Stir® (Kingswood Labs) spray
• Moi-Stir® oral swabsticks (Kingswood Labs) swabs • Hormonal disorders: Endocrine disorders, menopause.
• Optimoist® (Colgate-Palmolive) spray • Metabolic disorders: Gout, diabetes mellitus.
• Saliva substitute® (Roxane Labs) liquid • Autoimmune disorders: Sjögren’s syndrome, Waldenstrom
• Salivart® (Gebauer) preservative-free aerosol macroglobulinemia.
• Salix® (Scandinavian Natural Health and Beauty) • Syndromes: Aglossia-adactylia syndrome, Heerfordt’s
tablets syndrome, Felty’s syndrome.
• VA oralube® (Oral Dis. Res. Lab) sodium-free; • Miscellaneous: Hepatic disease, starvation, alcoholism,
liquid inflammation, benign lymphoepithelial lesion, adiposity,
• Xero-Lube® artificial saliva (Scherer) sodium-free; hyperthermia, oligomenorrhea and certain drugs.
spray • Of these menopause, diabetes mellitus, Sjögren’s syndrome,
Mucopolysaccharide solutions: Waldenstrom macroglobulinemia, Heerfordt’s syndrome,
Mouthkote ® (Parnell) spray Felty’s syndrome, alcoholism, benign lymphoepithelial
10. Using a sialogogue such as pilocarpine (Salagen), lesion, adiposity, hyperthermia, oligomenorrhea are usually
Cevimeline (cholinergic agonist), yohimbine (alpha-2 not seen in the pediatric population.
304 Essentials of Pediatric Oral Pathology

CLINICAL FEATURES ABERRANCY

• Usually asymptomatic. Aberrancy is simply that situation in which salivary glands
• Usually involves the minor salivary glands of the palate. are found farther than normal from their usual location.
• Presents as a small localized swelling measuring from The normal accessory salivary glands in the oral cavity
several millimeters to around a centimeter in diameter, have such a widespread distribution that it is really difficult
usually on the hard palate or at the junction of the hard to pinpoint the condition of aberrancy. Fortunately, the only
and the soft palate. The lesion has an intact surface and is clinical significance aberrant salivary glands may have is
firm, sessile and normal in color. that they may be the site of development of a retention cyst
• Age and gender predilection have not been established as or a neoplasm. Cases have been reported in which salivary
yet. gland tissue has been found within the body of the
• Arafat et al, 1981,11 reported 10 cases of salivary gland mandible. This condition known as the “developmental
hyperplasia. There were no associated abnormalities and lingual mandibular salivary gland depression” has been
no evident causative factors. Contrary to previous reports, described separately.
one of their cases involved the glands of the retromolar area
rather than the palate. DEVELOPMENTAL LINGUAL SALIVARY
GLAND DEPRESSION
HISTOPATHOLOGIC FEATURES
Also known as Staffne cyst/defect, static bone cyst, latent
• The histopathologic picture does not differ significantly bone cyst, static bone cavity, lingual mandibular bone cavity.
from normal mucous gland structure. The developmental lingual mandibular salivary gland
• Closely packed collections of normal appearing mucous depression is an unusual form of slightly aberrant salivary
acini with the usual intermingling of normal ducts is seen. gland tissue wherein a developmental inclusion of glandular
• There is no inflammation, no spillage of mucin and no tissue is found within, or more commonly, adjacent to the
fibrosis. lingual surface of the body of the mandible within a deep and
well-circumscribed depression. This entity is named after
Management Staffne who first recognized this phenomenon in 1942. 12
1. Primary mode of treatment is an excision biopsy for However, the oldest description is in a skull dated to the sixth
microscopic examination as the lesion could also be to fourth century B.C.
a salivary gland neoplasm of the area.
2. Recurrence has not been reported as yet.
CLINICAL FEATURES

ATRESIA • Incidence is 0.1 to 1.3 percent.

• Predilection of males over females is seen.
It is the congenital occlusion or absence of one or more of the • Although it is a congenital defect, it is not very commonly
major salivary gland ducts. observed in children.
• It is an exceedingly rare condition. • The radiograph reveals an ovoid radiolucency located
• Usually the submandibular duct in the floor of the mouth between the inferior alveolar canal and the inferior border
fails to canulate during embryological development. of the mandible in the region of second or third molars
• Within two to three days of age, the newborn infant presents (Fig. 11.3).
with submandibular swelling on the affected side due to
the presence of a retention cyst. DIAGNOSIS
• A relatively severe xerostomia may also be present.
• Differential diagnosis includes the traumatic or hemorrhagic
bone cyst from which it can be differentiated by the location
DIVERTICULI of the traumatic bone cyst superior to the inferior alveolar
A diverticulum is an outpouching of the ductal system of one canal.
of the major salivary glands. Its clinical significance lies in • A rare anterior variant of the Staffne’s cyst has also been
the fact that it may lead to recurrent episodes of acute reported presenting as a round or ovoid radiolucency in
parotitis. the area between the central incisors and first premolars.
Salivary Gland Lesions in Children 305

heterotropic salivary gland tissue was considered to have

given rise to tumors in 12 percent of the 110 cases of
heterotropic tissue. When heterotropia was associated with
a salivary gland tumor, the majority were mucoepidermoid
carcinomas (46%). The others were mixed tumors (23%),
Warthin’s tumor (23%) and adenoma (8%).
• Heterotropic salivary gland tissue in the paraparotid area
shows intranodal occurrence easily explained by the
entrapment of salivary gland tissue in lymph nodes during
embryonic development.
• The propensity for occurrence in the paraparotid nodes
could be related to the latent encapsulation of the parotid
glands. However, Bernier and Bhaskar, 1978, 15 and
Azzopardi and Hou, 1964,16 have argued over whether
salivary gland tissue in paraparotid lymph nodes is true
heterotropia rather than a variation of normal tissue.
FIGURE 11.3: Ovoid radiolucency located between the inferior
alveolar canal and the inferior border of the mandible
HISTOPATHOLOGIC FEATURES
HETEROTROPIC SALIVARY GLANDS • Histopathologically, the tissue mainly consists of either
mixed seromucinous glands or more commonly serous
Heterotropic salivary glands are also referred to as ectopic
glands with randomly scattered well-formed ducts.
salivary gland tissue or salivary gland choristomas. They
• It is important not to interpret a well-differentiated metastatic
include the salivary gland tissue located at sites other than those
tumor in paraparotid nodes as heterotropic salivary gland
appropriate for normal anatomic distribution of the major
tissue.
salivary glands, oral mucosa and pharynx.
HETEROTROPIC SALIVARY GLAND TISSUE OF
ETIOLOGY THE LOWER NECK
• Embryogenesis of the heterotropic salivary gland tissue is CLINICAL FEATURES
often unclear and is related to the anatomic site.
• Willis, 1968, has proposed three general explanations for • Heterotropic salivary gland tissue of the lower neck has a
heterotropia:13 unique clinical presentation, but unexplained embryogenesis.
1. Abnormal persistence and development of vestigial • Usually presents as branchial cleft sinuses in the lower
structures. anterolateral neck, medial to the border of the sterno-
2. Dislocation of a portion of a definitive organ rudiment cleidomastoid muscle.
during mass movement of development. • Bilateral presentation, as well as, existence at birth is a
3. Heteroplasia which is abnormal differentiation of the common feature.
local tissues. • Most characteristic feature is presence of a draining sinus
that secretes a mucoid, creamy or clear saliva like fluid.
SITE Drainage is minimal but may increase at meal times.
• The sinus tract is usually less than 2 cm and emanates from
• The most common locations are the paraparotid lymph a nodular or lobulated gray or yellow soft tissue mass,
nodes, middle ear and lower neck. which occasionally resembles normal salivary gland tissue.
• Less frequent locations are the upper neck, mandible, • Swelling may be apparent.
external auditory canal, mediastinum, cerebellopontine • Some lesions present as deep seated cysts that communicate
angle, pituitary gland, prostate gland, vulva, rectum, through the sinus.
thyroglossal duct, thyroid gland and parathyroid capsules.
HISTOPATHOLOGIC FEATURES
CLINICAL FEATURES
• Histopathologically, the sinus may be lined by pseudo-
• According to a survey of 20,000 salivary gland lesions by stratified ciliated columnar epithelium with metaplastic
the Armed Forces Institute of Pathology (AFIP), 14 stratified squamous epithelium. The heterotropic salivary
306 Essentials of Pediatric Oral Pathology

gland tissue may be identical with normal oral salivary • However, cases involving children have been documented
gland tissue and thus, may contain serous, mucous or mixed with no cases reported by Uemura et al, 1976.20
glandular acini. Cartilage may also be present and is
possibly attributed to branchial origin. HISTOPATHOLOGIC FEATURES
• Youngs and Scofield, 1967,17 proposed that ectopic salivary
gland tissue in the lower neck results from the overgrowth Histopathologically, the tissue is primarily mucous acini and
of the second branchial arch, which overlaps the second, third some cases are contiguous with the sublingual gland.
and fourth branchial clefts, thus forming the precervical sinus
of His. HETEROTROPIC SALIVARY GLAND TISSUE OF
• Himalstein, 1981, has suggested that this tissue is a OTHER SITES
residuum of the tenth nerve ganglion placodal duct after
the sinus of His has dissipated.18 CLINICAL FEATURES
• Heterotropic salivary gland tissue of other sites include
HETEROTROPIC SALIVARY GLAND TISSUE gingival choristomas which manifest as yellow swellings
OF THE MIDDLE EAR at the mucogingival junction and are composed of
seromucous or pure mucous glands.
CLINICAL FEATURES • May arise from pluripotential gingival epithelium or from
• Heterotropic salivary gland tissue of the middle ear was a mechanical disturbance during development.
first reported by Taylor and Martin, 1961.19 • Likewise, rare cases of heterotropic salivary gland tissue
• Its pathogenesis is thought to be related to errant have been reported at the cerebellopontine angle, external
development of the first and second branchial arches at auditory canal, posterior lobe of the pituitary, mediastinal
some time before the fourth month of intrauterine life. lymph node, rectum and vulva.
• It is quite a rare lesion and presents with unilateral Heterotropia and associated salivary gland tumors refers
conductive hearing loss. to the numerous examples of salivary gland tumors originating
• Usually presents in the first and second decades of life in heterotropic salivary gland tissue. These tumors include
arising in close association with the facial nerve. acinic cell carcinoma, adenoid cystic carcinoma, mucoepi-
• Ossicle deformities (mainly incus and stapes), cholestea- dermoid carcinoma, adenocarcinoma, monomorphic adenoma
tomas and oval window absence are seen. and mixed tumor.
• Usually appears as a gray to yellow, lobulated or smooth
surfaced, soft mass that is approximal to the facial nerve. ACCESSORY PAROTID GLANDS

HISTOPATHOLOGIC FEATURES The term accessory parotid glands refers to lobules of parotid
salivary tissue that are separated from the main body of the
• Histopathologically, mixed serous and mucous glands are gland but that drain into Stenson’s duct.
seen with excretory ducts in some cases. The salivary gland
elements are intermixed with mature adipose tissue and CLINICAL FEATURES
loosely arranged fibrous connective tissue.
• Although recurrence is minimal, careful dissection is • Typically ranges from 0.5 to 3 cm and may be spherical or
recommended to avoid damage to the facial nerve. oblong.
• The accessory parotid tissue is usually bound to the fascia of
the masseter muscle at an average distance of 6mm from the
INTRAOSSEOUS HETEROTROPIC SALIVARY anterior edge of the parotid gland usually on or above the duct.
GLAND TISSUE • Usually seen anterior to the border of the masseter, resting
CLINICAL FEATURES on the buccal pad fat.
• This parotid tissue is normally connected to Stenson’s duct
• Intraosseous heterotropic salivary gland tissue is usually by a single accessory duct, but there may be 2 to 10 ducts.
found in close association with the mandible and occurs in • Frommer, 1977, showed that 21 percent out of 96 cadaver
crypt like invaginations of the lingual surface dissections demonstrated accessory parotid salivary gland
• First described by Staffne in 1942,12 and has been discussed tissue. He also pointed out that anterior extensions of
previously in this section parotid gland tissue along the parotid duct are normal
• Pathogenesis for this lesion could be embryonic, congenital, variants of the parotid gland tissue and should not be
aneurysmal or developmental considered as accessory tissue.21
Salivary Gland Lesions in Children 307

• Clinically present as masses in the cheek that occur in the fulfill the histologic criteria of an adenoma, it should not be
central third of a line drawn from the middle of the tragus called so.25 Interestingly, a lot of authors have used the term
to a point midway between the ala of the nose and the adenomatoid hyperplasia.
vermillion border of the upper lip.
• The primary clinical significance of the recognition of CLINICAL FEATURES
accessory parotid tissue is that any pathologic process that can
occur in the main gland can also occur in the accessory tissue • Occurs in the age range of 5 to 81 years. However, usually
and incorrect diagnosis may lead to inadequate treatment. found in middle aged group with an average age of 41.7
• Perzik and White, 1966, reported that in 7.7 percent of years.
591 parotid tumors, an accessory parotid gland was • Male predilection has been reported.
involved.22 • Usually presents as an asymptomatic palatal mass that is
• The most common malignant salivary gland neoplasm in not ulcerated and that is normal or bluish in color.
accessory parotid tissue has been low grade mucoepidermoid • Generally firm and sessile, although, has been variously
carcinoma. Intermediate and high grade mucoepidermoid reported as soft or hard.
carcinoma, acinic cell carcinoma, malignant mixed tumor and
adenoid cystic carcinoma have also been reported. HISTOPATHOLOGIC FEATURES
• The most common benign salivary gland neoplasm in
• Mucosa appears normal although pseudoepitheliomatous
accessory parotid tissue has been a mixed tumor although cases
of Warthin’s tumor and papillary cystadenoma have occurred. hyperplasia has been reported.
• Sialography and palpation of the area while passing a • Underlying connective tissue contains enlarged lobules of
lachrymal probe through the Stenson’s duct may be used normal appearing mucous acini and ducts.
in the diagnosis of accessory parotid salivary gland lesions. • Salivary gland tissue has been described as hyperplastic,
present in greater than normal amounts and crowded.
HISTOPATHOLOGIC FEATURES • Inflammation and fibrosis are not significant features.
Histopathologically, the tissue is identical to the primary parotid • Aufdemorte et al, 1985, reported that cytologic features
tissue of the individual. Histological character of the neoplasm of fine needle aspirate of adenomatoid hyperplasia
developing in the accessory salivary gland tissue is identical to resemble low grade mucoepidermoid carcinoma and
that seen in lesions arising in the gland proper. cautioned that misin terpretation may result in
inappropriate treatment.25
Management
Management
1. Primary surgical management of lesions of accessory
parotid tissue must include complete removal , 1. Total excision of the lesion is recommended.
acceptable cosmetic result, avoidance of external 2. Recurrence is not expected.
salivary fistulas and preservation of the buccozygo- 3. However, association with mucoepidermoid carcinoma
matic rami of the facial nerve. has been suggested.
2. Polayes and Rankow, 1979, recommend a standard 4. Arafat et al, 1981, reported a patient in whom mucoe-
preauriculocervical flap that gives adequate exposure pidermoid carcinoma developed twelve years after the
for a superficial or total parotidectomy and that patient was diagnosed with mucinous salivary gland
provides an opportunity to preserve facial nerve hyperplasia.24
function.23
3. For high grade malignancy, they recommend total POLYCYSTIC (DYSGENETIC) DISEASE OF
parotidectomy with excision of the accessory gland. THE PAROTID GLANDS
4. For inflammatory or benign lesions, they recommend
excision of the accessory gland or excision of the Polycystic (dysgenetic) disease is the least common of the
accessory gland and superficial parotidectomy. benign cystic lesions of the parotid gland and is considered to
be a developmental malformation of the duct system.
ADENOMATOID HYPERPLASIA OF
MUCOUS GLANDS CLINICAL FEATURES
Adenomatoid hyperplasia of mucous glands was described by • High predilection in females.
Arafat et al, 1981, as representing a hyperplastic phenomenon • Usually evident during childhood and is bilateral in nature.
or a hamartomatous proliferation.24 However, Aufdemorte et • Typical history is that of a recurrent painless swelling of
al, 1985, argued that since adenomatoid hyperplasia does not the involved gland.
308 Essentials of Pediatric Oral Pathology

• No salivary or any other apparent abnormality of other • Other viruses causing this infectious condition are cyto-
salivary glands is evident. megalovirus, lymphocytic choriomeningitis virus, coxsackie-
• Seifert et al, 1981, reported that it has not been conclusively virus A, echovirus and parainfluenza virus type C, etc.
established whether polycystic (dysgenetic) disease of the • Acute suppurative sialadenitis is most commonly bacterial
parotid glands is associated with dysgenetic cyst of the in origin.
kidney, liver, lung or pancreas.26 • The causative agent most frequently implicated is
• Although the multifocal cystic pattern seen in this lesion Staphylococcus aureus. Other aerobic organisms isolated
may suggest a differential diagnosis of mucoepidermoid are Streptococcus pneumoniae, Hemophilus influenzae and
carcinoma, acinic cell adenocarcinoma and cystadeno- Escherichia coli. Anaerobic bacteria such as Bacteroides
carcinoma, the histologic features of widespread involve- have been found to be involved as well. Stasis of saliva,
ment of salivary gland parenchyma, variable epithelial often as a result of dehydration, is thought to be an integral
lining, presence of spheroliths and microliths and the component in the pathogenesis of this condition perhaps
relative lack of inflammation tilt the diagnosis in favor of secondary to obstruction or decreased production.
polycystic (dysgenetic) disease of the parotid glands. • Decreased salivary flow with stasis is a key factor in chronic
sialadenitis.
HISTOPATHOLOGIC FEATURES • Its development is often associated with a previous episode
of acute suppurative inflammation with subsequent
• Architecture of the glands remains undisturbed; however, glandular destruction. Another possibility is recurrent
the lobules are markedly distended and nearly replaced by parotitis of childhood which has continued into adulthood.
epithelium lined cysts, which impart a honey-combed or
lattice-like appearance. CLINICAL FEATURES
• Small residual islands of glandular acini are present
• Infectious sialadenitis caused by paramyxovirus group
between the cysts which vary in size and are variably lined
(mumps) typically involves the parotid glands bilaterally.
by flattened, cuboidal or columnar epithelium. The
• Children are most often affected.
columnar cells have abundant eosinophilic cytoplasm and
• Occurs in the age group of four to six years.
rounded luminal borders. These features give the
• Transmission is via infected respiratory droplets.
appearance of apocrine cells. • The parotid swelling is accompanied by constitutional
• Occasional ducts open directly into the cysts and some symptoms such as fever and malaise and many cases are
acinar units communicate with the cysts. These features mild and subclinical in nature. Stenson’s duct may be
suggest that the cysts arise from intercalated ducts. partially occluded.
• Most cyst lumina contain flocculent, eosinophilic material • Onset of symptoms usually follows a two to three week
and a few scattered macrophages. incubation period and infection may be documented by a
• Many cysts also contain spheroliths and microliths. rise in convalescent serum titers to viral antigens or to
isolation of the virus from the urine for a period from
Management approximately one week prior and two weeks after.
No treatment is necessary, given the purely benign nature • Serious sequelae are fortunately rare and often stem from
of this process. involvement of other organ systems such as the CNS,
pancreas and gonads.
SIALADENITIS
HISTOPATHOLOGIC FEATURES (FIG. 11.4)
Sialadenitis, defined as inflammation of a salivary gland, is a
common benign condition seen in children. • Lesional area shows scattered infiltration of salivary gland
parenchyma by lymphocytes and plasma cells.
ETIOLOGY • There is also acinar atrophy, ductal dilatation and fibrosis.
• May arise from various etiological factors, which may either Management
be infectious or non-infectious.
• Infectious sialadenitis may be of viral or bacterial origin. 1. Treatment is symptomatic in case of infectious
• Mumps is the most common viral infection causing sialadenitis of viral origin.
infectious sialadenitis. This illness is caused by an RNA 2. Complete bed rest is recommended.
3. Analgesics are prescribed.
virus from the paramyxovirus group.
Salivary Gland Lesions in Children 309

FIGURE 11.4: Histopathologic picture of chronic sialadenitis FIGURE 11.5: Sarcoidosis showing symmetric infiltrative
showing inflammation and fibrosis violaceous plaques on both the eyelids

4. In severe cases, corticosteroids are recommended for CLINICAL FEATURES

sialadenitis of viral origin. • Occurs at any age, most common in second to fourth decade.
5. Sialogogues, heat, massage of the affected gland and • Pulmonary manifestations are most characteristic of this
the administration of an appropriate intravenous
disease.
antibiotic, usually a penicillinase-resistant anti-
staphylococcal antibiotic are preferred treatment
• Parotid swelling occurs either unilaterally or bilaterally.
modalities for sialadenitis of bacterial origin. • Skin lesions occur in approximately 25 percent of the cases
6. Improvement is expected within the first 24 to 48 in the form of erythema nodosum and symmetric infiltrative
hours. If this does not occur, operative intervention violaceous plaques on the nose, cheeks, ears, forehead and
may be indicated. This usually consists of a standard hands; a term known as ‘lupus perino’.
parotidectomy—skin incision and flap followed by • Ocular involvement is variable.
creation of several openings within the substance of • Hepatic involvement occurs in 60 percent of cases.
the gland parallel to the course of the facial nerve. A • Osseous lesions are mostly seen in 5 percent of cases.
drain is then placed over the gland and the wound
• Granulomas may occur in nasal sinuses, pharynx, epiglottis
closed.
and larynx.

SARCOIDOSIS (FIG. 11.5) DIAGNOSIS

Sarcoidosis takes its name from the Greek words, sarx, meaning Kveim test was widely used earlier, but has largely been
“flesh” and osis, meaning “condition.” Hence, sarcoidosis is replaced by detection in serum of the levels of calcium,
nothing but a flesh (skin) condition. Sarcoidosis is a granulomatous angiotensin I converting enzyme (ACE), lysozyme and
disease of unknown etiology which might affect many systems.27 adenosine deaminase in recent times.
Although the disease is generally encountered between 20 to 40
years of age, it might be seen at any age.28 A few cases of HISTOPATHOLOGIC FEATURES (FIG. 11.6)
sarcoidosis in children have been reported by Siltzbach and • Histopathologically, non-caseating granulomas consisting of
Greenberg, 196829 and Jasper and Denny, 1968.30 epitheloid macrophages and multinucleated giant cells are
seen.
ETIOLOGY
• These granulomas may contain stellate inclusions (asteroid
• Unknown. bodies) and concentrically laminar calcifications
• Molecular biologic techniques suggest a possible role of (Schaumann bodies).
mycobacterium tuberculosis or a related organism as a • Periphery of the lesion shows diffuse lymphocytic
causative agent. infiltrate.
310 Essentials of Pediatric Oral Pathology

phase consists of the layered deposition of organic and

inorganic material.
• Submandibular stones are thought to form around a nidus
of mucous, whereas parotid stones are thought to form most
often around a nidus of inflammatory cells or a foreign body.
• Another theory that has been proposed states that an
unknown metabolic phenomenon can increase the salivary
bicarbonate content, which alters calcium phosphate
solubility and leads to precipitation of calcium and
phosphate ions.35
• A retrograde theory for sialolithiasis has also been
proposed. Some substances or bacteria within the oral
cavity might migrate into the salivary ducts and become
the nidus for further calcification. A case in which stone
formation around a vegetal nidus was histologically proven
has been reported. Salivary stagnation, increased alkalinity
FIGURE 11.6: Histopathologic picture of sarcoidosis showing central
area of calcification (Schaumann bodies), surrounded peripherally
of saliva, infection or inflammation of the salivary duct or
by diffuse lymphocytic infiltrate gland, and physical trauma to salivary duct or gland may
predispose to calculus formation.
• Submandibular sialolithiasis is more common as its saliva
Management is (i) more alkaline, (ii) has an increased concentration of
1. Corticosteroids seem to be the drug of choice in calcium and phosphate, and (iii) has a higher mucous content
treating symptomatic pulmonary sarcoidosis. than saliva of the parotid and sublingual glands. In addition,
2. Chloroquine, either alone or in combination with the submandibular duct is longer and the gland has an
corticosteroids may appear beneficial. antigravity flow. Stone formation is not associated with
3. Immunosuppressive drugs may be used for patients systemic abnormalities of calcium metabolism. Electrolyte
not responding to steroid treatment. and parathyroid hormone studies in patients with sialolithiasis
have not shown any significant abnormalities.36
SIALOLITHIASIS
CLINICAL FEATURES
It is estimated that sialolithiasis affects 12 in 1000 of the adult
population but is not very frequently seen in the pediatric • Males are affected twice as much as females.
population.31 Sialolithiasis accounts for more than 50 percent • Children are rarely affected but a review of the literature
of diseases of the large salivary glands and is thus the most reveals 100 cases of submandibular calculi in children aged
common cause of acute and chronic infections.32 3 weeks to 15 years.37
• More than 80 percent occur in the submandibular gland or
ETIOLOGY its duct, 6 percent in the parotid gland and 2 percent in the
• The exact etiology and pathogenesis of salivary calculi is sublingual gland or minor salivary glands.
largely unknown. • Salivary calculi are usually unilateral and are not a cause
• Genesis of calculi lies in the relative stagnation of calcium of dry mouth.38
rich saliva. They are thought to occur as a result of • Clinically they are round or ovoid, rough or smooth and of
deposition of calcium salts around an initial organic nidus a yellowish color (Fig. 11.7). They consist mainly of
consisting of altered salivary mucins, bacteria and calcium phosphate with small amounts of carbonates in the
desquamated epithelial cells.33 form of hydroxyapatite and smaller amounts of magnesium,
• For stone formation it is likely that intermittent stasis potassium and ammonia. This mix is distributed evenly
produces a change in the mucoid element of saliva, which throughout.39
forms a gel. This gel produces the framework for deposition • Submandibular stones are 82 percent inorganic and 18
of salts and organic substances creating a stone. percent organic material whereas parotid stones are
• Traditional theories suggest that the formation occurs in composed of 49 percent inorganic and 51 percent organic
two phases: a central core and a layered periphery.34 material. The organic material is composed of various
• The central core is formed by the precipitation of salts, carbohydrates and amino acids. Bacterial elements have not
which are bound by certain organic substances. The second been identified at the core of a sialolith.
Salivary Gland Lesions in Children 311

DIAGNOSIS
• Careful history and examination are important in the
diagnosis of sialolithiasis. Pain and swelling of the
concerned gland at mealtimes and in response to other
salivary stimuli are especially important. Complete
obstruction causes constant pain and swelling, pus may be
seen draining from the duct and signs of systemic infection
may be present.
• Bimanual palpation of the floor of the mouth, in a posterior
to anterior direction, reveals a palpable stone in a large
number of cases of submandibular calculi formation.
Bimanual palpation of the gland itself can be useful, as a
uniformly firm and hard gland suggests a hypo-functional
or non-functional gland. Recently Chung et al, 2007, in their
study reported that because of large proportion of relatively
FIGURE 11.7: Salivary duct stone small and distal sialolithiasis in pediatric patients, careful
bimanual palpation of the oral cavity is mandatory in the
• Sialolithiasis typically causes pain and swelling of the diagnostic approach for children suspicious of
involved salivary gland by obstructing the food related sialolithiasis.40
surge of salivary secretion. Calculi may cause stasis of • For parotid stones, careful intraoral palpation around
saliva, leading to bacterial ascent into the parenchyma of Stenson’s duct orifice may reveal a stone. Deeper parotid
the gland and therefore infection, pain and swelling of the stones are often not palpable.
gland. • When minor salivary glands are involved they are usually
• Some may be asymptomatic until the stone passes forward in the buccal mucosa or upper lip, forming a firm nodule
and can be palpated in the duct or seen at the duct orifice. It that may mimic a tumor.
may be possible that obstruction caused by large calculi is • Imaging studies are very useful for diagnosing sialolithiasis.
sometimes asymptomatic as obstruction is not complete and Occlusal radiographs are useful in showing radiopaque
some saliva manages to seep through or around the calculus. stones (Fig. 11.8). It is very uncommon for patients to have
• Long term obstruction in the absence of infection can lead a combination of radiopaque and radiolucent stones;
to atrophy of the gland with resultant lack of secretory
function and ultimately fibrosis.
Recently, Chung et al, 2007 in their study differentiated
between pediatric and adult sialolithiasis in a total of 210
patients with sialolithiasis confirmed by surgical treatment.
Twenty-nine of the 210 patients were of pediatric age group
(age < or = 18 years) and 181 were adult patients (age >19
years). Comparison of pediatric and adult sialolithiasis was
performed in terms of subject characteristics, clinical
manifestations, salivary calculi characteristics, treatment
modalities and outcomes. They reported that postprandial
recurrent swelling was the most frequent complaint in
pediatric sialolithiasis patients, similar to that in adult
patients. However, duration of symptoms was shorter in
pediatric patients (mean 14.1 months versus 30.7 months
in adults). Most calculi were less than 1cm in pediatric
patients (93.1 percent), compared to 56.3 percent of the
adult patients. The calculi were located more in the distal
duct (62.0 percent) than in proximal duct and gland in the
pediatric patients, whereas 44.7 percent were present in the FIGURE 11.8: Occlusal radiograph showing
distal duct in adult patients.40 radiopaque salivary duct stone
312 Essentials of Pediatric Oral Pathology

40 percent of parotid stones may be radiolucent. intraglandular stones. This is reserved for patients
Sialography is thus useful in patients showing signs of whose symptoms do not respond to conservative
sialadenitis related to radiolucent stones or deep sub- therapy and suffer from recurrent pain and swelling.
mandibular/parotid stones. Sialography is, however, 7. Alternative methods of treatment have emerged such
contraindicated in acute infection or in significant patient as the use of extracorporeal shock wave lithotripsy
contrast medium allergy. (ESWL) and more recently the use of endoscopic
intracorporeal shockwave lithotripsy (EISWL), in which
shockwaves are delivered directly to the surface of the
Management
stone lodged within the duct without damaging adjacent
1. Patients presenting with sialolithiasis may benefit from tissue (piezoelectric principle). Both extra and
a trial of conservative management, especially if the intracorporeal lithotripsy is gaining increasing
stone is small. The patient must be well hydrated and importance in the treatment of salivary stone disease.32
the clinician must apply moist warm heat and gland 8. In extracorporeal piezoelectric lithotripsy, the average
massage, while sialogogues are used to promote saliva size of fragments produced is about 0.7 mm. Duct
production and flush the stone out of the duct. With diameters are greater than 0.7 mm in general except
swelling of the gland and sialolithiasis, infection should for at the ostium. Therefore, fragments produced by
be assumed and a penicillinase resistant anti- ESWL would not be prohibited by duct diameters.
staphylococcal antibiotic prescribed. Most stones will Findings have also suggested that best results in
respond to such a regimen, combined with simple salivary stone lithotripsy are achieved when the
sialolithotomy when required.41 maximum size of stone fragments does not exceed
2. Almost half of the submandibular calculi lie in the distal 1.2 mm.43
third of the duct and are amenable to simple surgical 9. Extracorporeal salivary lithotripsy provides another
release through an incision in the floor of the mouth, therapeutic option that carries fewer risks than surgical
which is relatively simple to perform and not usually removal of the affected gland, such as the risks of a
associated with complications.42 Recently, Chung et al, general anesthetic administration, facial nerve damage,
2007, in their study have also suggested that intra-oral surgical scar, Frey’s syndrome and causes little
approach is an effective treatment procedure for most discomfort to the patient whilst preserving the gland.44
of the cases of sialolithiasis in children.40 10. A retrospective study of patients treated endoscopically
3. If the stone is sufficiently forward it can be milked and from 1994 to 1999 showed a success rate of 83 percent
manipulated through the duct orifice. This can be done with no severe complications.45
with the aid of lacrimal probes and dilators to open the 11. Endoscopy is a minimally invasive technique for removal
duct. Once open, the stone can be identified, milked of calculi from salivary glands as well as an excellent
forward, grasped and removed. The gland is then milked diagnostic procedure, as miniaturised endoscopes
to remove any other debris in the more posterior portion conforming to the physiological widths of the ducts are
of the duct. used to directly view and then deliver shock waves to
4. The duct may need opening to retrieve the stone. This the stones.46
involves a transoral approach where an incision is made
directly onto the stone. In this way more posterior BENIGN NEOPLASMS
stones, 1 to 2 cm from the punctum, can be removed
by cutting directly onto the stone in the longitudinal axis PLEOMORPHIC ADENOMA
of the duct. Care is taken as the lingual nerve lies deep,
but in close association with the submandibular duct It is a mixed tumor which shows epithelial and connective tissue
posteriorly. Subsequently, the stone can be grasped and features. Major and minor salivary glands are most commonly
removed. No closure is done leaving the duct open for involved. Pleomorphic adenoma was believed to originate from
drainage. cells of more than one germ layer hence was variously termed
5. If the gland has been damaged by recurrent infection as branchioma, enclavoma etc. WHO termed it as pleomorphic
and fibrosis, or calculi have formed within the gland, it adenoma because of a varied appearance under microscopic
may require removal.
field. The term “Pleomorphic” was first suggested by Willis,
6. Parotid stone management is more problematic as only
a small segment of Stenson’s duct is approachable
1948.47 It is usually more common in the 4th to 6th decades,
through an intraoral incision. In addition, opening hence not discussed in detail.
Stenson’s duct can be complicated by subsequent
stenosis of the duct whereas this is rare in the WARTHIN’S TUMOR
submandibular gland. As a result, parotidectomy is the
mainstay of surgical management for the majority of
1st described by Hildebrad in 189548 as congenital cyst of the
neck. Warthin in 192949 termed it as “papillary cystadenoma
Salivary Gland Lesions in Children 313

lymphomatosum”. Also called as cystic papillary adenoma, Regional lymph nodes (N)
branchiogenic adenoma, branchial cysts of parotid. It has rarely NX: Regional lymph nodes cannot be assessed.
been reported in the pediatric age group. The literature shows N0: No regional lymph node metastasis.
its incidence as less than one percent of all salivary gland N1: Metastasis in a single ipsilateral lymph node, 3 cm or
neoplasms in children. This warrants only a brief description less in greatest dimension.
of the tumor. N2: Metastasis in a single ipsilateral lymph node, more than
3 cm but not more than 6 cm in greatest dimension; or
MALIGNANT NEOPLASMS in multiple ipsilateral lymph nodes, none more than 6
cm in greatest dimension; or in bilateral or contralateral
MUCOEPIDERMOID CARCINOMA lymph nodes, none more than 6 cm in greatest dimension.
N2a: Metastasis in a single ipsilateral lymph node, more than
Mucoepidermoid carcinoma is the most common salivary gland
3 cm but not more than 6 cm in greatest dimension.
malignancy. It comprises of 5 to 9 percent of salivary neoplasms.
N2b: Metastasis in multiple ipsilateral lymph nodes, none more
De and Tribedi, 1939 first described mixed epidermoid and
than 6 cm in greatest dimension.
mucous secreting carcinoma of parotid.50 Stewart, Foot and
N2c: Metastasis in bilateral or contralateral lymph nodes, none
Becker, 1945, gave the term “Mucoepidermoid carcinoma.”51
more than 6 cm in greatest dimension.
N3: Metastasis in lymph node more than 6 cm in greatest
CLINICAL FEATURES
dimension.
• Age: 3rd to 8th decades, peak in 5th decade
• Slight female predilection is seen Distant metastasis (M)
• Most common salivary gland malignancy of children MX: Presence of distant metastasis cannot be assessed.
• Five to nine percent of salivary neoplasms M0: No distant metastasis.
• Site: Parotid-45-70 percent of cases, palate - 18 percent of M1: Distant metastasis.
cases Stage grouping
• More often seen in Caucasian than African American Stage 1: T1a N0 M0
population T2a N0 M0
• Presents as low grade and high grade tumors Stage 2: T1b N0 M0
• Low-grade: Slow growing, painless mass T1b N0 M0
• High-grade: Rapidly enlarging, pain may or may not be T1b N0 M0
present Stage 3: T3b N0 M0
• In minor salivary glands it may be mistaken for benign or T4a N0 M0
inflammatory process such as: Any T (except T4b) N1 M0
— Hemangioma
Stage 4: T4b Any N M0
— Papilloma
Any T N2N3 M0
— Tori.
Any T Any N M1
CLINICAL STAGING GROSS PATHOLOGY
American Joint Committee in 1988 proposed the staging • Well-circumscribed to partially encapsulated to unencap-
criteria based on four clinical variables viz; tumor size, local sulated mass.
extension of tumor, the palpability of and suspected metastasis • Solid tumor with cystic spaces.
to regional lymph nodes and the presence or absence of distant
metastasis. HISTOPATHOLOGIC FEATURES
Primary tumor (T) Grading criteria
TX: Primary tumor cannot be assessed. • Grading criteria are based on:
T0: No evidence of primary tumor. — Degree of cyst formation as opposed to solid growth.
T1: Tumor 2 cm or less in greatest diameter. — Proportion of cell types.
T2: Tumor more than 2 cm but not more than 4 cm in greatest — Presence or absence of cytomorphologic atypia.
dimension. • Divided into three types:
T3: Tumor more than 4 cm but not more than 6 cm in greatest 1. Low grade.
dimension. 2. Intermediate grade.
T4: Tumor more than 6 cm in greatest dimension. 3. High grade.
314 Essentials of Pediatric Oral Pathology

FIGURE 11.9: Histopathologic picture of low grade FIGURE 11.11: Histopathologic picture of high grade muco-
muc oepidermoid carc inoma s howing mucous cells epidermoid carcinoma showing predominant epidermoid cells with
predominating epidermoid cells increased mitotic figures and cellular pleomorphism

— Fewer and smaller cysts.

— Increasing pleomorphism and mitotic figures
• High-grade tumor (Fig. 11.11):
— Epidermoid cells predominate mucus cells.
— Solid tumor cell proliferation.
— Cytologic pleomorphism.
— Numerous mitotic figures.
— May be mistaken for squamous cell carcinoma.

Management
1. Conservative surgical approach with preservation of
facial nerve for stage I and stage II mucoepidermoid
carcinomas.
2. Aggressive surgical approach for stage III and stage
IV mucoepidermoid carcinomas.
3. Radical neck dissection performed in patients with
cervical node metastasis.
4. For minor salivary gland mucoepidermoid carcinomas,
FIGURE 11.10: Histopathologic picture of intermediate
treatment is primarily surgical.
grade mucoepidermoid carcinoma showing increased 5. In case if bone is not involved, wide excision down to
cellular pleomorphism in epidermoid cells periosteum with 1 or 2 cm tumor free lateral margins
is recommended.
6. Use of radiotherapy is controversial as most of the
• Low-grade tumor (Fig. 11.9): researchers think that these lesions are radioresistant.
— Mucus cells predominate epidermoid cells. Also, radiotherapy is contraindicated as it may give
— Prominent cysts. rise to other neoplasms such as sarcomas.
— Mature cellular elements. 7. Kaplan et al52 and Suen and Johns53 have suggested
• Intermediate- grade tumor (Fig. 11.10): that high grade mucoepidermoid carcinoma may show
similarity to squamous cell carcinoma.
— Mucus and epidermoid cells are equal in number.
Salivary Gland Lesions in Children 315

ACINIC CELL ADENOCARCINOMA

Acinic cell adenocarcinoma is the second most common parotid
and pediatric malignancy.

CLINICAL FEATURES
• Most common in the 5th decade
• Female predilection is seen
• Bilateral parotid disease is seen in three percent of the cases
• Solitary, slow-growing, often painless mass.

GROSS PATHOLOGY
• Well-demarcated tumor mass.
• Most often homogeneous.

HISTOPATHOLOGIC FEATURES FIGURE 11.12: Histopathologic picture of acinic cell

• Polyhedral cells. adenocarcinoma showing microcystic growth pattern
• Small, dark, eccentric nuclei.
Management
• Basophilic granular cytoplasm.
1. Surgical excision is the treatment of choice.
Four growth patterns may be seen: 2. If the tumor is confined to the superficial lobe of the
• Solid parotid gland, superficial parotidectomy is performed.
• Microcystic 3. When deep lobe is involved, total parotidectomy is
• Papillary cystic performed.
• Follicular. 4. Local enucleation is avoided.
5. In case of involvement of submandibular gland, total
Solid growth pattern resection of the gland is done.
• Large number of well-differentiated acinar cells mostly 6. In case of involvement of minor salivary glands,
resembling normal parotid gland parenchyma. complete local excision is required.
• Absence of striated ducts distinguish a solid growth from 7. Radical neck dissection is done only in cases of
normal salivary gland parenchyma. cervical lymph node involvement.

Microcystic (Fig. 11.12)

• More commonly seen growth pattern REFERENCES
• Small cystic spaces are seen 1. Regezi JA, Sciubba J, Jordan RCK. Textbook: Oral and
• Well-differentiated acinar cells, vacuolated and intercalated maxillofacial pathology: Clinical-pathologic correlations. 5th
duct-like cells (edn). WB Saunders Co., Philadelphia, PA, 2008.
• Microcystic space is due to coalescence of intracellular 2. Ellis GL, Auclair PL, Gnepp DR. Chapter 2: Developmental
vacuoles of ruptured cells. diseases. In: Ellis GL, Auclair PL, Gnepp DR, (Eds). Surgical
pathology of the salivary glands. WB Saunders Company:
Papillary cystic
10-26.
• One or more cystic structures that contain proliferation of
3. Mc Donald FG, Mantas J, Mc Ewen CG, Ferguson MM. Salivary
epithelium
gland disorder: An ectodermal disorder? J Oral Pathol 1986;15:
• Few folds of lining epithelium projecting into the lumina 115-7.
may be seen. 4. Bertram V Xerostomia. Acta Odontol Scand 1967;25(Suppl 49).
Follicular 5. Fox RI. Sjögren syndrome: New approaches to treatment. [www
• Least frequently encountered growth type document] (nd 2/1/01). Available from URL: www.medscape. com/
• Round cystic spaces lined by cuboidal or low columnar Medscape/rheumatology/TreatmentUpdate/2000/tu01/pnt-
epithelial cells tu01.html
• Intercystic space contains non-specific glandular cells with 6. Greenspan D. Xerostomia: Diagnosis and management.
some vacuolated and acinic type of cells. Oncology 1996;10(Suppl):7-11.
316 Essentials of Pediatric Oral Pathology

7. Sreebny LM, Valdini A. Xerostomia: A neglected symptom. patients with newly detected pulmonary sarcoidosis -results of
Arch Intern Med 1987;147:1333-7. a cooperative study in former West Germany and Switzerland.
8. McDonald E, Marino C. Dry mouth: Diagnosing and treating Sarcoidosis Vasc Diffuse Lung Dis 1998;15:178-82.
its multiple causes. Geriatrics 1991;46:61-3. 28. Merten DF, Kirks DR, Grossman H. Pulmonary sarcoidosis in
9. Dyke S. Clinical management and review of Sjögren’s childhood. AJR 1980;135:673-9.
syndrome. Int J Pharm Compound 2000;4:338-41. 29. Siltzbach LE, Greenberg M. Childhood sarcoidosis -a study of
10. Giansanti JS, Baker GO, Waldron CA. Intraoral mucinous, 18 patients. N Engl J Med 1968;279:1239-45.
minor salivary gland lesions presenting clinically as tumors. Oral 30. Jasper PL, Denny FW. Sarcoidosis in children. J Pediatr 1968;
Surg 1971;32:918. 73:505-12.
11. Arafat A, Brannon RB, Ellis GL. Adenomatoid hyperplasia of 31. Leung AK, Choi MC, Wagner GA. Multiple sialoliths and a
mucous salivary glands. Oral Surg 1981;52:52. sialolith of unusual size in the submandibular duct. Oral Surg,
12. Staffne EC. Bone cavities situated near the angle of the Oral Med, Oral Path, Oral Radiol, Endo 1999;87:331-3.
mandible. J Am Dent Assoc 1942;29:1969-72. 32. Zenk J, Benzel W, Iro H. New modalities in the management
13. Willis RA. Some unusual developmental heterotropias. Br Med of human sialolithiasis. Minimally invasive therapy 1994;3:
J 1968;3:267-72. 275-84.
14. Thackray AC, Lucas RB. Tumors of the major salivary glands. 33. Carr SJ. Sialolith of unusual size and configuration. Oral Surg,
Atlas of tumor pathology, 2nd series, fascicle 10. Washington, Oral Med, Oral Pathol 1965;20:709-12.
DC, Armed Forces Institute of Pathology, 1974;1-2. 34. Marchul F, Kurt AM, Dulguerov P, Lehmann W. Retrograde
15. Bernier JL, Bhaskar SN. Lymphoepithelial lesions of the Theory in sialolithiasis formation. Archives of Otolaryngology-
salivary glands. Cancer 1978;11:1156-78. Head and Neck Surgery 2001;127:66-8.
16. Azzopardi JG, Hou LT. The genesis of adenolymphoma. J Pathol 35. Blatt I. Studies in sialolithiasis: III. Pathogenesis, diagnosis, and
1964;88:213-8. treatment. South Med J 1964;57:723-8.
17. Youngs LA, Scofield HH. Heterotropic salivary gland tissue in 36. Work WP, Hecht DW. Inflammatory diseases of the major
the lower neck. Arch Pathol 1967;83:550-6. salivary glands. In: Papperalla MM, Shumrick DF, (Eds).
18. Himalstein MR. Letter to the editor. Laryngoscope, 1981; Otolaryngology Philadelphia, WB Saunders 1980;3:2235-
91:1200-1. 43.
19. Taylor G, Martin H. Salivary gland tissue in the middle ear. Arch 37. Steiner M, Gould AR, Kushner GM, Weber R, Pesto A.
Otolaryngol 1961;73:49-51. Sialolithiasis of the submandibular gland in an 8-year-old child.
20. Uemura S, Fujishita M, Fuchihata H. Radiographic Oral Surg, Oral Med, Oral Pathol, Oral Radiol, Endod 1997;
interpretation of so called developmental defect of mandible. 83:188.
Oral Surg, Oral Med, Oral Pathol 1976;41:120-8. 38. Cawson RA, Odell EW. Essentials of oral pathology and oral
21. Frommer J. The human accessory parotid gland: Its incidence, medicine 6th edn, Edinburgh: Churchill Livingstone 1998, p.
nature and significance. Oral Surg, Oral Med, Oral Pathol 1977; 239-40 .
43:671-6.
39. Williams MF. Sialolithisis. Otolaryngologic Clinics of North
22. Perzik S.L., White I.L. Surgical management of preauricular America 1999;32:819-34.
tumors of the accessory parotid apparatus. Am J Surg 1966;
40. Chung MK, Jeong HS, Ko MH, Cho HJ, Ryu NG, Cho DY, et
112:498-503.
al. Pediatric sialolithiasis: What is different from adult
23. Polayes I. M., Rankow R. M. Cysts, masses and tumors of the sialolithiasis? Int J Pediatr Otorhinolaryngol 2007;71(5):787-91.
accessory parotid gland. Plast Reconstr Surg 1979; 64: 17-23.
41. Pietz DM, Bach DE. Submandibular sialolithisis. General
24. Arafat A, Brannon RB, Ellis GL. Adenomatoid hyperplasia of
Dentistry 1987;35:494-6.
mucous salivary glands. Oral Surg, Oral Med, Oral Pathol 1981;
42. McGurk M, Esudier M. Removing salivary stones. Br J Hosp
52:51-5.
Med 1995;54:184-5.
25. Aufdemorte TB, Ramzy I, Holt GR, Thomas JR, Duncan DL.
43. Zenk J, Werner G, Hosemann MD, Iro H. Diameters of the main
Focal adenomatoid hyperoplasia of salivary glands: A
excretory ducts of the adult human submandibular and parotid
differential diagnostic problem in fine needle aspiration biopsy.
Acta Cytol 1985;29:23-8. gland – a histological study. Oral Surg, Oral Med, Oral Pathol,
Oral Radiol, Endod 1998;85:576-80.
26. Seifert G, Thomsen ST, Donath K. Bilateral dysgenetic parotid
glands. Morphological analysis and differential diagnosis of a 44. Iro H, Schneider HTh, Fodra C, et al. Shockwave lithotripsy of
rare disease of the salivary glands. Virchows Arch [A] 1981; salivary duct stones. Lancet 1992;339:1333-6.
390:273-88. 45. Nahlieli O, Baruchin AM. Long-term experience with endoscopic
27. Loddenkemper R, Kloppenborg A, Schoenfeld N, Grosser H, diagnosis and treatment of salivary gland inflammatory diseases.
Costabel U (WATL Study Group). Clinical findings in 715 Laryngoscope 2000;110:988-93.
Salivary Gland Lesions in Children 317

46. Nahlieli O, Baruchin AM. Sialendoscopy: Three years 50. De MN, Tribedi BP. A mixed epidermoid and mucus secreting
experience as a diagnostic and treatment modality. J Oral carcinoma of the parotid gland. J Pathol Bact 1939;49:432-33.
Maxillofac Surg 1997;55:912-8. 51. Stewart FW, Foot FW, Becker WF. Mucoepidermoid tumors of
47. Willis RA. Pathology of tumors. St. Louis’, CV Mosby Co, 1948;321. salivary glands. Ann Surg 1945;122:820-44.
48. Hildebrad O. Veber Angeborne Epitheliale Cysten und Fisteln 52. Kaplan MJ, Johns ME, Cantrell RW. Chemotherapy for salivary
des Halse. Arch F Clin Chir 1895;49:167-92. gland cancer. Otolaryngol Head Neck Surg 1986;95:165-70.
49. Warthin AS. Papillary cystadenoma lymphomatosum. A rare 53. Suen JY, Johns ME. Chemotherapy for salivary gland cancer.
teratoid of the parotid region. J Cancer Res 1929;14:116-25. Laryngoscope 1982;92:235-9.
12
318 Essentials of Pediatric Oral Pathology

Skin Lesions
in Children
Mayur Chaudhary, Shweta Dixit Chaudhary, Amol Gulhane

CHAPTER OVERVIEW
Introduction White sponge nevus
Ectodermal dysplasia Epidermolysis bullosa
Dyskeratosis congenita Pemphigus:
Incontinentia pigmenti Pemphigus foliaceous
Pachyonychia congenita Pemphigus vegetans
Ehlers-Danlos syndrome Pemphigus erythematosus
Osler-Weber-Rendu syndrome Paraneoplastic pemphigus
Peutz-Jeghers syndrome IgA pemphigus

INTRODUCTION insulation, shock absorption, and a reserve of calories. Both

sensory and motor nerves (autonomic fibers) are found in the
Skin is the soft and pliant membrane which covers the entire
dermis and the subcutaneous tissue.
surface of the body. It is man’s front-line protective barrier
Any abnormality within the structure or function of the
between internal structures and the external environment. It is
various layers of skin, may be genetic or environmental, may
tough, resilient, and virtually impermeable to aqueous solutions,
lead to skin diseases or disorders. The diseases of the skin are
bacteria, or toxic compounds. It protects against trauma,
divided into: Inflammation of the skin; Enlargement of the
regulates body temperature, serves as an organ of excretion
papillae of the skin; Disorders of the vessels of the skin;
and sensation, and synthesizes vitamin D in the presence of
Disorders of the sensibility of the skin; Disorders of the color
ultraviolet light. It has three primary layers: epidermis, dermis,
producing function of the skin.
and subcutaneous tissue. The epidermis (the outermost layer)
produces keratin as its primary function. The epidermis contains
two sublayers: the stratum corneum, an outer horny layer of ECTODERMAL DYSPLASIA
keratin that protects the body against harmful environmental The ectodermal dysplasias (EDs) comprise a large,
substances and restricts water loss, and the cellular stratum, heterogeneous group of inherited disorders that are defined by
where keratin cells are synthesized. The basement membrane primary defects in the development of two or more tissues
lies beneath the cellular stratum and serves to attach the derived from embryonic ectoderm. The tissues primarily
epidermis to the dermis. The dermis, the second primary layer involved are the skin, hair, nails, eccrine glands and teeth.
of the skin, consists of two fibrous proteins, fibroblasts, and Although Thurnam1 published the first report of a patient with
an intervening ground substance. The proteins are collagen, ectodermal dysplasia in 1848, the term ectodermal dysplasia
which strengthens the skin to prevent it from tearing, and elastin
was not coined until 1929 by Weech.2
to give it resilience. The ground substance, which makes the
skin soft and compressible, contains primarily dermis (top
CLASSIFICATION
layer) and the reticular dermis (bottom layer). Subcutaneous
tissue, the third primary layer of the skin, consists mainly of Freire-Maia and Pinheiro proposed the first classification
fat (containing mostly triglycerides), which provides heat system of the ectodermal dysplasias in 1982.3 Their original
Skin Lesions in Children 319

classification system stratified the ectodermal dysplasias into • X-linked hypohidrotic ectodermal dysplasia (Christ-
different subgroups according to the presence or absence of: Siemens-Touraine syndrome) is the most common
1. Hair anomalies or trichodysplasias ectodermal dysplasia. The patients suffering from this
2. Dental abnormalities condition show the following features:
3. Nail abnormalities or onychodysplasias, and — The typical facies, which is often not recognized until
4. Eccrine gland dysfunction or dyshidrosis. infancy, is characterized by frontal bossing, sunken
Priolo and Laganà, 2001, 4 reclassified the ectodermal cheeks, saddle nose, thick and everted lips, wrinkled,
dysplasias into two main functional groups: (1) Defects in hyperpigmented periorbital skin and large, low-set ears
developmental regulation/epithelial-mesenchymal interaction; (Fig. 12.1).
and (2) Defects in cytoskeleton maintenance and cell stability. — Dental manifestations include conical or pegged teeth,
Lamartine5 reclassified the ectodermal dysplasias into the hypodontia or complete anodontia and delayed eruption
following four functional groups based on the underlying of permanent teeth (Fig. 12.2).
pathophysiologic defect: (1) Cell-to-cell communication and — Most patients have fine, sparse, lusterless, fair hair (Fig.
signaling; (2) Adhesion; (3) Development; and (4) Others. 12.3).
Other classification systems categorize the ectodermal — Onychodystrophy may occur but is not common.
dysplasias based on defects in cell-cell communication and — Extensive scaling of the skin and unexplained pyrexia
signaling, adhesion, transcription regulation, or development.6 secondary to anhidrosis may occur in the neonatal
period. The development of a chronic eczematous
ETIOLOGY dermatitis is common.
— Other common signs are short stature, eye abnor-
Various genes responsible for signs and symptoms of ED have malities, decreased tear secretions and photophobia.
been found such as: • Hidrotic ectodermal dysplasia (Clouston syndrome) is
• Keratitis, ichthyosis, deafness (KID) syndrome is inherited in an autosomal dominant manner; the homozygous
caused by mutations in the GJB2 gene, which encodes state may be lethal. The patients suffering from this condition
connexin 26. show the following features:
• Margarita Island ectodermal dysplasia is caused by — Scalp hair is very sparse, fine, and brittle and alopecia
mutations in the PVRL1 gene, which encodes nectin-1. is common. Eyebrows are thinned or absent.
• Ectodermal dysplasia with skin fragility is caused by — Nail dystrophy is common. Persistent paronychial
mutations in the PKP1 gene, which encodes plakophilin 1. infections are frequent. Polydactyly, syndactyly and
bulbous fingertips may be present.
CLINICAL FEATURES — Patients have normal facies, no specific dental defects
and normal sweating.
• X-linked recessive hypohidrotic ectodermal dysplasia has
full expression only in males. Female carriers outnumber
affected men, but females show little or no signs of the
condition. Carriers always outnumber live affected patients
because of spontaneous abortion of the highly affected
fetuses.
• Many patients are not diagnosed until infancy or childhood,
when dental anomalies, nail abnormalities or alopecia
become apparent.
• Dry, hypopigmented skin is a feature. A chronic eczematous
dermatitis may be present.
• Sweating may be absent or reduced.
• Sparse, fair, brittle hair with alopecia is a feature, as are
absent or diminished body hair and sparse or absent
eyebrows and eyelashes.
• Nail dystrophy is a feature.
• Dental features may include hypodontia or anodontia;
malformed, rudimentary, or pegged teeth; and/or enamel
defects and frequent dental caries. FIGURE 12.1: Ten-year-old child with ectodermal dysplasia showing
• Diminished lacrimation and salivation are reported. hyperpigmented periorbital skin, thick, everted lips due to decreased
• Dysmorphic facies is a feature. vertical height of the jaws, depressed nasal bridge and saddle nose
320 Essentials of Pediatric Oral Pathology

conjunctivitis may develop. Nails are absent or dys-

trophic; pegged teeth are common. Mild hypohidrosis
is common. Hair may be sparse and coarse.
• Ectodactyly-Ectrodermal dysplasia-Cleft syndrome (EEC)
syndrome is inherited as an autosomal dominant trait of
low penetrance and variable expressivity. Many sporadic
cases have been reported. Ectrodactyly with tetramelic
3 to 4 syndactyly results in the characteristic lobster-claw
deformity of the hands and feet. Hypoplastic metacarpal
or metatarsal bones may be present. Cleft lip and palate
create a characteristic nasal contour.
— Other ectodermal anomalies include mild hypohidrosis,
coarse, dry hair with hypotrichosis, xerostomia,
dystrophic nails, dental enamel hypoplasia and
microdontia.
— Associated defects include blepharophimosis, lacrimal
FIGURE 12.2: Three-year-old child with ectodermal dysplasia duct anomalies, strabismus, deafness, choanal atresia
showing peg shaped incisors and abnormalities of the genitourinary tract.
• Rapp-Hodgkin ectodermal dysplasia is an autosomal
dominant syndrome. Felding noted that high forehead,
narrow nose, cleft lip or palate, and maxillary hyperplasia
produce a distinctive facies.8 Hypohidrosis is severe enough
to result in heat intolerance. Dental defects include conical
teeth and hypodontia. Hair are sparse, has a steel-wool
texture, and may show pili torti or pili canaliculi. Many
patients present with recalcitrant, inflammatory scalp
dermatitis followed by scarring alopecia. Nails are narrow
and dystrophic. Occasional abnormalities include deafness,
eye defects, and hypospadias.

HISTOPATHOLOGIC FEATURES
• Lesional area shows decrease in number of sweat glands,
hair follicles and sebaceous glands.
• Adnexal structures present are hypoplastic.
FIGURE 12.3: Three-year-old child with ectodermal dysplasia
• Epidermis is thin and flattened.
showing sparse hair
Management
— Other reported findings include reticulate hyper- 1. For patients with anhidrosis / hypohidrosis, encourage
pigmentation of the knees, elbows, and fingers; frequent consumption of cool liquids to maintain
palmoplantar keratoderma; and eccrine poromatosis. adequate hydration and thermoregulation.
• Hay-Wells syndrome is inherited as an autosomal dominant 2. Dhanrajani PJ recommends that for patients with
trait of variable expressivity. The patients suffering from dental defects, early dental evaluation and
this condition show the following features:7 intervention and routine dental hygiene be performed.
Dentures may be indicated as early as age two years.
— Scaling and erythema may be present at birth. The
Multiple replacements may be needed as the child
characteristic facies is due to ankyloblepharon
grows, and dental implants may eventually be
(congenital adhesion of the upper and lower eyelid required. Advise orthodontic treatment for cosmetic
margins by fibrous bands); a broad nasal bridge; and a reasons and to ensure adequate nutritional intake.9
sunken, hypoplastic maxilla. Cleft palate is common; 3. Patients with xerosis or eczematous dermatitis may
cleft lip is rare. benefit from the use of topical emollients.
— Recalcitrant, crusted, inflammatory scalp dermatitis 4. Patients with scalp erosions should be treated with
may cause scarring alopecia. Chronic blepharitis and topical and systemic antibiotics as needed. General
Skin Lesions in Children 321

scalp care may involve the use of weekly dilute bleach • Progressive nail dystrophy begins with ridging and
baths or acetic acid soaks to minimize bacterial longitudinal splitting. Progressive atrophy, thinning,
colonization of the scalp. Application of special scalp pterygium, and distortion eventuate in small, rudimentary,
dressings may be helpful. or absent nails.
5. Use artificial tears to prevent damage to the cornea • Mucosal leukoplakia occurs in approximately 80 percent
in patients with reduced lacrimation. of patients and typically involves the buccal mucosa, tongue
6. Protect nasal mucosa with saline sprays followed by (Fig. 12.5), and oropharynx. The leukoplakia may become
the application of petrolatum.
verrucous, and ulceration may occur. Patients also may have
7. Patients with ectodermal dysplasia with immuno-
an increased prevalence and severity of periodontal disease.
deficiency should be monitored for infection and
treated with therapeutic and/or prophylactic antibiotics
when appropriate.
8. Dupuis-Girod S et al report that allogeneic stem cell
transplantation has been performed in a small number
of patients with autosomal dominant ectodermal
dysplasia with immunodeficiency (EDA-ID); poor
engraftment and post-transplant complications were
common.10
9. Other midfacial defects or hand/foot deformities may
be surgically corrected in order to improve function
and reduce physical disfigurement.

DYSKERATOSIS CONGENITA
Dyskeratosis congenita (DKC), also known as Zinsser-Engman-
Cole syndrome, is a rare, progressive bone marrow failure
syndrome characterized by the triad of reticulated skin
hyperpigmentation, nail dystrophy, and oral leukoplakia. Mutations
in DKC1 have been shown to cause the X-linked form of
DKC. The inheritance pattern of most cases of DKC is
X-linked recessive, but autosomal dominant and recessive patterns FIGURE 12.4: Dyskeratosis congenita showing
have been reported. Autosomal dominant DKC is associated with dystrophic nail beds
TERC and TERT mutations in some cases, and NOP10 has been
associated with some cases of autosomal recessive DKC.11

CLINICAL FEATURES
• The primary finding is abnormal skin pigmentation, with tan-
to-gray hyperpigmented or hypopigmented macules and
patches in a mottled or reticulated pattern. Reticulated
pigmentation occurs in approximately 90 percent of patients.
Poikilodermatous changes with atrophy and telangiectasia are
common.
• The cutaneous presentation may clinically and histologi-
cally resemble graft versus host disease. The typical
distribution involves the sun-exposed areas, including the
upper trunk, neck and face.
• Other cutaneous findings may include alopecia of the scalp,
eyebrows, and eyelashes; premature graying of the hair;
hyperhidrosis; hyperkeratosis of the palms and soles; and
adermatoglyphia (loss of dermal ridges on fingers and toes).
• Nail dystrophy is seen in approximately 90 percent of
patients, with fingernail involvement often preceding toenail FIGURE 12.5: Dyskeratosis congenita showing
involvement (Fig. 12.4). hyperkeratosis of dorsal tongue mucosa
322 Essentials of Pediatric Oral Pathology

• Other mucosal sites may be involved (e.g. esophagus,

urethral meatus, glans penis, lacrimal duct, conjunctiva,
vagina, anus). Constriction and stenosis can occur at these
sites, with subsequent development of dysphagia, dysuria,
phimosis, and epiphora.
• Approximately 90 percent have peripheral cytopenia of one
or more lineages. In some cases, this is the initial
presentation, with a median age of onset of 10 years.
• Bone marrow failure is a major cause of death, with
approximately 70 percent of deaths related to bleeding and
opportunistic infections as a result of bone marrow failure.
• Approximately 20 percent of individuals with DKC develop
pulmonary complications, including pulmonary fibrosis and
abnormalities of pulmonary vasculature.
• The recommendation is that DKC patients avoid taking
drugs with pulmonary toxicity (e.g. busulfan) and that they FIGURE 12.6: Histopathologic picture of dyskeratosis congenita
have their lungs shielded from radiation during bone showing atrophic epidermis with areas of hyperpigmentation in the
marrow transplant (BMT). basal layer, and melanophages, telangiectasia, and mild infiltration
of inflammatory cells in the superficial dermis. Blister beneath the
• Patients have an increased prevalence of malignant mucosal
epidermis is due to liquefaction and degeneration of the basal cells
neoplasms, particularly squamous cell carcinoma of the
mouth, nasopharynx, esophagus, rectum, vagina, or cervix.
These often occur within sites of leukoplakia.
• The prevalence of squamous cell carcinoma of the skin is
also increased. Other malignancies reported include
Hodgkin’s lymphoma, adenocarcinoma of the gastro-
intestinal tract, and bronchial and laryngeal carcinoma.
• Malignancy tends to develop in the third decade of life.
• Patients may have learning difficulties and mental
retardation.
• Dyskeratosis congenita reportedly is associated with
conjunctivitis, blepharitides, and pterygium. Lacrimal duct
stenosis resulting in epiphora (i.e. excessive tearing) occurs FIGURE 12.7: Incontinentia pigmenti showing macular brown
in approximately 80 percent of patients. lesions on the skin of limb
• Patients may have mandibular hypoplasia, osteoporosis,
avascular necrosis, and scoliosis. 3. Short-term treatment options for bone marrow failure
• Gastrointestinal system findings: These may include in patients with DKC include anabolic steroids (e.g.:
esophageal webs, hepatosplenomegaly, and cirrhosis. oxymetholone), granulocyte macrophage colony-
• Hypospastic testes, hypospadias, and ureteral stenosis have stimulating factor, granulocyte colony-stimulating
also been reported. factor, and erythropoietin.12
4. The only long-term, curative option is hematopoietic
stem cell transplantation (SCT).
HISTOPATHOLOGIC FEATURES (FIG. 12.6)
• Lesional area in dyskeratosis congenita shows atrophic INCONTINENTIA PIGMENTI (FIG. 12.7)
epidermis with areas of hyperpigmentation in the basal
layer, and melanophages, telangiectasia, and mild Incontinentia pigmenti (IP) is an X-linked dominant neuro-
infiltration of inflammatory cells in the superficial dermis. cutaneous syndrome with cutaneous, neurologic, ophthalmologic,
• Blister beneath the epidermis is evident due to liquefaction and dental manifestations. Garrod reported the first probable
degeneration of the basal cells. case of incontinentia pigmenti in 1906 and described it as a
peculiar pigmentation of the skin in an infant.13
Management
1. Symptomatic treatment. ETIOLOGY
2. Routine medical examination to rule out development Incontinentia pigmenti is caused by mutations in the NEMO/
of aplastic anemia. IKK -gamma gene, which is located on chromosome Xq28.
Skin Lesions in Children 323

CLINICAL FEATURES involving the calves. Atrophic lesions usually develop

during adolescence and persist into adulthood. Atrophic
Incontinentia pigmenti is an X-linked dominant, male lethal
lesions have been reported to occur in 30 to 75 percent
syndrome. More than 95 percent of reported cases of inconti-
nentia pigmenti occur in females. of incontinentia pigmenti patients.
The proposed diagnostic criteria for incontinentia pigmenti — Hair changes include scarring alopecia and are seen in
are as follows: 28 to 38 percent of patients. An absence or hypoplasia
In the absence of a family history, the presence of at least one of the eyebrows and eyelashes has also been reported.
major criterion is necessary. The presence of minor criteria Finally, hair are sparse in early childhood; later, it has
supports the diagnosis of incontinentia pigmenti. a lusterless, wiry, and coarse appearance.
• Major criteria — Nail features include nail dystrophy, which ranges from
— Typical neonatal vesicular rash with eosinophilia mild pitting or ridging of the nail plate to hyperkeratosis
— Typical blaschkoid hyperpigmentation on the trunk, and onycholysis.14 This is observed in 7 to 40 percent
fading in adolescence of incontinentia pigmenti patients, and usually multiple
— Linear, atrophic hairless lesions. fingernails and toenails are affected. Nail dystrophy
• Minor criteria may improve with age. Subungual and periungual
— Dental anomalies keratotic tumors associated with pain, bony deformities,
— Alopecia and lytic lesions involving the underlying phalanges
— Wooly hair also may be seen, usually in older children and adults.
— Abnormal nails. The fingers are most commonly affected.
Ectodermal changes seen in incontinentia pigmenti. • Dental abnormalities are seen in 80 percent of patients and
— Skin features occur in four stages: can involve both deciduous and permanent teeth. Dental
anomalies are permanent and thus serve as a very useful
Stage 1 (vesicular) is characterized by the development
diagnostic finding in older patients. Delayed dentition,
of red papules and vesicles on an erythematous base.
partial anodontia, and conical or pegged teeth are the most
Lesions are seen predominantly on the extremities but
common dental findings. Poor enamel quality leading to
may also occur on the trunk or on the head and neck.
an increased incidence of dental caries has been reported
The vesicular stage has been reported to occur in 90 to
historically, but this association has been questioned.15,16
95 percent of patients. In most patients (>90%), lesions
• Ophthalmologic findings:
are present at birth or develop within the first two weeks
of life. They resolve within several months. — Ophthalmologic findings occur in 20 to 35 percent of
patients, and asymmetric involvement is common. Loss
Stage 2 (verrucous) is characterized by thickened, of visual acuity and blindness are significant
warty-appearing linear and whorled plaques on an complications. Blindness has been reported to develop
erythematous base. In general, lesions develop on the in 7 percent of incontinentia pigmenti patients.17
extremities and trunk but may also be seen on the head — Ophthalmologic manifestations may become evident
and neck. Verrucous lesions have been reported to occur within the first few weeks to months of life and may
in 70 to 80 percent of incontinentia pigmenti patients. progress rapidly to permanent visual deficits.
In most patients, verrucous lesions develop in the first — Retinal vaso-occlusive events with resultant ischemia
few weeks to months of life and subsequently resolve are believed to be the primary mechanism underlying
over weeks to months. ocular pathology.
Stage 3 (hyperpigmented) is characterized by the — Retinal manifestations include retinal detachment,
development of streaks and whorls of brown or slate- proliferative retinopathy, fibrovascular retrolental
gray pigmentation. This occurs in 90 to membranes, foveal hypoplasia, vitreous hemorrhages,
98 percent of incontinentia pigmenti patients. and atrophy of the ciliary body.
Hyperpigmented lesions usually involve the trunk but — Nonretinal manifestations include strabismus, optic nerve
may also involve the extremities, the skin folds, or the atrophy, conjunctival pigmentation, microphthalmia,
head and neck. Hyperpigmented lesions generally keratitis, cataracts, iris hypoplasia, nystagmus, and
develop within the first few months of life and resolve uveitis.
slowly by adolescence. • Neurologic abnormalities:
Stage 4 (atrophic / hypopigmented) is characterized — Neurologic complications occur in 30 percent of
by hypopigmented, atrophic, and reticulate or linear incontinentia pigmenti patients and often manifest
patches observed on the lower extremities, usually within the neonatal period.18
324 Essentials of Pediatric Oral Pathology

— Seizures are the most common neurologic complication dominant or recessive but sporadic cases do occur. Munro was
and usually develop within the first few weeks of life. the first to propose that the genetic defect in PC is linked to
— Neurologic complications may result in part from the keratin gene cluster on chromosome 17.21
microvascular vaso-occlusive ischemic events involving
the CNS. Involvement of the cerebral hemispheres, ETIOLOGY
cerebellum, and corpus callosum may occur. Progressive
It is now clear that pachyonychia congenita results from
periventricular hemorrhagic infarcts have been reported.
mutations in the genes encoding epidermal keratinocyte
— Other neurodevelopmental manifestations include
keratins, specifically the 1A and 1B helical encasing regions
developmental delay, mental retardation, ataxia, spastic
of keratins K6a, K6b, K16, and K17. The cause for sporadic
paralysis, microcephaly, cerebral atrophy, porencephaly,
pachyonychia congenita remains unknown. Currently two
hypoplasia of the corpus callosum, and periventricular
distinct syndromes of pachyonychia congenita are recognized:
cerebral edema.
(1) PC-1, or the Jadassohn-Lewandowsky type, and (2) PC-
• Other anomalies that have been reported to occur with
2, or the Jackson-Lawler type.22 Jadassohn-Lewandowsky
increased frequency in patients with IP include supernume-
type, or PC-1, is the more common variant. Mutations of the
rary nipples, nipple hypoplasia, and breast hypoplasia.
genes encoding keratins K6a and K16, which disrupt the keratin
filament assembly, characterize the disorder. In PC-2, mutations
HISTOPATHOLOGIC FEATURES (FIGS 12.8 AND 12.9)
in the keratin genes K6b and K17 are found.
• Lesional area in incontinenta pigmenti shows spongiosis
that manifest as epidermal intercellular edema with
exocytosis of numerous eosinophils and mononuclear cells
both within the epidermis as well as in spongiotic foci.
• Dyskeratotic keratinocytes are presented adjacent to spon-
giotic microvesicles. An interstitial infiltrate of lymphocytes
and eosinophils is present in the papillary dermis.

Management
1. Treatment is not usually required for the cutaneous
lesions. The vesicles of the inflammatory stage should
be left intact, and the skin should be monitored for
the development of secondary bacterial infections.
Emollients and topical antibiotics may be used as
needed.
2. Oral hygiene and regular dental care is necessary, FIGURE 12.8: Histopathologic picture of incontinentia pigmenti
and dental restoration may be indicated. Seizures showing marked edema of the upper epidermis
should be treated with anticonvulsants.
3. Routine neurodevelopmental assessments should be
made, with referral to occupational and physical
therapists as warranted.
4. Frequent ophthalmologic evaluations are required,
especially during the first year of life, in order to
diagnose and treat potential ophthalmologic
complications.
5. Abnormal retinal fibrovascular proliferation can be
treated with xenon laser photocoagulation or
cryosurgery.19
6. Retinal detachments may be treated using
vitreoretinal surgery.

PACHYONYCHIA CONGENITA
Pachyonychia congenita (PC) is a rare form of hereditary
palmoplantar keratoderma (PPK). Müller made the first FIGURE 12.9: Histopathologic picture of incontinentia pigmenti
documented observation in 1904.20 It may occur as autosomal showing epidermal spongiosis and an eosinophil-rich cellular infiltrate
Skin Lesions in Children 325

CLINICAL FEATURES
• The affected nails are usually present at birth, but they
may appear later, as they do in pachyonychia congenita
tarda.
• Paller et al described five patients in whom the disfigured
nails appeared when they were aged 10 to 30 years, as they
do in pachyonychia congenita tarda.23
• Pachyonychia congenita occurs equally in males and
females.
• The nail changes are the most prominent feature. The nail
plate is substantially thickened and brownish-gray with a
rough surface. This is thought to be due to deposition of
keratinaceous material in the nailbeds (Fig. 12.10).
• Circumscribed or diffuse hyperkeratoses are expressed on
the palms and soles. FIGURE 12.10: Pachyonychia congenita showing thickened and
• Follicular hyperkeratosis is observed on the face (e.g. atrophied nailbeds
temples, front, eyebrows) and on the extensor aspect of the
proximal parts of the extremities.
• Leucokeratosis of the oral mucosa is a prominent sign
especially of PC-1. Patchy whitish areas may be seen on the
back of the tongue, on the buccal mucosa, and, sometimes,
on the gingiva. In some patients, early tooth decay is observed.
• PC-2 is characterized by natal teeth and the consistent
presence of steatocystoma multiplex.
• A new pachyonychia congenita subtype was described in
two patients which included severe and generalized
hypotrichia with thickened nails.24
• Sebaceous cysts may appear later in life in case of
PC-2.

HISTOPATHOLOGIC FEATURES (FIG. 12.11)

• Lesional areas show marked hyperkeratosis, mostly FIGURE 12.11: Histopathologic picture of pachyonychia
congenita showing hyperkeratotic epithelium
parakeratosis, acanthosis and perinuclear clearing of
epithelial cells.
• Premalignant changes are not observed. the nail plate becomes thinner and its surface becomes
smoother.
5. Surgical ablation is not recommended, but it can be
Management
performed upon a patient’s insistence.
1. The thickened nail plate can be softened by using
20 percent salicylic acid ointment or 20 to 40 percent EHLERS-DANLOS SYNDROME (FIGS 12.12A AND B)
urea and 10 percent salicylic acid in an emulsifying
ointment with occlusive dressings. The Ehlers-Danlos family of disorders is a group of related
2. After the nail plate is softened, it can be mechanically conditions that share a common decrease in the tensile strength
abraded, which is sometimes performed by patients and integrity of the skin, joints, and other connective tissues.
even without softening. This abrasion may be followed The first detailed clinical description of the syndrome is
by additional softening of the nail plate. attributed to Tschernogobow in 1892.25 The syndrome derives
3. A five percent 5-fluorouracil cream can be applied its name from reports by Edward Ehlers, a Danish
twice daily. 5-fluorouracil cream can be applied after dermatologist, in 1901 and by Henri-Alexandre Danlos, a
thinning or abrasion of the nail plate to suppress the French physician with expertise in chemistry of skin disorders,
hyperproliferative activity of the nail matrix. in 1908. It is thought to be caused by various abnormalities in
4. Systemic treatment with retinoids (acitretin, retinoic
the synthesis and metabolism of collagen (a component of the
acid) in a daily dose of 1 mg/kg is partially effective;
matrix) and other connective tissue proteins.
326 Essentials of Pediatric Oral Pathology

MOLECULAR BASIS OF THE DISEASE OSLER-WEBER-RENDU SYNDROME

See Table 12.1. (FIGS 12.13A AND B)
Osler-Weber-Rendu syndrome, also known as hereditary
CLASSIFICATION
hemorrhagic telangiectasia (HHT), is an autosomal dominant
Types of Ehlers–Danlos Syndrome arranged in accordance to disorder typically identified by the triad of telangiectasia,
its new and previous nomendatures have been elaborated in recurrent epistaxis, and a positive family history for the disorder.
Table 12.2.
ETIOLOGY
CLINICAL FEATURES • The disease is caused by genetic defects with an autosomal
• It is thought to occur with a frequency of 1 per 5000 to dominant inheritance. So far, two primary loci have been
1 per 10,000. identified associated with Osler-Weber-Rendu syndrome:
• Ehlers-Danlos syndromes are heritable disorders. As such, one on chromosome arm 9q33-34 (HHT1) and a second
the disorders are present at birth. on chromosome arm 12q11-14 (HHT2).
• Most of the patients with this syndrome show malformed • Two more genes have recently been implicated; MADH4
teeth, large pulp stones and hypoplastic enamel. gene mutation in patients with a combined syndrome of
Osler-Weber-Rendu syndrome and juvenile polyposis and
Management an unidentified HHT3 gene linked to chromosome 5.27
1. Any type of medical treatment is unsatisfactory.
2. Extreme caution is mandatory in any surgical maneuver. CLINICAL FEATURES
3. Plastic re-excision of scars sometimes provides • The worldwide prevalence is 1 case per 5,000 to 10,000
acceptable cosmetic results. population.

FIGURES 12.12A and B: Ehlers-Danlos syndrome showing hypermobility of the joints

TABLE 12.1: Molecular basis of Ehlers-Danlos syndrome

Type Old nomenclature Protein abnormality Gene abnormality Chromosome locus

Classic Type I/II Type V collagen COL5A1, 9q34.2-34.3

COL5A2 2q31

Hypermobility Type III Unknown Unknown Unknown

Vascular Type IV Type III collagen COL3A1 2q31

Kyphoscoliosis Type VI Lysyl hydroxylase deficiency PLOD1 1p36.3-36.2

(some)

Arthrochalasia Type VII A/B Type I collagen COL1A1 17q31-22.5

COL1A2 7q22.1

Dermatosparaxis Type VIIC N-proteinase ADAMST2 5q23-24

Skin Lesions in Children 327

TABLE 12.2: Types of Ehlers-Danlos syndromes26

Type Inheritance Previous nomenclature Major diagnostic criteria Minor diagnostic criteria

Classic Autosomal Types I and II Skin hyperextensibility, wide Smooth, velvety skin; easy bruising;
dominant atrophic scars, joint hypermobility molluscoid pseudotumors; subcuta-
neous spheroids; joint hypermobility;
muscle hypotonia; pos toperative
complication (e.g. hernia); positive
family history; manifes tations of
tissue fragility (e.g. hernia, prolapse)

Hypermobility Autosomal Type III Skin involvement (soft, smooth Recurrent joint dislocation; chronic
dominant and velvety), joint hypermobility joint pain, limb pain, or both;
positive family history

Vascular Autosomal Type IV Thin, translucent skin; arterial/ Acrogeria,hypermobile small joints;
dominant intestinal fragility or rupture; tendon/muscle rupture; clubfoot;
extensive bruising; characteristic early onset varicose veins;
facial appearance arteriovenous, carotid-cavernous
sinus fistula; pneumothorax; gingival
recession; positive family history;
sudden death of close relative

Kyphoscoliosis Autosomal Type VI – lysyl Joint laxity, severe hypotonia at Tissue fragility, easy bruising,
recessive hydroxylase deficiency birth, scoliosis, progressive arterial rupture, marfanoid,
scleral fragility or rupture of microcornea, osteopenia, positive
globe family history (affected sibling)

Arthrochalasia Autosomal Type VII A, B Congenital bilateral dislocated Skin hyperextensibility, tissue
dominant hips,severe joint hypermobility, fragility with atrophic scars, muscle
recurrent subluxations hypotonia, easy bruising,
kyphoscoliosis, mild osteopenia

Dermatosparaxis Autosomal Type VII C Severe skin fragility; saggy, Soft, doughy skin; easy bruising;
recessive redundant skin premature rupture of membranes;
hernias (umbilical and inguinal)

FIGURES 12.13A and B: Osler-Weber-Rendu syndrome showing red, nodular and hemorrhagic areas on lip and cheek
328 Essentials of Pediatric Oral Pathology

• It occurs with equal frequency and severity in both sexes. procedures in all patients with known pulmonary
• It may be congenital in nature. arteriovenous malformations or positive contrast
• Epistaxis is the most common manifestation of the disease. echocardiography findings (agitated saline solution
• Recurrent painless gastrointestinal bleeding. transthoracic contrast echocardiography [TTCE]
• Dyspnea, hemoptysis may be present. grade 1 or higher).
• Migraine headaches, headache, seizures, stroke and brain
abscess. PEUTZ-JEGHERS SYNDROME (FIGS 12.14A AND B)
• Visual disturbances may be noted.
Peutz-Jeghers syndrome (PJS) is an autosomal dominant
• Patients may be cyanotic because of right-to-left pulmonary
inherited disorder characterized by intestinal hamartomatous
shunting or pale because of anemia.
polyps in association with mucocutaneous melanocytic
macules. The syndrome was described in 1921 by Jan Peutz,
Management
a Dutch physician who noted a relationship between the
1. Septal dermoplasty can reduce the severity of intestinal polyps and the mucocutaneous macules in a Dutch
epistaxis by 75 percent. This procedure is performed family. Harold Jeghers (1904–1990), an American physician is
by replacing the nasal mucosa with autologous skin credited with the definitive descriptive reports of the syndrome
grafts. Telangiectasias may also develop on the
when he published “Generalized intestinal polyposis and
autologous skin grafts.
melanin spots of the oral mucosa, lips and digits” in 1949 with
2. Pulsed dye laser treatment may also be used to
photocoagulate telangiectasias in the nasal mucosa. McKusick and Katz.29 The eponym Peutz-Jeghers syndrome
3. Endovascular embolization for treatment of severe (PJS) was introduced by the radiologist Andre J. Bruwer in
acute epistaxis is also a treatment modality.28 1954. The cause of Peutz-Jeghers syndrome (PJS) appears to
4. Septectomy combined with septal dermoplasty may be a germline mutation of the STK11/LKB1 (serine/threonine
also be a viable option for patients with severe kinase 11) tumor suppressor gene in most cases (66–94%),
transfusion-dependent epistaxis. located on band 19p13.3.
5. Embolization, ligation, or surgical excision is indicated
for enlarging or symptomatic pulmonary arteriovenous CLINICAL FEATURES
malformations.
• It shows equal predilection for males and females.
6. Life-threatening gastrointestinal bleeds are
often effectively treated by segmental bowel resection. • Although the average age at which Peutz-Jeghers syndrome
7. Embolization of the hepatic artery in selected patients (PJS) appears is 23 years in men and 26 years in women,
with liver involvement may be used, as well as liver pigmented lesions are present in the first years of life and
transplantation. may fade at puberty, except for lesions on the buccal
8. Recent recommendations also advocate the use of mucosa, making the diagnosis possible in pediatric patients.
antibiotic prophylaxis prior to surgical or dental • Repeated bouts of abdominal pain in patients younger than
25 years.

FIGURES 12.14A and B: Pigmented melanin spots on lip and gingiva

Skin Lesions in Children 329

• Unexplained intestinal bleeding in a young patient. CLINICAL FEATURES

• Prolapse of tissue from the rectum.
• White sponge nevus almost always presents during
• Menstrual irregularities in females (due to hyper-
childhood.
estrogenism from sex cord tumors with annular tubules).
• There is no gender predilection.
• Gynecomastia in males (possible due to the production of
• It is characterized by soft, white, and spongy plaques in
estrogens from Sertoli cell testicular tumors).
• Precocious puberty. the oral mucosa. The surface of the plaque is thick, folded,
and may peel away from the underlying tissue (Fig. 12.15).
• Mucocutaneous pigmentation and melanin spots are
typical of patients with Peutz-Jeghers (PJS) syndrome, • The buccal mucosa is the most commonly affected site,
and they are present in more than 95 percent of cases. followed by lips, alveolar ridges, and the floor of the
They appear as small, flat, brown or dark blue spots with mouth.
an appearance of freckles, most commonly in the
peribuccal area. HISTOPATHOLOGIC FEATURES
• Cutaneous pigmentation is usually located in the perioral • The histopathologic features of white sponge nevus include
region, crossing the vermilion border, in the perinasal and epithelial thickening, hyperparakeratosis, and vacuolization
perioral areas, fingers and the toes, on the dorsal and velar of the keratinocytes in the suprabasal layers (Fig. 12.16).
aspects of the hands and the feet. • The entire spinous layer may show intracellular edema with
“basket-weave” and “cell-within-a-cell” appearances.
HISTOPATHOLOGIC FEATURES • There are few mitotic figures and there is never any
• Lesional areas from the tissue in Peutz-Jeghers syndrome evidence of dysplasia.
shows overgrowths in intestinal glandular epithelium • In cytological smears occasional cells will have condensed
supported by core of smooth muscle. eosinophilic cytoplasm immediately surrounding the
• Cutaneous lesions show acanthotic epithelium with nucleus.
elongation of rete ridges.
Management
Management 1. No specific treatment is required for white sponge
1. Annual physical examination that includes evaluation nevus as it remains essentially unchanged after the
of the breasts, abdomen, pelvis, and testes along with first few months of onset, except for the rare
genetic counseling. example of a plaque which extends onto the lip
2. Annual complete blood cell (CBC) count. vermilion and is surgically removed for aesthetic
3. Push enteroscopy and interoperative enteroscopy reasons.
with polypectomy are used to remove larger polyps 2. The occasional mildly symptomatic case may respond
and may defer the need for repeated small bowel to topical applications of tetracycline.
resections.
4. Laparotomy and resection, as indicated, for small
intestinal intussusception, obstruction, or persistent
intestinal bleeding may be necessary.
5. Surgical treatment of extraintestinal cancers detected
by surveillance and diagnosis is required.

WHITE SPONGE NEVUS

White sponge nevus (WSN) is a rare autosomal dominant
disorder that predominantly affects nonkeratinizing stratified
epithelia such as the oral mucosa. This disease was first
described by Hyde,30 but the term “white sponge nevus” was
coined in 1935 by Cannon.31

ETIOLOGY
White sponge nevus is thought to be caused by CK4 or CK13 FIGURE 12.15: White sponge nevus showing diffuse white area
point mutations. on lateral border of tongue
330 Essentials of Pediatric Oral Pathology

CLINICAL FEATURES
Three major categories of epidermolysis bullosa have been
identified: The onset of dystrophic epidermolysis bullosa is at
birth or early childhood.
1. Epidermolysis bullosa simplex
2. Junctional epidermolysis bullosa
3. Dystrophic epidermolysis bullosa.

Epidermolysis Bullosa Simplex (Fig. 12.17)

• The onset of epidermolysis bullosa simplex is at birth or
early infancy.
• The generalized form is inherited as an autosomal dominant
trait.

FIGURE 12.16: Histopathologic picture of white sponge nevus

showing hyperkeratosis and vacuolization of the keratinocytes in
the suprabasal layers

EPIDERMOLYSIS BULLOSA
Epidermolysis bullosa (EB) is a rare group of inherited
disorders that manifests as blistering or erosion of the skin and,
in some cases, the epithelial lining of other organs, in response
to little or no apparent trauma.

CLASSIFICATION WITH MOLECULAR BASIS OF

EPIDERMOLYSIS BULLOSA32
FIGURE 12.17: Epidermolysis bullosa simplex showing multiple
See Table 12.3. vesicles in buttock region

TABLE 12.3: Classification with molecular basis of epidermolysis bullosa

Level of skin cleavage Major type Target gene (protein) Known targeted protein

Intraepidermal (epidermolytic) Epidermolysis bullosa PKP1 (plakophilin1) Keratins 4 and 14; plectin; 6 4 integrin;
DSP (desmoplakin) plakophillin-1 desmoplakin
KRT5 (keratin-5)
KRT14 (keratin-14)
PLEC1 (plectin)
ITGA6, ITGB4 ( 6 4 integrin)

Intra-lamina lucida Junctional LAMA3, LAMB3, LAMC2 Laminin-332(laminin 5); type XVII collagen;
(lamina lucidolytic) epidermolysis bullosa (laminin-332) 6 4 integrin
LAMA3, LAMB3, LAMC2
(laminin-332)
COL17A1 (type XVII collagen)
ITGA6, ITGB4 ( 6 4 integrin)
COL17A1 (type XVII collagen)

Sub-lamina densa Dystrophic COL17A1 (type XVII collagen) Type VII collagen
(dermolytic) epidermolysis bullosa

Mixed Kindler syndrome KIND1 (kindling-1) Kindlin-1

Skin Lesions in Children 331

• It is characterized by formation of vesicles and bullae on • The patients show positive Nikolsky sign, i.e. separation
hands and feet. These vesicles rupture and form ulcers. of the epithelium with tangential pressure on the skin
These ulcers heal within one to two weeks without scarring. surface.
• The localized form is also called as familial form that • Oral manifestations occur in the form of bullae which is
occurs on hands and feet, and also shows recurrence. preceded by white spots and localized areas of inflam-
• It occurs during childhood or later in life, shows vesicles mation.
that get ulcerated and heals without scar formation. • Apart from these, rudimentary teeth, congenitally absent
teeth and hypoplastic teeth are seen.
Junctional Epidermolysis Bullosa (Fig. 12.18)
HISTOPATHOLOGIC FEATURES (FIG. 12.19)
• The onset of junctional epidermolysis bullosa is at birth.
• There is absence of any pigmentation and death occurs • Lesional area in simplex form shows intraepithelial clefting.
within three months of age. • Junctional and dystrophic forms show subepithelial clefting.
• The lesions occur as vesicles that rupture and form ulcers • Ultramicroscopically, clefting of basement membrane in
to such an extent that there occurs shedding of the sheets junctional form occurs at the level of lamina lucida whereas
of skin. in dystrophic form it occurs below lamina densa.
• Similar forms of ulcers are seen in the oral cavity and some-
times there is defect in formation of enamel and dentin.33 Management
1. The treatment of epidermolysis bullosa (EB) is
Dystrophic Epidermolysis Bullosa primarily preventive and supportive. Once blistering
It occus in two forms: has occurred, the blister should be punctured with a
1. Dominant and sterile needle or a blade. This may prevent the
2. Recessive. accumulation of fluid and pressure and may thus
prevent the blister from extending.
Dominant form 2. Open wounds should be covered with nonadherent
• It occurs in infancy. dressings such as petrolatum-impregnated gauze,
• The lesions occur on ankles, elbows, feet and head. hydrogels, fenestrated silicone dressings or absorbent
• The vesicles rupture forming ulcers that heal with formation foam silicone dressings. Tape and any significant
of scar. pressure to the skin must be avoided. Dressings can
be held in place with rolled gauze (such as Kerlix),
• Other clinical features include hyperhidrosis, hypertrichosis
with tape applied only to the dressing itself or by
and ichthyosis. stockinette (such as Surgifix or Spandage).34
• Sometimes bullae occur in the oral cavity.
Recessive form
• It is also known as classic form.
• It is seen at birth and characterized by presence of bullae
at buttocks, feet, scapulae, elbows and fingers.

FIGURE 12.18: Junctional epidermolysis bullosa showing shed FIGURE 12.19: Histopathologic picture of epidermolysis
sheet of skin in upper limb bullosa showing subepithelial clefting
332 Essentials of Pediatric Oral Pathology

3. Some authors recommend daily application of

polymyxin, bacitracin, or silver sulfadiazine topical
ointments to treat open or partially healed wounds,
which should be covered with petrolatum-impregnated
gauze or nonadherent synthetic dressing. Gentamicin
soaks (480 mg/L saline), acetic acid soaks (white
vinegar), and the addition of small amounts of bleach
to the bath water (e.g.: 1/8 cup per full tub) have been
used to decrease the overgrowth of pseudomonas
and staphylococcal organisms.
4. Surgical procedures can correct the deformities of
epidermolysis bullosa caused by repeated episodes
of blistering and scarring of the hand. Esophageal
dilatation or insertion of a gastrostomy tube may be
required if esophageal strictures develop.
5. Patients with limited donor sites for a skin graft may
need advanced therapy with bioengineered skin FIGURE 12.20: Pemphigus vulgaris showing multiple
products. Several products (e.g.: composite cultured vesicles on forearm
skin [CCS], Graftskin, Dermagraft) have been used
in the treatment of patients with epidermolysis bullosa.
• Nikolsky sign, i.e. separation of the epithelium with
tangential pressure on the skin surface is positive.
PEMPHIGUS • Involvement of mucous membrane, mainly the oral mucosa,
Pemphigus is a group of autoimmune diseases of the skin and/or has been seen in almost all cases and usually precedes the
mucous membranes that cause burn-like lesions or blisters that cutaneous lesions.
do not heal. The term was originally named by Wichman in • Oral lesions are seen in the form of superficial, ragged
1791.35 erosions and ulcerations distributed along the palate, labial
mucosa, ventral surface of the tongue and gingiva.
CLASSIFICATION • Ocular involvement occurs in the form of bilateral
conjunctivitis.
There are basically three types of pemphigus:
• Pemphigus vulgaris (PV) (Fig. 12.20)
• Pemphigus vegetans HISTOPATHOLOGIC FEATURES
• Pemphigus foliaceous (PF).
• The blistering or cleavage in this disease occurs above
CLINICAL FEATURES the basal layer of the epithelium, typically leaving a layer
of basal cells with rounded tops, reminiscent of
• The clinical features of pemphigus are essentially similar “tombstones”.
in children to that of adults. • The intact bulla is filled with serum and occasional
• In children, most commonly seen pemphigus is fogo
sloughed, rounded, keratinocytes resembling “fried eggs”,
selvagem which is a subtype of pemphigus foliaceous,
with large, hyperchromatic nuclei and a rim of eosinophilic
followed by pemphigus vulgaris.
cytoplasm. The latter acantholytic cells, called Tzanck
• Mean age of onset is 12 years.36
• Boys have a lower mean age of onset as compared to girls. cells, are best seen in the smear sample of a fresh blister
• Occurrence is most common in males with a male to female (Tzanck test). The suprabasal bulla frequently contains
ratio of 1:0.96. chronic and acute inflammatory cells, including eosinophils
• Certain aggravating factors have been associated with (Fig. 12.21).
childhood pemphigus vulgaris similar to adults. This • Direct immunofluorescence of perilesional mucosa shows
includes drugs like enalapril37 and monteleukast38 and a lacy or chicken-wire pattern of deposits around individual
vaccination with diphtheria-tetanus toxin.39 spinous cells of the epithelium (Fig. 12.22).
• Lesions appear as flaccid vesicles on erythematous or • IgG is almost always the deposited immunoglobulin, but
normal skin which break easily to form erosions and crust. IgM and IgA are seen in almost half of all cases.
Skin Lesions in Children 333

4. Topical and intralesional steroids have been used for

single recalcitrant lesion to avoid escalation of
systemic dose.
5. When a high dose prednisolone is required to control
the disease, multiple adjuvants such as azathioprine
have been used in children. Bjarnason et al suggested
2 mg/kg/day to be used initially in two divided doses
followed by maintenance dose of 1 mg/kg/day.
6. Paltzik et al successfully used gold in intramuscular
form as aurothioglucose or aurothiomalate or in oral
form as auronofin in children in dose of 15 mg/week
for 32 weeks.
7. Methotrexate was used by Faure et al in a dose of
20 mg IM/week.41
8. Other adjuvants that have been used are dapsone
100 to 200 mg per day, cyclophosphamide in dose
FIGURE 12.21: Histopathologic picture of pemphigus vulgaris
of 50 mg/day and plasmapheresis. Recently a 17-
showing suprabasilar bulla containing inflammatory cell infiltrate
year-old female with 10 year history of pemphigus
vulgaris refractory to azathiopurine, mycophenolate
mofetil (2 gm/day), prednisolone (2 mg/kg/day), IVIg
(1gm/kg) and plasmapheresis was treated success-
fully with anti CD 20 antibody Rituximab (375 mg/
m 2sub) with decline in circulating anti Dsg 1 and
Dsg 3.42

PEMPHIGUS FOLIACEOUS
Pemphigus foliaceous (PF) is generally a benign variety of
pemphigus. It is an autoimmune skin disorder characterized by
the loss of intercellular adhesion of keratinocytes in the upper parts
of the epidermis (acantholysis), resulting in the formation of
superficial blisters. Pierre Louis Alphee Cazenave documented
FIGURE 12.22: Direct immunofluorescence showing intercellular
the first description of pemphigus foliaceous in 1844.
immunoglobulin G throughout the epidermis of a patient with
pemphigus vulgaris
CLASSIFICATION
Pemphigus foliaceous has the following six subtypes:
Management
1. Pemphigus erythematosus (PE)
1. Oral corticosteroids have been used with some 2. Pemphigus herpetiformis (PH)
success but, Cushing’s syndrome, growth retardation 3. Endemic pemphigus foliaceous
and infection are the most common side effects seen 4. Endemic pemphigus foliaceous with antigenic reactivity
in children. characteristic of paraneoplastic pemphigus (but with no
2. Bjarnason et al have suggested an initial dose in the neoplasm)
range of 2 to 3 mg/kg/day with slow tapering to 5-8
5. Immunoglobulin A (IgA) pemphigus foliaceous, and
mg/kg/day in approximately two weeks following
6. Drug-induced pemphigus foliaceous.
alternate day schedule for further reduction.40
3. Methylprednisolone pulse (1 gm/day IV for 5 days)
Endemic pemphigus foliaceous or fogo selvagem occurs
and dexamethasone pulse (136 mg of dexametha- most commonly in children and thus has been described here in
sone/day IV for 3 consecutive days) has also been detail. The role of genetic factors is evident in fogo selvagem in
used successfully in severe cases with high antibody which a strong association exists with some human leukocyte
burden. antigen DRB1 (HLA-DRB1) haplotypes, including DRB1*0404,
1402, 1406, and 1401.
334 Essentials of Pediatric Oral Pathology

CLINICAL FEATURES In both types, oral lesions are common. This is a rare lesion
and only three cases of pemphigus vegetans have been reported
• Endemic pemphigus foliaceous, or fogo selvagem, occurs
till 2004 in children.
with a high frequency in central and southwestern Brazil
and in Colombia. The Terena reservation in Brazil, a Neumann type: In this type, antibody is directed against 130
recently identified focus, has a prevalence of 3.4 percent and 85 kDa (kilo Dalton) polypeptides of the pemphigus
of the population.43 vulgaris antigen.
• Fogo selvagem often occurs in children, young adults, and • Clinical features: Initially, the lesions appear vesicular
genetically related family members. Metry et al 2002, in a erosive resembling pemphigus vulgaris evolving into
review of 28 cases reported that the average age at presen- vegetating plaques.
tation of this lesion was 7.7 years.44 • Histopathologic features: In the Neumann type, there are
• Childhood pemphigus foliaceous is slightly more common intraepidermal vesicles with suprabasilar acantholysis.
in boys with M:F ratio of 1.33:1. No eosinophilic microabscesses are present.
• Triggering factors for occurrence of this lesion are sunlight, Hallopeau type: In this type, antibody is directed against
drugs, bacterial infection, cytomegalovirus infection and 130 kDa (kilo Dalton) polypeptide.
otitis. • Clinical features: Initially, the lesions appear pustular
• Majority of children are asymptomatic. with a benign course and few relapses.
• Metry et al, 2002 reported the presence of crusted plaques, • Histopathologic features: In the Hallopeau type,
and erosions were the most common primary lesions in their eosinophilic spongiosis and microabscesses are present.
review of 28 cases. In the later vegetative plaques, there is prominent
• Lesions appear vesiculobullous, which later on rupture to epidermal hyperplasia with hyperkeratosis, papillo-
produce painful ulcers. matosis, and occasional acantholysis.
• An unusual circinate/arcuate/polycyclic pattern which is a
specific presentation in children has been described in a Management
few patients.45
• When direct immunofluorescence is performed, the Higher doses of steroids are usually prescribed.
antibody titer does not appear to correlate with the severity
and extent of skin disease. PEMPHIGUS ERYTHEMATOSUS

HISTOPATHOLOGIC FEATURES Pemphigus erythematosus (PE), also known as Senear-Usher

syndrome, is an overlap syndrome with features of lupus
• Lesional areas in pemphigus foliaceous show acanthosis erythematosus (LE) and pemphigus foliaceous.
in upper layers of epithelium.
• Separation of epithelium occurs without bulla formation. ETIOLOGY
• Epithelium may show hyperkeratosis or parakeratosis and Certain HLA haplotypes (A10 or A26, DRW6) are thought to
dyskeratotic cells within the granular cell layer. be associated, suggesting a genetic predisposition. In
• Dermal lymphocytic infiltration with presence of pemphigus erythematosus, the target antigen for the antibodies
eosinophils is also evident. is desmoglein 1.
Management CLINICAL FEATURES
1. Use of topical steroid is often recommended. • Kumar KA, 2008, reported a high prevalence of 4.4 cases
2. Dapsone is the most commonly used adjuvant. per million population, with a high preponderance (61.5%)
3. Other therapies include erythromycin, chloroquine, in females.46
methotrexate, sulfapyridine, azathioprine and • Pemphigus erythematosus is considered a variant of
hydroxychloroquine. pemphigus foliaceous rather than a distinct entity.
• Pemphigus erythematosus may occur at any age, but it is
PEMPHIGUS VEGETANS unusual in children. Four cases of pemphigus erythematosus
in children have been reported till date.
Pemphigus vegetans is a localized form of pemphigus vulgaris. • Lesions typically involve the scalp, face, upper part of the
CLASSIFICATION chest, and back.
• Patients with pemphigus erythematosus classically present
This rare form of pemphigus vulgaris has two variants: with small, flaccid bullae with scaling and crusting.
• Neumann and Occasionally, the appearance may suggest a papulo-
• Hallopeau. squamous disorder.
Skin Lesions in Children 335

• Secondary infection may occur, resulting in impetiginiza- CLINICAL FEATURES

tion, healing with pigment changes, and scarring.
• On the face, pemphigus erythematosus presents on the • Paraneoplastic pemphigus affects children and adolescents
bridge of the nose and on the malar areas as in the butterfly aged 8 to 18 years.
distribution seen in lupus erythematous. • Lesions are seen in both sexes equally.
• The earliest and most constant clinical finding in paraneo-
HISTOPATHOLOGIC FEATURES plastic pemphigus is painful oral erosions. The erosions can
The histology is identical to pemphigus foliaceous with a occur anywhere in the mouth, including the buccal, labial,
subcorneal blister and acantholysis. gingival, and lingual mucosae. All surfaces of the
oropharynx can be affected.
Management • Epistaxis is seen due to nasal ulcers.
• Patients may present with diffuse erythema, vesiculobullous
1. Topical corticosteroids are useful for patients with
lesions, papules, scaly plaques, exfoliative erythroderma,
limited disease or as an adjunct to systemic
therapy.
erosions, or ulcerations. The erythema can be macular,
2. Systemic steroids have been the mainstay of therapy urticarial, or targetoid, and it may be polymorphous.
for widespread pemphigus since their first use in Patients may initially present with erythema, and they may
1950. Prednisone 1 to 2 mg/kg/d as a single morning subsequently develop bullae and erosions.
dose or as intravenous pulses may control the • Large areas of denudation with a positive Nikolsky sign
disease. can occur.
3. Dapsone is effective in some patients with pemphigus
erythematosus. But pediatric dose had not been HISTOPATHOLOGIC FEATURES
established yet.
Mimouni et al 2002, reported four patterns of involvement of
4. Azathioprine is a potent immunosuppressive agent
that has been used as a steroid-sparing agent. Initial
the lesional tissue histopathologically:48
dose of 2 to 5 mg/kg/d PO/IV and maintenance dose 1. Sulli
of 1 to 2 mg/kg/d PO/IV has been administered. 2. Lichenoid infiltrate with cell necrosis
3. Intraepithelial acantholysis
PARANEOPLASTIC PEMPHIGUS (FIG. 12.23) 4. Combination of the above.
Pattern 3 was found to be most common.
Anhalt et al 1990, first described paraneoplastic pemphigus
in five patients with underlying neoplasms who developed oral Management
erosions and bullous skin eruptions.47 By immunoprecipitation,
target antigens were identified from skin extracts with 1. Warm compresses, nonadherent wound dressings,
and topical antibiotic ointment are helpful.
molecular weights of 250, 230, 210, and 190 kDa.
2. Potent immunosuppressive agents are required to
decrease blistering, but they are often ineffective.
High-dose corticosteroids are first-line therapy,
followed by steroid-sparing agents such as azathio-
prine, cyclosporine, and mycophenolate mofetil.49
3. Other therapeutic options include plasmapheresis,
immunophoresis, intravenous gammaglobulin, and
stem cell ablation therapy with high-dose cyclo-
phosphamide without stem cell rescue.
4. Rituximab has been tried in several patients with
mixed results.
5. For solid neoplasms, curative resection should be
attempted when appropriate.

IMMUNOGLOBULIN A PEMPHIGUS
Immunoglobulin A (IgA) pemphigus is a group of newly
characterized immune-mediated intraepidermal blistering skin
FIGURE 12.23: Paraneoplastic pemphigus showing oral erosions diseases.
336 Essentials of Pediatric Oral Pathology

ETIOLOGY 5. Lamartine J. Towards a new classification of ectodermal

dysplasias. Clin Exp Dermatol 2003;28(4):351-5.
Unlike typical immunoglobulin G (IgG)–mediated pemphigus, 6. Itin PH, Fistarol SK. Ectodermal dysplasias. Am J Med Genet
IgA pemphigus is characterized by tissue-bound and circulating C Semin Med Genet 2004;131C(1):45-51.
IgA autoantibodies that target the desmosomal proteins of the 7. Hay RJ, Wells RS. The syndrome of ankyloblepharon,
epidermis. At least three desmosomal components, including ectodermal defects and cleft lip and palate: an autosomal
desmoglein 3 (in intraepidermal neutrophilic–type [IEN-type] dominant condition. Br J Dermatol 1976;94(3):277-89.
IgA pemphigus), desmoglein 1, and desmocollin 1 (in 8. Felding IB, Bjorklund LJ. Rapp-Hodgkin ectodermal
subcorneal pustular dermatosis–type [SPD-type] IgA dysplasia. Pediatr Dermatol 1990;7(2):126-31.
pemphigus foliaceous), have been identified as target antigens 9. Dhanrajani PJ, Jiffry AO. Management of ectodermal dysplasia:
in IgA pemphigus. a literature review. Dent Update 1998;25(2):73-5.
10. Dupuis-Girod S, Cancrini C, Le Deist F, et al. Successful
CLINICAL FEATURES allogeneic hemopoietic stem cell transplantation in a child who
had anhidrotic ectodermal dysplasia with immuno-
• It is rarely seen in children and eight cases have been deficiency. Pediatrics 2006;118(1):e205-11.
reported in children till date including a 1 month old infant. 11. Mason PJ, Wilson DB, Bessler M. Dyskeratosis congenita—a
• A review of 28 cases reported from 1982-1997 revealed a disease of dysfunctional telomere maintenance. Curr Mol
male-to-female ratio of 1:1.33. Med 2005;5(2):159-70.
• Lesions form within erythematous plaques but also can 12. Giri N, Pitel PA, Green D, Alter BP. Splenic peliosis and rupture
form in skin without plaques. The initial, clear, fluid-filled in patients with dyskeratosis congenita on androgens and
blisters fill with neutrophils and transform into pustules. granulocyte colony-stimulating factor. Br J Haematol 2007;
• The trunk and proximal extremities, scalp and postauricular 138(6):815-7.
areas, and intertriginous areas (axillary and inframammary 13. Garrod AE. Peculiar pigmentation of the skin of the infant. Trans
areas) are involved. Clin Soc London 1906;39:216.
• Mucous membranes, palms, and soles usually are spared. 14. Nicolaou N, Graham-Brown RA. Nail dystrophy, an unusual
presentation of incontinentia pigmenti. Br J Dermatol 2003;
HISTOPATHOLOGIC FEATURES 149(6):1286-8.
15. Macey-Dare LV, Goodman JR. Incontinentia pigmenti: seven
Histopathologically, epidermal acantholysis and neutrophil cases with dental manifestations. Int J Paediatr Dent 1999;9(4):
infiltration predominate, hence the synonyms intraepidermal 293-7.
neutrophilic IgA dermatosis, intraepidermal IgA pustulosis, IgA 16. Minic S, Novotny GE, Trpinac D, Obradovic M. Clinical
herpetiform pemphigus, and intercellular IgA vesiculopustular features of incontinentia pigmenti with emphasis on oral and
dermatosis have been used to describe this group of diseases. dental abnormalities. Clin Oral Investig 2006;10(4):343-7.
17. Holmström G, Thorén K. Ocular manifestations of incontinentia
Management pigmenti. Acta Ophthalmol Scand 2000;78(3):348-53.
1. Generally, corticosteroids are the mainstay of 18. Aydingöz U, Midia M. Central nervous system involvement in
treatment. incontinentia pigmenti: cranial MRI of two siblings. Neuro-
2. Dapsone also may be helpful because of its radiology 1998;40(6):364-6.
antineutrophilic effects. 19. Nguyen JK, Brady-McCreery KM. Laser photocoagulation in
3. Rapid response to treatment with adalimumab and preproliferative retinopathy of incontinentia pigmenti. J
mycophenolate mofetil was reported in 2005.50 AAPOS 2001;5(4):258-9.
20. Muller C. On the causes of congenital onychogryphosis. Mcn
Med Wochenschr 1904;49:2180-2.
REFERENCES 21. Munro CS, Carter S, Bryce S, et al. A gene for pachyonychia
1. Thurnam J. Two cases in which the skin, hair and teeth were congenita is closely linked to the keratin gene cluster on 17q12-
very imperfectly developed. Proc RM Chir Soc 1848;31: q21. J Med Genet 1994;31(9):675-8.
71-82. 22. McKusick V. Mendelian Inheritance in Man. 11th ed. Baltimore,
2. Weech AA. Hereditary ectodermal dysplasia (congenital Md: J Hopkins University Press; 1994.
ectodermal defect). Am J Dis Child 1929;37:766-90. 23. Paller AS, Moore JA, Scher R. Pachyonychia congenita tarda.
3. Freire-Maia N, Pinheiro M. The ectodermal dysplasias. Arch A late-onset form of pachyonychia congenita. Arch
Dermatol 1982;118(4):215-6. Dermatol 1991;127(5):701-3.
4. Priolo M, Lagana C. Ectodermal dysplasias: a new clinical- 24. van Steensel MA, Koedam MI, Swinkels OQ, et al. Woolly hair,
genetic classification. J Med Genet 2001;38(9):579-85. premature loss of teeth, nail dystrophy, acral hyperkeratosis and
Skin Lesions in Children 337

facial abnormalities: possible new syndrome in a Dutch 37. Thami GP, Kaur S, Kanwar AJ. Severe childhood PV aggravated
kindred. Br J Dermatol 2001;145(1):157-61. by enalapril. Dermatology 2001;200:341.
25. Tschernogobow A. Ein fall von cutis laxa. Jahresber Ges 38. Cetkovska P, Piringer K. Childhood pemphigus associated with
Med 1892;27:562. montelukast administration. Clin Exp Dermatol 2003;28:328-9.
26. Beighton P, De Paepe A, Steinmann B, et al. Ehlers-Danlos 39. Cozzani E, Cacciapuoti M, Parodi A, Rbora A. Pemphigus
syndromes: revised nosology, Villefranche, 1997. Ehlers- following diphtheria and tetanus vaccination. Br J Dermatol
Danlos National Foundation (USA) and Ehlers-Danlos Support 2002;147:188-9.
Group (UK). Am J Med Genet 1998;77(1):31-7. 40. Bjarnason B, Flosadotter E. Childhood, neonatal and stillborn
pemphigus vulgaris. Int J Dermatol 1999;38:680-8.
27. Abdalla SA, Letarte M. Hereditary haemorrhagic telangiectasia:
current views on genetics and mechanisms of disease. Journal 41. Faure M, Cambazard F, Mauduit G. Pemphigus juvenile a
propos d’une observation. Lyon Med 1982;247:405-8.
of Medical Genetics 2006;43(2):97-110.
42. Kong HH, Prose NS, Ware RE, Hall RP. Successful treatment
28. Layton KH, Kallmes DF, Gray LA, Cloft HJ. Endovascular
of refractory childhood PV with anti CD20 monoclonal antibody
treatment of epistaxis in patients with hereditary hemorrhagic
(Rituximab). Pediatr Dermatol 2005;22:461-4.
telangiectasia. American Journal of Neuroradiology 2007;
43. Warren SJ, Lin MS, Giudice GJ, et al. The prevalence of
28(5):885-8.
antibodies against desmoglein 1 in endemic pemphigus
29. Jeghers H, McKusick VA, Katz KH. Generalized intestinal
foliaceous in Brazil. Cooperative Group on Fogo Selvagem
polyposis and melanin spots of the oral mucosa, lips and digits;
Research. N Engl J Med 2000;343(1):23-30.
a syndrome of diagnostic significance. N Engl J Med 1949;
44. Metry DW, Hebert AA, Jordan RE. Nonendemic pemphigus
241(25):993-1005, illust; passim.
foliaceous in children. J Am Acad Dermatol 2002;46:419-22.
30. Hyde JN. An unusual naevus of the tongue in a five-year-old
45. Jone SK, Schwab HP, Norris DA. Childhood pemphigus
boy. J Cutan Dis 1909 p. 256.
foliaceous: a case report and review of literature. Pediatr
31. Cannon AB. White sponge nevus of the mucosa (nevus spongious Dermatol 1986;3:459-63.
albus mucosa), Arch Dermatol Syphilol 1935;31: 365-70.
46. Kumar KA. Incidence of pemphigus in Thrissur district, south
32. Fine JD, Eady RA, Bauer EA, et al. The classification of India. Indian J Dermatol Venereol Leprol 2008;74(4):349-51.
inherited epidermolysis bullosa (EB): Report of the Third 47. Anhalt GJ, Kim SC, Stanley JR, Korman NJ, Jabs DA, Kory
International Consensus Meeting on Diagnosis and M, et al. Paraneoplastic pemphigus. An autoimmune muco-
Classification of EB. J Am Acad Dermatol 2008;58(6):931- cutaneous disease associated with neoplasia. N Engl J
50. Med 1990;323(25):1729-35.
33. Wright JT, Fine JD, Johnson L. Hereditary epidermolysis 48. Mimouni D, Anhalt GJ, Lazarova Z, Aho S, Kazerounian S,
bullosa: oral manifestations and dental management. Pediatr Kouba DJ, et al. Paraneoplastic pemphigus in children and
Dent 1993;15(4):242-8. adolescents. Br J Dermatol 2002;147:725-32.
34. Mellerio JE, Weiner M, Denyer JE, et al. Medical management 49. Martínez De Pablo MI, Iranzo P, Mascaró JM, Llambrich A,
of epidermolysis bullosa: Proceedings of the IInd International Baradad M, Herrero C. Paraneoplastic pemphigus associated
Symposium on Epidermolysis Bullosa, Santiago, Chile, with non-Hodgkin B-cell lymphoma and good response to
2005. Int J Dermatol 2007;46(8):795-800. prednisone. Acta Derm Venereol 2005;85(3):233-5.
35. Thivolet J. Pemphigus: Past, present and future. Dermatology 50. Howell SM, Bessinger GT, Altman CE, Belnap CM. Rapid
1994;189:26-9. response of IgA pemphigus of the subcorneal pustular dermatosis
36. Bjarnason B, Flosadotter E. Childhood, neonatal and stillborn subtype to treatment with adalimumab and mycophenolate
pemphigus vulgaris. Int J Dermatol 1999;38:680-8. mofetil. J Am Acad Dermatol 2005;53(3):541-3.
13
338 Essentials of Pediatric Oral Pathology

Infectious Diseases
in Children
Mayur Chaudhary, Shweta Dixit Chaudhary, Ragini Gulhane

CHAPTER OVERVIEW
Introduction Mycotic infections of the oral cavity:
Bacterial infections of the oral cavity: Candida albicans
Scarlet fever Viral infections of the oral cavity:
Diphtheria Herpes simplex virus
Tuberculosis Smallpox
Leprosy Measles (Rubeola)
Syphilis Rubella
Actinomycosis Herpangina
Noma Cytomegalovirus infection
Tetanus AIDS

INTRODUCTION occurring in chains (Fig 13.1). Incubation period for this

bacterium is three to five days. These bacteria produce a clear,
The oral flora is one of the most diverse microbial populations
colorless zone of hemolysis within which red cells are
known to man. It contains at least 350 different cultivable species.
completely lysed (Fig. 13.2).
The oral cavity provides some unique habitats for bacterial
colonization including teeth, mucosal surfaces, gingival crevices,
CLINICAL FEATURES
etc. Normally the oral flora exists in harmonious relationship with
the host. This relationship can be altered if normal oral environment • Occurrence in children is most common
is changed. The term infection is used to refer specifically to • Clinical manifestations of fever, malaise, chills and
infectious diseases, which are clinically evident diseases that result vomiting are seen
from the presence of pathogenic microbial agents, including • Enlargement of regional lymph nodes, especially cervical
viruses, bacteria, fungi, protozoa, multicellular parasites, and lymph nodes
aberrant proteins known as prions. Disease word itself implies any • Presence of diffuse, bright red skin rash particularly in areas
deviation from or interruption of the normal structures or function of skin folds. Such lines appear on 2nd or 3rd day and are
of any body part, organ, or system that is manifested by a known as “Pastias lines”
characteristic set of symptoms and signs and whose etiology, • Rash begins on upper trunk, spreads towards the
pathology and prognosis may be known or unknown. This chapter extremities, sparing the palms and soles
focuses on various diseases pertaining to children. • Desquamation of skin after a few days denotes terminal
stages of the disease
BACTERIAL INFECTIONS OF THE ORAL CAVITY • Palate and throat appear fiery red and congested
• Tonsils and faucial pillars are congested, swollen and show
SCARLET FEVER (SCARLATINA)
the presence of a grayish membrane
Scarlet fever is an infection produced by group A -hemolytic • Initially the tongue appears whitish due to presence of a
streptococci, which are gram-positive microorganisms coating on it. Later on due to loss of the coating, the
Infectious Diseases in Children 339

FIGURE 13.1: Streptococci (occurring in chains) FIGURE 13.3: Raspberry tongue

COMPLICATIONS
If left untreated, it may lead to peritonsillar abscess, rhinitis,
sinusitis, otitis media, meningitis, pneumonia, glomerulo-
nephritis, rheumatic fever and arthritis.

DIPHTHERIA
It is an acute, life-threatening, contagious bacterial infection
caused by gram-positive bacillus, Corynebacterium diphtheria
showing ‘wneiform appearance’ in Gram stain (Fig. 13.4).
Humans being the sole reservoirs, the infection mainly spreads
via droplet inhalation. Incubation period ranges from two to five
days.

CLINICAL FEATURES
• Occurrence in children is most common, especially during
FIGURE 13.2: Group A -hemolytic streptococci winter season
• Clinical manifestations of fever, malaise, chills, headache,
hyperemic and swollen fungiform papillae appear projecting anorexia and vomiting are seen
on the tongue, giving the tongue “Raspberry” appearance • Enlargement of regional lymph nodes, especially cervical
(Fig. 13.3). lymph nodes
• A patchy, yellowish-white thin film covered by grayish
DIAGNOSIS adherent membrane is seen known as “diphtheritic
membrane” (Fig. 13.5)
• Made on the basis of cultures from the throat secretions • Raw bleeding surface is seen left when this membrane is
• Detection of antigens specific for group A -hemolytic stripped off
streptococci • Due to involvement of soft palate, uvula, larynx and
• Dick test which is a test to determine susceptibility or immunity trachea, there occurs sore throat, stridor and respiratory
to scarlet fever by an injection of scarlet fever toxin. difficulties
• In severe cases, paralysis of soft palate can be seen.
Management
1. Use of antibiotics. DIAGNOSIS
2. Topical relief of symptoms by application of mupirocies • Made on the basis of cultures from the surface of the
ointment.
membrane and also from nasal secretions.
340 Essentials of Pediatric Oral Pathology

FIGURE 13.4: Corynebacterium diphtheriae FIGURE 13.6: Tubercle bacilli (arrow)

“cuneiform appearance”

2. Secondary tuberculosis: Occurs in people who have been

previously infected in their later life due to activation of
the micro-organism
3. Miliary tuberculosis: In which there is diffuse dissemi-
nation through vascular system.

CLINICAL FEATURES
• Fever, malaise, chills
• Night sweats, loss of weight, easy fatigability
• Persistent cough, lymphadenopathy
• ‘Scrofula’ is the term given for infected and enlarged
submandibular and cervical lymph nodes (Fig. 13.7)
• Primary tuberculosis of skin appearing as papular eruptions
FIGURE 13.5: Grayish adherent “diphtheritic membrane (arrows)”
and having a tendency for ulceration is termed as ‘Lupus
vulgaris’ (Fig. 13.8)
Management • Involvement of tongue in oral cavity is most common
1. Prophylactic active immunization with diphtheria toxoid. • Tuberculosis may also involve the bone of the maxilla or
2. Use of antitoxin in combination with antibiotics. mandible
• Although rare, lesion in the oral cavity may occur either
COMPLICATIONS due to hematogenous spread (anachoretic effect) or due to
If left untreated, may lead to myocarditis, polyneuritis and acute exposure to the infected sputum
interstitial nephritis. • The gingival lesion appears as diffuse, hyperemic and
nodular
TUBERCULOSIS • In case of an open cavity, if the micro-organism enters the
It is a chronic granulomatous disease caused by acid fast periapical region, there is formation of periapical
bacillus Mycobacterium tuberculosis which appears as straight granuloma termed as ‘Tuberculoma’.
or slightly curved slender rods (Fig. 13.6).

CLASSIFICATION HISTOPATHOLOGIC FEATURES (FIG. 13.9)

There are three types of tuberculosis: Lesional tissue reveals presence of granuloma containing
1. Primary tuberculosis: Occurs in people who were not central area of caseous necrosis surrounded by epithelioid cells,
exposed previously to the infection lymphocytes and giant cells.
Infectious Diseases in Children 341

FIGURE 13.7: Scrofula (Tuberculous lymphadenitis) FIGURE 13.9: Histopathologic picture showing caseation seen in
tuberculosis

LEPROSY (HANSEN’S DISEASE)

Leprosy is a chronic granulomatous disease caused by infection
with Mycobacterium leprae. M. leprae was first described in
1873 when Gerhard Armauer Henrik Hansen discovered it
while examining lymph nodes and other tissues obtained from
patients with leprosy. The microorganism appears as a straight
or slightly curved rod, similar to tubercle bacilli, but are less
acid fast as compared to tubercle bacilli (Fig. 13.10).

PATHOGENESIS
• The exact mechanism of M. leprae transmission remains
unknown; however, direct human-to-human contact, contact
with respiratory secretions from infected individuals, and
vertical transmission have been considered the most likely
routes of transmission. Most pathophysiological changes
observed in leprosy are caused by the ability of M. leprae
FIGURE 13.8: Lupus vulgaris to survive in macrophage cells. Although the incubation
period of M. leprae can be several decades, it generally
DIAGNOSIS averages 5 to 7 years.1
• Mantoux test (tuberculin test) • Once infected, both cell-mediated and humoral responses
• Sputum examination for micro-organisms with the help of are elicited by bacterial antigen DNA glycolipids.
Ziehl-Neelsen stain. Lipoarabinomannan, a component of the cell membrane,
induces immune suppression by inhibiting the interferon
Management gamma mediated activation of macrophages.
1. Two multiagent protocols are recommended as first
line therapy against drug susceptible tuberculosis:
CLINICAL FEATURES
a. Isoniazid + Rifampin for 9 months • The WHO has estimated the global prevalence of leprosy
b. Isoniazid (INH), rifampin and pyrazinamide for to be 10 to 12 million cases, with most reported in Africa
2 months followed by INH and Rifampin for and Asia, particularly in the Indian subcontinent.
4 months.
• The worldwide incidence rate is 2 cases per 10,000
2. Other first line medications include ethambutol and
streptomycin. Adverse effects limit the use of these
population. In some areas, as many as 10 percent of cases
drugs in children. develop in children younger than 15 years, among whom
paucibacillary forms are more frequent.2
342 Essentials of Pediatric Oral Pathology

FIGURE 13.10: Leprae bacilli FIGURE 13.11: Lepromatous leprosy

• Hypoesthesia is the clinical manifestation of peripheral • Other clinical manifestations of lepromatous leprosy
nerve involvement and is present in as many as 70 percent include corneal opacifications, keratitis, iritis,
of children with this condition. testicular atrophy and kidney disease resulting in renal
• The male-to-female ratio is 2:1 to 3:1. failure.
• Depending on the number of lesions and the number of • Patients with lepromatous leprosy present with
bacilli observed on the lesion’s smear, leprosy can be characteristically abnormal cell-mediated responses to
classified into the following three groups: M. leprae antigens.
1. Borderline leprosy: This is characterized by the • Erythema nodosum leprosum (ENL) is a condition
presence of single or multiple skin lesions with a raised
observed in patients with borderline and lepromatous
central area.
leprosy. It is usually associated with neuritis, fever and
2. Paucibacillary (tuberculoid) leprosy:
arthralgias. The development of ENL has been suggested
• Tuberculoid leprosy presents with one or few
(usually < 5) hypopigmented and hypoesthetic to be caused by the presence of immune complexes.
lesions. Sensory loss is frequently observed around • The development of nonhealing ulcers in the lower extremities
the lesions. of patients with lepromatous leprosy secondary to arthritis is
• Patients with tuberculoid leprosy have a known as the Lucio phenomenon. These ulcerations are more
characteristic normal cell-mediated response to common in individuals of Latin American descent and are
M. leprae antigens. associated with a high mortality rate.
3. Multibacillary (lepromatous) leprosy: • Neuropathic pain can be sequelae of multibacillary leprosy
• Lepromatoid leprosy usually presents with multiple that can present more than 10 years after completion of therapy.
(> 5) poorly defined, hypopigmented or erythematous • Apart from these generalized signs and symptoms, orofacial
lesions associated with hypoesthesia. It is also lesions observed are:
associated with the presence of papules, macules and — Facial paralysis
nodular lesions. Necrotizing erythema nodosum has — Lepromas
occasionally been reported in children. — Gingival hyperplasia
• Patients with advanced lepromatous leprosy may — Loosening of teeth.
present with loss of eyelashes or eyebrows and nasal
septum perforation. The constellation of disfiguring
facial features associated with this disease is named HISTOPATHOLOGIC FEATURES (FIG. 13.12)
leonine facies (Fig. 13.11). • Lesion is in the form of a granulomatous nodule.
• The peripheral neuropathy observed in lepromatous • It shows a collection of epitheloids, histiocytes, lymphocytes,
leprosy causes muscle weakness and atrophy and has Langhans type giant cells, vacuolated macrophages (lepra
been associated with clawhands and foot drops. cells).
Infectious Diseases in Children 343

FIGURE 13.12: Histopathologic picture showing FIGURE 13.13: Spirally arranged Treponema pallidum
granulomatous nodule seen in leprosy

DIAGNOSIS members of this genus include T. pallidum, Treponema pertenue

and Treponema carateum. Between 1905 and 1910, Schaudinn
• Diagnosis is usually made on the basis of culture of
organisms from the lesional area, nasal smears and and Hoffman identified T. pallidum as the cause of syphilis.3
scrapings, skin biopsy and nerve biopsy, inoculation in foot
pad of nine banded armadillo, histamine test, serological CLASSIFICATION
tests like Enzyme linked immunosorbent assay (ELISA) • Acquired syphilis
and Polymerase chain reaction (PCR). • Congenital syphilis

Management ACQUIRED SYPHILIS

1. Prolonged therapeutic regimens have traditionally been The acquired form of syphilis is contracted primarily as a
recommended in the treatment of leprosy; however, venereal disease, after sexual intercourse with an infected
recent recommendations by the WHO focus on partner, but it may also be acquired by professionals like dental
regimens with shorter duration for both tuberculoid surgeons, nurses or other individuals while carelessly handling
(paucibacillary) and lepromatous (multibacillary) leprosy.
2. The drugs that are more frequently used in the
the infected patients.
treatment of leprosy include rifampin, dapsone, There are three stages in which acquired syphilis occurs:
clofazimine, ofloxacin, minocycline and clarithromycin. 1. Primary syphilis
Multidrug therapy is required in all cases to prevent 2. Secondary syphilis
antimicrobial resistance. 3. Tertiary syphilis.
3. For multibacillary leprosy, the standard regimen
should include rifampin, dapsone, and clofazimine. PRIMARY SYPHILIS
For paucibacillary leprosy, rifampin is usually
prescribed in combination with dapsone. For single- • It has an incubation period averaging about 21 days.
lesion paucibacillary leprosy, a single dose of rifampin • The characteristic primary lesion of syphilis is called
combined with single doses of ofloxacin and “chancre” and it is a solitary, painless, indurated, non-
minocycline is recommended. tender, non-hemorrhagic ulcerated or eroded lesion.
4. Recent studies suggest that vaccination with Bacille • The oral lesions of primary syphilis occur on the lips,
Calmette-Guérin (BCG) during the neonatal period
tongue, palate, gingiva, uvula, tonsils, etc. usually three
may be protective against leprosy.
weeks after contact with the organism.
• Lymph nodes are enlarged bilaterally.
SYPHILIS
HISTOPATHOLOGIC FEATURES
Syphilis is a sexually transmitted disease (STD) caused by
infection with a spirochete named ‘Treponema pallidum • Primary syphilis: Skin and mucosal lesions show a
(Fig. 13.13) (T. pallidum).’ The genus name, Treponema, is perivascular and perijunctional infiltrate of lymphocytes,
derived from the Greek term for “turning thread.” Pathogenic plasma cells and macrophages.
344 Essentials of Pediatric Oral Pathology

• At times, capillary endothelial proliferation and subsequent • Small numbers of neutrophils may be included in the
obliteration of small blood vessels may be appreciable. perijunctional infiltrate and neutrophils may also be present
Focal erosion or ulceration is common. in an expanded overlying stratum corneum. Organisms are
readily demonstrable using T. pallidum immunoperoxidase
SECONDARY SYPHILIS staining during the secondary stage.
• The symptoms of secondary syphilis usually appear about six TERTIARY SYPHILIS
to eight weeks after the appearance of the primary infection.
• Symptoms include fever, malaise, rash, anorexia, arthralgias • It occurs about 5 to 10 years after the primary infection
and generalized painless lymphadenopathy. and it affects nearly every organ of the body.
• Renal, hepatic and ophthalmologic manifestations may be • Tertiary syphilis manifestations are divided into the following
present. subgroups:
— In Benign tertiary syphilis, gummatous lesions are
• Meningitis occurs in 30 percent of patients with secondary
found in skin and bones but rarely in other organs.
syphilis but may be asymptomatic. Symptomatic aseptic
Gummas are considered benign because they rarely
meningitis occurs in one to two percent of patients and is involve vital body structures.
characterized by headache, stiff neck, nausea and vomiting. — Cardiovascular tertiary syphilis can cause aortitis,
It is characterized by skin lesions, mucosal lesions and aortic aneurysm, coronary stenosis, aortic insufficiency
lymphadenopathy. and myocarditis.
• The oral lesions in this stage are called “mucous patches” — Meningitic tertiary neurosyphilis manifests with signs
that are seen over tongue, gingiva, tonsils, larynx, pharynx of meningitis and is differentiated from other causes
and cheek. of aseptic meningitis by a reactive result on a Venereal
• Multiple mucous patches in the oral cavity coalesce Disease Research Laboratory (VDRL) test of
together and form “snail track” like ulcers. cerebrospinal fluid (CSF); this test is termed a ‘CSF
• It is a highly contagious stage. VDRL.’
• Late neurosyphilis may present with focal neurological
HISTOPATHOLOGIC FEATURES (FIG. 13.14) findings suggestive of a stroke.
• Typical lesions of Tertiary syphilis are called “gumma”
• Secondary syphilis: Skin lesions are typified by a “lichenoid- which is a localized, chronic granulomatous lesion.
psoriasiform” configuration with a perijunctional infiltrate of • Intraoral lesions of tertiary syphilis are seen on the hard
lymphocytes, histiocytes and plasmacytes. Often the histiocytic and soft palate, lips and tongue (Fig. 13.15).
component of the infiltrate is prominent and thus the biopsy • Tongue lesions in syphilis are termed as ‘Syphilitic glossitis.’
may assume a “lichenoid-granulomatous” configuration. • It is a non-contagious stage.

FIGURE 13.14: Histopathologic picture of secondary syphilis showing FIGURE 13.15: Tertiary syphilis causing erosion of palate
a lichenoid infiltrate, vacuolar alteration of the superjacent epithelium
and subjunctional infiltrate rich in histiocytes and plasmacytes
Infectious Diseases in Children 345

• Saddle-nose is seen due to destruction of nasal septum and

collapse of nasal cartilage.

HISTOPATHOLOGIC FEATURES
• Tertiary lesions: Gummas consist of granulomatous
inflammation with central necrosis flanked by plump or
palisaded macrophages and fibrocytes surrounded by large
numbers of mononuclear leukocytes, including many
plasma cells.
• Treponemas may be scant in gummas and are sometimes
difficult to demonstrate.
• Aortitis reveals inflammatory scarring of the tunica media,
secondary to obliterative endarteritis of the vasa vasorum.
Uneven loss of the medial elastic fibers and muscle cells
may be evident.
FIGURE 13.16: A diffuse maculopapular desquamative rash
CONGENITAL SYPHILIS involving extensive sloughing of the epithelium

It is transmitted to the offspring only by an infected mother

and is not inherited.

CLINICAL FEATURES
• It is divided into:
— Early onset congenital syphilis
— Late onset congenital syphilis
Early onset congenital syphilis
• Early manifestations of congenital infection vary and
involve multiple organ systems. Mucous patches, rhinitis
and condylomatous lesions are highly characteristic features
of mucous membrane involvement in congenital syphilis.
• A diffuse maculopapular desquamative rash that involves FIGURE 13.17: Screwdriver shaped maxillary central incisors in
congenital syphilis
extensive sloughing of the epithelium, particularly on the
palms and soles and around the mouth and anus are
common findings. These lesions are highly infectious (Fig. • Oral manifestations show presence of ‘mulberry molars’
13.16). with constricted and atropic cusps, ‘screwdriver shaped’
• Hepatomegaly is seen. incisors (Fig. 13.17).
Late-onset congenital syphilis • Rhagades, i.e. fissuring and scarring of corners of mouth
• Manifestations include neurosyphilis and involvement of may be seen.
the teeth, bones, eyes and the eighth cranial nerve. • Frontal bossae and saddle nose are the characteristic
• The transmission usually occurs transplacentally or by features in infants suffering from syphilis.
sexual contact. • Hutchinson’s triad of hypoplasia of incisors and molar teeth,
• Vertical transmission of early syphilis during pregnancy eight nerve deafness and interstitial keratitis in eyes is seen.
results in a congenital infection in at least 50 to 80 percent
DIAGNOSIS
of exposed neonates.
• Other modes of transmission include contact with Diagnosis is based on detection of bacteria in smear by dark
contaminated blood or infected tissues. ground illumination microscopy, bacterial culture in artificial
• Children encounter two forms of syphilis: acquired syphilis, media and serological tests like Veneral Disease Research
which is almost exclusively transmitted by sexual contact Laboratory (VDRL), Fluorescent treponemal antibody (FTA),
and congenital syphilis, which results from transplacental Rapid plasma reagin (RPR) and Treponema pallidum
transmission of spirochetes. hemagglutination assay.
346 Essentials of Pediatric Oral Pathology

Management ACTINOMYCOSIS
1. Primary, secondary and early latent diseases are Actinomycosis is a chronic granulomatous, suppurative and
treated with a single intramuscular (IM) dose of fibrosing disea se. Th e causative microorganisms,
benzathine penicillin G (50,000 U/kg; not to exceed actinomyces, are related to mycobacteria and corynebacteria.
2.4 million U). Traditionally they are considered as a transition between
2. In patients with primary syphilis, doxycycline and bacteria and fungi. They are gram-positive, non-motile, non-
tetracycline have shown a high serological treatment sporing, non-capsulated filaments called as ‘ray fungus’ (Fig.
success rate, comparable to penicillin.4 13.18).
3. Azithromycin has also demonstrated a high cure rate Bollinger, 1877, for the first time found a mould like
in a long-term follow-up.5 organism in a lesion of lumpy jaw in cattle.6 Later, Wolff and
4. Patients who are allergic to penicillin and do not have Israel, 1891, isolated an anaerobic bacillus from human
neurosyphilis and are not pregnant may be treated lesions. 7 This was named Actinomyces israelii. The
with either doxycycline (100 mg oral [PO] bid for 2 actinomyces species is present as a commensal in mouth,
wk) or tetracycline (500 mg PO qid for 2 wk). Shorter- intestine and vagina.
acting forms of penicillin must be used to treat
neurosyphilis to produce reliably therapeutic levels in
ETIOPATHOGENESIS
the cerebrospinal fluid (CSF).
5. Aqueous crystalline penicillin G is recommended if • Actinomyces israelii, Actinomyces naeslundii, Actinomyces
congenital syphilis is proved or is highly suspected. odontolyticus, Actinomyces viscosus and Actinomyces meyeri
The recommended dosage is 100,000 to 150,000 U/ most frequently cause human actinomycosis. Actinomyces
kg/d IV every 8 to 12 hours to complete a 10-day to gerencseriae may also cause disease in humans.
14-day course. Procaine penicillin G (50,000 U/kg IM) • According to the Center for Disease Control and
has been recommended as an alternative to treat Prevention (CDC), three former coryneform bacteria now
congenital syphilis. have been added to the Actinomyces group and are thought
6. Congenital syphilis in older infants and children is to be pot ential causes for disea se; th ese
treated with aqueous crystalline penicillin (200,000– include Actinomyces neuii,8 Actinomyces radingae and
300,000 U/kg/d IV divided every 6 h for 10–14 d).
Actinomyces turicensis.
7. Syphilis in pregnancy is treated with penicillin,
• Actinomyces radicidentis, a recently described species, has
regardless of the stage of pregnancy. Patients are
been isolated with polymerase chain reaction from patients
administered 3 doses of benzathine penicillin (2.4
with endodontic infections.9
million U IM at 1-wk intervals).
• Predisposing factors for Actinomyces infection include:
8. Erythromycin treatment for pregnant patients who are
allergic to penicillin is not a reliable treatment for the
— Poor dental hygiene
fetus. — Oral surgical procedures
9. Early-acquired syphilis (i.e. primary, secondary, latent — Dental treatment
syphilis of < 1 y duration) is treated with a single dose
of IM benzathine penicillin G in a total dose of 50,000
U/kg (not to exceed 2.4 million U).
10. Syphilis greater than 1 year duration is treated with
benzathine penicillin G, 50,000 U/kg IM (not to exceed
2.4 million U) weekly for three successive weeks.
11. The recommended treatment for neurosyphilis is
aqueous crystalline penicillin G (200,000–300,000
U/kg/d IM [50,000 U/kg every 4–6 h]) for 10 to14 days
(adult dose, 12–24 million U/d [2–4 million U every 4
h]), followed by a single dose of benzathine penicillin
(50,000 U/kg/dose, not to exceed 2.4 million U) in 3
weekly doses.
12. Surgical correction of facial defects gives good
esthetic results.
13. Correction of dental defects using veneers or partial
or full coverage crowns may be done.
FIGURE 13.18: Actinomyces ‘Ray fungus’
Infectious Diseases in Children 347

— Oral trauma Thoracic actinomycosis

— Chronic mastoiditis • The most common route for infection is aspiration of
— Chronic otitis oropharyngeal or gastrointestinal secretions.
— Chronic tonsillitis • Patients may have a history or risk factors for aspiration.
— Any risk factor for aspiration • Other less common routes of infection include extensions from
— Alcohol or drug intoxication cervicofacial disease or spread from the abdomen and rarely,
— Altered mental status dissemination through blood from other sites of infection.
• The most common clinical presentation is a chronic,
— Neurologically devastated patients
indolent, slowly progressing pneumonia with or without
— Previous gastrointestinal surgery
pleural involvement. Patients present with a productive
— Perforation of the bowel, appendix, or colon cough, fever, chest pain and weight loss. The condition can
— Abdominal trauma mimic tuberculosis or malignancy.
— Foreign bodies • The disease can spread to the mediastinum and cause
— Typhoid fever tracheoesophageal fistulas, pericarditis, myocarditis or
— Amoebic dysentery endocarditis.
— Prior Actinomyces infection at a distant site, such as • Posterior mediastinal involvement can lead to vertebral
lungs, abdomen, or pelvis infection with bone destruction or disease of paraspinal
— Extension from contiguous source, such as cervicofacial muscles and soft tissues.
actinomycosis, paranasal infection or middle ear infection • Specific clinical or radiologic findings are not recognized.
— Prolonged use of intrauterine contraceptive devices • Physical examination reveals diffuse rales and rhonchi.
— Spread from intestinal infection. Decreased breath sounds can be appreciated if a pleural
empyema is present.
• The patient appears chronically ill.
CLINICAL FEATURES • Radiographic findings have the appearance of pneumonitis
• Infection can develop in individuals of all ages or a mass lesion and frequently, a pleural effusion is present.
• More commonly seen in males as compared to females • Hilar adenopathy can often be observed.
• Usual pattern of disease is abscess formation which drains • Extension to adjacent tissues with involvement of chest wall
by sinus tracts muscles and soft tissues may lead to the formation of sinus
tracts extending to the skin. This finding should always raise
• In human beings, infection occurs in four clinical forms.
the possibility of actinomycosis.
Cervicofacial actinomycosis
Abdominal actinomycosis
• This is the most common and recognized presentation of • Abdominal actinomycosis is the most covert and indolent
the disease. of all forms of the disease.
• Actinomyces species are commonly present in high • Diagnosis is rarely suspected or made on clinical grounds.
concentrations in tonsillar crypts and gingivodental Usually, the laboratory or the pathologist provides the diagnosis.
crevices. Many patients have a history of poor dentition, • The infection usually develops after gastrointestinal
oral surgery or dental procedures, or trauma to the oral mucosal integrity is broken from surgical procedures or
cavity. trauma, although, on many occasions, the inciting
• Chronic tonsillitis, mastoiditis and otitis are also important conditions may not be apparent.
risk factors for actinomycosis. • Conditions such as typhoid fever, amoebic dysentery and
• Periostitis or osteomyelitis can develop if the infection the presence of foreign bodies, such as chicken and fish
extends to facial and maxillary bones. The mandible bones, have precluded the development of actinomycosis.
appears to be one of the most common osteomyelitis sites. • Appendicitis with perforation is the most common
• Meningitis can also develop if the process extends into the predisposing event and as a result, right-sided abdominal
infection is far more common than left-sided abdominal
cranial bones through sinus tracts.
infection. The inciting event can precede the diagnosis by
• Patients may present with an acute form of the disease months to years.
characterized by the formation of a painful pyogenic • Patients present with nonspecific symptoms and findings, such
abscess with trismus and fistulas that drain the characteristic as fever, weight loss, diarrhea or constipation and abdominal
sulfur containing granules. pain. Extension to the perirectal space is not uncommon and
• More commonly, patients present with a painless indurated mass these patients present with defecation symptoms.
at the angle of the jaw or submandibular region with one or • Hepatic, renal and splenic disseminations are uncommon
several draining sinus tracts that discharge sulfur granules. complications of abdominal actinomycosis.
348 Essentials of Pediatric Oral Pathology

• An abscess or a hard, firm mass fixed to underlying tissue Management

can be palpated in all abdominal quadrants, more
commonly in the right lower quadrant. 1. Surgery is indicated for resection of necrotic tissue,
• Sinus tracts that extend to the abdominal wall or perirectal debulking of large masses, sinus tract excision,
tissues may be found sporadically. Patient experiences incision and drainage of empyemas and abscesses
localized or diffuse abdominal tenderness. and bone curettage.
2. Surgery alone is not curative and the use of prolonged
• Hepatomegaly and jaundice can be found with liver
courses of antibiotics is always required.
involvement. Appearance of symptoms is indolent and the
3. For most complicated cases, 4 to 6 weeks of intra-
patient may have had symptoms for months before seeking
venous penicillin G followed by 6 to 12 months of oral
attention. penicillin V is indicated. For patients with penicillin
Pelvic actinomycosis allergy, clindamycin, ceftriaxone, chloramphenicol and
• This condition is extremely rare in the pediatric population tetracyclines are good alternatives.
and is almost exclusively observed in patients who present 4. Parenteral and oral combinations of these drugs have
with prolonged use of intrauterine contraception devices, been successful. Because co-infection with
usually for longer than two years. A. actinomycetemcomitans is common, antibiotic
• Pelvic actinomycosis may develop from extension of intestinal cover for this organism when present is important,
especially in patients who are critically ill.
infection, commonly from indolent ileocecal disease.
5. Actinomycosis is susceptible to third-generation
• Patients present with an indolent history of vaginal discharge,
cephalosporins, rifampin, trimethoprim-sulfamethoxa-
abdominal or pelvic pain, menorrhagia, fever, weight loss
zole, ciprofloxacin, tetracyclines and chlorampheni-
and prolonged use of an intrauterine contraceptive device. col.
• Upon physical examination, a pelvic mass can be felt on
the adenexa and a vaginal or cervical discharge can be
NOMA
observed with speculum examination.
A fifth variety has been found recently that involves the Noma or cancrum oris is a rapidly spreading, gangrenous
central nervous system. stomatitis occurring in debilitated or nutritionally deficient
persons. Predisposing factors such as infections like diphtheria,
CNS disease
dysentery, measles, syphilis, tuberculosis, blood dyscrasias, etc.
• Clinical features are indistinguishable from those of other
infections of the CNS. may play an important role in development of this condition.
• The findings in patients without meningeal involvement are The microorganisms responsible for this condition are thought
typically those of a space-occupying lesion with focal to be fusospirochetal as seen in acute necrotizing ulcerative
neurologic defects and increased intracranial pressure. gingivitis, which are followed by streptococci, staphylococci
• The specific signs and symptoms are attributed to the and diphtheria bacilli.
anatomic location of the abscess, empyema or actino-
mycoma. CLINICAL FEATURES
• Patients with chronic meningitis have an indolent picture
• It occurs mostly in children who are malnourished, but may
that is no different from other chronic meningitides with
headaches, low toxicity and subtle neurologic findings also be seen in adults
dominating the picture. • The lesion begins as a small ulcer on gingival mucosa and
spreads rapidly involving the surrounding tissues.
HISTOPATHOLOGIC FEATURES • The tissues become inflamed, necrotic and finally a
demarcation is seen between the normal and the pathologic
• Pus from the lesion when examined on a clean slide shows tissues (Fig. 13.19)
sulfur granules • Fever, malaise, nausea are common symptoms
• Central area shows abscess within which colonies of micro-
• Foul odor from oral cavity is evident
organisms are seen
• If left untreated, patient may die of toxemia.
• Colonies appear to be floating in a sea of neutrophils associated
with multinucleated giant cells and macrophages.
Management
DIAGNOSIS 1. Maintenance of proper nutrition is the prime aim to
prevent this condition.
• Clinical features in a patient 2. The prognosis is better if antibiotics are administered
• Demonstration of organism in tissue section or smear before the patient reaches the final stages.
• Culture of tissue section or smear.
Infectious Diseases in Children 349

present with cranial nerve palsies. The infection may be

localized or may become generalized.
Neonatal tetanus is a major cause of infant mortality in
underdeveloped countries. Infection results from cord
contamination during unsanitary delivery conditions, coupled
with a lack of maternal immunization. At the end of the first
week of life, infected infants become irritable, feed poorly and
develop rigidity with spasms. This form of tetanus has a very
poor prognosis for survival.

ETIOPATHOGENESIS
• Tetanus results from infection with C. tetani, a motile,
spore-forming, anaerobic, gram-positive bacillus. This
bacillus is found in or on soil, manure, dust, clothing, skin
and 10 to 25 percent of human gastrointestinal tracts. The
FIGURE 13.19: Noma showing necrosis of
spores need tissue with the proper anaerobic conditions
the skin on the cheeks
to germinate; the ideal media are wounds with tissue
TETANUS necrosis.
• Under anaerobic conditions, the spores of C. tetani
The word tetanus comes from the Greek tetanos, which is
germinate and produce two toxins: tetanolysin (a hemolysin
derived from the term teinein, meaning to stretch. Nicolaier
with no recognized pathologic activity) and tetanospasmin,
discovered the anaerobic bacillus Clostridium tetani in 1885.10
which is responsible for tetanus. These toxins bind
In 1889, Koch’s pupil, Kitasato, obtained the bacillus of tetanus
irreversibly to the axon terminals. The spores give the
in pure culture and associated the disease to animals.10
bacilli a ‘drumstick appearance’ (Fig. 13.20).
• Once the toxin is synthesized, it moves from the contami-
CLASSIFICATION
nated site to the spinal cord in 2 to 14 days. When the toxin
There are four clinical types of tetanus, viz: reaches the spinal cord, localized or cephalic tetanus may
1. Generalized occur initially, followed by generalized tetanus.
2. Localized
CLINICAL FEATURES
3. Cephalic
4. Neonatal. • Neonatal tetanus accounts for 50 percent of the tetanus-
Approximately 50 to 75 percent of patients with related deaths in developing countries.
generalized tetanus present with trismus secondary to masseter • It may occur at any age.
muscle spasm. Nuchal rigidity and dysphagia also are early • Common first signs of tetanus are headache and muscular
complaints that cause risus sardonicus, the ironic smile of stiffness in the jaw (i.e. lockjaw), followed by neck stiffness,
tetanus, resulting from facial muscle involvement. As the
disease progresses, patients have generalized muscle rigidity
with intermittent reflex spasms in response to stimuli (i.e. noise,
touch). Tonic contractions cause opisthotonus (i.e. flexion and
adduction of the arms, clenching of the fists and extension of
the lower extremities). During these episodes, patients have
intact sensorium and feel severe pain. The spasms can cause
fractures, tendon ruptures and acute respiratory failure.
Patients with localized tetanus present with persistent
rigidity in the muscle group close to the injury site. The
muscular rigidity is caused by a dysfunction in the interneurons
that inhibit the alpha motor neurons of the affected muscles.
No further CNS involvement occurs and this form has very
low mortality rates.
Cephalic tetanus is uncommon and usually occurs FIGURE 13.20: Clostridium tetani showing a
following head trauma or otitis media. Patients with this form ‘drumstick appearance’
350 Essentials of Pediatric Oral Pathology

difficulty in swallowing, rigidity of abdominal muscles,

spasms and sweating.
• Patients often are afebrile.
• Severe tetanus results in opisthotonos, flexion of the arms,
extension of the legs, periods of apnea resulting from spasm
of the intercostal muscles and diaphragm and rigidity of
the abdominal wall (Fig. 13.21).
• Late in the disease, autonomic dysfunction develops, with
hypertension and tachycardia alternating with hypotension
and bradycardia.

Management
1. Passive immunization with human tetanus immune
globulin (TIG) shortens the course of tetanus and may
lessen its severity. A dose of 500 U appears as
effective as larger doses. FIGURE 13.21: Tetanic rigidity
2. Supportive therapy may include ventilatory support important to discuss about methods of sampling and techniques
and pharmacologic agents that treat reflex muscle
currently available for identification of isolates. Candida albicans
spasms, rigidity and tetanic seizures.
3. Benzodiazepines have emerged as the mainstay of is a common inhabitant of the oral cavity. The thallus of Candida
symptomatic therapy for tetanus. To prevent spasms consists of yeast cells. It is an ovoid or spherical budding cell,
that last longer than 5 to 10 seconds, administer mainly responsible for oppurtunistic infections like candidiasis.
diazepam intravenously, typically 10 to 40 mg every
1 to 8 hours. Vecuronium (by continuous infusion) or CLASSIFICATION
pancuronium (by intermittent injection) are adequate Candidal infection is frequently classified into two types:
alternatives.
1. Mucocutaneous candidiasis (oral or oropharyngeal,
4. Penicillin G, which has been used widely for years,
is not the drug of choice. Metronidazole (e.g. 0.5g intestinal, esophageal, etc.)
q6h) has comparable or better antimicrobial activity 2. Systemic candidiasis (involves eyes, kidneys and other
and penicillin is a known antagonist of GABA, as is visceral organs).
tetanus toxin.
5. Physicians also use sedative hypnotics, narcotics, ETIOLOGY
inhalational anesthetics, neuromuscular blocking
Various predisposing factors favor the development of
agents and centrally acting muscle relaxants (e.g.
intrathecal baclofen).
candidiasis.
6. To date, reports indicate that more than 26 adults with • Acute and chronic diseases like tuberculosis, diabetes
severe tetanus have been treated with intrathecal mellitus and anemia
baclofen. A representative dose of the continuous • Myxedema, hypoparathyroidism and Addison’s disease
infusion is 1750 mcg per day. Case reports and small • Immunodeficiency like AIDS
case series outline the efficacy of intrathecal baclofen • Nutritional deficiency like Fe, Vit. B6, Vit. A, etc
in controlling muscle rigidity. The effects of baclofen • Prolonged hospitalization for chronic illness, debilitating
begin within 1 to 2 hours and persist for 12 to 48 disease
hours. The half-life elimination of baclofen in CSF • Radiation therapy
ranges from 0.9-5 hours. After lumbar intrathecal
• Prolonged use of antibiotics, steroids and cytotoxic drugs.
administration, the cervical-to-lumbar concentration
ratio is 1:4. The major adverse effect of baclofen is a
depressed level of consciousness (LOC) and
ORAL MANIFESTATIONS
respiratory compromise. • Acute pseudomembranous candidiasis: This is the form
most often seen in children. It occurs mainly in debilitated
MYCOTIC INFECTIONS OF THE ORAL CAVITY or chronically ill children. Oral lesions include soft, white,
slightly elevated plaque occurring on buccal mucosa,
CANDIDA ALBICANS tongue, gingiva and floor of the mouth called as thrush
In recent years there is an emergence of opportunistic infections (Fig. 13.22). The plaques consist of masses of fungal
caused by Candida. Furthermore, many resistant forms of hyphae with intermingled desquamated epithelium, keratin,
Candida have emerged in the past several years. Hence, it is fibrin, necrotic debris and leukocytes.
Infectious Diseases in Children 351

FIGURE 13.22: Soft, white, curd-like plaques FIGURE 13.23: Creamy, convex colonies on
Sabouraud’s dextrose agar

• Acute atrophic: The lesions appear red or erythematous

rather than white thus appearing pseudomembranous where
membrane has been wiped off. It is the only type of
candidiasis which is painful according to Lehner.11
• Chronic hyperplastic: Lesion consists of white persistent
plaques on lips, cheeks and tongue. It is often called as
leukoplakia type of candidiasis.
• Chronic mucocutaneous: Presents oral lesions similar in
appearance and at similar sites as chronic hyperplastic
candidiasis.
• Chronic atrophic: Also called as denture sore mouth, a
diffuse inflammation of denture bearing areas often
occurring with angular cheilitis.

DIAGNOSIS
Diagnosis is usually on the basis of clinical features and culture.
FIGURE 13.24: Germ tube formation in serum
Various techniques for recovery of Candida from oral cavity are:
• Smear
• Method by Taschdjan et al, 1960, is the standard laboratory
• Swab
method for identifying C. albicans. 13 Test involves
• Concentrated oral rinse
induction of hyphal outgrowths from yeast cultured in
• Imprint culture
serum for 2 to 4 hours at 37 degree celsius. Approximately
• Impression culture technique
95 percent C. albicans produce germ tubes (Fig. 13.24)
• Salivary culture technique
along with C. stellatoidea, C. tropicalis and C. dubliniensis.
• Culture media that can be used:
— Most used is Sabouraud’s dextrose agar (Odds 1991)
Management
which permit growth of Candida and suppress growth
of other species of oral bacteria due to its low pH.12 It 1. Improved oral hygiene is of importance which includes
is incubated aerobically at 37 degree celsius for 24 to control of caries, keeping pacifiers and appliances
48 hours. Candida develops as creamy, convex colonies clean, replacing contaminated tooth brushes.
on Sabouraud’s dextrose agar (SDA) (Fig. 13.23). 2. Topical antifungal agents like compounded
— Pagano-Levin agar: Distinguishes between Candida clotrimazole suspension (10 mg/ml) and nystatin oral
species based on reduction of triphenyltetrazolium suspension (100,000 U per ml) may be swished for 2
minutes and swallowed/expectorated four times daily
chloride.
for two weeks. The patient should not eat or drink for
— Albicans ID: Differentiation is done on the basis of 30 minutes afterwards. Adolescents can use 1 to 2
chromogenic substrate for hexoseaminidase detection.
352 Essentials of Pediatric Oral Pathology

pastilles (200,000 U) slowly dissolved in the mouth 5 PRIMARY HSV INFECTION

times daily.
3. All antifungal agents formulated for topical use contain • The lesion occurs in patients with no prior infection with
sweeteners and may promote caries if used for an HSV1.
extended period. Nystatin solution contains 30 to 50 • Transmitted by contact with infected saliva.
percent of sucrose. Daily use of topical fluoride is • Transferred from the mouth to other parts of the body by
recommended to reduce the caries potential. touching the blister with fingers and then touching other
4. Clotrimazole troches (10 mg) also very rich in sucrose areas.
can be used by slowly dissolving in mouth, one troch • When the virus is transferred to the eye, ‘ocular herpes’
every 3 hours while awake (5 per day) for 14 days. The
child must be of age and maturity to comprehend and
may result which is painful and dangerous.
follow instructions to use troch vehicle. Liver toxicity has • When herpes blister forms on the fingers or the hand, the
been reported in patients using clotrimazole. Further lesion is called a ‘herpetic whitlow’.
clinical studies are required to establish the safety of
the drug in children less than 3 years. PATHOGENESIS
5. Systemic antifungal drugs are advantageous when
other topically delivered medications are administered • HSV-1 prefers to live in the trigeminal nerve root where it
concurrently. It is usually reserved for children either causes lesions in the oral cavity and on the face.
not tolerating or failing topical treatment or those at • After infection, the virus travels to the nerves in the face
risk of systemic infections. (trigeminal ganglia), where it lies dormant until triggered
6. Systemic agents include clotrimazole 6 mg/kg every through either stress or disease.
12 to 24 hours for 5 to 7 days; adolescents can use
• While less contagious when in its dormant state,
a 200 mg loading dose and then 100 to 200 mg once
a day for about a week.
transmission is still possible with no visible symptoms.
7. Ketoconazole may also be used in children at 5 to
10 mg/kg every 12 to 24 hours and in adolescents CLINICAL FEATURES
200-400 mg every 24 hours for 5 to 7 days. It is highly
• Primary infection occurs most often in children between
effective and has the advantage of good patient
compliance. Fluconazole is generally preferred over two and five years of age and is usually subclinical
ketoconazole which has a greater risk of associated • In case of clinical presentation, vesicles are formed. Vesicle
hepatotoxicity. Itraconazole and voriconazole are two rupture leads to ulceration (Fig. 13.25)
additional azoles with excellent activitiy against • Symptoms like fever, malaise, pain, nausea, flu, aches,
Candida. regional lymphadenitis are other clinical findings
8. Common side effects with systemic use include • HSV lesions erupt on intraoral tissues
nausea, vomiting, pruritis, skin rash, abdominal
• They always appear on attached tissue (attached gingiva,
discomfort, headache, abnormal liver function test and
drug induced hepatitis.
the hard palate and the dorsal surface of the tongue)
9. Chlorhexidine gluconate 0.12 percent can be used as • HSV when involves the tongue, occurs only on the
antimicrobial rinse and is most useful for maintenance ventral (top) surface and it causes red, swollen fungiform
purposes. papillae.
10. Antifungal ointments and cream include nystatin,
clotrimazole, myconozole and ketoconazole.
11. For chronic cases of angular cheilitis, Mycolog II is
the best choice when applied to the corners of the
mouth 3 times a day for 5 days.

VIRAL INFECTIONS OF THE ORAL CAVITY

HERPES SIMPLEX VIRUS
Herpes simplex virus (HSV) typically causes an acute infectious
disease characterized by the formation of blisters on the skin or
mucous membranes. Tissue preferentially involved by HSV
(Herpesvirus Hominis) consists of ectodermal derivatives such
as skin, mucous membrane, eyes, CNS.
• Two immunologically different types of HSV are:
1. HSV1 or HHV1
2. HSV2 or HHV2. FIGURE 13.25: Multiple ulcers on cheek and corner of mouth
Infectious Diseases in Children 353

HISTOPATHOLOGIC FEATURES PATHOGENESIS

• Lesional area shows an intraepidermal vesicle containing • Reactivation of latent HSV 1
ballooned, acantholytic keratinocytes in which there are • Triggers include physical trauma, sunlight, stress and
intranuclear inclusion bodies (Fig. 13.26). immunosuppression.

DIAGNOSIS CLINICAL FEATURES

• Diagnosis is based on clinical history, HSV isolation by culture • Affects perioral skin, lips, gingiva, palate
and by examining antibody titer in serum of the patients. • Self-limiting entity
• Lesion is preceded by tingling and burning sensation and
Management feeling of tautness, swelling or slight soreness and
1. Symptomatic treatment is often provided to facilitate
subsequent development of vesicle
intake of nutrition and relief from pain. • Edema at the site of the lesion is common occurrence.
2. Prevention of dehydration is of utmost importance in
children. Management
3. Acyclovir [9-(2-hydroxyethoxymethyl) guanine] if used 1. Symptomatic treatment is often provided to facilitate
early is found to be effective. intake of nutrition and relief from pain.
4. Vidarabine (adenine arabinoside) and Idoxuridine 2. Possible control with Acyclovir.
(5-iodo-2’-deoxyuridine) are also being used as 3. Systemic treatment is superior to topical treatment.
specific antiviral chemotherapeutic agents.
5. Healthy and nutritional diet can help boost the body’s
natural ability to control the disease. HERPES ZOSTER
Herpes zoster is an acute vesicular inflammation characterized
COMPLICATIONS by inflammation of dorsal root ganglia, or extramedullary
• Eczema herpeticum cranial nerve ganglia. It is associated with vesicular eruptions
• Corneal scarring and permanent damage to eyesight of the skin or mucus membrane in areas supplied by the affected
• Encephalitis. sensory nerves.

ETIOLOGY
RECURRENT HSV INFECTION
• Usually seen in adult patients and manifests clinically as Primary infection by varicella-zoster virus results clinically in
an attenuated form of primary disease chicken pox, while a recurrent infection results clinically in
• Vesicles precede ulcers. Ulcers are multiple and confluent herpes zoster (shingles).
• Occurs in keratinized mucosa.
CLINICAL FEATURES
• Chicken pox is more common in childhood whereas herpes
zoster lesions are more common in adult life (Fig. 13.27).
• Occurs with equal frequency in males and females.
• Herpes zoster (shingles) is usually diagnosed by the
distribution of the rash it causes.
• The distribution of the rash always corresponds to the
distribution of a single major nerve branch.
• The distribution of the rash usually involves exactly one
half of one of the cervical or thoracic dermatomes,
wrapping from the midline of the back, around to the
midline on the front of the body.
• When the infection involves the lumbar or sacral
dermatomes, the rash involves only one of the affected limbs.
• Shingles (herpes zoster) is not considered to be contagious.
FIGURE 13.26: Histopathologic picture of herpes simplex showing • This is because a healthy person who is exposed to a person
viral cytopathic effect with both Cowdry type A (arrowhead) and suffering from an active shingles infection will not contract
Cowdry type B (short arrow) inclusions shingles.
354 Essentials of Pediatric Oral Pathology

FIGURE 13.27: Chickenpox presenting as rashes on the skin FIGURE 13.28: Ballooning degeneration in epithelial cells

• On the other hand, he or she may become infected with Management

chickenpox if he/she has never been inoculated against it,
either by having had chickenpox during childhood, or being 1. Acyclovir is the treatment of choice.
inoculated with the vaccine. 2. Corticosteroids may be used with caution as clinical
course is more severe in immunocompromised
• The first symptom is usually one-sided pain, tingling, or
patients.
burning. 3. Antihistamines may be used to reduce itching.
• The pain and burning may be severe. 4. Skin should be kept clean, and contaminated items
• Red patches on the skin form, followed by small blisters should not be reused.
that look very similar to early chickenpox.
• The blisters break, forming small ulcers that begin to dry SMALLPOX
and form crusts. The crusts fall off in two to three weeks.
• Additional symptoms may include: Smallpox is also called as ‘variola’. Farr first accurately
— Abdominal pain predicted variola infection rates in the 1830s. Once the disease
— Chills and its method of spread were understood better, smallpox
— Difficulty moving some of the muscles of the face vaccination became mandatory in developed countries in the
— Ptosis early 1900s. On December 9, 1979, the WHO global
— Fever commission for certification of small pox declared that small-
— General ill-feeling pox eradication has been achieved throughout the world (Fig.
— Genital lesions 13.29).14 Hence, discussion of smallpox here is fortunately only
— Headache of historical interest.
— Hearing loss Variola is a member of the orthopoxvirus genus, of which
— Joint pain cowpox, monkeypox, orf and molluscum contagiosum also are
— Loss of eye motion (ophthalmoplegia) members. Poxviruses are the largest animal viruses, larger than
— Swollen glands (lymph nodes) some bacteria. They have a large genome, composed of 200
— Taste problems kilobase (kb) double-stranded DNA enclosed in a double
— Vision problems. membrane layer. Poxviruses are the only viruses that can
replicate in cell cytoplasm without the need of a nucleus
HISTOPATHOLOGIC FEATURES (Fig. 13.30).
The virus is acquired from inhalation, although virus
The histopathology of this lesion shows ballooning degeneration particles can remain viable on fomites (clothing, bedding,
of the epthelial cells (Fig. 13.28). surfaces) for approximately one week. The virus initially
Infectious Diseases in Children 355

FIGURE 13.29: Declaration of eradication of smallpox FIGURE 13.31: Multiple small, elevated,
yellowish green pustules

The minor criteria include (1) a centrifugal distribution of

lesions, with the first lesions on the oral mucosa or palate, face,
or forearms; (2) a toxic or moribund appearance; (3) the slow
evolution of lesions of 1 to 2 days per stage; and (4) lesions
that appear on the palms and soles.15

Management
1. Immediate contact and droplet isolation of the patient
is required.
2. The patient and any individual who came into contact
with the patient up to 17 days prior to the illness
(including the treating physician and nursing staff)
should remain in isolation until a definite diagnosis is
made. Presently, this requires sending a viral culture
to the CDC in Atlanta.
3. The most likely scenario of a variola outbreak is from
FIGURE 13.30: Variola viruses a terrorist attack. Given the highly infective nature of
the organism (not taking into account a genetically
replicates in respiratory tract epithelial cells. From there, a altered virus), researchers estimate that 1 infected
massive asymptomatic viremia ensues, resulting in focal patient can subsequently infect 20 new contacts
during the infectious stage of the illness.
infection of the skin, intestines, lungs, kidneys, and brain. The
4. The presentation of a clinically apparent case implies
multiplication in the skin epithelial cells first leads to a rash, that a larger population has probably been infected.
progressing into deep-seated pustules approximately 14 days 5. Because of the medicolegal and social implications
after inoculation (Fig. 13.31). A cell-mediated immune response of quarantine and isolation for a minimum of 17 days,
is responsible for pustule formation, as immunocompromised coordinated involvement on the federal, state and
rabbits do not produce these characteristic lesions. Patients who local levels is mandatory. In practicality, a strict
survive an initial infection often have severely deformed skin quarantine of a large segment of the population is
from the pustules and subsequent granulation tissue formation. probably not possible.
Currently, the clinical diagnosis of smallpox is based on 6. Once the disease is fully manifested, medical
treatment of variola is supportive care. Vaccinations
several criteria. The major criteria are: (1) a febrile prodrome
and postexposure interventions are the mainstays of
1 to 4 days before rash onset; (2) the classic smallpox lesions treatment. The various vaccines available and being
(i.e. deep-seated, firm, round, well-circumscribed lesions); and tested are divided into generations. First-generation
(3) lesions that are at the same stage of development.
356 Essentials of Pediatric Oral Pathology

vaccines are composed of vaccinia virus derived from Management

calf lymph or chicken embryos, have little attenuation 1. Since the complication rate is high, the best line of
and represent the majority of the vaccine stockpile. management for measles is vaccination — MMR
Second-generation vaccines are viruses taken from (mumps, measles, rubella).
first-generation vaccines that are then sterilely tissue- 2. Routine vaccination is recommended for all children
cultured with the aim of decreasing adverse between the ages of 12 and 15 months with a second
outcomes. Lastly, third-generation vaccines use dose administered between the ages of 4 and 6 years.
replication-deficient viruses that are highly attenuated, 3. In healthy patients, fluids and non-aspirin antipyretics
again with the aim of decreasing side effects and
are recommended for symptomatic relief.
adverse outcomes.
7. The vaccinia (smallpox) vaccine and vaccinia immune COMPLICATIONS
globulin (VIG) are available only through the CDC and
state and federal health agencies. Dryvax, and now Otitis, pneumonia, persistent bronchitis, diarrhea and subacute
ACAM200, are the only vaccines available, although sclerosing panencephalitis.
vaccines from other countries may be made available
in a smallpox outbreak. RUBELLA (GERMAN MEASLES)
Rubella is a mild viral infection caused by Toga virus. Its
MEASLES (RUBEOLA)
importance lies in its capacity to induce birth defects in the
Measles is an infection produced by a paramyxovirus. Until developing fetus. In the past, this infection occurred in cycles,
late 1980s, when a vaccine was produced against this infection, with localized epidemics every 6 to 9 years and pandemics
children used to suffer from measles very commonly. every 10 to 30 years. An effective vaccine, first released in
1969, is now used widely and has dramatically affected the
CLINICAL FEATURES epidemiology of the infection and broken the cycle of
• Most of the cases occur during spring and spread through occurrences.
respiratory droplets
ETIOLOGY
• Incubation period is from 10 to 12 days
• Prodromal symptoms of fever, malaise, coryza, • Rubella is caused by toga virus (RNA virus) (Fig. 13.32)
conjunctivitis and cough occur • Infection is transmitted by droplets as the virus is present
• Initial lesion occurs as a rash on the face, trunk and in throat infections.
extremities lasting for four to seven days. Rash is replaced • Congenital rubella results from transmission of virus from
by brown pigmentation infected mother to the fetus through transplacental route
• Oral manifestations occur in the form of Koplik’s spots
which occur as small, bluish to white macules on buccal
and labial mucosa and less often on soft palate
• These pathognomonic spots represent foci of epithelial
necrosis and have been described as “grains of salt” on a
red background
• Other manifestations include:
— Pitted enamel hypoplasia of developing permanent teeth
— Enlargement of accessory lymphoid tissues.

HISTOPATHOLOGIC FEATURES
• Initially Koplik’s spots represent areas of focal
hyperparakeratosis, epithelial spongiosis, intercellular
edema, dyskeratosis and epithelial syncytial giant cells
• As the spot ages, epithelium exhibits exocytosis by
neutrophils leading to microabscess formation, necrosis and
ulceration
• Within the hyperplastic lymphoid tissue, there are numerous
multinucleated giant lymphocytes termed as Warthin-
Finkeldey giant cells. FIGURE 13.32: Toga virus
Infectious Diseases in Children 357

FIGURE 13.33: Rash with pink macules FIGURE 13.34: Coxsackievirus

and fetus is/may be associated with congenital

• Pregnant women
abnormalities of eyes, heart and may cause retardation • Immunodeficient patients
• Acquired rubella infection affects older children and young • Patients with acute febrile illnesses
adults • Patients with a known allergy to components of
• The incubation period is 18 days. the vaccine.

CLINICAL FEATURES
HERPANGINA
• Malaise, headache, fever, mild conjunctivitis and
lymphadenopathy. Herpangina is an acute febrile illness associated with small
• The rash develops within 1 to 5 days of symptom onset, vesicular or ulcerative lesions on the posterior oropharyngeal
starting on the face and forehead and spreading caudally structures. Herpangina typically occurs during the summer and
to involve the trunk and extremities (Fig. 13.33). usually develops in children, occasionally occurring in young
• Lymphadenopathy may be present particularly in the posterior adults.
auricular, posterior cervical, and suboccipital chains.
• The rash consists of pink macules and papules, which may ETIOLOGY
become confluent, resulting in a scarlatiniform eruption.
• Petechiae of the soft palate, known as the Forchheimer • Various enteroviruses cause the condition.
sign, may be present. • Herpangina is usually caused by coxsackievirus A. Less-
common causes of herpangina include coxsackievirus B,
DIAGNOSIS
echovirus and enterovirus. Enteroviruses that cause
Diagnosis is based on virus isolation and serological tests. herpangina belong to the picornaviridae family.
• Infection occurs through injection, direct contact or through
Management
droplet spread and multiple cases in a single household are
1. No antiviral therapy for rubella is available. Treatment common.
is supportive. • Coxsackieviruses A 1-10, 16 and 22 represent the most
2. The goals of pharmacotherapy are to reduce morbidity
common pathogens that cause herpangina (Fig. 13.34).
and prevent complications.
3. Antipyretics may be used to decrease fever.
• Less-commonly, the viruses causing herpangina are
4. Antihistamines may be used to control itching. coxsackievirus B 1-5, echovirus 3, 6, 9, 11, 16, 17, 22, 25,
5. Prevention is carried out by Rubella vaccine that and 30 and enterovirus 71.
should be given to all children at the age of 15 months • Viruses that cause herpangina are typically spread via
(MMR vaccine). Second dose is given at the age of the fecal-oral route, although they may spread via the
11 to 13 years. The vaccine is contraindicated in the
respiratory route or through fomites. Freshwater sources
following groups:
(e.g. lakes) may act as a reservoir for transmission.
358 Essentials of Pediatric Oral Pathology

CYTOMEGALOVIRUS INFECTION
In 1904, Ribbert first identified histopathological evidence of
cytomegalovirus, probably in tissues from a congenitally
infected infant. Ribbert mistakenly assumed that the large
inclusion-bearing cells he observed at autopsy were from
protozoa (incorrectly named Entamoeba mortinatalium). In
1920, Goodpasture correctly postulated the viral etiology of
these inclusions. Goodpasture used the term cytomegalia to
refer to the enlarged, swollen nature of the infected cells.
Human cytomegalovirus (HCMV) was first isolated in tissue
culture in 1956 and the propensity of this organism to infect
the salivary gland led to its initial designation as a salivary gland
virus.16 In 1960, Weller designated the virus cytomegalovirus.17
During the 1970s and 1980s, knowledge of the role of
cytomegalovirus as an important pathogen with diverse clinical
FIGURE 13.35: Ulcers with gray base and inflamed manifestations increased steadily.18
periphery on the faucial pillars
ETIOLOGY
CLINICAL FEATURES
Cytomegalovirus is a member of a family of 8 human
• Herpangina is commonly seen in young children. herpesviruses, designated as human herpesvirus 5 (HHV-5).
• It commonly occurs in summer. Taxonomically, cytomegalovirus is referred to as a Betaherpes-
• It is comparatively mild and of short duration. virinae, based on its propensity to infect mononuclear cells and
• Herpangina typically has an incubation period of 7 to 14 lymphocytes and on its molecular phylogenetic relationship to
days. Viremia occurs after inoculation and subsequently other herpesviruses. Cytomegalovirus is the largest member of
results in distant sites of infection. the herpesvirus family, with a double-stranded DNA genome
• Clinical symptoms at secondary sites of infection occur after of more than 240 kbp, capable of encoding more than 200
viral replication. Bilateral, anterior, cervical potential protein products. The function of most of these
lymphadenopathy may occur, resulting from infection of the
proteins remains unclear. As with the other herpesviruses, the
posterior oropharynx. Coxsackievirus A may be recovered
structure of the viral particle is that of an icosahedral capsid,
from the nasopharynx, feces, blood, urine and cerebrospinal
surrounded by a lipid bilayer outer envelope.
fluid (CSF). After clinical symptoms have resolved,
asymptomatic enteroviral infection may persist in the
CLINICAL FEATURES
gastrointestinal tract.
• Begins with sore throat, cough, rhinorrhea, low grade fever, • Both sexes are equally susceptible to infection and
headache, vomiting and abdominal pain. morbidity from cytomegalovirus, although only women are
• Patients soon exhibit small vesicles which rupture to form at risk for transplacental transmission of infection.
crops of ulcers with a gray base and inflamed periphery on • Two age groups have higher rates of acquisition of infection:
the faucial pillars, posterior pharyngeal wall, buccal mucosa toddlers who attend group day care and adolescents.
and tongue (Fig. 13.35). • Congenital cytomegalovirus (CMV) infection: Current
• Vesicles preceding the ulcer are small and of short duration. estimates suggest that 30,000 to 40,000 infants are born
• Ulcers are not extremely painful. with congenital cytomegalovirus infection annually in the
• Erythema of the pharynx may range from mild to severe. United States, making cytomegalovirus by far the most
Pharyngitis in enteroviral infections may be associated with common and important of all congenital infections. The
pleurodynia, meningitis and/or exanthema. likelihood of congenital infection and the extent of disease
• Bilateral, anterior, cervical lymphadenopathy may develop. in the newborn depend on maternal immune status. If
primary maternal infection occurs during pregnancy, the
Management
average rate of transmission to the fetus is 40 percent;
Treatment is generally supportive and includes the approximately 65 percent of these infants have
following: cytomegalovirus disease at birth. With recurrent maternal
1. Hydration infection (i.e. cytomegalovirus infection that occurs in the
2. Antipyretics (e.g. acetaminophen, ibuprofen)
context of preconceptual immunity), the risk of transmission
3. Topical analgesics (e.g. topical lidocaine).
to the fetus is lower, ranging from 0.5 to 1.5 percent; most
Infectious Diseases in Children 359

of these infants appear normal at birth (i.e. silent infection). occurs postnatally. Primary infection in this context is
Hence, congenital infection may be classified as generally asymptomatic, although cytomegalovirus disease
symptomatic or asymptomatic in nature. may occur in certain risk groups as follows:
Symptomatic congenital cytomegalovirus/Cytomegalic Perinatal infection
inclusion disease (CID) • Perinatal acquisition of cytomegalovirus usually occurs
• Approximately 10 percent of infants with congenital secondary to exposure to infected secretions in the birth
infection have clinical evidence of disease at birth. The canal or via breastfeeding. Most infections are
most severe form of congenital CMV infection is referred asymptomatic. Indeed, in some reviews, cytomegalovirus
to as CID. acquired through breast milk has been referred to as a form
• CID almost always occurs in women who have primary of natural immunization.
cytomegalovirus infection during pregnancy, although rare • Some infants who acquire cytomegalovirus infection
cases are described in women with preexisting immunity perinatally may have signs and symptoms of disease,
who presumably have reactivation of infection during including lymphadenopathy, hepatitis and pneumonitis,
pregnancy.
which may be severe. Disease secondary to acquisition
• CID is characterized by intrauterine growth retardation,
by breast milk is generally limited to premature infants
hepatosplenomegaly, hematological abnormalities
with low birth weight. These infants may suffer
(particularly thrombocytopenia) and various cutaneous
considerable morbidity. Whether interventions, such as
manifestations, including petechiae and purpura (i.e.
freezing or pasteurization, are warranted to decrease
blueberry muffin baby). However, the most significant
the risk of transmission to these high-risk infants is
manifestations of CID involve the CNS. Microcephaly,
ventriculomegaly, cerebral atrophy, chorioretinitis and unclear. More studies of long-term neurodevelopmental
sensorineural hearing loss are the most common out com es of pr em a t ur e i n fa n t s wh o a cqui r e
neurological consequences of CID. cytomegalovirus infection perinatally from breast milk
• Intracerebral calcifications typically demonstrate a are needed.
periventricular distribution and are commonly encountered Cytomegalovirus mononucleosis
using CT scanning. The finding of intracranial calcifications • Typical cytomegalovirus mononucleosis is a disease found
is predictive of cognitive and audiologic deficits in later in young adults. Although cytomegalovirus mononucleosis
life and predicts a poor neurodevelopmental prognosis. may be acquired by blood transfusion or organ
• Most infants who survive symptomatic CID have significant transplantation, cytomegalovirus mononucleosis is usually
long-term neurological and neurodevelopmental sequelae. acquired via person-to-person transmission.
Indeed, it has been estimated that congenital • The hallmark symptoms of cytomegalovirus mononucleosis
cytomegalovirus may be second only to Down syndrome are fever and severe malaise. An atypical lymphocytosis
as an identifiable cause of mental retardation in children. and mild elevation of liver enzymes are present.
Asymptomatic congenital cytomegalovirus • Clinically differentiating cytomegalovirus mononucleosis
• Most infants with congenital cytomegalovirus infection are from Epstein-Barr virus (EBV)—induced mononucleosis
born to women who have preexisting immunity to may be difficult. Cytomegalovirus mononucleosis is
cytomegalovirus. These infants appear clinically healthy at typically associated with less pharyngitis and less
birth; however, although infants with congenital splenomegaly. As with EBV mononucleosis, the use of
cytomegalovirus infection appear well, they may have -lactam antibiotics in association with cytomegalovirus
subtle growth retardation compared to uninfected infants. mononucleosis may precipitate a generalized morbilliform
Although asymptomatic at birth, these infants, nevertheless, rash.
are at risk for neurodevelopmental sequelae. Transfusion-acquired cytomegalovirus infection
• The major consequence of inapparent congenital • Post-transfusion cytomegalovirus infection has a
cytomegalovirus infection is sensorineural hearing loss. presentation similar to that of cytomegalovirus
Approximately 15 percent of these infants will have mononucleosis. Incubation periods range from 20 to 60
unilateral or bilateral deafness. Routine newborn audiologic days.
screening may not detect cases of cytomegalovirus— • The use of seronegative blood donors, frozen
associated hearing loss because this deficit may develop deglycerolized blood, or leukocyte-depleted blood can
months or even years after birth. decrease the likelihood of transmission and is
• Acquired cytomegalovirus infection: In contrast to recommended for high-risk patients (e.g. neonates,
congenital infection, acquired cytomegalovirus infection immunocompromised patients).
360 Essentials of Pediatric Oral Pathology

Cytomegalovirus infections in immunocompromised Cytomegalovirus retinitis

patients • Before the advent of highly active antiretroviral therapy
Cytomegalovirus causes various clinical syndromes in (HAART) for HIV infection, cytomegalovirus retinitis
immunocompromised patients. Disease manifestations vary in was the most common cause of blindness in adult
severity depending on the degree of host immunosuppression. patients with acquired immunodeficiency syndrome
Infection may occur because of reactivation of latent viral (AIDS), with an overall lifetime prevalence of more than
infection or may be newly acquired via organ or bone marrow 90 percent.
transplant from a seropositive donor. Infections may also be • HIV-associated cytomegalovirus retinitis in children, in
mixed in nature, with donor and recipient isolates both present. contrast to adults, has been relatively rare, probably
Viral dissemination leads to multiple organ system involvement, reflecting overall differences in cytomegalovirus
with the most important clinical manifestations consisting of seroprevalence between the populations. Retinitis is less
pneumonitis, gastrointestinal disease and retinitis. common in transplant patients.
Cytomegalovirus pneumonitis • Cytomegalovirus produces a necrotic rapidly progressing
• Cytomegalovirus is a major cause of pneumonitis in retinitis with characteristic white perivascular infiltrate with
immunosuppressed children and adults. This disease may hemorrhage (brushfire retinitis).
be observed in the setting of HIV infection, congenital • Peripheral lesions may be asymptomatic and even advanced
immunodeficiency, malignancy and solid organ or bone disease does not cause pain. In children, strabismus or
marrow transplant. failure to fix and follow objects may be important clues to
• The mortality rate is based on the degree of the diagnosis.
immunosuppression, with mortality rates of at least 90 • The disease can progress to total blindness and retinal
percent reported in bone marrow transplant recipients. Solid detachment if left untreated. Cytomegalovirus
organ transplant recipients are at risk of developing chorioretinitis is also observed in symptomatic infants with
cytomegalovirus pneumonitis also, although mortality rates congenital infection, although the disease does not usually
are lower. progress to vision loss. The presence of chorioretinitis in
• The illness usually begins one to three months following an infant with congenital infection indicates a poor
transplantation and starts with symptoms of fever and dry, neurodevelopmental prognosis.
nonproductive cough. The illness progresses quickly with Other cytomegalovirus syndromes:
retractions, dyspnea, and hypoxia becoming prominent. • Various syndromes have been attributed to cytomegalovirus
• The illness is an interstitial pneumonitis, with a radiographic
infection, although cause and effect relationships are often
appearance of diffuse bilateral interstitial infiltrates.
difficult to establish.
Because the differential diagnosis of pneumonitis is
• Menetrier disease is a rare disorder characterized by
extensive in immunocompromised patients, consider
hyperplasia and hypertrophy of the gastric mucous glands,
performing a bronchoalveolar lavage or open lung biopsy
which results in massive enlargement of the gastric folds.
to confirm the diagnosis and direct appropriate therapy.
Most cases appear to be cytomegalovirus associated,
Cytomegalovirus gastrointestinal (GI) disease
although the pathogenesis is unknown.
• GI tract disease caused by cytomegalovirus can include
• In children with congenital HIV infection, co-infection
esophagitis, gastritis, gastroenteritis, pyloric obstruction,
with cytomegalovirus appears to accelerate the HIV
hepatitis, pancreatitis, colitis and cholecystitis.
disease progression and HIV-associated neurological
Characteristic signs and symptoms may include nausea,
disease. Accumul ating eviden ce suggests that
vomiting, dysphagia, epigastric pain, icterus and watery
diarrhea. cytomegalovirus infection may be a cofactor in the
• Stool may be hemoccult positive or frankly bloody. pathogenesis of atherosclerosis. In addition, the
Endoscopy and biopsy are warranted and characteristic phenomena of posttransplant vascular sclerosis and
cytomegalic inclusion cells may be observed in GI postangioplasty restenosis appear to be cytomegalovirus-
endothelium or epithelium. induced lesions.
• Although CMV enteritis does not carry the same ominous • The long-term health consequences of cytomegalovirus
prognosis as cytomegalovirus pneumonitis, antiviral therapy infection may include atherosclerosis, immunosenescence
is warranted. and an increased risk of malignancy. These associations
• Differentiating cytomegalovirus hepatitis from chronic require further study but provide a potential justification
rejection in liver transplant patients may be difficult, even for universal vaccination of both sexes against
with biopsy. cytomegalovirus.
Infectious Diseases in Children 361

term AIDS was not used to describe this new unexplained

immune deficiency syndrome. The syndrome had several
names, including “gay syndrome”, due to it being initially
identified in homosexuals.19
Researchers from the Viral Oncology Unit called on the
American team of Pr Gallo (National Cancer Institute, United
States) who had described the only human retrovirus known at
that time, HTLV 1. Pr Gallo informed them that he was also
looking for the virus causing what would be known as AIDS
and that he considered that it may be HTLV 1 (Human T-Cell
Leukemia Virus). However, initial comparisons suggested and
confirmed that this was false, particularly comparison by
immunoflorescence undertaken by Marie-Thérèse Nugeyre.19
In May 1986, the International Committee on the
FIGURE 13.36: Histopathologic picture of cytomegalovirus Taxonomy of Viruses ruled that a new name, HIV (Human
inclusion bodies showing ‘Owl’s eye’ appearance Immunodeficiency Virus) be given to the virus.19
Two-thirds of HIV/AIDS infections in Asia occur in India,
HISTOPATHOLOGIC FEATURES with an estimated 5.7 million infections (estimated 3.4–9.4
million) (0.9% of population), surpassing South Africa’s
Cells infected with CMV are enlarged and have inclusion estimated 5.5 million (4.9–6.1 million) (11.9% of population)
bodies which can be recognized from biopsy material. It is infections, making it the country with the highest number of HIV
known as having an “owl’s eye” appearance (Fig. 13.36). infections in the world. In 2005, five Indian states had high HIV/
AIDS prevalence—Andhra Pradesh, Karnataka, Maharashtra,
Management
Manipur and Nagaland as did 95 districts in the states.20
1. Medical care of cytomegalovirus (CMV) consists of
good nutritional support, vigorous supportive care for STRUCTURE OF HIV 1 (FIGS 13.37 AND 13.38)
end-organ syndromes (particularly pneumonia in
immunocompromised patients) and specific antiviral The HIV-1 virus is an icosahedral, enveloped, RNA virus of
therapy in select circumstances. the Lentivirinae subfamily of retroviruses that primarily infects
2. Some children with congenital cytomegalovirus human white blood cells. They are so called because their
require orthopedic interventions (cerebral palsy) and genetic material RNA must be transcribed to DNA before the
gastrostomy placement for enteral nutrition. virus can complete its replicative cycle. The genome of HIV
3. Nucleosides are the only true antiviral agents active contains two types of genes: the structural genes and the
against cytomegalovirus, although immunoglobulins
regulatory genes. The structural genes are responsible for
may provide some antiviral effect, particularly in
combination with these agents. These agents share
direction and synthesis of proteins and glycoproteins that will
a common molecular target, namely, the viral DNA give the virus its physical characteristics, i.e. shape, size,
polymerase. Biochemically, ganciclovir is an acyclic structural integrity, compartmentalization, etc. The regulatory
nucleoside analog, whereas cidofovir is an acyclic genes are responsible for subsequent production of proteins
nucleoside phosphonate. that can affect the activities of viral components or can
4. Ganciclovir is commonly used as preemptive therapy specifically turn other genes on and off. Among other activities,
in transplant recipients at high risk of developing the regulatory proteins have ability to increase or decrease the
disease (e.g. a cytomegalovirus-seronegative replication of HIV. The activity of this regulatory gene is
recipient of an organ transplant from a cytomegalo-
responsible for profound pathogenicity of HIV.21
virus-seropositive donor).
5. Alternatives to ganciclovir include trisodium
Structurally, HIV is composed of two major parts: an outer
phosphonoformate (PFA) and cidofovir. Pediatric envelope and inner core. The external envelope components are
experience with these agents is limited. embedded in the lipid matrix of the membrane and are involved
in the binding of the virus to the host cells during infections.
ACQUIRED IMMUNODEFICIENCY The outermost viral components are arranged as spikes or knobs
SYNDROME and extend from the lipid membrane. These external spikes are
proteins with sugar molecules and are therefore glycoproteins.
The first cases of acquired immunodeficiency syndrome (AIDS) The core components of the virus are located internal to the outer
were described in the United States in 1981. At that point, the membrane and are bound by a protein coat encompassing two
362 Essentials of Pediatric Oral Pathology

3. pol proteins (polymerase components that represents

enzymes that are involved in transcription, integration and
cleavage of other viral components).21
The most important gag proteins (p) are those having
molecular weights of 55,000 (p55), 24,000 (p24), 17,000 or
18,000 (p17) and 15,000 Da (p15). The p55 antigen is the
precursor molecule produced early in the infectious process
and is eventually cleaved to produce other core proteins. All
of the gag proteins are located in the nucleocapsid of the virus.
The p17 protein lies in the matrix between the protein core
and the envelope and is embedded within the internal portion
of the envelope. The p24 and p15 proteins make up the core
coat (capsid) that surrounds the internal nucleic acids. The
major capsid component is p24.
FIGURE 13.37: Structure of HIV In addition to p24, two additional core proteins, p9 and p7
which are nucleic acid binding proteins are also present.
Together these proteins make-up the nucleoid core.21
The env (envelope) antigens are glycoproteins and include
those components having molecular weights of 160,000
(gp 160), 120,000 (gp 120) and 41,000 Da (gp 41). The gp
160 is the precursor molecule and is not a structural component
(similar to the gag p55); it is a component that is produced
during infection, but is later cleaved to form gp120 and gp 41
(the structural envelope glycoproteins). These envelope
glycoproteins contain:
1. Conserved regions (sequences constant in all HIV-1
viruses).
2. Variable regions (sequences that may vary between
HIV-1 viruses).21
The gp41 antigen spans the inner and outer membrane of
the virus and thus is often referred to as a transmembrane
glycoprotein antigen. This particular antigen contains important
variable regions and therefore may be specific for each type
or strain of HIV-1 virus. The gp120 antigen is the major
component of external envelope and is responsible for 72 knobs
or spikes of the envelope. Significant variability in gp120 (as
high as 15%) between the HIVs occur in the hypervariable
FIGURE 13.38: Genomic composition of HIV regions of the molecule; this may be responsible for the inability
of the immune system to contain the virus. Together the gp41
identical copies of the nucleic acid RNA genome. Also within
and gp120 antigens are involved with the fusion and attachment
the core are the three viral enzymes: reverse transcriptase,
of HIV to the CD4 molecule on the host cells.21
integrase and protease. These enzymes are responsible for
Polymerase (pol) antigens include the proteins p66 (a
transcription, integration of the virus in the host and cleavage
subunit of reverse transcriptase enzyme that has RNase H
of other proteins respectively.21
activity), p51 (another subunit of reverse transcriptase enzyme),
The different structural antigens of HIV-1 are encoded by
and p31 (integrase or endonuclease). Polymerase antigens are
three major structural genes and therefore can be classified into
three major groups: located within the core of the virus and are closely associated
1. gag proteins (encoded by gag gene, or group associated with the nucleic acids. Collectively, these polymerase
antigen gene). components are responsible for:
2. env glycoproteins (encoded by the genes that determine the • Conversion of viral RNA to DNA (reverse transcription)
production of envelope components). • Integration of viral DNA into host cellular DNA
Infectious Diseases in Children 363

• Cleavage of precursor molecules into smaller active AIDS is divided into three phases:
components; accomplished by the proteases.21 • Early acute phase: Level of plasma viremia, CD8 count,
The precursor molecules gp160 and p55 are not structural CD4 count, sore throat, fever, etc.
components of the virus and are only included in the figure • Middle chronic phase: Seen after seroconversion stage,
for completeness. These precursor components are however viral replication in lymphoid tissue in early stage but in
true gene products produced during transcription and late stage, patient asymptomatic.
translation. It is later but prior to virus assembly that they are • Final crisis phase: CD4 count < 200/ l, fever > 1 month,
cleaved to form the other structural components.21 weight loss, chronic diarrhea > 1 month, opportunistic
Other components of virus may be antigenic and play a infections, aseptic meningitis, viral encephalitis, etc.
significant role in infection and the development of immune
ORAL MANIFESTATIONS OF AIDS
responses, but their exact roles are poorly understood. These
viral components includes the negative regulatory proteins nef Usually precede the general or systemic manifestations thus
(p27) and vpr (p15), both of which may limit viral replication they are of utmost importance for a dental professional.
and the positive regulatory proteins vpu (p16, increases the
maturation of the viral particles), tat (p14, transactivates gene CLASSIFICATION
expression), rev (p19, production of viral mRNA) and vif (p23,
Most widely accepted European Commission Clearinghouse
viral infectivity factor). Generally, these regulatory components
(EC Clearinghouse) classification based on:23
are responsible for modifying the expression of viral proteins
and for the replication of the virus. The negative regulatory • Clinical observation of the lesions and
factor may be responsible for limiting the degree of viral • Are a result of special investigations for absolute diagnosis.
replication (down regulation), thereby leading to the latency Divided into three broad groups:
period.21 Although the exact function of these regulatory factors (In Adults)
is unknown, they play an important role in the infectious • Group 1: Lesions strongly associated with the HIV
process and may govern the whole process of disease infection
progression.21 • Group 2: Lesions less commonly associated with the HIV
infection
REPLICATION CYCLE OF VIRUS
• Group 3: Lesions seen in the HIV infection.
Refer to Figure 13.39.
(In Children)
• Group 1: Lesions commonly associated with the HIV
CLINICAL FEATURES OF AIDS
infection in children
• Disease is divided in five clinical stages: • Group 2: Lesions less commonly associated with the HIV
1. Acute HIV infection infection in children
2. Asymptomatic/latent infection • Group 3: Lesions strongly associated with HIV infection
3. Persistent generalized lymphadenopathy but rare in children.
4. AIDS related complex
Group 1 (Adults): Lesions strongly associated with the HIV
5. Full blown AIDS.
infection in adults:
World Health Organization (WHO) classification • Candidiasis: Erythematous, pseudomembranous, angular
• Presence of 2 major signs and 1 minor sign: cheilitis
— Major signs: weight loss > 10 percent of body weight, • Hairy leukoplakia
chronic diarrhea > 1 month, prolonged fever > 1 month • Kaposis sarcoma
— Minor signs: persistent cough > 1 month, oral candidiasis, • Non-Hodgkin’s lymphoma
herpes zoster infection, persistent generalized • Periodontal disease: Linear gingival erythema, necrotizing
lymphadenopathy, other opportunistic infections. gingivitis, necrotizing periodontitis.
Center for Disease Control (CDC) Atlanta Classification22 Group 2 (Adults): Lesions less commonly associated with the
Classification is based on: HIV infection in adults:
• Etiologic agent • Tuberculosis
• Natural history • Melanotic hyperpigmentation
• Clinical manifestation and • Necrotizing stomatitis
• CD4 T-cell counts. • Salivary gland disease
364 Essentials of Pediatric Oral Pathology

FIGURE 13.39: Replication cycle of virus

• Thrombocytopenic purpura Group 2 (Children): Lesions less commonly associated with

• Ulceration not otherwise specified the HIV infection in children:
• Herpes simplex • Bacterial infections: A. israelii, E. coli, Klebsiella
• Human papilloma virus pneumoniae
• Varicella zoster virus • Periodontal disease: Necrotizing gingivitis, necrotizing
periodontitis
Group 3 (Adults): Lesions seen in the HIV infection in adults:
• Xerostomia
• Bacterial infections: A. Israelii, E. coli, Klebsiella
• Seborrheic dermatitis
pneumoniae
• Viral infections: Cytomegalovirus, molluscum contagio-
• Drug reactions: Ulcerative erythema multiforme, lichenoid
sum, human papillomavirus, varicella-zoster virus.
reaction
• Fungal infections: Cryptococcus neoformans, Histoplasma Group 3 (Children): Lesions strongly associated with HIV
capsulatum infection but rare in children:
• Neurologic disturbances: Facial palsy, trigeminal neuralgia • Hairy leukoplakia
• Recurrent aphthous stomatitis • Kaposis sarcoma
• Viral infections: Cytomegalovirus, molluscum contagiosum. • Non-Hodgkin’s lymphoma
• Tuberculosis related ulcers.
Group 1 (Children): Lesions commonly associated with the
HIV infection in children:
• Candidiasis: Erythematous, pseudomembranous, angular GROUP I LESIONS
cheilitis 1. Candidiasis (Fig. 13.40): Oral candidiasis is most
• Linear gingival erythema commonly associated with Candida albicans, although
• Herpes simplex other species, such as C. glabrata and C. tropicalis, are
• Salivary gland disease frequently part of the normal oral flora. A number of factors
• Recurrent aphthous stomatitis. predispose patients to develop candidiasis: infancy, old age,
Infectious Diseases in Children 365

FIGURE 13.40: Oral thrush FIGURE 13.41: Angular cheilitis

antibiotic therapy, steroid and other immunosuppressive slide and protected by a cover slip. The smear is examined
drugs, xerostomia, anemia, endocrine disorders and primary under the microscope and Candida is detected by finding
and acquired immunodeficiency. Candidiasis is a common hyphae and blastospores. Hyphae and spores are only seen
finding in people with HIV infection. Reports describe oral in smears from lesions and are rarely seen in the healthy
candidiasis during the acute stage of HIV infection, but it individual in the carrier state.
occurs most commonly with falling CD4+ T-cell count in • Cultures are grown on specific media, such as Sabouraud’s
middle and late stages of HIV disease.24 agar; they may be positive and yet reveal very low colony
The clinical appearances of oral candidiasis vary. The counts. This probably represents a carrier state rather than
most common presentations include pseudomembranous active infection. Culture is useful for establishing the
and erythematous candidiasis, which are equally predictive Candida species but may not be useful for diagnosis.
of the development of AIDS,25 and angular cheilitis. These
lesions may be associated with a variety of symptoms, Management
including a burning mouth, problems eating spicy food and 1. Oral candidiasis may be treated either topically or
changes in taste. All three of these common forms may systemically. Treatment should be maintained for 7
appear in one individual. days. Response to treatment is often good; oral
2. Erythematous Candidiasis: Erythematous candidiasis lesions and symptoms may disappear in a fairly short
appears as flat, red patches of varying size. It commonly period (ranging from 2 to 5 days), but relapses are
occurs on the palate and the dorsal surface of the tongue. common because of the underlying immuno-
Erythematous candidiasis is frequently subtle in appearance deficiency. As with other causes of oral candidiasis,
and clinicians may easily overlook lesions, which may recurrences are common if the underlying problem
persists.
persist for several weeks if untreated.
2. Topical Treatment: Topical treatments are preferred
3. Angular Cheilitis (Fig. 13.41): Angular cheilitis appears because they limit systemic absorption, but the
clinically as redness, ulceration and fissuring, either effectiveness depends entirely on patient compliance.
unilaterally or bilaterally at the corners of the mouth. It can Topical medications require that the patient hold
appear alone or in conjunction with another form of medications in the mouth for 20 to 30 minutes. If the
candidiasis. patient uses formulations containing sweetening
agents for long periods, consider as concurrent
Diagnosis treatment daily fluoride rinses (e.g. ACT or Fluorigard,
Candida is a commensal organism in the oral cavity. available as over-the-counter preparations) for one
• Candidiasis is diagnosed by its clinical appearance and by minute once a day to be then expectorated.
detection of organisms on smears. Smears taken from 3. Clotrimazole is an effective topical treatment (one oral
troche [10-mg tablet]) when dissolved in the mouth
clinical lesions are examined using potassium hydroxide
five times daily. Used less frequently, one vaginal
(KOH), PAS, or Gram’s stain. Smears are taken by gently troche can be dissolved in the mouth daily. Nystatin
drawing a wooden tongue depressor across the lesion. The preparations include a suspension, a vaginal tablet
specimen is then transferred into a drop of KOH on a glass
366 Essentials of Pediatric Oral Pathology

and an oral pastille. Regimens are nystatin vaginal

tablets (one tablet, 100,000 units, dissolved in the
mouth three times a day), or nystatin oral pastille
(available as a 200,000-unit oral pastille, one or two
pastilles dissolved slowly in the mouth five times a
day). Nystatin suspension has a high sugar content
and cannot be held in the mouth long enough to be
effective. Topical creams and ointments containing
nystatin, ketoconazole, or clotrimazole may be useful
in treating angular cheilitis. Another therapeutic choice
is amphotericin B (0.1 mg/ml). Five to 10 ml of oral
solution is used as a rinse and then expectorated
three to four times daily.
4. Systemic Treatment: Several agents are effective for
systemic treatment. Ketoconazole (Nizoral) is a 200-
FIGURE 13.42: Resolving herpetic lesions
mg tablet taken with food once daily. Patient
compliance is usually good. Careful monitoring of and form crusts. Recurrent intraoral herpes appears as clusters
liver function is necessary for long-term use because
of painful small vesicles that rupture and ulcerate and usually
of reported side effects, including hepatotoxicity.
heal within 1 week to 10 days. The lesions usually occur on
Lack of efficacy of ketoconazole may occur because
of poor absorption in those with an abnormally high the keratinized mucosa, such as the hard palate and gingiva,
gastric pH. although lesions may arise on the dorsal surface of the tongue.
5. Fluconazole (Diflucan) is a triazole antifungal agent
effective in treating candidiasis (100-mg tablet taken DIFFERENTIAL DIAGNOSIS
once daily for 2 weeks). Several studies suggest Rising antibody titers from initial and convalescent sera confirm
fluconazole is effective as a prophylactic agent, primary herpetic gingivostomatitis. Examining smears of
although the most effective prophylaxis dosing regimen
lesions (treated with Papanicolaou stain) for multinucleated
is still unclear.26 Numerous reports however, describe
giant cells confirms recurrent herpes. It is possible to
oral and esophageal candidiasis failing to respond to
treatment with fluconazole and in some of these cases demonstrate herpes simplex type 1 or type 2 by applying
investigators isolated resistant strains.27 Itraconazole monoclonal antibodies to smears from the lesions (the Syva
(100-mg capsules) may be used for the treatment of Kit, Syva Corporation, Palo Alto, CA).28 Swabs taken from
oral candidiasis (200 mg daily orally for 14 days). fluid-filled vesicles may grow herpes simplex in culture if
Itraconazole oral suspension is now available (200 mg vesicles are a few days or less old. Clinicians can distinguish
daily for 2 weeks). Salivary levels of itraconazole are between recurrent intraoral herpes simplex lesions, which
maintained for several hours after administration. always occur on keratinized mucosa (such as the hard palate
6. Ketoconazole, fluconazole, and itraconazole may and gingiva) and recurrent aphthous ulcers, which always
interact with other medications including rifampicin, appear on nonkeratinized mucosa. Recurrent intraoral herpes
phenytoin, cyclosporin A, terfenadine, digoxin,
may appear more frequently in HIV-infected patients. The
coumarin-like medications and oral hypoglycemic
lesions may be painful and slow to heal.
medications.

Herpes Simplex (Fig. 13.42) Management

Herpes simplex causes both primary and secondary or recurrent 1. No treatment will permanently eradicate oral herpes
disease in the oral cavity. Primary herpetic gingivostomatitis simplex infections, but acyclovir may shorten the
commonly occurs in children and young adults and may be healing time for individual episodes. The optimum oral
followed by frequent recurrences. Following the primary episode, dosage of acyclovir is 1000 to 1600 mg daily for 7 to
10 days.
the virus becomes latent in the trigeminal ganglion. Recurrent
2. Topical acyclovir is not useful for treating intraoral
oral herpes occurs at any age extraorally or intraorally. lesions and may not be effective for lesions on the
lips. Recurrent outbreaks of acyclovir-resistant herpes
CLINICAL FEATURES have been reported, including a case involving the
Recurrent herpes labialis occurs on the vermilion border of the facial skin, lips, nose, and mouth. In this case, the
lesions resolved after treatment with foscarnet.
lips. The patient may report a history of itching or pain,
Phosphonoformate may also prove effective.
followed by the appearance of small vesicles. These rupture
Infectious Diseases in Children 367

Linear Erythematous Gingivitis soft palate and oropharynx. The major RAU type appears as
extremely large (2-4 cm) necrotic ulcers. The major RAUs are
This entity appears as a 1 to 3 mm band of marginal gingival
very painful and may persist for several weeks.
erythema, often with petechiae. It is typically associated with
no symptoms or only mild gingival bleeding and mild pain.
DIAGNOSIS
Histological examination fails to reveal any significant
inflammatory response, suggesting that the lesions represent The ulcers may present a diagnostic problem. Herpetiform
an incomplete (aborted) inflammatory response, principally RAUs may resemble the lesions of coxsackievirus infection,
with only hyperemia present. There is no evidence to suggest and major RAUs may require biopsy to exclude malignancy,
that this entity will proceed to the far more destructive such as lymphoma, or opportunistic infection, such as
necrotizing periodontitis. Unlike conventional gingivitis, the histoplasmosis. The ulcers usually occur on nonkeratinized
erythema often persists following simple dental prophylaxis. mucosa; this characteristic differentiates them from those
Oral rinsing with chlorhexidine gluconate 0.12 percent often caused by herpes simplex.
reduces or eliminates the erythema and typically requires
prophylactic use to avoid recurrence. Management
Clinicians should refer patients to a periodontist or dentist The RAU type ulcers usually respond well to topical
for management. The following protocol has achieved steroids such as fluocinonide (0.05%) ointment mixed with
reasonable success: plaque removal, local debridement, equal parts Orabase applied six times daily or clobetasol
irrigation with povidone-iodine, scaling and root planing and (0.05%) ointment mixed with equal parts Orabase applied
maintenance with a chlorhexidine mouth rinse (Peridex-R) once three times per day. Dexamethasone elixir (0.5 mg/5 ml)
or twice daily. Studies show that the addition of chlorhexidine used as a mouth rinse and then expectorated two to three
to this regimen produces significant improvement in times daily is helpful for multiple ulcers and for those
periodontal condition. where topical ointments are hard to apply. 29 For HIV-
infected persons with oral and gastrointestinal aphthous-
Parotid Gland Disease like ulcers, systemic steroid therapy (prednisone 40 to
60 mg/day for 7 to 10 days) has been reported as helpful.
HIV infection is associated with parotid gland disease, The risks of steroid therapy, however, must be considered
characterized clinically by gland enlargement and diminished before administration to individuals in this population.
flow and histologically by lymphoepithelial infiltration and Thalidomide (50 to 200 mg) has been used in Europe
benign cyst formation. The enlargement typically involves the and is the subject of clinical trials in the United States.
tail of the parotid gland or, less commonly, the submandibular
gland and it may present uni- or bi-laterally with periods of GROUP II LESIONS
increased or decreased size. While the appearance may raise Although isolated cases of oral infection with Klebsiella
suspicion of malignancy (salivary gland or lymphoma) or pneumoniae, Enterobacter cloacae, Actinomyces israelii,
infection, aspiration of a yellow mucinous secretion supports Escherichia coli and Mycobacterium avium intracellulare have
the diagnosis of HIV-related salivary gland disease, thus been reported in patients with HIV infection, the most common
avoiding unnecessary biopsy or imaging diagnostics. oral lesions associated with bacterial infection are necrotizing
Occasional swelling can be managed simply by repeated ulcerative gingivitis and periodontitis and much less commonly,
aspiration and rarely is radical removal of the gland necessary. bacillary epithelioid angiomatosis and syphilis. In the case of
The pathophysiological mechanism is not known, though the periodontal infections, the bacterial flora is no different from
cytomegalovirus has been suggested to play a role. that of a healthy individual with periodontal disease. Thus, the
clinical lesion is a manifestation of the altered immune response
Oral Ulceration to the pathogens.
Oral ulcers resembling recurrent aphthous ulcers (RAUs) in
HIV-infected persons are reported with increasing frequency. Necrotizing Ulcerative Periodontitis (NUP)
The cause of these ulcers is unknown. Proposed causes include This unique periodontal lesion is characterized by generalized
stress and unidentified infectious agents. In HIV-infected deep osseous pain, significant erythema that is often associated
patients, the ulcers are well circumscribed with erythematous with spontaneous bleeding and rapidly progressive destruction
margins. The ulcers of the minor RAU type may appear as of the periodontal attachment and bone. The destruction is not
solitary lesions of about 0.5 to 1.0 cm. The herpetiform type self-limiting and can result in loss of the entire alveolar process
appears as clusters of small ulcers (1-2 mm), usually on the in the involved area. This very painful associated lesion
368 Essentials of Pediatric Oral Pathology

adversely affects oral intake of food, resulting in significant ulceration, but occurs in areas overlying bone and is associated
and rapid weight loss. Because the periodontal microflora is with severe immune deterioration. Unlike necrotizing ulcerative
no different from that seen in healthy patients, the lesion periodontitis, the lesion may occur in edentulous areas. As in
probably results from the altered immune response in HIV major aphthous ulceration, systemic corticosteroid medication
infection. More than 95 percent of patients with NUP have a or topical steroid rinse is the treatment of choice.
CD4 lymphocyte count of less than 200/mm3.
Xerostomia
Management Xerostomia is common in HIV disease, most often as a side
effect of antiviral medications or of the other antihypertensive,
1. Treatment consists of rinsing twice daily with
antidepressant, anxiolytic or analgesic medications commonly
chlorhexidine gluconate 0.12 percent, metronidazole
(250 mg orally four times daily for 10 days) and prescribed for patients with HIV infection. The oral dryness
periodontal debridement, which is performed after presents a significant risk factor for caries and can lead to rapid
antibiotic therapy has been initiated. dental deterioration. Xerostomia also contributes to oral
2. Within 36 to 48 hours of antibiotic therapy, relief of candidiasis, mucosal injury and dysphagia and is often
pain associated with NUP is obtained. associated with pain and reduced oral intake of food. Although
several saliva substitutes exist, compliance is often poor and
Bacillary Epithelioid Angiomatosis (BEA) relief inadequate. For patients with residual salivary gland
function, determined by gustatory challenge, oral pilocarpine
This recently described lesion appears to be unique to HIV
(5 mg up to 3 times daily) often provides improved salivary
infection and is often clinically indistinguishable from oral
flow and consistency. Oral hygiene instruction, regular
Kaposi’s sarcoma (KS). Since both may present as an erythe-
maintenance and the use of prescription-strength, fluoridated
matous, soft mass which may bleed upon gentle manipulation,
dentifrice (Prevident 5000 Plus®) is essential.
biopsy and histological examination are required to distinguish
BEA from KS. The presumed etiological pathogen, Rochalimaea
Herpes Zoster
henselae, can be identified using Warthin-Starry staining. Both
KS and BEA are histologically characterized by atypical vascular The reactivation of varicella-zoster virus (VZV) causes herpes
channels, extravasated red blood cells and inflammatory cells. zoster (shingles). The disease occurs in the elderly and the
However, prominent spindle cells and mitotic figures occur only immunosuppressed.
in KS. Erythromycin (erythromycin estolate, 500 mg 4 times
Clinical features: Oral herpes zoster generally causes skin
daily for at least 10 days) is the treatment of choice for BEA.
lesions. Following a prodrome of pain, multiple vesicles appear
on the facial skin, lips and oral mucosa. Skin and oral lesions
Syphilis
are frequently unilateral and follow the distribution of the
While the prevalence of syphilis infection has risen significantly maxillary and/or mandibular branches of the trigeminal nerve.
over the past decade, it is an uncommon cause of intraoral The skin lesions form crusts and the oral lesions coalesce to
ulceration, even in HIV infection. Its appearance is no different form large ulcers. The ulcers frequently affect the gingiva, so
from that observed in healthy individuals; it is a chronic, tooth pain may be an early complaint.
nonhealing, deep, solitary ulceration; often clinically indisting-
uishable from that due to tuberculosis, deep fungal infection, or Management
malignancy. Dark field examination may demonstrate treponema. Acyclovir limits the duration of the lesions. For herpes
Positive reactive plasma reagin (RPR) and histological zoster, the standard oral dosage is 800 mg five daily for
demonstration of Treponema pallidum is diagnostic. Patients with 7 to 10 days, which is considerably higher than that
newly diagnosed syphilis should be referred to their physicians recommended for treatment of herpes simplex.
for evaluation and treatment; combination treatment with
penicillin, erythromycin and tetracycline is the treatment of Molluscum Contagiosum (Fig. 13.43)
choice, the dosage and duration of treatment depending on
presence or absence of neurosyphilis. Molluscum contagiosum (MC) is a common, self-limiting viral
disease of the skin and mucous membranes. It was first
Necrotizing Stomatitis described by Bateman in 1817. It is caused by molluscipox-
virus, which belongs to unclassified genus of poxvirus species.
Necrotizing stomatitis is an uncommon acute, painful ulceration The mature virion is a brick-shaped particle measuring 150
which often exposes underlying bone and leads to considerable × 350 nm. Molluscum contagiosum has a usual incubation
tissue destruction. This lesion may be a variant of major aphthous period of 14 to 50 days, although there are reports of newborns
Infectious Diseases in Children 369

FIGURE 13.43: Dome shaped papule with FIGURE 13.44: Histopathologic picture of molluscum contagiosum
central umbilication showing enlarged altered keratinocytes with eosinophilic "Henderson
and Paterson' inclusion bodies

having lesions as early as seven days postpartum. The lesions The centers of these bulbous structures are filled with enlarged,
may persist for weeks to months suggesting that the virus altered keratinocytes with eosinophilic viral inclusions referred
provokes a minimal cell-mediated immunity. to as Henderson and Paterson inclusion bodies. The inclusion
It occurs predominantly in preadolescent children, sexually bodies are the result of a virally induced transformation process.
active adults, participants in sports with skin to skin contact Initially, the small virion particle is formed in the cytoplasm
and in individuals with impaired cellular immunity. of the epithelial cells above the basal layer. These eosinophilic
Although Molluscum contagiosum as a clinical entity is particles grow in size as they progress towards the granular
well-defined and commonly observed, few data regarding its cell layer causing compression of the nucleus to the periphery
epidemiology in the pediatric population exist. of the infected epithelial cells (Fig. 13.44).

CLINICAL FEATURES Management

1. Molluscum contagiosum is a self-limiting disease,
According to one survey, incidence of molluscum contagiosum
which, if left untreated, will eventually resolve in
in India in children less than 14 years of age was found to be
immunocompetent hosts but may be protracted in
2.5 percent. Immunocompromised individuals act as the ideal atopic and immunocompromised individuals. One of
hosts for this lesion. Transmission factors in children are the most common, quick, efficient methods of
accounted by a warm and humid environment, overcrowding, treatment is cryotherapy.
poor hygiene, sharing towels, clothes, etc. These are the 2. An easy method to remove the lesions is eviscerating
conditions that are usually seen in orphanages and other the core with an instrument such as a scalpel, sharp
institutions where children reside together. In adults, tooth pick, edge of a glass slide or any other
Molluscum contagiosum is usually sexually transmitted. instrument capable of removing the umbilicated core.
Signs of sexual abuse should always be looked for when Because of its simplicity, patients, parents and
lesions on genitalia are present in children. caregivers may be taught this method so that new
lesions can be treated at home.
The lesions of molluscum contagiosum appear dome-
3. Curettage, use of adhesive tape, 0.05 ml of 5 percent
shaped, flesh-colored papules, usually multiple and with central
podofilox in lactate-buffered ethanol, Cantharidin
umbilicated area. The umbilicated area consists of white, waxy (0.9% solution of collodion and acetone), Tretinoin 0.1
curd-like core. percent cream, oral cimetidine, 10 percent potassium
hydroxide, Imiquimod 5 percent cream, Cidofovir
HISTOPATHOLOGIC FEATURES either topically or intralesional injection have also
been used for the management of molluscum
The lesional area shows inverted lobules of hyperplastic,
contagiosum with varying success.
acanthotic squamous epithelium arranged in lobulated pattern.
370 Essentials of Pediatric Oral Pathology

Human Papillomavirus Lesions Hairy leukoplakia and progression of HIV disease:

Diagnosis of HL is an indication of both HIV infection and
Oral warts, papillomas, skin warts and genital warts are
immunodeficiency; it is an indication for a work-up to evaluate
associated with the human papillomavirus (HPV). Lesions
and treat HIV disease. HL correlates with a statistical risk for
caused by HPV are common on the skin and mucous membranes
more rapid progression of HIV disease. Those persons with
of persons with HIV disease. Anal warts have frequently been
hairy leukoplakia who progressed to CDC-defined AIDS most
reported among homosexual men. Because the HPV types found
rapidly, however, were more often anergic to Candida antigen
in oral lesions in HIV-infected persons are different from the
HPV types associated with anogenital warts, clinicians should at diagnosis of hairy leukoplakia, indicating significant
probably not use the term condyloma acuminata to describe immunosuppression at that time. Progression to CDC-defined
oral HPV lesions. AIDS was more rapid in those HIV-infected persons with hairy
leukoplakia than in those without hairy leukoplakia, even after
CLINICAL FEATURES adjustment for CD4+ T-cell count.

HPV lesions in the oral cavity may appear as solitary or PATHOGENESIS

multiple nodules. They may be sessile or pedunculated and
appear as multiple, smooth-surfaced raised masses resembling The Epstein-Barr virus (EBV) in hairy leukoplakia is both
focal epithelial hyperplasia or as multiple, small papilliferous unusual in that deletions in the EBNA 2 gene have been
or cauliflower-like projections. I have identified HPV types 7, described and in that to date no viral DNA is found in the basal
13 and 32 have been identified in some of these oral warts. layers of HIV. Intraepithelial Langerhans’ cells (LCs) are
Malignant transformation of HPV oral lesions has not been reduced or absent in the hairy leukoplakia lesion, which
reported, but the identification of four new HPV types in these correlates with the presence of viral antigens. It is not known
lesions warrants further study. whether the lack of LCs is a cause of hairy leukoplakia or a
consequence of EBV infection.
Cytomegalovirus In electron microscopic specimens, investigators have
found structures consistent with a herpes group virus. One
Oral ulcers caused by cytomegalovirus (CMV) have been structure consisted of 100-nm intranuclear virions and 240-nm
reported. These ulcers can appear on any mucosal surface and encapsulated virus particles. Other structures are 48- to 52-nm
may be confused with aphthous ulcers, necrotizing ulcerative particles visible in the suprabasal layer. Closer to specimen
periodontitis (NUP) and lymphoma. Unlike aphthous ulcers, surfaces, where the nuclei are more condensed, arrays of these
however, which usually have an erythematous margin, CMV particles and herpes group particles occurred in the same cell.
ulcers appear necrotic with a white halo. Diagnosis of CMV Several studies described the appearance of these particles in
ulcers is made from a biopsy. Immunohistochemistry may be HL biopsies.
helpful.
CMV ulcers in the oral cavity usually occur in individuals CLINICAL FEATURES
with disseminated CMV disease. Therefore, diagnosis of CMV-
infected oral ulcers should be followed by examination for the Hairy leukoplakia lesions vary in size and appearance and may
systemic disease. CMV ulcers resolve when ganciclovir is used be unilateral or bilateral. The surface is irregular and may have
to treat CMV disease. prominent folds or projections, sometimes markedly resembling
hairs. Occasionally, however, some areas may be smooth and
GROUP III LESIONS flat. Lesions occur most commonly on the lateral margins of
the tongue and may spread to cover the entire dorsal surface.
Hairy Leukoplakia and Epstein-Barr Virus: Oral hairy They may also spread downwards onto the ventral surface of
leukoplakia (HL), which presents as a nonmovable, corrugated the tongue, where they usually appear flat. Hairy leukoplakia
or “hairy” white lesion on the lateral margins of the tongue, lesions can also occur on the buccal mucosa, generally as flat
occurs in all risk groups for HIV infections, although less lesions. Rarely, lesions occur on the soft palate. Hairy
commonly in children than in adults. HL occurs in about 20 leukoplakia usually does not cause symptoms.
percent of persons with asymptomatic HIV infection and
becomes more common as the CD4+ T-cell count falls.30 No DIAGNOSIS
report describes HL in mucosal sites other than the mouth. HL
has occurred in non-HIV-infected people including recipients of • Hairy leukoplakia should be diagnosed by biopsy for
bone marrow, cardiac and renal transplants. definitive diagnosis.
Infectious Diseases in Children 371

• Experienced clinicians can make a presumptive diagnosis CLINICAL FEATURES

of hairy leukoplakia in association with HIV disease from
Kaposi’s sarcoma can appear as a red, blue, or purplish lesion.
the clinical appearance, although it can be confused with
It may be flat or raised solitary or multiple. The most common
oral candidiasis. The typical microscopic appearance of
oral site is the hard palate, but lesions may occur on any part
hairy leukoplakia includes acanthosis, marked parakeratosis
of the oral mucosa, including the gingiva, soft palate and buccal
with the formation of ridges and keratin projections, areas
mucosa and in the oropharynx. Occasionally, yellowish mucosa
of ballooning cells and little or no inflammation in the
surrounds the Kaposi’s sarcoma lesion. Oral Kaposi’s sarcoma
connective tissue. The ballooning changes resemble
lesions may enlarge, ulcerate and become infected. Good oral
koilocytosis. Cells are enlarged; some contain enlarged
hygiene is essential to minimize these complications.
ballooning cells with pyknotic nuclei. Some contain
perinuclear haloes.
Management
• Definitive diagnosis of hairy leukoplakia requires
demonstration of EBV. EBV may be readily demonstrated 1. Treatment is determined on the basis of the number,
in biopsy specimens by a variety of techniques. Cells taken size and location of the oral Kaposi’s sarcoma lesions.
from the hairy leukoplakia lesion by scraping can be used The choice of therapy depends on the effect of
treatment on the adjacent mucosa, pain associated
for a noninvasive diagnosis using in situ hybridization.
with treatment, interference with eating and speaking,
and the patient’s preference. It is important to perform
Management
thorough dental prophylaxis before initiating therapy
1. Hairy leukoplakia usually is asymptomatic and does for Kaposi’s sarcoma lesions involving the gingiva.
not require treatment. Response to therapy is improved if all local plaque
2. It is almost always a manifestation of HIV infection and calculus are removed. Local application of
and clinicians should arrange evaluation of HIV sclerosing agents may reduce the size of oral lesions.
disease and appropriate treatment for patients with 2. Local treatment is appropriate for large oral Kaposi’s
hairy leukoplakia. sarcoma lesions that interfere with eating and talking.
3. Hairy leukoplakia has disappeared in patients Oral lesions can be treated surgically or with localized
receiving high-dose acyclovir for herpes zoster, intralesional chemotherapy. Surgical removal is
presumably because of the anti-EBV activity of suitable for small, well-circumscribed lesions such as
acyclovir. Doses of acyclovir (2.5 to 3 mg per day for gingival or tongue lesions. Surgical removal can be
2 to 3 weeks) usually eliminate HL, but the lesion performed under local anesthesia with a blade or with
usually recurs with cessation of treatment. the carbon dioxide laser. Intralesional vinblastine is
4. Elimination or almost complete clinical resolution of useful for treating small lesions, particularly on the
the lesion has occurred in patients treated with agents palate or gingiva. Several studies have documented
such as desciclovir, an analog of acyclovir, the effectiveness of one or two injections of 0.1 to 0.2
phosphonoformate, Retin A and podophyllin resin, mg per ml solution of vinblastine. Post-treatment pain
although lesions tend to recur within a few months. is fairly common, but systemic effects are rare. The
5. Case reports describe hairy leukoplakia disappearing pain usually disappears several days after therapy.
during treatment with ganciclovir, zidovudine and 3. Radiation therapy may be indicated for large, multiple
aerosolized pentamidine. Katz and colleagues have lesions. A single dose of 800 cGy or an equivalent
shown that HL both appears and disappears in fractionated dose is frequently used and produces a
good response. Side effects include xerostomia and
patients receiving zidovudine, although no case-
mucositis, although both conditions usually improve
controlled studies are available.31
with cessation of radiation therapy.
6. Occasionally, Candida albicans may be found in hairy
leukoplakia lesions. Treatment consists of antifungal
medications. Lymphoma
Non-Hodgkin’s lymphoma is rare in the pediatric population,
Kaposi ’s Sarcoma but when present, it is a strong indicator of HIV infection.
Kaposi’s sarcoma may occur intraorally, either alone or in
CLINICAL FEATURES
association with skin and disseminated lesions. Intraoral lesions
have been reported at other sites and may be the first Diffuse, undifferentiated non-Hodgkin’s lymphoma (NHL) is
manifestation of late-stage HIV disease (AIDS). Kaposi’s a frequent HIV-associated malignancy. Most are of B-cell origin
sarcoma occurs most commonly in men but also has been and Epstein-Barr virus occurs in cells from several cases.
observed in women. Lymphoma can occur anywhere in the oral cavity and there
372 Essentials of Pediatric Oral Pathology

may be soft tissue involvement with or without involvement 15. Seward JF, Galil K, Damon I, Norton SA, Rotz L, Schmid S,
of underlying bone. The lesion may present as firm, painless et al. Development and experience with an algorithm to evaluate
swelling that may be ulcerated. Some oral lesions may appear suspected smallpox cases in the United States, 2002-2004. Clin
as shallow ulcerations. Oral NHL may appear as solitary lesions Infect Dis 2004;39(10):1477-83.
16. Goodpasture EQ, Talbot FB. Concerning the nature of
with no evidence of disseminated disease.
“protozoan-like” cells in certain lesions of infancy. Am J Dis
Management Child 1921;21:415.
17. Weller TH, Hanshaw JB. Virological and clinical observation
After diagnosis of the oral lesions, the patient must be of cytomegalic inclusion disease. N Engl J Med 1962;266:1233.
referred for further evaluation of disseminated disease and 18. Weller TH. The cytomegaloviruses: ubiquitous agents with
its subsequent treatment. protean clinical manifestations. In Engl J Med 1971;285(4):
203-14.
REFERENCES 19. www.tulane.edu/~dmsander/WWW/335/335Structure.html
20. Joint United Nations Programme on HIV/AIDS. Overview of
1. Cortes SL, Rodriguez G. Leprosy in children: Association the global AIDS epidemic, 2006 Report on the global AIDS
between clinical and pathological aspects. J Trop Pediatr 2004; epidemic.
50(1):12-5. 21. Constantine NT, Callahan JD, Watts DM. Chapter 3: Screening
2. Imbiriba EB, Hurtado-Guerrero JC, Garnelo L, Levino A, Cunha tests for HIV-1 infection. In: Constantine NT, Callahan JD, Watts
Mda G, Pedrosa V. Epidemiological profile of leprosy in DM, editors. Retroviral testing. Essentials for quality control and
children under 15 in Manaus (Northern Brazil), 1998-2005. Rev laboratory diagnosis. CRC Press Inc, Florida 1992:35-58.
Saude Publica 2008;42(6):1021-6. 22. CDC. Current Trends Classification System for Human
3. Schaudinn F, Hoffmann E. Syphilitischer. Dtsch Med Immunodeficiency Virus (HIV) Infection in Children Under 13
Wochenschr 1905;31:711-4. Years of Age. MMWR 1987;225-30:235-6.
4. Wong T, Singh AE, De P. Primary syphilis: Serological 23. Classification and diagnostic criteria for oral lesions in HIV
treatment response to doxycycline/tetracycline versus benzathine infection. EC-Clearinghouse on Oral Problems Related to HIV
penicillin. Am J Med 2008;121(10):903-8. Infection and WHO Collaborating Centre on Oral Manifesta-
5. Bai ZG, Yang KH, Liu YL, et al. Azithromycin vs. benzathine tions of the Immunodeficiency Virus. J Oral Pathol Med 1993;
penicillin G for early syphilis: A meta-analysis of randomized 22(7):289-91.
clinical trials. Int J STD AIDS 2008;19(4):217-21. 24. Dull JS, Sen P, Raffanti S, et al. Oral candidiasis as a marker
6. Bollinger O. Ueber eine neue Pilzkrankheit beim Rinde. of acute retroviral illness. South Med J 1991;84:733-5, 739.
Zentralblatt Medizinische Wissenschaft 1877;15:481-90. 25. Dodd CL, et al. Oral candidiasis in HIV infection: Pseudo-
7. Wolff M, Israel J. Ueber Reincultur des Actinomyces und seine membranous and erythema tous candidiasis show similar rates
Uebertragbarkeit auf Thiere. Archiv Pathologische Anatomie of progression to AIDS. AIDS 1991;5:1339-43.
1891;126:11-28. 26. Just-Nubling G, Gentschew G, Meissner K, et al. Fluconazole
8. Funke G, von Graevenitz A. Infections due to Actinomyces neuii prophylaxis of recurrent oral candidiasis in HIV-positive
(former “CDC coryneform group 1” bacteria). Infection 1995; patients. Eur J Clin Microbiol Infect Dis 1991;10:917-21.
23(2):73-5. 27. Akova M, Akalin HE, Uzun O, et al. Emergence of Candida
krusei infections after therapy of oropharyngeal candidiasis with
9. Siqueira JF, Rocas IN. Polymerase chain reaction detection of
fluconazole. Eur J Clin Microbiol Infect Dis 1991;10:598-9.
Propionibacterium propionicus and Actynomyces radicidentis
28. Fung JC, Shanley J, Tilton RC. Comparison of the detection of
in primary and persistent endodontic infections. Oral Surg Oral
herpes simplex virus in direct clinical specimens with herpes
Med Oral Pathol Oral Radiol Endod 2003;96:215-22.
simplex virus-specific DNA probes and monoclonal antibodies.
10. Chapter 1: History of medical microbiology. In: Essentials of
J Clin Microbiol 1985;22:748-53.
Medical Microbiology. Pub: Jaypee Brothers 1-5.
29. Phelan JA, Eisig S, Freedman PD, et al. Major aphthous-like
11. Lehner T. Oral thrush or acute pseudomembranous candidiasis. ulcers in patients with AIDS. Oral Surg Oral Med Oral Pathol
A clinicopathologic study of 44 cases. Oral Surg 1964;18:27. 1991;71:68-72.
12. Odds F. Sabourauds agar. J Med Vet 1991;29:355-9. 30. Feigal DW, Katz MH, Greenspan D, et al. The prevalence of
13. Taschdjian CL, Burchill JJ, Kozin PZ. Rapid identification of oral lesions in HIV-infected homosexual and bisexual men: three
Candida albicans by filamentation in serum and serum San Francisco epidemiological cohorts. AIDS 1991;5:519-25.
substitutes. Amer J Clin Path 1960;99:212-5. 31. Katz MH, Greenspan D, Heinic GS, et al. Resolution of hairy
14. World health. The magazine of the World Health Organization, leukoplakia: an observational trial of zidovudine versus no
May, 1980. treatment. J Infect Dis 1991;164:1240-1.
14 Hematological Disorders in Children 373

Hematological
Disorders in Children
Shweta Dixit Chaudhary, Mayur Chaudhary, Prashant Dixit

CHAPTER OVERVIEW
Introduction Monocytosis
Classification Basophilia
Anemia Agranulocytosis
Thalassemia Neutropenia
Hemophilia Lymphocytosis
Polycythemia Leukemia
Leukocytosis Thrombocytopenia
Neutrophilia Lymphoma
Eosinophilia

INTRODUCTION Classification by Cause of Anemia

Fortunately, most of the hematologic diseases are often • Blood loss anemia
dramatic and demonstrative in the type and presentation of • Aplastic anemia
orofacial signs and symptoms. Recognition of these signs and • Megaloblastic anemia
symptoms is important for a clinician in order to be able to • Hemolytic anemia
avoid any inadverdent dental mishaps. — Hereditary spherocytosis
— Sickle cell anemia
CLASSIFICATION — Erythroblastosis fetalis.
Bleeding disorders can be broadly classified as those:
1. Involving the red blood cells Classification by Histologic Picture
2. Involving the white blood cells • Macrocytic
3. Involving blood platelets • Normocytic
4. Involving specific blood factors. • Microcytic
• Hypochromic microcytic.
ANEMIA
DEFINITION Effect on the Circulatory System
Anemia is defined as an abnormal reduction in: Etiologic Classification by Wintrobe1
• Number of circulating red blood cells.
1. Loss of blood
• The quantity of hemoglobin.
a. Acute post hemorrhagic anemia.
• The volume of packed red cells in a given unit of blood.
b. Chronic post hemorrhagic anemia.
CLASSIFICATION 2. Excessive destruction of red corpuscles
It includes four subtypes: a. Extra corpuscular causes:
1. Classification by cause of anemia • Antibodies
2. Classification by histologic picture • Infection
3. Effect on the circulatory system. • Splenic sequestration and destruction
4. Etiologic classification by Wintrobe1 • Associated disease states
374 Essentials of Pediatric Oral Pathology

• Drugs, chemicals and physical agents • Dekeratinization occurs alongwith epithelial atrophy
• Trauma to RBCs. • Atrophy of filiform papillae of tongue
b. Intra corpuscular causes: • Ulceration and inflammation of the mucosa
• Hereditary • Gingivitis and periodontitis are also common
— Disorders of glycolysis • Angular cheilitis
— Faulty synthesis or maintenence of reduced • Susceptible to oral monilial infections.
glutathione
— Qualitative or quantitative abnormalities in NUTRITIONAL DEFICIENCY
synthesis of globin • Usually present in adolescent girls
— Abnormalities of RBC membrane • Exogenous iron needed during first two years of life and in
— Erythropoietic porphyria. adolescence
• Acquired • Cracking or splitting of nails
— Paroxysmal nocturnal hemoglobinuria • Painful tongue
— Lead poisoning. • Decreased healing after dental surgeries
3. Impaired blood production resulting from deficiency of • Mucosal pallor.
substances essential for erythropoiesis:
• Iron deficiency. FOLIC ACID DEFICIENCY ANEMIA
• Deficiency of various B vitamins (B12, B9, B6, B3). Folic acid deficiency anemia is a common, slowly progressive,
• Protein deficiency. megaloblastic anemia characterized by red blood cells that are
• Possibly ascorbic acid deficiency. larger than normal and hence the term macrocytic (Fig. 14.1)
4. Inadequate production of mature erythrocytes: when referring to this type. The red blood cells are also
• Deficiency of erythroblasts deformed, and both their rate of production and their lifespan
— Atrophy of bone marrow: aplastic anemia are diminished. Folic acid anemia occurs most often in infants,
i. Chemical or physical agents adolescents, pregnant and lactating females, alcoholics, the
ii. Hereditary elderly and in those with malignant or intestinal diseases.
iii. Idiopathic.
— Isolated erythroblastopenia: ETIOPATHOGENESIS
i. Thymoma
ii. Chemical Folic acid is needed for the orderly production of deoxyribo-
iii. Antibodies. nucleic acid (DNA) in all tissue cells and is a component of
three of the four DNA bases: thymine, adenine and guanine.
• Infiltration of bone marrow
In bone marrow, it is required for the normal production of the
— Leukemia, lymphomas
— Multiple myeloma
— Carcinoma, sarcoma
— Myelofibrosis.
• Endocrine abnormality
— Myxedema
— Addisonian adrenal insufficiency
— Pituitary insufficiency
— Hyperthyroidism.
• Chronic renal disease
— Infections
— Non-infectious diseases including granulomatous
and collagen diseases.
• Cirrhosis of liver.

COMMON ORAL MANIFESTATIONS IN ANEMIA

• They are mainly due to changes in metabolism in the FIGURE 14.1: Folic acid deficiency anemia showing macro
mucosal epithelium cytes similar to that seen in vitamin B deficiency anemia
Hematological Disorders in Children 375

red blood cells and for RNA synthesis. It is basically required • Overcooking of food, destroying valuable water-soluble
for the formation of heme, the pigmented, iron-containing nutrients, including a high percentage of folic acid.
portion of the hemoglobin in erythrocytes. A deficient intake • Limited storage capacity in infants.
of folic acid impairs the maturation of young red blood cells, • Prolonged drug therapy, especially from anticonvulsants
which results in anemia. Folic acid circulates through and is and estrogens.
stored in the liver and a deficiency is almost always because • Not addressing increased folic acid needs of certain age
of insufficient amounts in the diet. groups, as well as in patients with neoplastic diseases
Absorption of folic acid occurs primarily in the upper small and some skin disorders (e.g. chronic exfoliative
intestine and does not depend on intrinsic factor as vitamin B12 dermatitis).
does. A deficiency of folic acid is more common than a • Alcohol abuse (alcohol prevents absorption of several
cobalamin (B12) deficiency. Folic acid stores are also depleted nutrients especially the B vitamins).
more rapidly than cobalamin stores and without proper dietary • Poor diets (common in alcoholics, the elderly, those
intake, a megaloblastic anemia will develop. living alone or in poverty and infants, especially those
Folic acid deficiency anemia is common during pregnancy. with infections or diarrhea).
Both folate and iron are essential for red cell production and
during pregnancy there is an increased need to supply both the CLINICAL FEATURES
mother and the developing infant. This type of anemia is
Signs and symptoms of folic acid deficiency anemia gradually
common in newborns because of the increasing survival rates
produces clinical features similar to other megaloblastic
of premature infants. Not only can it be a danger to the mother,
anemias, but without neurologic manifestations of B12
but also contributes to fetal malformations. The most common
deficiency. Symptoms include the following:
birth defect resulting from a deficiency of this vitamin is spina
• Gastrointestinal dysfunction
bifida. Deficiency of folic acid is also one of the contributory
• Angular cheilitis
factors for the development of cleft lip and/or palate. This lays
• Ulcerative stomatitis
emphasis on the importance of prenatal counseling.
• Pharyngitis
During the 1980s, a considerable body of evidence
• Sore tongue
accumulated stating that spina bifida and other neural tube
• Diarrhea
defects were associated with folate deficiency. It is now widely
• Progressive fatigue
recognized that folate supplements are necessary and best
• Shortness of breath
started before pregnancy occurs since closure of the neural tube
• Heart palpitations
occurs by day 28 of pregnancy. This is generally long before
• Weakness
the woman knows she is pregnant. It was also established that
• Glossitis
receiving enough folate from fortified foods was nearly
• Nausea
impossible and supplements were highly recommended. Even
• Anorexia
though the studies successfully used the folate monoglutamate
• Headache
form of folic acid, it is the pteroylmonoglutamic acid form that
• Fainting
is used in vitamin supplements and fortified foods. Most
• Irritability
naturally occurring folates are pteroylpolyglutamates.
• Forgetfulness
Folic acid deficiency is also common in tropical areas
• Pallor
where poverty results in a poor diet. In North America and other
• Slight jaundice.
regions of the world where access to food is rarely a problem,
folic acid deficiency still occurs because dietary needs are not Blood Investigations
met, especially during the growth of children and adolescents
and during pregnancy. These age groups are more prone to folic • Evaluation is based on blood tests and measurement of
acid deficiency anemia because of their heavy use of folate- serum folate levels.
deficient cow’s milk, which also inhibits the absorption of iron, • Diagnosis is made following the Schilling test and a
causing an additional risk of iron-deficiency anemia as well. therapeutic trial of vitamin B12 injections to distinguish
Causes of folic acid deficiency anemia include: between folic acid deficiency anemia and pernicious
• Impaired absorption because of intestinal dysfunction anemia.
from such disorders as celiac disease, tropical sprue, • Significant blood test findings include macrocytosis,
regional jejunitis, Crohn’s disease or bowel resection. decreased reticulocyte count, abnormal platelets and a
• Bacteria competing for available folic acid. serum folate less than 4 mg/ml.
376 Essentials of Pediatric Oral Pathology

Management • Atrophy of taste buds on tongue is a common finding

(Fig. 14.2)
1. The oral administration of folic acid produces quick
improvement in all symptoms; an adequate diet
• Nonspecific persistent stomatitis.
results in cure in cases due to simple malnutrition. • Mucosa is intolerant to appliances.
2. Conventional treatment consists primarily of folic acid
supplements (about 400 mg three times daily) and, Blood Investigations
more importantly, the elimination of causes
contributing to deficiency. • R.B.C count < 10,00000 cells/cubic mm
3. Oral administrations of folate preparations are 1 to • Macrocytosis (Fig. 14.3)
5 mg daily.
• Poikilocytosis
4. Prophylactic doses are given in pregnancy or those
considering getting pregnant. • Hemoglobin content is increased
5. Parenteral administration of folic acid can relieve • Advanced cases—Polychromatophilic cells, stippled cells,
acute symptoms within 48 hours. Howell-Jolly bodies and Cabot’s rings-punctate basophilia
6. Blood transfusions are given to treat severe cardiac • WBCs are decreased in number but increased in size—
or respiratory distress as a result of severe deficiency. Macropolycytes
• Mild to moderate thrombocytopenia
PERNICIOUS ANEMIA AND VITAMIN B12 • Coexistent iron deficiency.
DEFICIENCY
It is a chronic hematologic disease with gastric atrophy and loss
of intrinsic factor production in gastric secretions. Intrinsic factor
is essential for absorption of vitamin B12 (erythrocyte–maturing
principle). It is also called as vitamin B12 deficiency, Addisonian
anemia, Hunter-Addison anemia, Lederer anemia, Biermer-
Ehrlich anemia, Addison-Biermer disease. Pernicious anemia is
a term reserved for patients with vitamin B12 deficiency due to
a lack of production of intrinsic factor in the stomach.

ETIOPATHOGENESIS
• Gastric atrophy
• Autoimmune disorder with antiparietal cell antibodies
associated with HLA types A2, A3 and B7 and A blood group.

CLINICAL FEATURES
• Usually rare before the age of 30 years, hence does not FIGURE 14.2: Pernicious anemia showing atrophy
warrant a detailed description here of taste buds on tongue
• Incidence in males > females
• Triad of symptoms—Weakness, sore painful tongue,
numbness of extremities
• Fatigue, headache, nausea, vomiting, pallor, loss of weight
and appetite
• Nervous system—Paresthesia of limbs, stiffness, irritability,
depression, drowsiness. Degeneration of peripheral nerves
and degeneration of myelin sheath.

Oral Manifestations
• Glossitis—Tongue is beefy red in color entirely or in
patches. Small shallow ulcers.
• Glossodynia, glossopyrosis, gradual loss of papillae of
tongue—Smooth or bald tongue called as Hunter’s glossitis
or Moeller’s glossitis. FIGURE 14.3: Pernicious anemia showing macrocytosis
Hematological Disorders in Children 377

Serum Investigations
• Indirect bilirubin elevated
• Serum lactate dehydrogenase increased.

Gastric Secretions
• Achlorhydria
• Intrinsic factor decreased or absent.

Bone Marrow
• Hypercellularity
• Trilineage differentiation
• Erythroid precursors are large and oval, nucleus is large
and contains coarse motley chromatin clumps providing a
“checker board appearance”
• Presence of giant platelets in smear.

Management
FIGURE 14.4: Purpuric rash on forearm
1. Administration of vitamin B12 and folic acid. seen in aplastic anemia
2. The mental and neurological damage can become
irreversible without therapy. Oral Manifestations
• Petechiae and purpuric spots on oral mucosa
APLASTIC ANEMIA • Spontaneous gingival hemorrhage
Aplastic anemia is a bone marrow failure syndrome charac- • Ulcerative lesions of oral mucosa and pharynx may result
terized by peripheral pancytopenia and generalized lack of in gangrene (lack of inflammatory response).
marrow activity.
Laboratory Investigations
Types • Pancytopenia
• Primary aplastic anemia • RBC count <10,00,000 cells/cu mm
• Secondary aplastic anemia. • Decreased hematocrit and Hb levels
• Prolonged bleeding time
ETIOLOGY • Hypoplasia of bone marrow
• Severe cases—Fatty replacement of marrow and increased
• Primary aplastic anemia: plasma cells and mast cells (Fig. 14.5).
— Unknown etiology
— Fanconi’s Syndrome: Congenital aplastic anemia + Management
bone abnormalities + microcephaly + hypogenitalism
1. Blood transfusion.
+ olive brown pigmentation of skin. 2. Bone marrow transplantation.
• Secondary aplastic anemia: 3. Immunosuppressive therapy.
— Known etiology with a better prognosis 4. Consult patient’s physician before planning dental
— Exposure to various drugs, chemical substances, radiant treatment.
energy. Chemicals may be amidopyrine, chlorampheni- 5. Toothbrushing is contraindicated by most physicians.
col, colloidal Ag, Hg, etc. A soft toothbrush and gentle strokes may be
permitted.
CLINICAL FEATURES 6. Mouthwashes are found to be ineffective.
7. Gentle rubbing with wet gauze is recommended.
• Severe weakness, dyspnea, pallor of skin 8. Local anesthesia may be dangerous if platelet count
• Numbness and tingling of extremities is below 50,000/cu mm.
• Petechiae in skin and mucous membrane due to platelet 9. Prosthetic and orthodontic appliances are contra-
deficiency (Fig. 14.4) indicated.
10. Treatment contraindicated during an aplastic crisis.
• Increased infection due to neutropenia.
378 Essentials of Pediatric Oral Pathology

amino acid glutamic acid to be replaced with the hydrophobic

amino acid valine at the sixth position. The -globin gene is
found on the short arm of chromosome 11. The association of
two wild-type -globin subunits with two mutant -globin
subunits forms hemoglobin S (HbS). Under low-oxygen
conditions (being at high altitude, for example), the absence
of a polar amino acid at position six of the -globin chain
promotes the non-covalent polymerization (aggregation) of
hemoglobin, which distorts red blood cells into a sickle shape
and decreases their elasticity. Instead of being flexible and disc-
shaped, these cells are more stiff and curved in the shape of
the old farm tool known as a sickle— that is where the disease
gets its name. The shape is similar to a crescent moon. This
single amino acid change causes hemoglobin proteins to form
FIGURE 14.5: Aplastic anemia showing presence of more
number of fat cells as compared to red cells
fibers. In people heterozygous for HgbS (carriers of sickling
hemoglobin), the polymerization problems are minor, because
the normal allele is able to produce over 50 percent of the
11. Aplastic crisis usually self-limiting with recovery in
hemoglobin. In people homozygous for HgbS, the presence of
7 to 10 days.
long-chain polymers of HbS distort the shape of the red blood
12. For ulcerations:
• Soft bland diet cell from a smooth donut-like shape to ragged and full of spikes,
• Topical anesthetics making it fragile and susceptible to breaking within capillaries.
• Mouthrinses of 50:50 mixture of diphenhydramine Carriers have symptoms only if they are deprived of oxygen
and kaolin/pectin or dyclonine. (for example, while climbing a mountain) or while severely
dehydrated. Under normal circumstances, these painful crises
SICKLE CELL ANEMIA occur 0.8 times per year per patient.
The loss of red blood cell elasticity is central to the patho-
Sickle-cell disease or sickle-cell anemia (or drepanocytosis)
is a life-long blood disorder characterized by red blood physiology of sickle-cell disease. Normal red blood cells are
cells that assume an abnormal, rigid, sickle shape. Sickling quite elastic, which allows the cells to deform to pass through
decreases the cells’ flexibility and results in a risk of various capillaries. In sickle-cell disease, low-oxygen tension promotes
complications. red blood cell sickling and repeated episodes of sickling
Linus Pauling and colleagues were the first, in 1949, to damage the cell membrane and decreases the cell’s elasticity.
demonstrate that sickle-cell disease occurs as a result of an These cells fail to return to normal shape when normal oxygen
abnormality in the hemoglobin molecule.2 This was the first tension is restored. As a consequence, these rigid blood cells
time a genetic disease was linked to a mutation of a specific are unable to deform as they pass through narrow capillaries,
protein, a milestone in the history of molecular biology and it leading to vessel occlusion and ischemia (Figs 14.6A and B).
was published in their paper “sickle cell anemia, a molecular The actual anemia of the illness is caused by hemolysis,
disease”. the destruction of the red cells inside the spleen, because of
their misshape. Although the bone marrow attempts to
ETIOPATHOGENESIS compensate by creating new red cells, it does not match the
rate of destruction. Healthy red blood cells typically live
One-third of all indigenous inhabitants of sub-Saharan Africa
carry the gene, because in areas where malaria is common, there 90 to 120 days, but sickle cells only survive 10 to 20 days.
is a survival value in carrying only a single sickle-cell gene The allele responsible for sickle-cell anemia is autosomal
(sickle cell trait). Those with only one of the two alleles of incomplete dominant and can be found on the short arm of
sickle-cell disease are more resistant to malaria, since the chromosome 11. A person that receives the defective gene from
infestation of the malaria plasmodium is halted by the sickling both father and mother develops the disease; a person that
of cells which it infests. Sickle-cell gene mutation probably receives one defective and one healthy allele remains healthy,
arose spontaneously in different geographic areas, as suggested but can pass on the disease and is known as a carrier. If two
by restriction endonuclease analysis. parents who are carriers have a child, there is a 1-in-4 chance of
Sickling of erythrocytes occurs because of a point mutation their child’s developing the disease and a 1-in-2 chance of their
in the -globin chain of hemoglobin, causing the hydrophilic child’s being a carrier. Since the gene is incompletely recessive,
Hematological Disorders in Children 379

FIGURE 14.7: Diagrammatic representation of inheritance of

sickle cell anemia

child’s having sickle-cell disease (SS) and a 50 percent

chance of a child’s having sickle-cell trait (AS).
2. When both parents have sickle-cell trait (AS), a child has
a 25 percent chance (1 of 4) of sickle-cell disease (SS), as
shown in the diagram.
FIGURES 14.6A and B: Sickle cell anemia. (A) Showing normal
red blood cells flowing freely in a blood vessel. The inset image CLINICAL FEATURES
shows a cross-section of a normal red blood cell with normal
hemoglobin. (B) Showing abnormal, sickled red blood cells clumping • Sickle-cell disease usually presents in childhood and
and blocking blood flow in a blood vessel. (Other cells also may occurs more commonly in people (or their descendants)
play a role in this clumping process.) The inset image shows a from parts of tropical and sub-tropical regions where
cross-section of a sickle cell with abnormal hemoglobin malaria is or was common. The prevalence of the disease
in the United States is approximately 1 in 5,000, mostly
affecting Americans of sub-Saharan African descent,
carriers can produce a few sickled red blood cells, not enough according to the National Institute of Health.4 In the United
to cause symptoms, but enough to give resistance to malaria. States, about 1 in 500 black births have sickle-cell anemia.
Because of this, heterozygotes have a higher fitness than either • Life expectancy is shortened, with studies reporting an
of the homozygotes. This is known as heterozygote advantage. average life expectancy of 42 and 48 years for males and
Due to the adaptive advantage of the heterozygote, the females, respectively.5
disease is still prevalent, especially among people with recent • Uwakwe et al, 2002, found that caffeine could hasten
ancestry in malaria-stricken areas, such as Africa, the sickling as well as fragility of HbS erythrocytes in a
Mediterranean, India and the Middle East.3 concentration dependent manner.6
Sickle-cell disease is inherited in the autosomal recessive • No active hemolytic disease is generally seen before the
pattern (Fig. 14.7). age of two years.
1. If one parent has sickle-cell anemia (SS) and the other has • Medullary hypertrophy is seen in an attempt to replace the
sickle-cell trait (AS), there is a 50 percent chance of a lysed erythrocytes.
380 Essentials of Pediatric Oral Pathology

Oral Manifestations • Abnormal hemoglobin forms can be detected on

hemoglobin electrophoresis, a form of gel electrophoresis
• Robinson and Sarnat, 1952, stated that 79.2 percent
on which the various types of hemoglobin move at varying
patients show jaw abnormalities.7 speeds. The diagnosis can be confirmed with high-
• Mourshed and Tuckson, 1974, found radiographic changes performance liquid chromatography (HPLC).
in the mandible in 85 percent of patients.8 • Genetic testing is rarely performed, as other investigations
• Sanger and Bystrom, 1977, noted a loss of alveolar bone are highly specific for HbS and HbC.
in intra-oral periapical radiographs of all children • An acute sickle-cell crisis is often precipitated by infection.
examined.9 Therefore, a urinalysis to detect an occult urinary tract
• A coarse trabecular “stepladder” pattern was seen in the infection, and chest X-ray to look for occult pneumonia
jaw. should be routinely performed.
• Hypomineralization of dentin is seen.
• Interglobular dentin in the periapical area is evident with Bone Marrow
inclusions in the peripulpal dentinal tubules. Bone marrow is hyperplastic (Fig. 14.9)
• Abrupt alteration of dentinogenesis with denticle like
calcified bodies in the pulp chamber may be seen. Management
• Proliferation of cementum in the apical and midroot areas 1. Most people with sickle-cell disease have intensely
may be evident. painful episodes called vaso-occlusive crises. Painful
crises are treated symptomatically with analgesics;
Laboratory Investigations pain management requires opioid administration at
regular intervals until the crisis has settled. For milder
• In HbSS, the full blood count reveals hemoglobin levels crises, a subgroup of patients manage on NSAIDs
in the range of 6 to 8 g/dL with a high reticulocyte count. (such as diclofenac or naproxen). For more severe
In other forms of sickle-cell disease, Hb levels tend to be crises, most patients require inpatient management
higher. for intravenous opioids; patient-controlled analgesia
(PCA) devices are commonly used in this setting.
• A blood film may show features of hyposplenism (target
Diphenhydramine is also an effective agent that is
cells and Howell-Jolly bodies). frequently prescribed by doctors in order to help
• Sickling of the red blood cells, on a blood film, can be control any itching associated with the use of opioids.
induced by the addition of sodium metabisulfite (Fig. 14.8). 2. Children born with sickle-cell disease will undergo
• The presence of sickle hemoglobin can also be close observation by the pediatrician and will require
demonstrated with the “sickle solubility test”. A mixture management by a hematologist to assure they remain
of hemoglobin S (HbS) in a reducing solution (such as healthy. These patients will take a 1 mg dose of folic
sodium dithionite) gives a turbid appearance, whereas acid daily for life. From birth to five years of age, they
will also have to take penicillin daily due to the
normal Hb gives a clear solution.

FIGURE 14.8: Peripheral smear showing sickle-shaped red blood cell FIGURE 14.9: Sickle cell anemia showing hyperplastic marrow
Hematological Disorders in Children 381

immature immune system that makes them more 14. Prevention along with regular recalls is recommended
prone to early childhood illnesses. for periodontal disease.
3. Acute chest crisis is managed in a manner similar to 15. During general anesthesia, strict adherence to
vaso-occlusive crisis, with the addition of antibiotics general principles, will avoid the onset of a sickle cell
(usually a quinolone or macrolide, since wall-deficient crisis.
[atypical] bacteria are thought to contribute to the 16. Future treatments:
syndrome), oxygen supplementation for hypoxia, and • Various approaches are being sought for
close observation. Should the pulmonary infiltrate preventing sickling episodes as well as for the
worsen or the oxygen requirements increase, simple complications of sickle-cell disease. Other ways to
blood transfusion or exchange transfusion is modify hemoglobin switching are being investi-
indicated. The latter involves the exchange of a gated, including the use of phytochemicals such
significant portion of the patient’s red cell mass for as nicosan. Gene therapy is under investigation.
normal red cells, which decreases the percent of Another treatment being investigated is
hemoglobin S in the patient’s blood. Senicapoc.12
4. The first approved drug for the causative treatment • People who are known carriers of the disease
of sickle-cell anemia, hydroxyurea, was shown by often undergo genetic counseling before they
Charache et al, 1995, to decrease the number and have a child. A test to see if an unborn child has
severity of attacks10 and shown to possibly increase the disease takes either a blood sample from the
survival time in a study in 2003 by Steinberg et al.11 fetus or a sample of amniotic fluid. Since taking a
This is achieved, in part, by reactivating fetal blood sample from a fetus has greater risks, the
hemoglobin production in place of the hemoglobin S latter test is usually used.
that causes sickle-cell anemia. Hydroxyurea had
previously been used as a chemotherapy agent and
there is some concern that long-term use may be THALASSEMIA
harmful, but this risk has been shown to be either
Thalassemia is an autosomal recessive disorder that can affect
absent or very small and it is likely that the benefits
outweigh the risks.
the structure and synthesis of hemoglobin.
5. Bone marrow transplants have proven to be effective
in children. ETIOPATHOGENESIS
6. Fluid balance, body temperature regulation, patient
Beta halassemia major results in a failure to produce globin
positioning are extremely important.
7. Dietary cyanate, from foods containing cyanide
chains, impeding the synthesis of normal globin chains, leading
derivatives, has been used as a treatment for sickle to damage of the red cell membrane, followed by cell lysis and
cell anemia. In the laboratory, cyanate and severe anemia. The body responds by increasing the production
thiocyanate irreversibly inhibit sickling of red blood of erythrocytes, causing increased marrow capacity in bones
cells drawn from sickle cell anemia patients. However, and extra-medullary hematopoiesis, particularly in the spleen
the cyanate would have to be administered to the and liver.
patient for a lifetime, as each new red blood cell
created must be prevented from sickling at the time
CLINICAL FEATURES
of creation. Cyanate also would be expelled via the
urea of a patient in every cycle of treatment. • Clinical manifestations are seen within the first year of life.
8. Treatment during a sickle cell crisis is contraindicated. • Failure to thrive, splenomegaly, poor appetite are
9. Sedative drugs to be used should not affect the commonly seen.
respiratory rate.
• Varying degrees of anemia and jaundice (Fig. 14.10) may
10. Local anesthetics with vasoconstrictors are not contra-
indicated.
be present.
11. According to Stewart, N2O – O2 is not contraindicated • Recurrent ulcers on lower extremities may be seen.
if oxygen levels are closely monitored and 100 • Protrusion of the middle-third of face due to enlargement
percent O 2 administered immediately on of maxilla (“chipmunk facies”) (Fig. 14.11), tightness of
discontinuation of N2O – O2 sedation. the upper lip are evident. This leads to an increased overjet
12. Pre-operative blood transfusion is recommended if Hb and spacing of the upper incisors. The maxillary alveolar
< 5 g/dL. bone and the palate show a retruded position.
13. Presence of enamel hypomineralization dictates the • Mandible becomes less enlarged compared to the maxilla,
institution of preventive measures to avoid caries or
possibly because the dense cortical plates of the mandible
wearing away of tooth structure.
prevent the expansion.
382 Essentials of Pediatric Oral Pathology

• Ocular hypertelorism, epicanthal folds, bronzing of the skin • Medullary hyperplasia, generalized osteoporosis show a
may be seen. Frontal bossing is evident (Fig. 14.12). “hair on end” appearance on a lateral skull radiograph (Fig.
• Pain and swelling of the parotid glands and atrophic 14.13).
candidiasis have also been reported in these patients. • Lamina dura may be thinned out with shortening of roots
of the teeth.
RADIOGRAPHIC FEATURES
BLOOD INVESTIGATION
• Maxillary sinuses may be obliterated due to erythroid
hyperplasia. Thalassemia major shows small, pale (hypochromic),
• There is generalized rarefaction of alveolar bone, thinning abnormally-shaped red blood cells (Fig. 14.14). Thalassemia
of cortical bone, coarse trabeculae of the jaws and a minor shows small (microcytic), pale (hypochromic), variously-
“chicken-wire” appearance of enlarged marrow spaces. shaped (poikilocytosis) red blood cells (Fig. 14.15)

FIGURE 14.10: Thalassemia major showing yellowish FIGURE 14.12: Thalassemia major showing
discoloration of sclera frontal bossing in a child

FIGURE 14.11: Thalassemia major showing ‘chipmunk facies’ FIGURE 14.13: Thalassemia major showing widening of the diploic
space of the skull with thinning of the inner and outer tables produces
a “hair on end” appearance
Hematological Disorders in Children 383

5. Surgical correction of bony abnormalities of the

maxilla may be done, but a relapse can be expected
due to continued marrow expansion.
6. Dental treatment under local anesthesia and nitrous
oxide-oxygen inhalation may be carried out normally,
but decision on use of general anesthesia warrants
a detailed preoperative consultation with an
experienced anesthesiologist and the patient’s
physician due to cardiomyopathy, liver concerns and
endocrine complications.
7. Allogenic stem cell transplantation is recommended
and preferably should be done at an early age before
the child has developed disease and complications
associated with the therapies.

FIGURE 14.14: Thalassemia major showing small, pale HEMOPHILIA

(hypochromic), abnormally-shaped red blood cells
Hemophilia is a rare inherited disorder in which the blood does
not clot normally. The word hemophilia is derived from Greek
haima = blood, philia = friend.

CLASSIFICATION
Hemophilia may be of three types:
1. Hemophilia A—Also called as classic hemophilia—
deficiency of factor VIII or antihemophilic factor.
2. Hemophilia B—Also called as Christmas disease—
deficiency of factor IX or plasma thromboplastin
component.
3. Hemophilia C—Deficiency of factor XI or plasma
thromboplastin antecedent.
• Incidence of Factor VIII deficiency—80 percent of
patients suffering from hemophilia
FIGURE 14.15: Thalassemia minor showing small (microcytic), pale • Incidence of Factor IX deficiency—15 percent of
(hypochromic), variously-shaped (poikilocytosis) red blood cells. patients suffering from hemophilia.
These small red blood cells (RBCs) are able to carry less oxygen Hemophilia A and hemophilia B are classified into three
than normal RBCs groups based on the level of procoagulant present:
• Mild—Factor levels are 5 to 35 percent
Management • Moderate—Factor levels are 2 to 5 percent
1. Prevention of dental disease is paramount, hence the • Severe—Factor levels are 0 to 1 percent.
emphasis on pre- and post-natal counseling with Hemophilia C can be distinguished from hemophilia A
anticipatory guidance and close follow-up. (deficiency of factor VIII) and hemophilia B (deficiency of
2. In order to manage the organomegaly and skeletal factor IX) by the absence of bleeding into joints and muscles
changes, children start regular monthly blood and by its occurrence in individuals of either sex.
transfusions from an early age (6–9 months), which Unlike the bleeding tendency in hemophilia A or B, which
continue through life if a hematopoietic cell transplant is clearly related to the factor level, the bleeding risk in
is not available. hemophilia C is not always influenced by the severity of the
3. Iron overload, the main cause of morbidity and deficiency, especially in individuals with partial deficiency. This
mortality, is controlled by the iron-chelating drug unpredictability makes hemophilia C more difficult to manage
desferrioxamine.
than hemophilia A or B.
4. Conventional treatment related complications include
post transfusional viral infections and multiple VON WILLEBRAND’S DISEASE
endocrine dysfunction, progressive liver fibrosis and
cardiac disease due to iron overload. It is a hereditary bleeding disorder resulting from an
abnormality of von Willebrand factor (VWF) that is found in
384 Essentials of Pediatric Oral Pathology

plasma, platelets, megakaryocytes and endothelial cells. It

circulates with factor VIII and is important in platelet adhesion
to the subendothelium via collagen and is therefore important
in formation of primary platelet plug. There is a qualitative and
quantitive defect in the VWF. VWF is composed of subunits
called as multimers. von Willebrand’s disease is divided into
subtypes based on platelet and plasma multicentric structure
of the VWF. Impaired formation of the platelet plug may result
in bleeding from the skin and mucosa, bruising, epistaxis,
prolonged bleeding after surgical procedures and menorrhagia.
This is in contrast to factors VIII and IX deficient hemophilia
in which bleeding tends to involve muscles and joints.

ETIOLOGY

Transmission (Figs 14.16 and 14.17)

• Factor VIII and IX deficiency—X-linked recessive
• Factor XI deficiency—Autosomal recessive
• Von Willebrand disease—Autosomal dominant. FIGURE 14.17: Schematic representation of inheritance pattern
Hemophilia almost always affects boys because the disease of hemophilia (hemophilic father and normal mother)
is an X-linked genetic disorder, passed from mother to son.
Boys get an X chromosome from their mother and a Y in the daughter becoming a hemophilia carrier. So it’s possible
chromosome from their father. If the mother carries the gene one of her sons could have the disease.
for hemophilia on one of her X chromosomes, each of her sons
will have a 50 percent chance of having hemophilia. CLINICAL FEATURES
A mother who is a carrier also has a 50 percent chance of • Usually no spontaneous hemorrhage during the first year
giving the faulty X chromosome to her daughter. That does of life.
not give the daughter the hemophilia disease, but it does result • Ambulation often brings evidence of bruising.
• People who have hemophilia may bleed for a long time
after an injury or accident.
• They also may bleed into their knees, ankles and elbows
(hemarthroses) (Fig. 14.18). Bleeding in the joints causes
pain and, if not treated, can lead to arthritis.
• Muscle atrophy, osteoporosis may be present.
• Spontaneous bleeding in severe cases is seen.
• Mulkey, 1976 13 and Stoneman and Deierl, 1975, 14
described hemophilic pseudotumors of the jaws.
• Intraosseous and subperiosteal hemorrhage is generally
seen.
• Tooth displacement may be present.
• Mucous membrane hemorrhage may be present.
• Petechiae, ecchymoses are very common.
• Bleeding in the brain, a very serious complication of
hemophilia requires emergency treatment.
• Babies who are learning to crawl usually bump into things,
but ordinarily this doesn’t cause swelling in their joints. If
they do, it is a tip-off that there may be bleeding in the
joints, which usually does not happen from normal activities
FIGURE 14.16: Schematic representation of inheritance pattern or minor bumps.
of hemophilia (normal father and carrier mother) • Excessive bleeding during menstruation may occur.
Hematological Disorders in Children 385

13. Slow atraumatic injections reduce post-operative

complications.
14. Rubber dam clamps should be atraumatic to gingival
tissues.
15. Evans, 1977, recommends a thin rubber dam to
minimize clamp torque.
16. Saliva ejection is avoided to minimize suction
hematoma.15
17. Wedges and matrices are crucial to retract papillae
from the operative site.
18. Retraction cord during crown preparation is helpful
in exposing marginal areas.
19. Powell, 1974, recommended that during pulp therapy,
filing and filling should be 1 to 2 mm short of the radio-
graphic apex.16
FIGURE 14.18: Hemarthrosis seen in hemophilia 20. Most oral surgeries can be accomplished at a 30 to
50 percent replacement level.
• Psychosocial problems due to inability to play and mix with 21. Just before surgery, start with 7 to 10 day regimen of
other children of the same age group and frequent visits to EACA (epsilon-amino caproic acid)—Loading dose
the hospital. 100-200 mg/kg followed by 100 mg/kg for 7 to 10 days.
22. Clear liquids for the first day after surgery.
23. Utensils, straws and hard foods should not be inserted
RADIOGRAPHIC FEATURES
in the mouth.
• Enlargement of bony structures 24. Milk and dairy products may aggravate clot dissolution.
• Delicate trabeculae 25. Tissue borne primary tooth can be extracted using
• Areas of radiolucency. topically applied thrombin or other hemostatic agent
and local pressure.
Management 26. Cellulose bandage may be applied to protect the
wound.
1. Main principles of management are: 27. For a primary tooth with good bone support, extraction
• Prevention of dental disease. considerations should be same as that for a
• Prevention of hemorrhage while the disease is permanent tooth.
being eliminated. 28. Placement of oxidized cellulose dipped in powdered
2. Brushing with a soft toothbrush and careful flossing. or liquid thrombin in the apical portion of sockets helps
3. Fluorides for prevention of dental caries. control the hemorrhage.
4. Careful prophylaxis may not require replacement 29. Topical application of concentrated antihemophilic
therapy. factor and microfibrillar collagen hemostat to sockets
5. Deep scaling may require transfusion of factor VIII also helps in controlling hemorrhage after extraction.
concentrate or factor IX concentrate (monoclonal and 30. Sutures may be used when appropriate.
recombinate). 31. For mild factor VIII deficient hemophilia, DDAVP
6. Ultrasonic instruments are advocated for professional (1-deamino-8-D-arginine vasopressin) may be used
oral hygiene procedures. for minor hemorrhagic episodes to achieve
7. Gingival packing may expose deep calculus to easier hemostasis. DDAVP is a synthetic analog of
removal and avoid replacement therapy. vasopressin. This drug when given intravenously,
8. Infiltration local anesthesia is possible without factor subcutaneously or intranasally causes a rise in the
coverage. factor VIII activity and the VWF often to the hemostatic
9. Intrapulpal injections are possible without factor range. Peak levels are obtained approximately
coverage. one hour post administration. Repeated use of
10. Aspiration of blood or hematoma formation following DDAVP may result in tachyphylaxis. DDAVP used to
injection requires factor coverage and local control treat hemorrhagic disorders may also be associated
with ice packs. with water retention, hyponatremia and rarely
11. Block anesthesia requires 30 to 50 percent factor seizures. DDAVP may also be used to achieve
levels. hemostasis in type I von Willebrand’s disease. Type
12. Mulkey, 1976, advocated peridental injection to avoid I von Willebrand’s disease is a quantitative deficiency
requirement of replacement therapy.13 of the von Willebrand factor with all multimers present.
386 Essentials of Pediatric Oral Pathology

32. Orthodontic therapy usually does not present a ETIOPATHOGENESIS

problem. In normal hematopoiesis, myeloid stem cells give rise to
33. Drugs like aspirin, meperidine, phenacetin, oxycodone,
erythrocytes, platelets, granulocytes, eosinophils, basophils
ibuprofen, naproxen sodium are contraindicated.
and monocytes. The production of each lineage is a function
34. Acetaminophen, propoxyphene, pentazocine may be
prescribed for pain.
of cell proliferation, differentiation and apoptosis. These
35. Patients with joint replacement may require antibiotic various stages of differentiation rely on multiple interrelated
prophylaxis. processes. Protein growth factors, known as cytokines,
36. Patient with an inhibitor presents a tremendous stimulate proliferation of the multilineage cells (e.g.
challenge. Inhibitors are antibodies that may develop interleukin [IL]-3, granulocyte-macrophage colony-
in approximately 28 percent of patients with severe stimulating activity [GM-CSF]). Other factors primarily
factor VIII deficiency and in 35 percent of patients with stimulate the growth of committed progenitors (e.g. GM-CSF,
severe factor IX deficiency. Patients with low macr ophage colon y-stimulating factor [M-CSF],
responding inhibitors may continue to be treated with erythropoietin [Epo]).
factor concentrate, whereas those with high Erythropoiesis (Fig. 14.19) is a carefully ordered sequence
responding inhibitors require the use of bypassing of events.
products (either PCC or activated PCC). Hemophilics Initially occurring in fetal hepatocytes, the process is taken
with inhibitors pose a challenging treatment problem over by bone marrow in the child and adult. Although multiple
and should be managed in conjunction with a regional cytokines and growth factors are dedicated to the proliferation
hemophilia comprehensive center, since hemostasis
of the RBC, the primary regulator is Epo. Red cell development
may be difficult to achieve.
is initially regulated by stem cell factor (SCF), which commits
37. Scientists are working on developing a gene therapy
for people with hemophilia. Hemophilia is considered
hematopoietic stem cells to develop into erythroid progenitors.
a good test for gene therapy because it is caused by Subsequently, Epo continues to stimulate the development and
only one defective gene. This could be a way to terminal differentiation of these progenitors. In the fetus, Epo
change the way someone’s body works so that the is produced by monocytes and macrophages found in the liver.
body can produce the missing clotting factors on its After birth, Epo is produced in the kidneys; however, Epo
own. messenger RNA (mRNA) and Epo protein are also found in
the brain and in RBCs, suggesting that some paracrine and
POLYCYTHEMIA autocrine function is present as well.
Erythropoiesis escalates as increased expression of the EPO
Polycythemia vera, also known as “erythremia”, is a blood gene produces higher levels of circulating Epo. EPO gene
disorder in which the bone marrow makes too many red blood expression is known to be affected by multiple factors,
cells. Polycythemia vera may also result in the overproduction including hypoxemia, transition metals (Co2+, Ni2+, Mn2+) and
of white blood cells and platelets. Most of the health concerns iron chelators. However, the major influence is hypoxia,
associated with polycythemia vera are caused by a blood- including factors of decreased oxygen tension, RBC loss and
thickening effect that results from an overproduction of red increased oxygen affinity of hemoglobin. In fact, Epo
blood cells.

TYPES
Relative polycythemia (pseudoerythrocytosis) is secondary to
fluid loss or decreased fluid intake resulting in hemoconcen-
tration. Two basic categories of polycythemia are recognized:
• Primary polycythemias due to factors intrinsic to red cell
precursors, including primary familial and congenital
polycythemia (PFCP), idiopathic erythrocytosis and
polycythemia vera.
• Secondary polycythemias are caused by factors extrinsic
to red cell precursors and include a physiologic-appropriate
erythropoietin (Epo) production in response to tissue
hypoxia and physiologic-inappropriate erythropoietin
production not in response to tissue hypoxia. FIGURE 14.19: Bone marrow film demonstrating erythropoiesis
Hematological Disorders in Children 387

production has been observed to increase as much as

1000-fold in severe hypoxia.
Primary polycythemia is due to factors intrinsic to red cell
precursors caused by acquired and inherited mutations. It
includes the diagnoses of primary familial and congenital
polycythemia, idiopathic erythrocytosis and polycythemia
vera.
Currently, the diagnosis of polycythemia vera is based on
the 2008 World Health Organization (WHO) criteria, which
has integrated molecular diagnostics into the evaluation and
screening for polycythemia vera.17 A diagnosis of polycythemia
vera is made when both major and one minor criterion are
present or when the first major criterion is present with any
two minor criteria. The current criteria include the following:
FIGURE 14.20: Polycythemia vera showing atypical large mega-
Major Criteria karyocytes with abnormal nuclear lobulation and eosinophilic
nucleoli
1. Hemoglobin level of more than 18.5 g/dL in men (> 16.5
g/dL in women) or other evidence of increased red cell
volume.
Or
2. Hemoglobin or hematocrit level higher than 99th percentile
of method-specific reference range for age, sex, altitude,
of residence.
Or
3. Hemoglobin level of more than 17 g/dL in men (> 15 g/dL
in women) if associated with a documented and sustained
increase of at least 2 g/dL from an individual’s baseline value
that cannot be attributed to correction of iron deficiency.
Or
4. Elevated red cell mass greater than 25 percent above mean
normal predicted value (Fig. 14.20).
Or
5. Presence of JAK2V617F or similar mutation (e.g. JAK2 exon FIGURE 14.21: Bone marrow biopsy in polycythemia vera showing
12 mutation). increased cellularity with predominantly erythroid hyperplasia

Minor Criteria Familial clustering suggests a genetic predisposition.

1. Bone marrow trilineage myeloproliferation. Whether these mutations are responsible for the development
2. Subnormal serum Epo levels. of polycythemia vera in pediatric patients is unclear. Some
3. Endogenous erythroid colony growth (Fig. 14.21). groups have reported lower rates of JAK2 mutations in children
Until recently, the pathophysiology of polycythemia vera was compared with adults, whereas other groups have seen similar
unclear. In 2005, significant progress in the understanding of rates with complete or near complete presence of JAK2V617F
polycythemia vera was made with the discovery of a gain of and other JAK2 mutations. The prevalence of familial cases of
function mutation in the tyrosine kinase JAK2 (JAK2V617F ), chronic myeloproliferative disease is thought to be at least
which now appears to cause most primary cases in 7.6 percent, and the pattern of inheritance is consistent with
adults. JAK2V617F is detectable in more than 95 percent of an autosomal dominant pattern with decreased penetrance.
patients diagnosed with polycythemia vera. Several other Evidence of disease anticipation is noted in the second
mutations of JAK2 have since been described (e.g. exon 12, generation, presenting at a significantly younger age. However,
JAK2H538-K539delinsI ). The JAK2 mutations cause the enzyme to clinical and hematological features in familial cases have not
be constitutively active allowing cytokine independent been shown to differ from those of sporadic mutations.
proliferation of cell lines that express Epo receptors causing these Secondary polycythemia may result from functional
cells to be hypersensitive to cytokines.18 hypoxia induced by lung disease, heart disease, increased
388 Essentials of Pediatric Oral Pathology

altitude (hemoglobin increase of 4 percent for each 1000 m as 10 to 15 years with phlebotomy alone. Blood count
increase in altitude), congenital methemoglobinemia and other 6 to 7 million/cu mm.
high-oxygen affinity hemoglobinopathies stimulating increased • In the neonatal period, polycythemia-induced hyper-
Epo production. Secondary polycythemia may also result from viscosity can lead to altered blood flow and subsequently
increased Epo production secondary to benign and malignant affect organ function. Infants with polycythemia are at
Epo-secreting lesions. increased risk for necrotizing enterocolitis, renal
High altitude erythrocytosis is evident within the first week dysfunction, hypoglycemia and increased pulmonary
of high-altitude exposure. A sharp increase in Epo production vascular resistance with resultant hypoxia and cyanosis.
is noticeable, with associated mobilization of iron stores with Although initially thought to cause neurologic dysfunction,
evidence of iron-deficient erythropoiesis. Abnormal high- the decrease in cerebral blood flow seen in newborns with
affinity hemoglobin mutations characterized by left shift in the polycythemia is a physiologic response and does not appear
oxygen-hemoglobin dissociation curves lead to erythrocytosis. to cause cerebral ischemia.
Secondary polycythemia of the newborn is fairly common
and is seen in 1 to 5 percent of all newborns in the United Management
States. It results from either chronic or acute fetal hypoxia or
1. Primary polycythemia: The goals of therapy are to
delayed cord clamping and stripping of the umbilical cord.
maximize survival while minimizing the complications
Aberrant erythropoietin production is seen with various renal, of therapy as well as of the disease itself.
liver, CNS disorders and leads to physiologically inappropriate • Phlebotomy and myelosuppressive chemotherapy
secondary polycythemia. Renal disorders frequently associated are the cornerstones of therapy and have
with polycythemia include renal cell carcinoma, Wilms tumor, produced a median survival time of 9 to 14 years
polycystic kidneys, and renal transplantation. Erythrocytosis after the beginning of treatment.
has also been documented in patients with hepatocellular • The goal of phlebotomy is to maintain normal red
carcinoma. cell mass and blood volume, with a target
hematocrit of less than 45 percent for men and
CLINICAL FEATURES less than 42 percent for women. The mean
survival time of adult patients treated solely with
• Primary polycythemia is rare; the overall prevalence of phlebotomy is 13.9 years; however, a high risk of
polycythemia vera is 22 cases per 100,000 people. thromboembolic complications is observed.
• Only 0.1 percent of cases of polycythemia vera are • In the past, the use of anticoagulants, including
observed in individuals younger than 20 years. antiplatelet drugs such as aspirin and
• The male-to-female ratio is 1.2 to 2.2:1 in adults and 1:1 dipyridamole (Persantine), was associated with an
in children. increased risk of bleeding without an associated
• Symptoms of headache, vertigo, insomnia, weakness or decrease in thrombotic events; therefore,
malaise, pruritus (especially after exposure to warm water), anticoagulants have not previously been
bruising, ruddy or red appearance, erythromelalgia (burning recommended. However, a large European study,
pain, warmth and redness of extremities), diaphoresis/ results of which were published in the New
dyspnea, visual disturbance, ringing in the ears, pares- England Journal of Medicine by Landolfi et al,19
thesias, arthropathies, weight loss, fullness of the abdomen, showed a decrease in thrombotic events in those
thirst, abdominal discomfort, constipation may be seen. patients receiving low-dose aspirin therapy and
recommended aspirin therapy for those patients
• Signs of rubor, especially facial rubor and sparing of the
for whom no contraindications were noted.
trunk, skin plethora, hypertension, both systolic and
• Hydroxyurea as a myelosuppressive agent is also
diastolic, hepatomegaly, splenomegaly (usually feels hard widely used in high-risk patients with polycythemia
and smooth and occurs in more than two-thirds of patients), vera (i.e. > 60 y, history of thrombosis) who
conjunctival plethora (engorged vessels in the bulbar require cytoreductive therapy, reducing the need
conjunctiva), ecchymosis and cardiac hypertrophy (rarely for phlebotomy. However, similarly to those
observed) may be seen. treated with chlorambucil, these patients also
• The complications found in polycythemia vera are related experience higher rates of malignancy.
to two primary factors. The first includes complications • Interferon alpha is effective in eliminating
related to hyperviscosity. The second involves bone JAK2V617F expression and inducing hematologic
marrow–related complications. Untreated, the median remission. Its use is limited by side effects, cost
survival time for these patients is 18 months. However, if and route of administration. The pegylated form
patients are treated, survival is greatly extended, as many and low dose treatment has decreased the rate
Hematological Disorders in Children 389

of discontinuation of the drug secondary to side • Hydroxyurea (Hydrea): Inhibitor of deoxynucleotide

effects. synthesis. PO antineoplastic agent used in CML,
• In the past, patients have been treated with melanoma, ovarian carcinoma and some head and
chlorambucil and busulfan. However, these neck carcinomas. Pediatric dose: 20 to 30 mg/kg/
patients exhibited the highest rates of secondary d PO (same as in adults).
malignancy including acute leukemia, lymphocytic • Busulfan (Myleran): Potent cytotoxic drug that,
lymphomas and skin and GI carcinomas. The at recommended dosage, causes profound
rates of malignancy appear lower with busulfan mye losu ppre ssio n. A s al kyla ting age nt,
than with the other alkylating agents. Currently, mechanism of action of active metabolites may
these agents are rarely used. inv olve cro ss-l inkin g of DNA , wh ich may
• Patients treated with phosphorus-32 (32P) tolerate interfere with growth of normal and neoplastic
treatment well and have prolonged periods of cells. Pediatric dose: 0.06 to 0.12 mg/kg/d or
remission. However, these patients also exhibit 1.8 to 4.6 mg/m 2/d PO; titrate dose to maintain
increased rates of acute leukemias (10-15%). The WBC > 40,000/mL; reduce dose by 50 percent
mean survival time with 32P treatment is 10.9 if WBC is 30,000 to 40,000/mL; discontinue if
years; therefore, phosphorous is rarely used. WBC < 20,000/mL.
• Current recommendations for treatment of young
patients primarily rely on phlebotomy because the LEUKOCYTOSIS
thrombosis is far less likely to occur in children
and the long-term risks of leukemia over a longer
Leukocytosis is an abnormal increase in the number of
life span are increased. circulating white blood cells. This condition may occur as a
2. Secondary polycythemia: Phlebotomy is used for result of the reaction of the body to any pathologic situation.
symptomatic hyperviscosity. The goal is to treat the It may be caused by an increase in (1) neutrophil count (i.e.
underlying cause of polycythemia. neutrophilia), (2) lymphocyte count (i.e. lymphocytosis),
3. Surgery is not typically indicated. Occasionally, (3) monocyte count (i.e. monocytosis), (4) eosinophilic
splenectomy is performed late in the course of the granulocyte count (i.e. eosinophilia), (5) basophilic granulocyte
disease if massive splenomegaly causes adverse count (i.e. basophilia), or (6) immature cells (e.g. blasts). A
effects such as early satiety, anemia, or combination of any of the above may be involved.
thrombocytopenia from sequestration.
4. Please note that these patients have a high risk of
NEUTROPHILIA (FIG. 14.22)
complications during surgical procedures.
5. Consult a neurologist and neurosurgeon if evidence Neutrophils are phagocytic granulocytes that constitute an
of a stroke is present. important component of the rapid “non-specific” immune
6. Diet is unrestricted. defences. They, like other leucocytes, are derived from
7. Contact sports and other activities should be limited pluripotent stem cell progenitors in the bone marrow.
for individuals in hypercoagulable and hypocoagul-
able states.
8. Current recommendations for treatment of young
patients with polycythemia primarily rely on
phlebotomy.
9. Antineoplastic agents: The following medications are
not approved for pediatric polycythemia but are
extrapolated from other pediatric treatment regimens,
including leukemia and myelodysplastic syndrome.
• Interferon alfa 2a and 2b (Roferon-A [alfa-2a],
Intron A [alfa-2b]): A recombinant purified protein
used IV for CML, hairy cell leukemia and Kaposi
sarcoma. Inhibits cellular growth and alters cell
differentiation. Pediatric dose: 2.5 to 5 million
U/d IM/SC.
• Chlorambucil (Leukeran): Antineoplastic alkylating
agent of nitrogen mustard type used for CLL, giant
follicular lymphoma, Hodgkin lymphoma and
FIGURE 14.22: Increased number of neutrophils
lymphosarcoma. Pediatric dose is not established.
in neutrophilia
390 Essentials of Pediatric Oral Pathology

Upon stimulation by colony stimulating factors, including • Inflammation

stem cell factor, granulocyte-monocyte colony stimulating factor • Acute hypersensitivity
(GMCSF) and granulocyte colony stimulating factor • Ulcerative colitis
(G-CSF), phagocyte precursors proliferate and develop in the • Crohn’s disease.
bone marrow to form mature segmented neutrophils. Increase
in the number of circulating neutrophils is termed as neutrophilia. MONOCYTOSIS (FIG. 14.24)
Neutrophilia is divided into four categories based on the
Monocytosis is defined as a monocyte count that exceeds the
mechanism of neutrophilia: (1) increased production,
(2) decreased egress from vascular space (demargination), (3) upper limit of the reference range of 0.95 × 199/L (950/µL).
Monocytosis is commonly caused by the following conditions:
increased mobilization from the marrow storage pool and
1. Certain bacterial infections, e.g. tuberculosis, SABE,
(4) reduced margination into the tissue.
syphilis.
ETIOLOGY 2. Viral infection.
3. Protozoal and rickettsial infections, e.g. malaria, typhus,
• Infection (bacterial, fungal) kala-azar.
• Trauma (surgery, burns) 4. Convalescence from acute infection.
• Malignancies (Hodgkin’s lymphoma, juvenile myelomono- 5. Hematopoietic disorders, e.g. monocytic leukemia,
cytic leukemia) lymphomas, myeloproliferative disorders, multiple
myeloma, lipid storage disease.
6. Malignancies, e.g. cancer of stomach, ovary, breast.
EOSINOPHILIA (FIG. 14.23) 7. Granulomatous diseases, e.g. sarcoidosis, inflammatory
An increase in absolute eosinophil count greater than 0.5 × 109/ bowel diseases.
L (500/µL) is generally considered eosinophilia. The following 8. Collagen vascular diseases.
are common causes of eosinophilia:
• Allergy BASOPHILIA
• Hay fever, asthma, eczema A basophil count that exceeds 0.10 to 0.15 × 109/L (100–
• Infection, Parasitic 150/µL) that leads to leukocytosis is rare.
• Skin disorders
• Connective tissue disorders
AGRANULOCYTOSIS
• Polyarteritis nodosa
• Malignancy solid tumor, lymphoma Total lack of granulocytes in the blood is termed as
• Myeloproliferative disease agranulocytosis. These children suffer frequent infections from
• Chronic myeloid leukemia bacteria which in the past led to death in three-quarters of cases

FIGURE 14.23: Increased number of granular eosinophils seen FIGURE 14.24: Increased number of monocytes seen in
in eosinophilia monocytosis
Hematological Disorders in Children 391

before three years of age. This disease is also known as severe susceptible to bacterial infections that can become life-threatening.
congenital neutropenia (SCN). SCN was first clearly described The clinical symptoms of cyclic neutropenia are same as
by Kostmann in 1956.20 It is now known to be caused by a neutropenia only the disorder occurs in a cyclic manner. The cycles
defect in a gene on chromosome 1 (in 1p35-p34.3) that codes of decrease in neutrophilic count occurs over a period of 21 days.
for what is called the granulocyte colony-stimulating factor The next few days, neutrophil count remains normal and then again
receptor (GCSFR). The inheritance of the disease is autosomal the cycle of decrease in neutrophil count is repeated.
recessive.
Children with SCN have no special problems with viral or ETIOLOGY
fungal infections. They do, however, have an increased risk of
Among children, neutropenia has a number of causes,
developing acute myelogenous leukemia or myelodysplasia, a
including:
bone marrow disorder. Aside from agranulocytosis, the bone
• Inadequate bone marrow production due to other blood
marrow and blood show a number of other abnormalities
disorders such as aplastic anemia or cancer such as leukemia.
(including maturational arrest of neutrophil precursors at the
• Response to radiation therapy or chemotherapy, which
promyelocyte stage, absolute monocytosis, eosinophilia and
destroys white cells. When this occurs, it could delay
thrombocytosis). The gamma globulin level in blood is low.
radiation or chemotherapy.
Other causes include cyclic neutropenia, irradiation by
• Inadequate white cells because of an autoimmune disease.
gamma rays, chemicals containing benzene or anthracene
• Bacterial infections, such as tuberculosis, or viral infections
nuclei.
like mononucleosis.
CLINICAL FEATURES
CLINICAL FEATURES
• Fever, chills, lymphadenopathy, weakness are common
• Fever
signs and symptoms.
• Shaking chills
• Oral mucosal ulcers and periodontal disease – most
• Sore throat
common,
• Cough or shortness of breath
• Ulcers resemble large deep scarring ulcers in major
• Nasal congestion
aphthous stomatitis • Diarrhea or loose bowels
• Periodontal manifestations range from marginal gingivitis • Burning during urination
to rapidly advancing bone loss. • Unusual redness, swelling or warmth at the site of an injury
• Ulceration of oral mucosa – most common (Fig. 14.25).
Management
• Lack surrounding inflammation and are characterized by
1. Treatment with recombinant human granulocyte necrosis
colony-stimulating factor (GCSF) elevates the granulo- • Oral ulcers, advanced periodontal disease, pericoronitis and
cyte counts, helps resolve preexisting infections and pulpal infection—potentially life threatening—can lead to
diminishes the number of new infections and results bacteremia and septicemia
in significant improvements in survival and quality of
life. Some patients have developed leukemia or
myelodysplastic syndrome following treatment with
GCSF.
2. Congenital neutropenia is due to diverse causes. Not
all patients with congenital neutropenia have
mutations in the GCSFR gene.
3. Alternative names for severe congenital neutropenia
(SCN) include: Kostmann’s disease or syndrome,
infantile genetic agranulocytosis and genetic infantile
agranulocytosis.

NEUTROPENIA
Neutropenia is a rare disorder that causes children to have lower
than normal levels of neutrophils, a type of white blood cell that
destroys bacteria in the blood. Neutropenia can be a very serious FIGURE 14.25: Ulcer on lateral border of tongue seen in
condition because without enough neutrophils, a child is neutropenia
392 Essentials of Pediatric Oral Pathology

• Peripheral blood smear reveals reduced number or absence LYMPHOCYTOSIS

of neutrophils (Fig. 14.26).
Lymphocytosis conventionally refers to a lymphocyte count
Management
greater than 4 × 109/L (4000/µL); however, a lymphocyte count
that exceeds this is physiologically present in infants and young
1. Treatment with granulocyte colony stimulating factors children. The upper normal limit of lymphocyte count in this
(G-CSFs) is the main option of treatment with severe age group has not been well defined in a healthy population.
congenital neutropenia. • Marked lymphocytosis is observed in individuals infected
2. Corticosteroids may help if the neutropenia is caused
with pertussis.
by an autoimmune reaction. Removing an enlarged
• Viral infection generally causes lymphocytosis (relative or
spleen may cure the neutropenia involved with
hypersplenism. absolute) with or without neutropenia. Typical examples
3. Bone marrow (or stem cell) transplantation may be include infectious mononucleosis or cytomegalo
recommended to treat certain serious causes of virus infection, respiratory syncytial virus infections, and
neutropenia, such as Kostmann syndrome, aplastic infectious hepatitis.
anemia or leukemia. • Chronic lymphocytic leukemia is extremely rare in children
and is usually not considered in the differential diagnosis of
CLINICAL IMPORTANCE OF WBC COUNT lymphocytosis.
ANC Significance
LEUKEMIA
> 1500 Normal
500–1000 Patient at some risk for infection; defer Leukemia is the progressive overproduction of white blood
elective procedures that could induce cells, which usually appear in the circulation in an immature
significant transient bacteremia form. The first published description of a case of leukemia in
200–500 Patient must be admitted to hospital if medical literature dates to 1827, when French physician Alfred-
febrile and given broad spectrum
Armand-Louis-Marie Velpeau described a 63-year-old florist
antibiotics; defer all elective dental
procedures who developed an illness characterized by fever, weakness,
< 200 At significant risk for sepsis urinary stones and substantial enlargement of the liver and
spleen. Velpeau noted that the blood of this patient had a
ANC = (% of neutrophils + % of bands) × total white count/100
consistency “like gruel” and speculated that the appearance of
the blood was due to white corpuscles.21 In 1845, a series of
LYMPHOCYTES
patients who died with enlarged spleens and changes in the
• Majority of lymphoid neoplasms are of B cell origin. “colors and consistencies of their blood” was reported by the
— T cell tumor—CD2, CD3, CD4, CD7 and CD8 surface Edinburgh-based pathologist JH Bennett; he used the term
markers used. “leucocythemia” to describe this pathological condition.22
— B cell tumor—CD10, CD19, CD20 and surface markers The term “leukemia” was coined by Rudolf Virchow, the
are used. renowned German pathologist, in 1856. As a pioneer in the
use of the light microscope in pathology, Virchow was the first
to describe the abnormal excess of white blood cells in patients
with the clinical syndrome described by Velpeau and Bennett.
As Virchow was uncertain of the cause of the white blood cell
excess, he used the purely descriptive term “leukemia” (Greek:
“white blood”) to refer to the condition.23 Wilhelm Ebstein
introduced the term “acute leukemia” in 1889 to differentiate
rapidly progressive and fatal leukemias from the more indolent
chronic leukemias.24 Finally, in 1900 the myeloblast, which is
the malignant cell in AML, was characterized by Naegeli, who
divided the leukemias into myeloid and lymphocytic.25,26

TYPES OF LEUKEMIA
• Myeloid Leukemia—Involving granulocyte series
• Lymphoid Leukemia—Involving lymphocyte series
FIGURE 14.26: Absence of neutrophils seen in neutropenia • Monocytic Leukemia—Involving monocyte series.
Hematological Disorders in Children 393

CLASSIFICATION TABLE 14.1: French-American-British classification27

1. Acute myeloblastic leukemia (AML) Lymphoblastic (ALL)
2. Acute lymphoblastic leukemia (ALL)
L1 Small monomorphic, high N:C ratio (Scores 0, 1, or
3. Chronic myeloid leukemia (CML) 2)
4. Chronic lymphocytic leukemia (CLL). L2 Large, heterogeneous, nucleolated, low N:C ratio
(Scores-1, 2, or 3)
ETIOLOGY L3 Burkitt-cell type, basophilic, vacuolated

1. Genetic factors—Identical twins. Congenital disorders— Myeloid (AML)

Down’s, Bloom’s, Klinefelters’s syndromes and Fanconi’s M0 Undifferentiated Myeloblastic (requires cell markers)
anemia M1 Myeloblastic without maturation (requires cytochemistry:
2. Environmental factors peroxidase or Sudan black B)
a. Ionizing radiation related to CML, AML and ALL but M2 Myeloblastic with maturation
M3 Hypergranular promyelocytic
not CLL. M3-variant Micro- or hypogranular bilobed promyelocytes
b. Chemical carcinogens—Benzene–AML. M4 Myelomonocytic with both granulocytic and monocytic
c. Certain drugs—Alkylating agents–AML. differentiation
3. Infection—HTLV-I and HTLV-II. M5 Monoblastic (M5a requires cytochemistry:Alpha-
naphthyl acetate esterase or Alpha-naphthyl butyrate
esterase) and promonocytic-monocytic (M5b)
PATHOGENESIS M6 Erythroleukemia, with > 50% erythroblasts and < 30%
• Malignant transformation of a single clone of cells of or > 30% blasts
M7 Megakaryoblastic (requires cell markers)
myeloid or lymphoid series, followed by proliferation of
the transferred clone.
• In acute leukemia—Defect in maturation process of c. Infection of mouth, throat, skin, respiratory, perianal
myeloid or lymphoid series. sites.
• In leukemic transformation, the basic defect lies in DNA. d. Fever attributed to infections but sometimes idiopathic.
• Most consistent chromosomal abnormality is Philadelphia 2. Due to organ infiltration:
(Ph) chromosome. a. Pain and tenderness of bones
• Leukemic cells accumulate in bone marrow and suppress b. Lymphadenopathy and enlargement of tonsils
normal hematopoietic stem cells. c. Splenomegaly
d. Hepatomegaly
ACUTE LEUKEMIAS e. Leukemic infiltration of the kidney
See Table 14.1. f. Gum hypertrophy
g. Meningeal involvement
ACUTE MYELOBLASTIC LEUKEMIA h. Chloroma or granulocytic sarcoma
i. CNS manifestations.
Acute myeloid leukemia (AML), also known as acute Acute myeloid leukemia (ALL), peripheral blood of a child,
myelogenous leukemia, is a cancer of the myeloid line of blood Pappenheim stain shows angel-wing—lobated nucleus and auer
cells, characterized by the rapid growth of abnormal white rods (Figs 14.27 and 14.28).
blood cells that accumulate in the bone marrow and interfere
with the production of normal blood cells. AML is the most
common acute leukemia affecting adults, and its incidence ACUTE LYMPHOBLASTIC LEUKEMIA
increases with age. Hence this entity does not warrant a detailed Acute lymphoblastic leukemia (ALL) is a form of leukemia,
discussion here. or cancer of the white blood cells characterized by excess
lymphoblasts. Malignant, immature white blood cells
CLINICAL FEATURES continuously multiply and are overproduced in the bone
1. Due to bone marrow failure: marrow. ALL causes damage and death by crowding out
a. Anemia producing pallor, lethargy, dyspnea. normal cells in the bone marrow and by spreading
b. Bleeding manifestations due to thrombocytopenia (metastasizing) to other organs. It is the most common
causing spontaneous bruises, petechiae, bleeding from malignancy diagnosed in children, representing nearly one-third
gums. of all pediatric cancers.
394 Essentials of Pediatric Oral Pathology

Aldrich syndrome, congenital hypogammaglobulinemia, ataxia-

telangiectasia) that have an increased predisposition to develop
lymphoid malignancies.
In acute lymphoblastic leukemia, a lymphoid progenitor cell
becomes genetically altered and subsequently undergoes
dysregulated proliferation, survival and clonal expansion. In most
cases, the pathophysiology of transformed lymphoid cells reflects
the altered expression of genes whose products contribute to the
normal development of B-cells and T-cells. Several studies
indicate that leukemic stem cells are present in certain types of
acute lymphoblastic leukemia. Another one-third of the ALL
population exhibit one of four chromosomal translocations in
the absence of increases in chromosomal number which can
predict outcome. Three of the four translocations: t(1;19), mixed-
lineage leukemia (MLL) translocations and the t(9;22)
FIGURE 14.27: Acute myelogenous leukemia showing Philadelphia chromosome are associated with a poor prognosis
angel-wing–lobated nucleus (arrow) while the most common translocation, t (12;21), predicts a
favorable response.28

CLASSIFICATION
WHO proposed classification of acute lymphoblastic
leukemia. 28 The recent WHO International panel on ALL
recommends that the FAB classification be abandoned, since
the morphological classification has no clinical or prognostic
relevance. It instead advocates the use of the immunopheno-
typic classification mentioned below.
1. Acute lymphoblastic leukemia/lymphoma. Synonyms:
Former FAB L1/L2
• Precursor B acute lymphoblastic leukemia/lymphoma.
Cytogenetic subtypes:
— t (12;21)(p12,q22) TEL/AML-1
— t (1;19)(q23;p13) PBX/E2A
FIGURE 14.28: Acute myelogenous leukemia showing auer
— t (9;22)(q34;q11) ABL/BCR
rods (arrow) — T (V,11)(V;q23) V/MLL.
• Precursor T acute lymphoblastic leukemia/lymphoma.
ETIOPATHOGENESIS 2. Burkitt’s leukemia/lymphoma. Synonyms:Former FAB L3.
3. Biphenotypic acute leukemia.
Although a few cases are associated with inherited genetic
syndromes (i.e. Down syndrome, Bloom syndrome, Fanconi
CLINICAL FEATURES
anemia), the cause remains largely unknown. Many
environmental factors (i.e. exposure to ionizing radiation and • The annual incidence rate for acute lymphoblastic leukemia
electromagnetic fields, parental use of alcohol and tobacco) is 30.9 cases per million populations.
have been investigated as potential risk factors, but none has • The peak incidence occurs in children aged two to five years.
been definitively shown to cause acute lymphoblastic leukemia. • Acute lymphoblastic leukemia occurs slightly more
Various viruses may be linked to the development of leukemia, frequently in boys than in girls. This difference is most
particularly when prenatal viral exposure occurs in mothers pronounced for T-cell acute lymphoblastic leukemia.
recently infected with influenza or varicella. However, no direct • Children with acute lymphoblastic leukemia (ALL)
link has been established between viral exposure and the generally present with signs and symptoms that reflect bone
development of leukemia. marrow infiltration and extramedullary disease. Because
Acute lymphoblastic leukemia may also occur in children leukemic blasts replace the bone marrow, patients present
with various congenital immunodeficiencies (i.e. Wiskott- with signs of bone marrow failure, including anemia,
Hematological Disorders in Children 395

thrombocytopenia and neutropenia. They also have signs

of infection because of neutropenia.
• Clinical manifestations include fatigue and pallor, petechiae
and bleeding and fever. In addition, leukemic spread may
manifest as lymphadenopathy and hepatosplenomegaly.
• Other signs and symptoms of leukemia include weight loss,
bone pain and dyspnea.
• Signs and symptoms of CNS involvement, even when it
occurs, are rarely observed at the time of initial diagnosis.
The signs and symptoms include headache, nausea and
vomiting, lethargy, irritability, nuchal rigidity and
papilledema.
• Cranial nerve involvement, which most frequently involves
the seventh, third, fourth and sixth cranial nerves, may occur.
• Also, leukemia can present as an intracranial or spinal mass,
which causes numerous neurologic symptoms, most of
which are due to nerve compression. FIGURE 14.29: Acute lymphoblastic leukemia showing round,
• Testicular involvement at diagnosis is rare. However, if slightly oval and indented nuclei with light staining and evenly
present, it appears as painless testicular enlargement and dispersed nuclear chromatin
is most often unilateral.
• Careful neurologic examination to look for CNS involve- Management
ment is important because the treatment for leukemia with 1. With improvements in diagnosis and treatment, overall
CNS involvement is different. cure rates for children with acute lymphoblastic leukemia
• In male patients, testicular examination is necessary to look now approach 80 percent. Further refinements in
for testicular involvement of leukemia. therapy, including the use of risk-adapted treatment
• Gingival bleeding. protocols, may improve cure rates for patients at high
• Gingival hypertrophy. risk while limiting the toxicity of therapy for patients with
• Pain and numbness in the mandible. a low risk of relapse.
• Candidiasis, ANUG , herpes simplex infections. 2. Because leukemia is a systemic disease, therapy is
• Little effect of radiation treatment may be seen. primarily based on chemotherapy. Different forms of
• Effects of long term chemotherapy on oral development acute lymphoblastic leukemia (ALL) require different
are not well substantiated. approaches for optimal results. Excluding mature B-cell
• Side effects of antineoplastic drugs may cause jaw pain and acute lymphoblastic leukemia which is treated with
numbness. short-term intensive chemotherapy, including high-dose
methotrexate (MTX), cytarabine and cyclophosphamide,
Contrasting features between AML and ALL have been
acute lymphoblastic leukemia treatment typically
described in Table 14.2.
consists of a remission-induction phase, intensification
(consolidation) phase and continuation therapy targeted
RADIOGRAPHIC FEATURES at eliminating residual disease. The addition of
• Loss of lamina dura cyclophosphamide and intensive treatment with
• Resorption of alveolar bone asparaginase is also beneficial in the treatment of
• Alterations in the periodontal space T-cell acute lymphoblastic leukemia.
3. Tumor lysis syndrome
• Destruction of cancellous bone
• Before and during the initial induction phase of
• Alterations in crypts of developing teeth.
chemotherapy, patients may develop tumor lysis
Acute lymphoblastic leukemia (ALL), peripheral blood of syndrome, which refers to the metabolic
a child stained with Pappenheim stain shows round, slightly derangements caused by the systemic and rapid
oval and indented nuclei with light staining and evenly release of intracellular contents as chemotherapy
dispersed nuclear chromatin (Fig. 14.29). destroys leukemic blasts.
• Primary features of tumor lysis syndrome include
CHRONIC LEUKEMIAS hyperuricemia (due to metabolism of purines),
hyperphosphatemia, hypocalcemia and hyper-
These are generally not seen in the pediatric age group, hence kalemia. The hyperuricemia can lead to crystal
are out of the scope of this textbook (Table 14.2).
396 Essentials of Pediatric Oral Pathology

TABLE 14.2: Contrasting features of AML and ALL

Sr. No. Feature AML ALL

1. Common age Adults between 15–40 years; comprise Children under 15 years; comprise 80% of childhood
20% of childhood leukemias leukemias

2. Physical findings Splenomegaly + Splenomegaly +

Hepatomegaly + Hepatomegaly +
Lymphadenopathy + Lymphadenopathy +
Bony tenderness + Bony tenderness +
Gum hypertrophy + Meningeal involvement

3. Laboratory findings Low to high TLC; predominance of Low to high TLC; predominance of lymphoblasts in
myeloblasts and promyelocytes in blood and bone marrow; thrombocytopenia moderate
blood and bone marrow; thrombo- to severe
cytopenia moderate to severe

4. Cytochemical stains Cytosine arabinoside, anthracyclines PAS +, acid phosphatase (focal) +

(daunorubicin, adriamycin) and
6-thioguanine
Myeloperoxidase, lysozyme, CD68

5. Specific therapy Cytosine arabinoside, anthracyclines Vincristine, prednisolone, anthracyclines and

and 6-thioguanine L-asparginase

6. Response to therapy Remission rate low, duration of Remission rate high, duration of remission longer
remission shorter

7. Median survival 12–18 months Children without CNS prophylaxis 33 months, with
CNS prophylaxis 60 months; adults 12–18 months

formation with tubular obstruction and possibly, lymphoblastic leukemia, has 5 components:
acute renal failure requiring dialysis. Therefore, induction, consolidation, interim maintenance,
electrolyte and uric acid levels should be closely delayed intensification and maintenance. The goal
monitored throughout initial therapy. of induction is to achieve remission or less than
• To prevent complications of tumor lysis syndrome, 5 percent blasts in the bone marrow. Induction
patients should initially receive intravenous (IV) therapy generally consists of 3 to 4 drugs, which
fluids at twice the maintenance rates, usually without may include a glucocorticoid, vincristine,
potassium. asparaginase and possibly an anthracycline. This
— Sodium bicarbonate is added to the IV fluid to type of therapy induces complete remission based
achieve moderate alkalinization of the urine (pH on morphology in more than 98 percent of patients.
level, 7.5–8) to enhance the excretion of • Consolidation therapy is given soon after remission
phosphate and uric acid. A urine pH level higher is achieved, to further reduce the leukemic cell
than this should be avoided to prevent burden before the emergence of drug resistance and
crystallization of hypoxanthine or calcium relapse in sanctuary sites (i.e. testes, CNS). In this
phosphate. phase of therapy, the drugs are given at doses
— The standard treatment for malignancy- higher than those used during induction or the
associated hyperuricemia also includes patient is given different drugs (i.e. high-dose MTX
allopurinol. By blocking the enzyme xanthine and 6-mercaptopurine [6-MP]), epipodophyllotoxins
oxidase, allopurinol blocks uric acid formation. with cytarabine, or multiagent combination therapy.
— Rasburicase, a recombinant urate oxidase, has • In interim maintenance, oral medications are
demonstrated increased efficacy in pediatric administered to maintain remission and allow the
patients at high risk for tumor lysis by catalyzing bone marrow to recover. This occurs for 4 weeks and
the enzymatic oxidation of uric acid to a much is followed by delayed intensification, which is aimed
more urine soluble product, allantoin. at treating any remaining resistant leukemia cells.
4. Phases of therapy • The last phase of treatment is maintenance. This
• The treatment of childhood acute lymphoblastic consists of intrathecal MTX every 3 months, monthly
leukemia, with the exception of B-cell acute vincristine, daily 6-MP and weekly MTX.
Hematological Disorders in Children 397

5. Duration of therapy • Pediatric oncologist: Refer all patients to a

• Whereas B-cell acute lymphoblastic leukemia is subspecialist to direct their care.
treated with a 2-month to 8-month course of • Pediatric surgeon: Patients require placement of a
intensive therapy, achieving acceptable cure rates central venous catheter.
for patients with B-precursor and T-cell acute • Psychosocial team: Involve psychologists and social
lymphoblastic leukemia requires approximately 2 to workers in the care of patients with acute
2.5 years of continuation therapy. Attempts to lymphoblastic leukemia to aid them and their families
reduce this time, results in high relapse rates after in navigating all of the difficult issues surrounding
therapy is stopped. their care.
• Most contemporary protocols include a continuation • Radiation oncologist: Depending on their risk group,
phase based on weekly parenterally administered some patients require craniospinal radiation as part
MTX given with daily, orally administered 6-MP of the treatment plan.
interrupted by monthly pulses of vincristine and a • Other subspecialists: Consultations with other
glucocorticoid. Although these pulses improve specialists (i.e. infectious disease specialist,
outcomes, they are associated with avascular nephrologist) may be appropriate, depending on the
necrosis of the bone. Patients with high-risk acute clinical circumstances.
lymphoblastic leukemia may also benefit from 10. Oral and dental considerations in leukemia:
intensified continuation therapy that includes the • For patient whose 1st remission has not been
rotational use of drug pairs. obtained or is in relapse, all elective dental
• Improvements in relapse-free survival gained by procedures should be deferred.
intensification with anthracyclines or epipodophyllo- • However, potential source of infection to be
toxins must be weighed against the late sequelae removed, e.g. extraction of grossly carious tooth.
of these agents, which include cardiotoxicity and • Patient with remission but undergoing chemo-
treatment-related acute myeloid leukemia.
therapy can undergo routine restorative and
6. Treatment of subclinical CNS leukemia is an essential
surgical procedures but blood profile to be taken
component of acute lymphoblastic leukemia therapy.
before procedure.
Although cranial irradiation effectively prevents overt CNS
• Patient with complete remission for two years is
relapse, concern about subsequent neurotoxicity and
treated normally.
brain tumors has led many investigators to replace
• Endodontic treatment not recommended in leukemic
irradiation with intensive intrathecal and systemic
patients.
chemotherapy for most patients. This strategy has
• Topical treatment to stop gingival bleeding includes
produced excellent survival outcomes, with CNS relapse
removal of local irritants and direct pressure.
rates of less than 2 percent in some studies.
• Absorbable gelatin or collagen sponges, topical
7. High-risk patients: Optimal treatment for patients with
thrombin or placement of microfibrillar collagen can
very high-risk acute lymphoblastic leukemia has not
been found. Some centers recommend allogenic stem- be helpful.
cell transplantation (SCT) soon after first remission is Oral rinses of antifibrinolytic agents such as
achieved. For patients without a matched family donor, tranexamic acid or -aminocaproic acid to stop
transplantation of marrow from an unrelated donor is a gingival bleeding have been reported.
reasonable treatment option. Results of SCT, often • Platelet transfusion—Last resort to stop significant
reported from single institutions, have been inconsistent gingival hemorrhage.
and sometimes disappointing. Large, multi-institutional, • Treatment of caries and other dental infections
controlled trials are clearly needed to determine the should be definitive.
effectiveness of this therapy for patients without a • Removal of local irritants and scupulous oral
matched donor. hygiene for gingivitis.
8. Molecular targeted therapy: A drug targeted at the • Supragingival scaling if patient in remission.
underlying molecular defect that is unique to certain • Saline rinses if patient not in remission.
leukemias can have potent and specific antileukemic • Prophylactic antibiotic coverage.
activity while producing minimal toxicity to normal cells. • Bacterial infections—Penicillin.
The best example of molecular targeted therapy is • Fungal infections—Nystatin.
imatinib mesylate, a selective BCR-ABL tyrosine kinase • Severe mycotic infections—Amphotericin B.
inhibitor. • Ulcers—Topical viscous lidocaine – 2 percent or
9. Numerous consultations should be obtained, depending dyclonine – 0.5 percent.
on the clinical circumstances of patients with newly • Artificial saliva products (explained in the section on
diagnosed acute lymphoblastic leukemia. xerostomia).
398 Essentials of Pediatric Oral Pathology

Guidelines for Oral and Dental Management of •Prophylactic antibiotic therapy to prevent post-
Leukemia operative infection (if severe neutropenia is present).
5. Emergency dental care
1. Detection
a. Treatment of oral ulcers:
a. History • Antibiotics
b. Examination • Bland mouth rinse
c. Screening laboratory tests: • Antihistamine solutions
• White cell count • Orabase.
• Differential white cell count b. Oral candidiasis—Treat with antifungal medication.
• Smear for cell morphologic study c. Conservative management of pain and infection
• Hemoglobin or hematocrit level • Antibiotic sensitivity testing
• Platelet count. • Antibiotics for infection
2. Referral • Strong analgesics for pain.
a. Medical diagnosis
b. Treatment. DISORDERS OF HEMOSTASIS
3. Consultation before any dental care is rendered
PLATELET DISORDERS
a. Current status
b. Review of dental treatment needs See Figure 14.30.
c. Dental management plan.
4. Routine dental care THROMBOCYTOPENIA
a. None for patient with acute symptoms Thrombocytopenia is a condition in which the blood contains
b. Once disease is under control, patient may receive a reduced number of platelets, which are necessary for blood
indicated dental care clotting. Thrombocytopenia, defined as a platelet count of less
c. Scaling and surgical procedures: than 150,000/ L, is clinically suspected when there is a history
• Bleeding time on day of procedure; if normal, of easy bruising or bleeding in a child. It may also present as
proceed; if prolonged, delay or obtain platelet an incidental finding during routine evaluation or during
replacement laboratory investigations performed for other reasons.

FIGURE 14.30: Schematic representation of classification of platelet disorders

Hematological Disorders in Children 399

Idiopathic thrombocytopenic purpura (ITP), also known as — Bruising tendency

primary immune thrombocytopenic purpura and autoimmune — Nonpalpable petechiae, which mostly occur in
thrombocytopenic purpura, is defined as isolated thrombo- dependent regions
cytopenia with normal bone marrow and the absence of other — Hemorrhagic bullae on mucous membranes
causes of thrombocytopenia. The two distinct clinical — Purpura (Fig. 14.31)
syndromes manifest as an acute condition in children and a — Gingival bleeding
chronic condition in adults. — Signs of Gastrointestinal bleeding
ITP is a decrease in the number of circulating platelets in — Menometrorrhagia, menorrhagia
the absence of toxic exposure or a disease associated with a — Retinal hemorrhages
low platelet count. — Evidence of intracranial hemorrhage, with possible
neurologic symptoms
PATHOPHYSIOLOGY — The prevalence of palpable spleen in patients with ITP
The normal platelet count for children ranges from 150,000 to is approximately the same as that in the non-ITP
450,000/ L. In general, the risk of bleeding does not increase population (i.e. 3% in adults, 12% in children)
until the platelet count falls significantly below 1, 00,000/ L. — Spontaneous bleeding when platelet count is less than
For example, surgical bleeding solely due to a decreased 20,000/mm3.
platelet count typically does not occur until the platelet count Blood picture in thrombocytopenia shows low platelet
is less than 50,000/ L; whereas, spontaneous bleeding does count (Fig. 14.32). Thrombocytopenia may occur commonly
not occur until the platelet count is less than 20,000/ L. in children as the bone marrow contains only one day storage
ITP is primarily a disease of increased peripheral platelet of platelets.
destruction, with most patients having antibodies to specific
platelet membrane glycoproteins. Relative marrow failure may Management
contribute to this condition, since studies show that most 1. Life-threatening bleeding requires conventional critical
patients have either normal or diminished platelet production. care interventions.
Acute ITP often follows an acute infection and has a 2. In a patient with known ITP, high-dose parenteral
spontaneous resolution within two months. Chronic ITP persists glucocorticoids and IV immunoglobulin (IVIg), with or
longer than six months without a specific cause. without platelet transfusions, are appropriate.
3. Platelet transfusion is indicated for controlling severe
CLINICAL FEATURES hemorrhage. Send a blood specimen to the lab for type
and screen in case platelet transfusion is necessary.
• Hemorrhage represents the most serious complication; 4. Platelet survival is increased if the platelets are
intracranial hemorrhage is the most significant. The transfused immediately after IVIg infusion.
mortality rate from hemorrhage is approximately one 5. A consultation with a hematologist may be required to
percent in children and five percent in adults. make a decision regarding the transfusion of platelets.
• Spontaneous remission occurs in more than 80 percent of
cases in children but is uncommon in adults.
• In acute ITP (children), distribution is equal between males
(52%) and females (48%).
• Peak prevalence occurs in children aged two to four years.
Approximately 40 percent of all patients are younger than
10 years.
• History of bleeding, other illnesses, medications like
heparin, quinidine/quinine, sulfonamides may be present.
• Common signs, symptoms and precipitating factors include
the following:
— Abrupt onset (childhood ITP)
— Gradual onset (adult ITP)
— Menorrhagia
— Epistaxis
— Recent live virus immunization (childhood ITP) FIGURE 14.31: Thrombocytopenia showing purpuric rashes on
— Recent viral illness (childhood ITP) whole body of an infant
400 Essentials of Pediatric Oral Pathology

they show promise for raising platelet counts, there are

potential safety concerns such as thrombocytosis and
rebound thrombocytopenia.
14. Dental considerations:
a. Post extraction antifibrinolytic agents should be
administered (e.g. to tranexamic acid, 25 mg/kg
t.i.d.) for three to five days.
b. Local measures to limit and control hemorrhage
should be employed.
c. Patient should be kept on a soft diet for seven days.

LYMPHOMA
Cancer can basically be divided into leukemia (mainly in blood
and bone marrow) and solid tumors, where one or more lumps
are the likely origin of disease. Lymphomas are solid tumors
which can involve many different parts of the body and can
FIGURE 14.32: Thrombocytopenia showing low platelet count
present at different ages. Some lymphomas resemble leukemias
in their pattern of spread and are treated with leukemia-type
6. Guidelines for transfusion dosage: treatment protocols. Because the various types of lymphomas
a. 6 to 8 U of platelet concentrate, or 1 U/10 kg. behave in different ways, it is best to consider them under
b. 1 U of platelets to increase count of a 70-kg adult by individual groupings.
5-10,000/mm3 and an 18-kg child by 20,000/mm3. A number of different classification systems exist for
7. Splenectomy is reserved for patients in whom medical lymphoma. In the mid 1990s, the Revised European-American
therapy fails. Emergent splenectomy is indicated in
Lymphoma (REAL) Classification attempted to apply
patients with life-threatening bleeding in whom medical
therapy fails.
immunophenotypic and genetic features in identifying distinct
8. Glucocorticoids and IVIg are the mainstays of medical clinicopathologic NHL entities.29
therapy. Indications for use, dosage and route of
administration are based on the patient’s clinical NEW WHO CLASSIFICATION OF LYMPHOID
condition, the absolute platelet count and the degree of
NEOPLASMS (2008) 30
symptoms. Consultation with a hematologist may be
needed prior to starting therapy. Precursor Lymphoid Neoplasms
9. Children who have platelet counts > 30,000/mm3 and
are asymptomatic or have only minor purpura do not
• B lymphoblastic leukemia/lymphoma NOS
require routine treatment. Children who have platelet • B lymphoblastic leukemia/lymphoma with recurrent
counts < 20,000/mm3 and significant mucous membrane genetic abnormalities
bleeding and those who have platelet counts < 10,000/ • B lymphoblastic leukemia/lymphoma with t(9;22); bcr-abl1
mm 3 and minor purpura should receive specific • B lymphoblastic leukemia/lymphoma with t(v;11q23);
treatment. MLL rearranged
10. Steroid use, usage of immunosuppressive drugs and • B lymphoblastic leukemia/lymphoma with t(12:21); TEL-
splenectomy may be undesirable because of their AML1 and ETV6-RUNX1
associated complications. • B lymphoblastic leukemia/lymphoma with hyperploidy
11. According to one study , using a combination of weekly • B lymphoblastic leukemia/lymphoma with hypodiploidy
vincristine, weekly methylprednisolone, both until
• B lymphoblastic leukemia/lymphoma with t(5;14); IL3-
platelet counts reached 50,000/mm3 and cyclosporine
orally twice daily until the platelet count is normal for
IGH
3 to 6 months seems promising. • B lymphoblastic leukemia/lymphoma with t(1;19); E2A-
12. Other therapies, such as cyclophosphamide, danazol, PBX1 and TCF3-PBX1
dapsone, interferon alfa, azathioprine, vinca alkaloids, • T lymphoblastic leukemia/lymphoma.
accessory splenectomy and splenic radiation have been
studied. Mature B-Cell Neoplasms
13. Clinical trials have shown promise for agents that
directly stimulate platelet production, such as • Chronic lymphocytic leukemia/small lymphocytic
thrombopoietin (TPO) receptor-binding agents. While lymphoma
Hematological Disorders in Children 401

• B-cell prolymphocytic leukemia • Peripheral T-cell lymphoma, NOS

• Splenic marginal zone lymphoma • Angioimmunoblastic T-cell lymphoma
• Hairy cell leukemia • Anaplastic large cell lymphoma, ALK+ type
• Lymphoplasmacytic lymphoma/Waldenstrom macro- • Anaplastic large cell lymphoma, ALK– type.
globulinemia
• Heavy chain disease Hodgkin Lymphoma (Hodgkin Disease)
• Plasma cell myeloma Nodular lymphocyte-predominant Hodgkin lymphomas
• Solitary plasmacytoma of bone • Classic Hodgkin lymphomas
• Extraosseous plasmacytoma • Nodular sclerosis Hodgkin lymphoma
• Extranodal marginal zone B-cell lymphoma of mucosa- • Lymphocyte-rich classic Hodgkin lymphoma
associated lymphoid tissue (MALT) type • Mixed cellularity Hodgkin lymphoma
• Nodal marginal zone lymphoma • Lymphocyte depletion Hodgkin lymphoma.
• Follicular lymphoma
• Primary cutaneous follicular lymphoma Post-Transplant Lymphoproliferative Disorders (PTLD)
• Mantle cell lymphoma
• Diffuse large B-cell lymphoma, NOS • Plasmacytic hyperplasia
• (T-cell/histiocyte-rich type; primary CNS type; primary leg • Infectious mononucleosis like PTLD
skin type and EBV+ elderly type) • Polymorphic PTLD
• Diffuse large B-cell lymphoma with chronic inflammation • Monomorphic PTLD (B and T/NK cell types)
• Lymphomatoid granulomatosis • Classic HD type PTLD.
• Primary mediastinal large B-cell lymphoma
• Intravascular large B-cell lymphoma Histiocytic and Dendritic Cell Neoplasms
• ALK+ large B-cell lymphoma • Histiocytic sarcoma
• Plasmablastic lymphoma • Langerhans cell histiocytosis
• Large B-cell lymphoma associated with HHV8+ • Langerhans cell sarcoma
Castleman disease • Interdigitating dendritic cell sarcoma
• Primary effusion lymphoma • Follicular dendritic cell sarcoma
• Burkitt lymphoma • Fibroblastic reticular cell tumor
• B-cell lymphoma, unclassifiable, Burkitt-like • Indeterminate dendritic cell sarcoma
• B-cell lymphoma, unclassifiable, Hodgkin lymphoma-like. • Disseminated juvenile xanthogranuloma.

Mature T-Cell and NK-Cell Neoplasms HODGKIN’S DISEASE

• T-cell prolymphocytic leukemia Hodgkin’s lymphoma causes the cells in the lymphatic system
• T-cell large granular lymphocytic leukemia to abnormally reproduce, eventually making the body less able
• Chronic lymphoproliferative disorder of NK-cells. to fight infection and cause swelling in the lymph nodes.
• Aggressive NK-cell leukemia Thomas Hodgkin published the first description of lymphoma
• Systemic EBV+ T-cell lymphoproliferative disorder of in 1832, specifically of the form named after him; Hodgkin’s
childhood lymphoma.31 Hodgkin’s lymphoma cells can also spread to
• Hydroa vacciniforme-like lymphoma other organs and tissue and cause metastasis.
• Adult T-cell lymphoma/leukemia
• Extranodal T-cell/NK-cell lymphoma, nasal type ETIOLOGY
• Enteropathy-associated T-cell lymphoma • The specific cause of Hodgkin’s lymphoma is unknown.
• Hepato-splenic T-cell lymphoma • It is possible that a genetic predisposition and exposure to
• Subcutaneous panniculitis-like T-cell lymphoma viral infections may increase the risk for developing
• Mycosis fungoides Hodgkin’s lymphoma.
• Sézary syndrome • There is a slightly increased chance for Hodgkin’s
• Primary cutaneous CD30+ T-cell lymphoproliferative lymphoma to occur in siblings and cousins of patients.
disorder • There has been much investigation into the association of
• Primary cutaneous gamma-delta T-cell lymphoma the Epstein-Barr virus (EBV) which causes infection
402 Essentials of Pediatric Oral Pathology

mononucleosis; as well as of human immunodeficiency

virus (HIV), which causes acquired immune deficiency
syndrome (AIDS). Both of these infectious viruses have
been correlated with a greater incidence of children
diagnosed with Hodgkin’s lymphoma, although the direct
link is unknown.

CLINICAL FEATURES
• Hodgkin’s lymphoma accounts for about five percent of
childhood cancers
• Hodgkin’s lymphoma occurs most often in people between
the ages of 15 and 34 and in people over age 55
• The disease, for unknown reasons, affects males more than
twice as often as females
• Painless swelling of the lymph nodes in neck, underarm,
groin and chest FIGURE 14.33: Hodgkin’s lymphoma showing
Reed-Sternberg cell
• Difficulty in breathing (dyspnea) due to enlarged nodes in
the chest
• Fever iii. Mixed cellularity
• Night sweats iv. Lymphocyte depletion
• Tiring easily (fatigue) v. Unclassifiable.
• Weight loss/decreased appetite • Lesional area in case of lymphoma consists of inflammatory
• Itching skin (pruritus) cell infiltrate interspersed with large, atypical neoplastic
• Frequent viral infections (i.e. cold, flu, sinus infection). lymphoid cells. Classical Hodgkin’s lymphoma consists of
‘Reed-Sternberg’ cells that are binucleated (resembling an
STAGING OF LYMPHOMA ‘owl- eye’) or multinucleated atypical cells with prominent
nucleoli33 (Fig. 14.33)
The Ann Arbor classification is named after Ann Arbor, • Nodular lymphocyte predominant Hodgkin’s lymphoma
Michigan, where the Committee on Hodgkin’s Disease Staging shows ‘popcorn cells’ showing resemblance of nuclei to
Classification met in 1971.32 that of kernel of popped corn.
• Stage I—Usually involves a single lymph node region or • Lymphocyte rich subtype of classical Hodgkin’s lymphoma
structure. shows sheets of small lymphocytes with few ‘Reed-
• Stage II—Involves two or more lymph node regions or Sternberg’ cells.
structures on the same side of the body. • Nodular sclerosis subtype shows ‘Reed-Sternberg’ cells
• Stage III—Involves lymph node regions or structures on within the clear space and thus they are referred to as
both sides of the body and is further classified depending lacunar cells.
on the organs and areas involved. • Mixed cellularity subtype shows mixture of lymphocytes,
• Stage IV—Involves the disease in other areas (metastasis), plasma cells, eosinophils and histiocytes with abundant
in addition to the lymphatic system involvement. Reed-Sternberg cells (Fig. 14.34).
• Stages are also noted by the presence or absence of • Lymphocyte depletion subtype as the name suggests shows
symptoms of the disease: bizarre Reed-Sternberg cells with less or reduced number
— Asymptomatic (A) of lymphocytes.
— Symptomatic (B). • When lymphomas do not fit in any of the above subtypes,
they are termed as unclassifiable.
HISTOPATHOLOGIC FEATURES
DIAGNOSIS
• It is comprised of two main forms:
1. Nodular lymphocyte predominant Hodgkin’s lymphoma Lymphoma is diagnosed by:
2. Classical Hodgkin’s lymphoma • Blood and urine tests
i. Lymphocyte rich • X-rays of the chest
ii. Nodular sclerosis • Lymph node biopsy
Hematological Disorders in Children 403

ETIOLOGY
• The specific cause of non-Hodgkin’s lymphoma is unclear.
It is possible that genetics and exposure to viral infections
may increase the risk for developing this malignancy.
• Non-Hodgkin’s lymphoma has also been linked to
chemotherapy and radiation therapy. Non-Hodgkin’s may
be a secondary malignancy as a result of treatment for
certain cancers.
• There has been much investigation into the association of
the Epstein-Barr virus (EBV) that causes the mononucleosis
infection; as well as the human immunodeficiency virus
(HIV), which causes acquired immune deficiency syndrome
(AIDS). Both of these infectious viruses have been linked
to the development of Burkitt’s lymphoma.
FIGURE 14.34: Hodgkin’s lymphoma of mixed cellular variety • The majority of Burkitt’s lymphoma cases result from a
chromosomal rearrangement between chromosome 8 and
• Computed tomography scan of the abdomen, chest and 14, which causes genes to change positions and function
pelvis differently, promoting uncontrolled cell growth. Other
• Lymphangiogram (LAG) chromosomal rearrangements have been seen in non-
• Bone marrow biopsy/aspiration. Hodgkin’s lymphoma (all types) that are also thought to
promote excessive cell growth.
Management • Children and adults with other hereditary abnormalities
have an increased risk of developing non-Hodgkin
1. Chemotherapy is the mainstay of treatment which can
lymphoma, including patients with ataxia telangiectasia,
often lead to remission.
2. Patients who have limited disease (stages 1 and 2) X-linked lymphoproliferative disease, or Wiskott-Aldrich
are managed by local radiation therapy alone. syndrome.
3. Recent treatment trends however, combine less The Working Formulation is a classification of non-
extensive radiotherapy fields with milder multiagent Hodgkin lymphomas published in 1982.34 It has since been
chemotherapy regimens. replaced by other lymphoma classifications but is still used by
4. Patients with stage 3 or 4 disease require chemo- cancer agencies for compilation of lymphoma statistics.
therapy; radiation therapy is used conjointly if
significant mediastinal involvement or residual
GRADES
disease is detected.
5. Widely used regimen to treat Hodgkin’s lymphoma is Low grade
known as MOPP (Mechlorethamine, Oncovin, • Malignant Lymphoma, small lymphocytic (chronic
Procarbazine, Prednisone). Due to long term side lymphocytic leukemia)
effects of this regimen, another regimen known as
ABVD (Adriamycin, Bleomycin, Vinblastine, DTIC) is
• Malignant Lymphoma, follicular, predominantly small
often used. cleaved cell
6. Surgery. • Malignant Lymphoma, follicular, mixed (small cleaved and
7. Bone marrow transplant. large cell).
8. Supportive care (for pain, fever, infection and nausea
Intermediate grade
or vomiting) is required.
9. Continued follow-up care (to determine response to
• Malignant Lymphoma, follicular, predominantly large cell
treatment, detect recurrent disease and manage side • Malignant Lymphoma, diffuse, small cleaved cell
effects of treatment) is essential. • Malignant Lymphoma, diffuse, mixed small and large cell
• Malignant Lymphoma, diffuse, large cell.
NON-HODGKIN’S LYMPHOMA High grade
Non-Hodgkin’s lymphoma is a cancer of the lymphatic system • Malignant lymphoma, large cell, immunoblastic
in which the cells in the lymphatic system reproduce abnormally, • Malignant lymphoma, lymphoblastic
eventually causing tumors to grow. Non-Hodgkin’s disease cells • Malignant lymphoma, small non-cleaved cells (Burkitt’s
can also spread to other organs and tissues in the body. lymphoma).
404 Essentials of Pediatric Oral Pathology

Miscellaneous the cases, involves the T-cells and usually presents with a
• Composite mass in the chest, swollen lymph node(s), with or without
• Mycosis fungoides bone marrow and central nervous system involvement.
• Histiocytic • Burkitt’s or non-Burkitt’s lymphoma: Burkitt’s or non-
• Extramedullary plasmacytoma Burkitt’s lymphoma is a non-Hodgkin’s disease in which
• Unclassifiable. the cells are undifferentiated and diffuse. This has also been
referred to as small non-cleaved cells. Burkitt’s and non-
CLINICAL FEATURES Burkitt’s lymphoma accounts for about 40 to 50 percent
of the cases and is usually characterized by a large
• Lymphomas are the third most common childhood abdominal tumor and may have bone marrow and central
cancer. They occur most often in children between the ages nervous system involvement.
of 7 and 11, but can occur at any age from infancy to • Large cell or diffuse histiocytic non-Hodgkin’s lymphoma:
adulthood. Large cell or diffuse histiocytic non-Hodgkin’s lymphoma
• Non-Hodgkin’s lymphoma affects males more often than involves the B-cells and T-cells and accounts for about 25
females and is more common among Caucasian children percent of the cases. Children with this type of non-
than among African-American children and children of Hodgkin’s lymphoma usually have lymphatic system
other races. involvement, as well as involvement of a non-lymph
• Some children with non-Hodgkin’s lymphoma have structure (i.e. lung, jaw, brain, skin and bone).
symptoms of an abdominal mass and have complaints of • Large cell anaplastic lymphoma: Large cell anaplastic
abdominal pain, fever, constipation and decreased appetite lymphoma is a less common type of lymphoma in children.
due to the pressure and obstruction a large tumor in this Treatment for this type is the same as for large cell
area can cause. lymphoma.
• Some children with non-Hodgkin lymphoma have
symptoms of a mass in their chest and have complaints of Staging of non-Hodgkin’s Lymphoma32
respiratory problems, pain with deep breaths (dyspnea), • Stage I—Involves the tumor at one site, either nodal or
cough and/or wheezing. elsewhere in the body.
• Because of the rapid onset of this malignancy, any • Stage II—Involves the tumor at two or more sites on the
respiratory symptoms can quickly worsen, causing a life- same side of the body.
threatening emergency. • Stage III—Involves tumors in any number that occur on
• Other symptoms include: both sides of the body, but does not involve bone marrow
— Painless swelling of the lymph nodes in neck, chest, or the central nervous system.
abdomen, underarm, or groin • Stage IV—Any stage of tumor that also has bone marrow
— Fever and/or central nervous system involvement. Stage IV is also
— Sore throat subdivided depending on the amount of blasts (cancer cells)
present in the bone marrow.
— Fullness in groin area from node involvement
— Bone and joint pain
HISTOPATHOLOGIC FEATURES (Fig. 14.35)
— Night sweats
— Tiring easily (fatigue) • Non-Hodgkin’s lymphomas are classified as low grade,
— Weight loss/decreased appetite intermediate grade and high grade histopathologically.
— Itching of the skin • Low grade lesions show well-differentiated small
— Recurring infections. lymphocytes.
• High grade lesions show less differentiated cells.
CLASSIFICATION • The histologic pattern may be nodular in which large
clusters of cells are seen or diffuse in which cells are
Staging and classification of non-Hodgkin’s lymphoma is based monotonous with no evidence of germinal center formation.
on the extent of the disease and the specific cells involved. • Two cell types are seen in nodular type: Centrocytes that
Non-hodgkin’s lymphoma in children is almost always one of are small, irregular with cleaved nuclei and scant cytoplasm
three types: and centroblasts that are large cells with open nuclear
chromatin, several nucleoli and moderate amount of
• Lymphoblastic non-Hodgkin’s lymphoma: Lymphoblastic cytoplasm. The cells in the diffuse form have open vesicular
non-Hodgkin’s lymphoma accounts for about 30 percent of nuclei but sometimes cleaved nucleus may be present.
Hematological Disorders in Children 405

accompanied by proptosis. He noted that some children had

huge abdominal masses, sometimes accompanied by disease
in the facial bones, although there was usually no lymph node
involvement. This malignancy, initially thought to be a
sarcoma35-37 and later established to be a lymphoma and given
the name Burkitt’s lymphoma, was the most common tumor in
children in that area. This lymphoma was found to occur
throughout tropical Africa except at high altitudes or in areas
where the climate was relatively cool. Occurrence was greater
in areas with greater rainfall. In 1961, Burkitt made the
acquaintance of Epstein, an experimental pathologist and shared
samples of the lymphoma with him. Within these lymphomas,
Epstein and colleagues identified the virus that has come to be
known as Epstein-Barr virus (EBV); this was the first
description of a virus involved in the pathogenesis of a tumor
FIGURE 14.35: Non-Hodgkin’s lymphoma showing scant in humans. In the setting of the florid reactive lymphoid
cytoplasm and irregular shaped nuclei of lymphocytes
hyperplasia that occurs in response to malaria, it was proposed
that EBV could be oncogenic.36
DIAGNOSIS
Diagnosis of non-Hodgkin’s lymphoma is made by: Classification According to Clinical Features
• Blood and urine tests
• X-rays of the chest In the World Health Organization (WHO) Classification, three
• Computed tomography scan of the abdomen, chest and pelvis clinical variants of Burkitt’s lymphoma are described:
• Positron emission tomography (PET) scan 1. Endemic
• Lymph node biopsy 2. Sporadic
• Lymphangiogram 3. Immunodeficiency-associated types.
• Bone marrow aspiration and/or biopsy • Endemic Burkitt’s lymphoma refers to those cases occurring
• Lumbar puncture. in African children, usually four to seven years old, with a
male: female ratio of 2:1, involving the bones of the jaw
Management and other facial bones, as well as kidneys, gastrointestinal
tract, ovaries, breast and other extranodal sites.38
Treatment may include (alone or in combination):
1. Chemotherapy
2. Radiation therapy
3. Surgery
4. Close monitoring of blood work
5. Bone marrow transplant
6. Bone marrow examinations
7. Lumbar punctures/spinal taps
8. Antibiotics (to prevent or treat infections)
9. Supportive care (for side effects of treatment)
10. Long-term follow-up care (to determine response to
treatment, detect recurrent disease, and manage late
effects of treatment).

BURKITT’S LYMPHOMA (FIG. 14.36)

HISTORY
In the middle of the last century, when Denis Burkitt, a surgeon,
was working in central Africa in Kampala, he noted children
with grossly distorted faces, with lesions involving one or both FIGURE 14.36: Burkitt’s lymphoma showing swelling
sides of the face and upper and lower jaws, sometimes in the right mandibular area
406 Essentials of Pediatric Oral Pathology

• Sporadic Burkitt’s lymphoma occurs worldwide; it includes lymphoma occurring in transplant recipients tends to occur
those cases occurring with no specific geographic or after a relatively long interval post-transplant (mean, 4.5 years
climatic association. It accounts for 1 to 2 percent of in one series). Most patients are solid organ recipients, but
lymphoma in adults and up to 40 percent of lymphoma in recipients of stem cells are rarely affected as well. EBV is
children in the U.S. and Western Europe.39 The abdomen, commonly but not uniformly present.
especially the ileocecal area, is the most common site of With respect to morphology, the WHO Classification
involvement; the ovaries, kidneys, omentum, Waldeyer’s describes classic Burkitt’s lymphoma and two variants: Burkitt’s
ring, and other sites may also be involved. Bilateral lymphoma with plasmacytoid differentiation and atypical
involvement of the breasts may occur in association with Burkitt’s/Burkitt-like lymphoma.38
the onset of puberty or with lactation. Lymph node • Classic Burkitt’s lymphoma is found in cases of endemic
involvement is more common among adults than among Burkitt’s lymphoma and most cases of sporadic Burkitt’s
children. Patients may also have malignant pleural effusions lymphoma affecting children but in only a minority of
or ascites. Rarely, patients present with disease that is sporadic and immunodeficiency-associated adult cases.
primarily leukemic (classified as acute lymphoblastic Neoplastic cells are uniform and medium-sized (their nuclei
leukemia [ALL], L3 type in the French-American-British are no larger than the nuclei of admixed histiocytes), with
[FAB] Classification). Neoplastic cells are EBV positive in round nuclei and several or multiple small basophilic
15 to 30 percent of cases, or fewer in some series.40 nucleoli. Cytoplasm is moderately abundant and with
• Immunodeficiency-associated Burkitt’s lymphoma occurs formalin fixation there may be slight cytoplasmic retraction,
mainly in patients infected with HIV but also occurs in leading to squared-off edges between neighboring cells. The
allograft recipients and individuals with congenital RNA-rich cytoplasm is deep blue on Giemsa or Wright stain
immunodeficiency. In the early years of the AIDS epidemic, and usually shows multiple vacuoles because of the presence
several cases of Burkitt’s lymphoma were described in of lipid, when marrow aspirates or Wright-stained touch preps
homosexual men;41,42 these were the first descriptions of non- are available. The mitotic rate is unusually high.
Hodgkin’s lymphoma arising in association with what was Characteristically there are numerous admixed tangible body
later recognized as HIV infection. Information accumulated macrophages, phagocytosing abundant apoptotic debris,
since that time indicates that Burkitt’s lymphoma accounts creating a ‘starry-sky’ pattern. Many cases of sporadic
for 30 to 40 percent of non-Hodgkin’s lymphoma in HIV Burkitt’s lymphoma occurring in adults have Burkitt-like
morphology. Those with plasmacytoid differentiation tend
positive patients. In a study performed before widespread
to occur in association with immunodeficiency.
use of highly active antiretroviral therapy (HAART),
• Burkitt-like lymphoma and Burkitt’s lymphoma with
Burkitt’s lymphoma was estimated to be 1,000 times more
plasmacytoid differentiation, both tend to have greater
common in HIV positive individuals than in the general
nuclear pleomorphism than classic Burkitt’s lymphoma and
population. Compared with other HIV positive patients with
both tend to have a smaller number of more prominent
non-Hodgkin’s lymphoma of the diffuse large B-cell type,
nucleoli. The plasmacytoid variant has, in addition,
those with Burkitt’s lymphoma are younger, less often carry
monotypic cytoplasmic immunoglobulin. Burkitt’s
a prior diagnosis of AIDS and have higher mean CD4 counts lymphoma, regardless of subtype, typically expresses
(usually > 200 cells/µL). The diagnosis of Burkitt’s monotypic surface IgM, pan-B-cell antigens, including
lymphoma in an HIV positive individual often represents the CD19, CD20, CD22 and CD79a and coexpresses CD10, Bcl-
first AIDS-defining criterion.43,44 HIV-associated Burkitt’s 6, CD43 and p53, but not CD5, CD23, Bcl-2, CD138, or
lymphoma shares some pathogenetic features with endemic TdT. The proliferation fraction is very nearly 100 percent;
Burkitt’s lymphoma. HIV infection, analogous to malaria, accordingly, the doubling time of the tumor is very short,
leads to polyclonal B-cell activation and permits poorly between 24 and 48 hours. Rare cases have been reported that
controlled proliferation of EBV positive B-cells. The genetic lack surface immunoglobulin,8 including some occurring in
instability of the EBV+/–, aberrantly regulated B-cells leads allograft recipients. The immunophenotype suggests follicle
to a greater risk of c-myc rearrangement and then to center origin for this lymphoma.
lymphoma. HIV is not directly involved in lymphomagenesis A defining feature of Burkitt’s lymphoma is the presence
but is indirectly involved via cytokine deregulation, chronic of a translocation between the c-myc gene and the IgH
antigenic stimulation and decreased immune surveillance. gene (found in 80% of cases [t(8;14)]) or between c-myc and
Lymphoma often involves lymph nodes, bone marrow and the gene for either the kappa or lambda light chain (IgL) in
extranodal sites, most often in the abdomen. Burkitt’s the remaining 20 percent [t(2;8) or t(8;22), respectively]. The
Hematological Disorders in Children 407

myc/Ig translocation may not be detected by routine

cytogenetics; performing fluorescence in situ hybridization
(FISH) or long-segment polymerase chain reaction may
increase the chance of identifying the translocation. In
endemic Burkitt’s lymphoma, the break point in c-myc is
more than 100 kb upstream from the first coding exon and
the break point in the IgH gene is in the joining segment. In
sporadic and HIV-associated Burkitt’s lymphoma, the break
point in myc is between exon 1 and 2 and the break point in
IgH is in the switch (Sµ) region, suggesting a different
pathogenesis and suggesting that neoplastic transformation
affects B-cells at different maturational stages for these
subtypes of Burkitt’s lymphoma. It should be noted however,
that results have not been uniform; in a study of sporadic
Burkitt’s lymphoma, most presenting as leukemia, one-third FIGURE 14.37: Burkitt’s lymphoma showing ‘starry sky’
appearance
of cases had chromosomal break points in the joining region
of IgH.40
disease have a > 90 percent 5-year survival rate and
children with widespread disease (including leukemic
STAGING
presentation) may achieve a > 90 percent complete
Staging is performed using the Ann Arbor or, more often, the response (CR) rate, with an event-free survival (EFS)
St Jude/Murphy staging system.45 Approximately 30 percent rate at 4 years of 65 percent in patients with leukemic
of patients present with limited-stage disease (I or II), while presentation, and 79 percent for those presenting with
70 percent present with widespread disease (stage III or IV). stage IV lymphoma reported in one series.46
3. The institution of the CODOX-M/IVAC regimen
Patients often present with bulky disease, frequently with an
(Magrath protocol)—two cycles of CODOX-M
elevated lactate dehydrogenase (LDH) level. Patients with any
(cyclophosphamide, vincristine, doxorubicin, high-
of the three clinical variants are at risk for spread to the central dose metho-trexate and intrathecal therapy)
nervous system (CNS) and bone marrow. The bone marrow is alternating with IVAC (ifosfamide, etoposide, high-
positive in 30 to 38 percent, and the CNS is involved in dose cytarabine and intrathecal therapy)—for high-risk
13 to 17 percent of adult cases. In a study of children with disease and for those with low-risk disease (e.g. one
disseminated disease, 12 percent had CNS involvement and extranodal site or completely resected abdominal
22 percent had marrow involvement.46 disease with normal LDH), three cycles of CODOX-
M, represented a major step forward in the treatment
HISTOPATHOLOGIC FEATURES of Burkitt’s lymphoma. Children and adults treated with
this regimen had similar outcomes; the EFS rate at 2
• Histopathologic features represent an undifferentiated, years was 92 percent for the group as a whole. 47
small, non-cleaved B-cell lymphoma. However, there were significant associated toxicities,
• The cells appear homogeneous with round nuclei and including frequent myelosuppression, mucositis,
prominent nucleoli and presence of abnormal mitosis. neuropathy and some treatment-related deaths. In
• A characteristic ‘starry sky’ appearance is seen due to addition, because of the rapid, effective tumor cell
presence of macrophages within the tumor with abundant killing with this aggressive protocol, careful monitoring
clear cytoplasm (Fig. 14.37). is required to avoid the complication of tumor lysis
syndrome.
4. The Dana-Farber Cancer Institute has treated patients
Management
with a modified Magrath protocol, aimed at decreasing
1. Sporadic and immunodeficiency-associated Burkitt’s toxicity while maintaining good outcome. In this
lymphoma do not share endemic Burkitt’s lymphoma’s modification, the schedule of fractionated
exquisite sensitivity to chemotherapy, so that cyclophosphamide was altered and the vincristine
historically the prognosis had been poor, particularly dose was capped, but the dose of doxorubicin was
among adults. escalated.
2. Short-duration, high-intensity chemotherapy, 5. Rituximab, a monoclonal anti-CD20 antibody, may
sometimes combined with CNS prophylaxis, yields improve outcome; a study of a small series from the
excellent survival in children: patients with localized M.D. Anderson Cancer Center used rituximab in
408 Essentials of Pediatric Oral Pathology

conjunction with hyper-CVAD (hyperfractionated 13. Mulkey TF. Outpatient treatment of hemophiliacs for dental
cyclophosphamide, vincristine, doxorubicin extractions. J Oral Surg 1976;34:428-34.
and dexamethasone), with CNS prophylaxis and 14. Stoneman DW, Deierl CD. Pseudotumor of hemophilia in the
achieved a complete remission rate of 89 percent. mandible. Oral Surg 1975;40:811.
6. Novel therapies that have not been tested but could 15. Evans BE. Dental treatment for hemophiliacs: evaluation of
be useful include those targeted against the c-myc dental program (1975-1976) at the Mount Sinai Hospital
gene, DNA methyltransferase inhibitors, proteasome International Hemophilia Training Center. Mt Sinai J Med 1977;
inhibitors, cyclin-dependent kinase inhibitors and 44:409-37.
others. 16. Powell D, Bartle J. The hemophiliac: Prevention is the key, Dent
7. Among pediatric patients, a poorer prognosis is Hyg(Chic) 1974;48:214-9.
associated with age over 15 years. 48 A good 17. Spivak JL, Silver RT. The revised World Health Organization
prognosis is associated with resectable abdominal diagnostic criteria for polycythemia vera, essential
disease. Failure to achieve a clinical remission is a thrombocytosis, and primary myelofibrosis: An alternative
very poor prognostic sign. proposal. Blood 2008;112(2):231-9.
8. HIV positive patients may be treated with intensive 18. Jones AV, Kreil S, Zoi K, et al. Widespread occurrence of the
therapy but require especially close observation, with JAK2 V617F mutation in chronic myeloproliferative disorders.
transfusion support and antibiotic therapy as needed. Blood 2005;106:2162-8.
19. Raffaele Landolfi, Roberto Marchioli, Jack Kutti, Heinz
Gisslinger, Gianni Tognoni, Carlo Patrono, et al. Efficacy and
REFERENCES safety of low-dose aspirin in polycythemia vera. New England
1. Wintrobe MM. Clinical Hematology. 10th edn. Williams and Journal of Medicine 2004;350(2):114-24.
Wilkins 1999;2:1862-82. 20. Kostmann R. Infantile genetic agranulocytosis (agranulocytosis
infantilis hereditaria): A new recessive lethal disease in
2. Pauling L, Itano HA, Singer SJ, Wells IC. Sickle cell anemia,
man. Acta Pediatr Scand 1956;45:1-78.
a molecular disease. Science 1949;110:543-8.
21. Hoffman, Ronald, et al. Hematology: Basic Principles and
3. Kwiatkowski DP. “How malaria has affected the human genome
Practice, 4th edn, St Louis, Mo: Elsevier Churchill Livingstone
and what human genetics can teach us about malaria”. Am J
2005. p. 1071. ISBN 0-443-06629-9.
Hum Genet 2005;77(2):171-92.
22. Bennett JH. “Two cases of hypertrophy of the spleen and liver,
4. h t t p :/ / w w w. n h l bi. n i h . g ov/ h e alt h / dci / D ise as es/ Sc a/
in which death took place from suppuration of blood”.
SCA_Summary.html. Edinburgh Med Surg J 1845;64:413.
5. Platt OS, Brambilla DJ, Rosse WF, et al. “Mortality in sickle 23. Virchow R. “Die Leukämie”. In: Virchow R (in German).
cell disease. Life expectancy and risk factors for early death”. Gesammelte Abhandlungen zur Wissenschaftlichen Medizin.
N Engl J Med 1994;330(23):1639-44. Frankfurt: Meidinger., 1856, 190.
6. Uwakwe AA, Onwuegbuke C, Nwinuka NM. Effect of Caffeine 24. Ebstein W. “Über die acute Leukämie und Pseudoleukämie”.
on the Polymerization of HbS and Sickling Rate/Osmotic Deutsch Arch Klin Med 1889;44:343.
Fragility of HbS Erythrocytes. Journal of Applied Sciences and 25. Naegeli O. “Über rothes Knochenmark und Myeloblasten”.
Environmental Management 2002;6(1):69-72. Deutsch Med Wochenschr 1900;26:287.
7. Robinson IB, Sarnat BG. Roentgen studies of the maxilla and 26. Zhen-yi, Wang. “Ham-Wasserman Lecture: Treatment of Acute
mandible in sickle-cell anemia. Radiology 1952;58:517. Leukemia by Inducing Differentiation and Apoptosis”.
8. Mourshed F, Tuckson CR. A study of the radiographic features Hematology 2003;2003:1.
of the jaws in sickle cell anemia. Oral Surg 1974;37:812. 27. Lilleyman JS, Hann IM, Stevens RF, Eden OB, Richards SM.
9. Sanger RG and Bystrom EB. Radiographic bone changes in French American British (FAB) morphological classification of
sickle cell anemia. J Oral Med 1977;32:32. childhood lymphoblastic leukaemia and its clinical importance.
10. Charache S, Terrin ML, Moore RD, et al. “Effect of hydroxyurea J Clin Pathol 1986;39(9):998-1002.
on the frequency of painful crises in sickle cell anemia. 28. Randolph, Tim R. Advances in Acute Lymphoblastic Leukemia.
Investigators of the Multicenter Study of Hydroxyurea in Sickle Clinical Laboratory Science, Fall, 2004.
Cell Anemia”. N Engl J Med 1995;332(20):1317-22. 29. www.emedicine.com on Lymphoma, Non-Hodgkin
11. Steinberg MH, Barton F, Castro O, et al. “Effect of hydroxyurea 30. The 2008 WHO classification of lymphomas: implications for
on mortality and morbidity in adult sickle cell anemia: Risks clinical practice and translational research. Hematology 2009;
and benefits up to 9 years of treatment”. JAMA 2003;289(13): 2009(1):523-31.
1645-51. 31. Hellman Samuel, Mauch PM editors. Hodgkin’s Disease,
12. Ataga KI, Stocker J. “Senicapoc (ICA-17043): a potential Chapter 1: Lippincott Williams and Wilkins. 1999:5.
therapy for the prevention and treatment of hemolysis-associated 32. Carbone PP, Kaplan HS, Musshoff K, Smithers DW, Tubiana
complications in sickle cell anemia”. Expert Opin Investig M. “Report of the Committee on Hodgkin’s Disease Staging
Drugs 2009;18(2):231-9. Classification”. Cancer Res 1971;31(11):1860-1.
Hematological Disorders in Children 409

33. Hellman S. “Brief Consideration of Thomas Hodgkin and His 41. Doll DC, List AF. Burkitt’s lymphoma in a homosexual. Lancet
Times”. In: Hoppe RT, Mauch PT, Armitage JO, Diehl V, Weiss 1982;1:1026-7.
LM. Hodgkin Lymphoma, (2nd ed.). Philadelphia: Wolters 42. Ziegler JL, Drew WL, Miner RC, et al. Outbreak of Burkitt’s-
Kluwer Health/Lippincott Williams and Wilkins 2007, 3-6. like lymphoma in homosexual men. Lancet 1982;2:
34. The non-Hodgkin’s lymphoma pathological classification 631-3.
project. National Cancer Institute sponsored study of 43. Knowles DM. Etiology and pathogenesis of AIDS-related non-
classification of non-Hodgkin’s lymphomas: Summary and Hodgkin’s lymphoma. Hematol Oncol Clin North Am
description of working formulation for clinical usage. Cancer 2003;17:785-820.
1982;49:2112-35. 44. Martinez-Maza O, Breen EC. B-cell activation and lymphoma
35. Burkitt D. A sarcoma involving the jaws in African children. in patients with HIV. Curr Opin Oncol 2002;14:528-32.
Br J Surg 1958;46:218-23. 45. Murphy SB, Hustu HO. A randomized trial of combined
36. Burkitt DP. The discovery of Burkitt’s lymphoma. Cancer modality therapy of childhood non-Hodgkin’s lymphoma.
1983;51:1777-86. Cancer 1980;45:630-7
37. Burkitt D, Wright D. Burkitt’s lymphoma, 1st edn. Edinburgh 46. Cairo MS, Sposto R, Perkins SL, et al. Burkitt’s and Burkitt-
and London: E and S Livingstone, 1970;1-251. like lymphoma in children and adolescents: A review of the
38. Diebold J. Burkitt lymphoma. In: Jaffe E, Harris N, Stein H, Children’s Cancer Group experience. Br J Haematol
et al, editors. Pathology and Genetics of Tumours of 2003;120:660-70.
Haematopoietic and Lymphoid Tissues. Washington, DC: IARC 47. Bowman WP, Shuster JJ, Cook B, et al. Improved survival for
Press 2001;181-4. children with B-cell acute lymphoblastic leukemia and stage
39. Blum KA, Lozanski G, Byrd JC. Adult Burkitt leukemia and IV small non-cleaved-cell lymphoma: A Pediatric Oncology
lymphoma. Blood 2004;104:3009-20. Group study. J Clin Oncol 1996;14:1252-61.
40. Burmeister T, Schwartz S, Horst HA, et al. Molecular 48. Patte C, Auperin A, Michon J, et al. The Societe Francaise
heterogeneity of sporadic adult Burkitt-type leukemia/lymphoma d’Oncologie Pediatrique LMB89 protocol: Highly effective
as revealed by PCR and cytogenetics: Correlation with multiagent chemotherapy tailored to the tumor burden and initial
morphology, immunology and clinical features. Leukemia response in 561 unselected children with B-cell lymphomas and
2005;19:1391-8. L3 leukemia. Blood 2001;97:3370-9.
15
410 Essentials of Pediatric Oral Pathology

Forensic Odontology
in Children
Shweta Dixit Chaudhary, Mayur Chaudhary, Syed Ahmed Taqi

CHAPTER OVERVIEW
History Bite mark analysis
Terminologies Child abuse
Forensic odontology Bite marks
Role of the pedodontist in forensic odontology Forensic anthropology
Oral autopsy protocol Development of the human dentition by Schour and Massler
Scientific methods of identification Sex determination of skeletal remains
Technologies for age determination The fetal skeleton
Saliva: an identification tool Assessment of skeletal remains
Palatal rugae pattern Chronology of dental development and age assessment
Lip prints and their use for identification Age estimation in prenatal, neonatal and early postnatal child
Reconstruction of the facial tissue Review of the various development surveys
DNA identification Forensic photography
Importance of blood group determination Role of forensic dentistry in mass disasters
Dental tissues and their role in forensic science Conclusion

HISTORY been buried on the battlefield by their teeth and dental work.
Revere was able to identify Dr. Joseph Warren, the man who
The history of Forensic Odontology dates back around 4500 sent him on his famous ride, because he had made him a partial
years. One of the first dental identification recorded was in denture out of silver wire and pieces of hippo tusk.
2500 BC when two molars linked together by gold wire were The first truly celebrated American dental identification took
found by Junker in a tomb located at Gizu. place in Boston in 1850. The identity of murdered physician,
In 49 BC, Agrippina ordered the death of her rival Lollia Dr George Parkman, was established through dental evidence.
Paulina, who was in competition with her to be the wife of The murderer, Dr. John Webster, a Harvard professor, had
Emperor Claudius. Agrippina demanded to see Lollia Paulina’s attempted to dispose of the body by incineration in his laboratory
head as proof of her death, but she wasn’t sure that her rival furnace. However, a partial denture and a portion of jaw were
was dead until she noticed Lollia Paulina’s distinctive recovered from a privy where they had been disposed of after
discolored front teeth. burning. Although one dental expert testified that in his opinion
A new era in Forensic Odontology began in the 17th century it was unlikely that a dentist would remember the dentistry from
when a body was identified from the dental details of a previous patients, the jury believed Dr. Nathan Cooley Keep who
deceased personally known to the dentist. demonstrated how the partial denture and recovered bone
In Medieval England, teeth were supposedly used to seal fragments fit a cast of Dr. Parkman’s jaw and jury was convinced.
important documents. Dr. Webster was hanged.
Another famous foray into forensic dentistry was that of The first case in which a great number of victims died
Paul Revere, who in addition to being a blacksmith was also a occurred in Paris in 1897. A fire during charity bazaar resulted
dentist. He helped identify Revolutionary War dead who had in 126 fatalities. After routine methods of identification of the
Forensic Odontology in Children 411

time (visual and personal effects succeeded for less than 100 unique features present in an individual’s dental structures.
of the victims), dental identification was used. Forensic dentistry plays a major role in identification in
Dr Oscar Amoedo presented a paper about identification manmade or natural disasters – events that result in multiple
of the case in 1897, published in Dental Cosmos in 1897, and fatalities that may not be identifiable through conventional
authored a textbook first published in 1898 on the field of methods such as fingerprints (Figs 15.1 and 15.2).
Forensic Dentistry.1 Since that time, dental identification has Major fields of forensic odontology:
become an important part of any identification process when • Civil non-criminal.
multiple deaths occur. • Criminal:
A recent review of appellate decisions, presented by Judge — Identification of persons from their dentition or
Haskell Pitluck of Illinois (1989) revealed 132 American cases, teeth.
in the history of dental involvement in the field of bite mark — Dealing with bite marks identification.
analysis.2 • Research.
Battered child syndrome entered the lexicon of America Forensic odontology has three major areas of utilization:
in 1962, publicizing a problem that dates back to early 1. Diagnostic and therapeutic examination and evaluation
civilizations.3 of injuries to jaws, teeth and oral soft tissues;
German dictator Hitler and in recent times Pakistani
President General Zia-ul-Haq were identified only with the help
of dental evidence.
The Royal Mail Ship (RMS) ‘Titanic’ had a brief and
inglorious history that culminated with her striking an iceberg
and sinking on April 15, 1912, while on her maiden voyage.
The teeth were instrumental in determining the identity of an
‘Unknown Child’.
Recently, on 26th December, 2004, Tsunami caused
devastation and loss of life around Indian Ocean. 1,474
deceased have been identified. Dental comparison has been the
primary identifier in 79 percent of cases and a contributor in
another 8 percent, a total of 87 percent.

TERMINOLOGIES

FORENSIC SCIENCE FIGURE 15.1: Forensic anthropologists can help to identify skeleto-
nized human remains, such as those found lying in shrub in Western
Forensic science is defined as the study and practice of the Australia, circa 1900-1910
application of science to the purpose of law.4

FORENSIC MEDICINE
Forensic medicine is defined as the science of medicine as
related to the law.5

FORENSIC ODONTOLOGY 5,6

Forensic odontology is the branch of odontology, which deals
with the proper handling and examination of dental evidence
and with the proper evaluation, and presentation of dental
findings in the interest of justice.

FORENSIC ODONTOLOGY
Forensic odontology or forensic dentistry is one of the most
unexplored and intriguing branches of forensic sciences. It FIGURE 15.2: Searching for body fragments of badly burnt
primarily deals with identification, based on recognition of bodies at the site of a mass disaster
412 Essentials of Pediatric Oral Pathology

2. Identification of individuals, especially casualties in the organs, to determine the cause of death. Autopsy has a
criminal investigations and/or mass disasters; and systematic protocol starting with critical examination of the
3. Identification, examination, and evaluation of bite external features of the body such as gender, ethnicity, build,
marks which occur with some frequency in sexual wound, scars, tattoos and body piercing. Photographs,
assaults, child abuse cases and in personal defense radiographs, fingerprints, fingernail scrapings and hair samples
situations. may be obtained according to the requirements.
Forensic odontologists delve into: The dental autopsy is a very important part of the
• Identifying unknown human remains through dental investigative procedure, which ideally will lead to identifica-
records and assisting at the location of a mass disaster. tion. A forensic odontologist may be required to perform a
• Eliciting the ethnicity and assisting in building up a dental or oral examination on a body in one of the following
picture of lifestyle and diet of skeletal remains at categories:
archaeological sites. Normal: Everything is normal except that the subject is
• Determining the gender of unidentified individuals. dead. Rigor mortis complicates the accessibility.
• Age estimation of both the living and the deceased. Rigor mortis time frames after death are:
• Recognition and analysis of bite marks found on victims < 12 hours – Commencement of rigor mortis
of attacks and on other substances such as foodstuffs. 12 hours – Complete rigor mortis
• Presenting evidence in court as an expert witness. 18-36 hours – Beginning to disappear
Classically, forensic dentistry can be considered a 48-60 hours – No longer present.
subspeciality of oral and maxillofacial pathology. This is Decomposed: Decomposition can be classified into
analogous to the relationship in medicine between forensic primary or advanced. If the body or specimen is refrigerated,
pathology and pathology. The requirements of forensic dental the odor remains minimal. Resected specimens will deodorize
fieldwork, however, often demand an interdisciplinary if they are soaked in formalin for 30 minutes or more.
knowledge of dental science. This has resulted in other dental Mutilated: The bodies in high-impact accidents, such as
specialists and general dentists joining oral and maxillofacial air crash would fit into this category where one finds tissue
pathologists in providing legal authorities with dental expertise. and bone destruction with fragmentation.
Burned: Due to severe burning, soft tissue may get scraped
ROLE OF THE PEDODONTIST IN off the bone to provide easier access to the oral cavity and the
FORENSIC ODONTOLOGY teeth. Bones may get fragile, they can be strengthened before
A pedodontist plays an important role in forensic odontology sectioning by spraying them with artist’s clear matte finish. The
by applying his expertise in the following areas: material is completely radiolucent, so radiographs are as clear
• Mass disaster. as they would be without the coating. An alternate method is
• Child abuse and neglect. to expose the anterior dentition carefully and, using an etching
• Accidental and non-accidental oral trauma. brush, coat the fire-damaged teeth with cyanoacrylate glue.
• Dental fraud. Skeletonized: The bones are without soft tissue or only
• Age determination. remnants remain in a few areas. This is the easiest to deal with
• Bite marks. in regard to accessibility for examination, radiographs,
• Lip prints. photographs and study model impressions.
• Poisoning.
• Dental records. AUTOPSY METHODS7
In recent years, the accelerated application of Scientific
(After securing proper permission)
Methodology to Criminalistics and to the Field of Law has led
Body beyond recognition (autolysis, fire, mutilation, etc.)
to the creation and subsequent recognition of a multitude of
• Photographs (properly identified), in situ.
special study disciplines, which comprise the forensic sciences,
• Incision—Corner of mouth to tragus of the ear.
thus forensic dentistry. The focus of forensic dentistry occupies
• Disarticulation of mandible or saw cuts posterior to third
a primary role within the total spectrum of methods applied to
molar area (Stryker electric saw or by bone saw).
medicolegal identification.
• Cuts into maxillary sinuses above post apices of teeth and
dissection of maxilla.
ORAL AUTOPSY PROTOCOL
• Wrap specimens in plastic or soak in 10 percent formalin
Autopsy, also known as necropsy or postmortem, involves and bleach solution until fixed and odor controlled. Check
examination of the deceased, usually with dissection to expose the specimens twice daily to prevent demineralization.
Forensic Odontology in Children 413

• Radiographs—Periapical films taped to area with decreased After removal, jaws can be placed in a heavy-duty plastic
settings on machine; or, split maxilla and mandible in bag containing a swab soaked in formalin and sealed with
midline and place on ring stand using occlusal film. wire or tape. Then detailed examination should be carried out.
• Photograph specimens. Ideally, jaws can be returned to the mortuary to be placed
• Chart all dental findings. with the body if there is a positive identification.8
• Return specimens to remainders of body unless written Feriera and associates presented a technique of oral
permission is granted to retain custody. autopsy, which included means of accessing the oral cavity
in burned human remains. Since teeth may be brittle in
VIEWABLE BODY (no mutilation, autolysis, etc.) burned cases, they need to be reinforced with cyanoacrylate
cement, polyvinyl acetate or clear acrylic spray paint prior
1. Photographs (properly identified) in situ.
to examination. Access for radiography in incinerated bodies
2. Utilize mouth props to open mouth or wait until rigor mortis
can be obtained by removing the tongue and contents of the
disappears. If prying methods are used to open mouth, be
floor of the mouth in a “tunneling” fashion from beneath
careful not to fracture teeth.
the chin. 9
3. Intraoral photographs (properly identified).
4. Radiographs—Periapicals: or, tape occlusal film on cheek
parallel to the alignment of the posterior teeth or to the crest SCIENTIFIC METHODS OF IDENTIFICATION
of the alveolar ridge if the jaw is edentulous. The central In the forensic sciences, a great deal of effort is spent on the
ray (dental or portable medical X-ray machine) is directed identity or confirmation of identity of the victims and
from a point below the inferior border of the mandible on perpetrators. Forensic identifications by their nature are
the side opposite to the first molar region of the side to be multidisciplinary team efforts relying on positive identification
examined. Distortions are present in this view. methodologies as well as presumptive or exclusionary
5. Charting—Use checklist system to ensure complete oral methodologies.10
examination. Legal certification of an individual’s identity is based on a
number of parameters most of which are centered about the
JAW RESECTION METHODS individual’s appearance and personal effects. As such, many
people are buried or cremated based on a visual identification
Several terms are used for this procedure, such as ‘resect’,
or other presumptive identification methods. Where possible,
‘remove’, ‘disarticulate’ and ‘exenterate.’
a positive identification is preferred to a presumptive
Two methods for jaw resection may be used:
identification in such medicolegal cases.
1. A mouth prop is required to hold the mandible in the
Positive identification traditionally involves a comparison
open position. This provides good access to all surfaces
of pre- and post-mortem data, which are considered unique to
of the teeth. Incision can be given on both sides, from
the individual. These methods include: (1) Dental comparisons;
each corner of the mouth to the posterior border of the
(2) Fingerprints, palm prints and footprints; (3) DNA identifi-
ramus of the mandible. It should be in line with occlusal
cations; and (4) Radiographic superimpositions (vertebrae,
plane and through the cheek to the oral cavity. Then make
cranial structures including frontal sinuses, pelvic structures,
vertical incision at either ends of the horizontal one, at
bone trabeculae and prostheses).
posterior border of ramus of the mandible. Dissect the
Presumptive identifications include: Visual recognition,
tissue superiorly beyond the apices of the longest teeth
personal effects, serology, anthropometric data, and medical
so that it also exposes the lower part of the lateral wall
history which do not usually identify unique characteristics of
of maxillary sinuses and outer surface of the ramus.
the individual but rather present a series of general or class
2. Second method used is Keiser-Nielsen’s method, 1980.
characteristics which may exclude others based on race, sex,
In this method, a horseshoe-shaped incision is made
build, age, blood group, etc. Most positive identifications today
below the inferior edge of the mandible, so that
are based on dental examinations and fingerprints and are
postoperatively, suture lines will not be visible.
fundamental procedures in medicolegal death investigations
Alternatively, dissection may be started from the upper
including mass disasters.10
chest area where the forensic pathologist’s autopsy began,
extending the incision laterally on both sides to the
TECHNOLOGIES FOR AGE DETERMINATION
hairline. This allows for the careful repositioning of the
flap and esthetic packing of the area where the jaws are Dental age estimation makes use of morphologic, radiographic,
missing in order to return the facial features as close as histologic, and biochemical methods to examine age dependent
possible to the original contours. changes in teeth.11
414 Essentials of Pediatric Oral Pathology

ASPARTIC ACID RACEMIZATION magnesium/zinc ratio was the most reliable, with zinc/sodium,
magnesium/sodium and chromium/sodium ratios useful for
Aspartic acid racemization has been used for age estimation
supplemental comparative studies. Furthermore, individual
based on its presence in human dentin. Most protein
trace elements, such as, arsenic complement this sorting process
components of body consist of L-amino acids, whereas D-
effort. The authors concluded that this ancillary procedure did
amino acids have been found in bone, teeth, brain, and the eye’s
not provide sufficient individuality to be used alone, but was a
crystalline lens. D-amino acids are believed to have a slower
valuable adjunct to standard anthropological techniques
metabolic turnover and subsequently a slower decomposition
typically used for sorting commingled remains.
rate. Aspartic acid has the highest racemization rate of all amino
acids. In 1976, Halfman and Bada used this information to
SEROLOGICAL PARAMETERS
study age estimation by comparing D/L aspartic acid dental
ratios in 20 subjects with good results (r = 0.979).10 Forensic serology has been applied to odontological
A high coronary D/L ratio was noted in the younger age investigations with reasonable success. In dental pulps, ABO
group, decreasing with age presumptively due to environmental blood groups and serum proteins Gm, Km and Gc are present
changes. This determination technique is based on a linear as well as eight polymorphic enzymes (PGM, PGD, ADA, AK,
regression equation: In (1+D/L) / (1–D/L) = 2K (Aspartic)T + EsD, Fuc, DiA3 and transferrin).
Constant, where K = 1st order kinetics and T = actual age. Kido et al in 1993, reported on the use of transferrin C
Subsequently, they found that better age estimations could be subtyping by isoelectric focusing electrophoresis in dental
achieved with fractionating the total amino acid fraction (TAA) pulps. Sensitive immunoblotting techniques had previously
into an insoluble collagen fraction (IC) and a soluble peptide identified transferrin subtypes in urine, blood stains and semen.
fraction (SP). SP had higher concentrations of aspartic acid and The Kido et al study showed good correlation between serum
glutamine, both hydrophilic acids. So, this has the most reliable and dental pulp specimens.15
age estimation because of a high racemization rate roughly In 1993, Lopez-Abadia and Ruiz de la Cuesta reported a
three times that of TAA.10 simplified method for phenotyping alpha-2-HS glycoprotein in
serum, blood stains and dental pulp using isoelectric focusing
DENTAL STRUCTURE IDENTIFICATION electrophoresis on neuramidase-treated specimens, with
Scanning electron microscopy (SEM) with and without energy- excellent results.16
dispersive X-ray (EDX) analysis has been used to identify teeth
by dentinal tubules and evidence of previous restorations, SALIVA: AN IDENTIFICATION TOOL
especially in incinerated remains. Use of SEM with EDX Saliva offers several routes of inquiry. Identification of inorganic
provided a profile of elements present which may identify a anions such as thiocyanate or nitrites and polymorphic enzymes
particular type of dental material. Fairgrieve in 1994 reported such as alkaline phosphatase and amylase, are directed towards
a similar case involving SEM on incinerated teeth to evaluate its identification in bite marks. Further individualization is
parallel striations in tooth enamel and dentine as evidence of directed towards classical serological parameters including the
previous dental restorations.12 detection of blood groups, serum polymorphic enzymes and
Smith in 1990 reported the application of SEM with EDS polymorphic studies unique to saliva based on electrophoretic
(Energy Dispersive X-ray Fluorescence Spectrometry) analysis variation of isoenzymes of hexose-6-phosphate dehydrogenase
on MIA remains from Southeast Asia based on examination (Sgd), amylase, acid protein (Pa), basic protein (Pb) double band
and analysis of proximal facets.13 Smith also noted that this protein (Db), and proline rich protein (Pr). In addition to ABH,
preliminary study indicated that it was possible to detect Lewis and Sda antigens, a number of serum polymorphic
restorative material residue on the proximal surfaces of isoenzymes are present in saliva: alkaline phosphatase, amylase,
unrestored teeth and indicate the antemortem existence of a esterases, G-6-PD, and parotid peroxidase (SAPX). Saliva also
restoration on the adjacent tooth surface. This knowledge could
contains an interesting polymorphic protein system based on
be valuable in presumptive identifications where the teeth with
parotid glycoproteins; most, if not all, are acidic and proline rich.
critical restorations were not recovered with the primary
Both Gm and Km proteins are present and have been used for
remains, but teeth proximal to those with restorations were
racial determinants. Harrington et al 1988, described their efforts
present.
to detect hemagglutinins in dried saliva stains for comparison
with blood typing.10 Salivary drug detection use has been
SORTING BY METAL RATIOS
explored, especially in monitoring therapeutic drug concentration
In 1986, Fulton et al,14 reported on the use of metal ratios in and detection of impaired drivers in a relatively non-invasive
the reassociation of scattered and mixed human bones. The fashion. Peel et al 1984, found measurable quantities of drugs
Forensic Odontology in Children 415

in saliva extracted with methanol and analyzed by EMIT (enzyme Apart from problem of intra-observer discrepancies in
multiple immunoassay technique) and gas chromatography / reading rugae patterns, there is no doubt that even greater
mass spectrophotometry. 10 A number of drugs such as discrepancies could exist between observers. The existence of
phenobarbital, amphetamine and morphine have been detected this unreliability brings into question the present usefulness of
in saliva and saliva stains by radioimmunoassay (RIA) by a descriptive rugoscopy in the fields such as forensic science.20
number of investigators. Smith described similar studies in KS Limson and R Julian assess the feasibility of palatal
bloodstains for drug content.17 rugae patterns for identification with the aid of computer and
software program known as RUG FP-ID Match. In circum-
PALATAL RUGAE PATTERN stances where the identification of an individual by fingerprints
or dental record comparison is difficult, palatal rugae may be
Palatal rugae, also called as plicae palatine transversae and
considered as an alternative source of comparative material.
rugae palatina refer to ridges on the anterior part of the palatal
Technological advances now available to the forensic dentists
mucosa on each side of the midpalatine raphe, behind the
incisive papilla. Palatal rugae are well protected by the lips, such as computers, image capturing devices and the ability to
cheek, tongue, buccal pad of fat and teeth in incidents of fire transfer information quickly have simplified the task of human
and high-impact trauma. Rugae patterns, like teeth, are identification in deceased individuals as well as in mass fatality
considered unique to an individual. They do not change shape situations. In their study of about 250 subjects, the success rate
with age and reappear after trauma or surgical procedures (Fig. of 92 to 97 percent was obtained.21
15.3).
Palatal rugae classification has spanned almost 100 years; LIP PRINTS AND THEIR USE FOR IDENTIFICATION
the one suggested by Lysell is quoted most often. 18 He This idea was first suggested by Le Moyne Snyder in 1950 in
measured rugae in a straight line from the terminus at the lateral his book Homicide Investigation. Santos et al in 1960 reported
and categorized them into three: that wrinkles and grooves found on the lip could be divided
1. Primary rugae (> 5 mm) into simple and compound types. Suzuki in his study found
2. Secondary rugae (3-5 mm)
none of his participants to have the same lip groove pattern.22
3. Fragmentary rugae (2-3 mm).
Tsuchihashi named the wrinkles and grooves visible on the
(Rugae < 2 mm is not taken into consideration).
lips as ‘sulci labiorum rubrorum’.23 The imprint produced by
Thomas and Kotze have further detailed the various
these grooves is termed as ‘lip prints’, the examination of which
patterns of primary rugae. These include branched, unified,
is referred to as ‘cheiloscopy’. These grooves are heritable and
crosslinked, annular and papillary.19
are supposed to be individualistic. Cheiloscopy is used in the
Sunita Kapali et al in their study observed that the length
of rugae increased significantly with age, but the total number same manner as fingerprints.
of rugae remained constant. They also found ethnic variation It is analogous to bite marks analysis.
in rugae, for example, straight rugae were common in Lip prints were first classified by Martin Santos, 1966, thus:
Caucasians while wavy forms were common in Aborigines.20 Simple wrinkles
Straight line
Curved line
Angled line
Sine-shaped curve.
Compound wrinkles
Bifurcated
Trifurcated
Anomalous.
Tsuchihashi later proposed a separate classification, dividing
the pattern of grooves into six types:
Type I: Clear-cut vertical grooves that run across the entire
lip.
Type I’: Similar to type I, but do not cover the entire lip.
Type II: Branched grooves.
FIGURE 15.3: Landmarks on a dental cast, showing 1st, 2nd and Type III: Intersected grooves.
3rd rugae with their medial and lateral points Type IV: Reticular grooves.
416 Essentials of Pediatric Oral Pathology

Type V: Grooves that cannot be morphologically differen- number of different powders or cyanoacrylate and photo-
tiated. graphed.
Lip prints are usually left at crime scenes and can provide Pattern on lips is quite mobile and print may vary in
a direct link to the suspect. In recent years, lipsticks have been appearance according to pressure, direction and method used in
developed that do not leave any visible trace after contact with making the print. Lipstick amount applied may affect outcome.
surfaces such as glass, clothing, cutlery or cigarette butts and Although lip prints have been used in court in isolated
have been called persistent lip prints. Although invisible, these cases, there needs to be far more research to support the claim
prints can be lifted using materials such as aluminium powder of uniqueness attributable to any such evidence. Actual use of
and magnetic powder. lip prints in court is rare; however, its acceptance is debatable.22
Types of lip prints (Fig. 15.4):
1. Vertical RECONSTRUCTION OF THE FACIAL TISSUE
2. Branched
3. Intersected (diamond grooves) Positive identification is achievable when the skull and facial
4. Reticular. bones are used as a foundation to reconstruct the facial soft
Minor differences have been observed between right and tissues. Three-dimensional computer images, computed
left sides and between upper and lower lips. tomography (CT) images and radiographs have been used in
Lip prints on drinking glasses, facial tissues, magazines and the replication of the face of a 5000-year-old person whose
undergarments have been used as evidence. remains were removed from glacial ice on the Austrian and
Italian border.24
RECORDING LIP PRINTS With knowledge of the anatomic relationships between the
skull and face, antemortem facial photographs or radiographs
There are a number of ways: can be superimposed and matched with the skull. Video
1. On a non-porous flat surface such as mirror, they can be superimposition with two television cameras and an electronic
photographed, enlarged and overlay tracings made of the mixing device has been used successfully in overlaying a
grooves. photograph of a human face on an image of a skull for
2. Photographed directly with no medium and tracings made, identification (Fig. 15.5).24,25
but this requires correct lighting.
3. Rouge can be applied to the lips and then the lips are DNA IDENTIFICATION
photographed.
Images are then observed through magnifying glass and Dental identification takes advantage of the polymorphic nature
traced onto cellophane. Lip prints can be developed using a of the hardest tissues in the body—precisely those structures,
which are most likely to remain available for identification
purposes. Although dental structures are more likely to survive
traumatic and decompositional changes than other traditional
means of identification such as fingerprints, scars, facial
appearance, etc. DNA has a still greater likelihood of survival.
A common obstacle to fingerprint and dental identification
is the lack of antemortem data for comparison. The common

FIGURE 15.4: Classification of lip prints FIGURE 15.5: Reconstruction of facial tissue
Forensic Odontology in Children 417

availability of families as sources of reference material for blood group types, red cell antigens and protein isoenzymes.
comparison purposes is a particularly important aspect of DNA Due to the degeneracy of genetic code, there will be more
identification. polymorphisms than the resultant phenotypes. The
discriminatory power of DNA is far greater than any set of
THE DNA MOLECULE traditional markers including HLA typing. It can be performed
on any tissue or fluid. DNA is less susceptible to environmental
The basis for all inheritance is found in the DNA genome of
insults so can be performed on far older specimens.27,28
cells. This information is coded within the chemical structure of
the DNA molecule or more accurately, the set of DNA molecules
RFLP Methods (Restriction Fragment Length
known as the genome. Nucleotide bases are arranged in specific
Polymorphisms)
sequences within the chemical structural scaffolding. Only four
bases (adenine, cytosine, guanine and thymine) make up the This DNA typing method that was first described and most
genetic alphabet that produces the words, sentences, paragraphs commonly employed by many crime labs initially is known as
and chapters, which are eventually read into proteins that restriction fragment length polymorphisms (RFLP) analysis.
comprise biological organisms. These bases are present in pairs The six steps in RFLP testing include:
in a complementary fashion to form base pairs, such that every 1. Extraction of DNA from a biologic source.
A is paired with a T, every C with a G, and vice versa. 2. Cutting the DNA into relatively small fragments at
Stability of DNA: DNA is a robust molecule which can specific sites with ‘restriction enzymes’.
tolerate a remarkable range of temperature, pH, salt, and other 3. Separating the fragments by size using agarose gel
factors that destroy classical serological markers. This electrophoresis.
ruggedness allows DNA longevity and has permitted DNA 4. Transferring and immobilizing the separated DNA
typing of Egyptian mummies and 30-million-year-old insects fragments onto a nylon membrane.
preserved in amber.26 5. Denaturation of the DNA into single strands and
hybridization to radioisotopically-labeled probes (small
DNA POLYMORPHISMS fragments of single-stranded DNA).
This can be length-based or sequence-based. Length-based 6. Autoradiography in which an X-ray film is placed over
polymorphisms are a characteristic of repetitive DNA that the membrane for several days resulting in exposure
generally does not code for any protein (so-called ‘junk’ DNA). of the film at the point of the probe.
DNA fragments vary in size between individuals due to the RFLP testing is often called as ‘Southern blotting” because
presence of variable numbers of tandem repeats (VNTRs), i.e.: the DNA transfer technique was first described by Professor
core of 7 bases may be repeated 3 times in one individual or Southern. Typically, RFLP testing will take several weeks to
12 times in the next individual. Traditional restriction fragment perform. For every probing, the membrane is stripped off the
length polymorphism (RFLP) analysis, as is commonly previous probe and rehybridized and autoradiography
associated with the DNA testing in crime labs, involves cut performed anew. However, alternatives to radioisotopic labels
fragments (restriction fragments), which include internal VNTR now exist, particularly chemiluminescent and fluorescent probe
region (loci) and thus vary in fragment length. VNTR fragments labels, which permit much faster testing.
can also be amplified instead of cut, then called as amplified Unfortunately, RFLP is not useful where the DNA is
fragment length polymorphisms (AmpFLPs). degraded; so, RFLP testing is of limited value in testing cadaver
DNA identity information is found not only in fragment tissue for identification of human remains, unless the remains
length variation, but also within the DNA sequence of similarly are fresh.26-28
sized DNA fragments. Sequence polymorphisms consist of
difference changes in one or more bases in a DNA sequence Polymerase Chain Reaction (PCR) Methods
at a particular location in the genome. Sequence variations can (Figs 15.6A and B)
manifest as regions of alternative alleles or base substitutions,
It is a method of copying or ‘amplifying’ a particular segment
additions or deletions. Most sequence polymorphisms are mere
of DNA. A few strands or a single strand of DNA can be used
point mutations. Sequence polymorphisms can be detected by
to reproduce millions of copies of target DNA fragments. Kary
DNA probes or by direct sequencing.26
Mullis was awarded the Nobel Prize in 1993 for the discovery
of the PCR process, which has led to a revolution in the life
DNA IDENTIFICATION METHODS
sciences. PCR amplification is a sample preparation technique,
DNA testing is far superior to those other tissue-typing which enables further testing to detect various polymorphisms.
techniques for a variety of reasons. DNA is the basis for all Nonamplified DNA becomes undetectable against the amplified
418 Essentials of Pediatric Oral Pathology

FIGURES 15.6A and B: PCR kit

background target sequence. PCR testing is sensitive, quicker, fragment. Commercial kits, i.e. DQ-alpha and Poly-Marker
less labor intensive and less tedious than RFLP testing. It is systems, are based on a dot/blot format for SSO typing and are
also used for degraded specimens because only a few copies currently in use by many crime labs. The resultant dot/blot strip
of relatively short segments need to remain intact. However, has a series of spots that turn blue if the reaction is positive and
PCR testing is susceptible to inhibition and the potential for in this way give a series of yes/no results. These dot/blot tests
cross contamination.27 are quite rapid and work reasonably well despite sample
Human X and Y chromosome alpha-satellite sequences degradation, but do not harbor the same discriminatory power
lying within higher order repeats were amplified by the as RFLP tests.26
polymerase chain reaction (PCR) in genomic deoxyribonucleic The sex determination of bloodstains was performed using
acid (DNA) isolated from blood, bone, and several other tissues a human Y chromosome-specific (DNA) fragment of 1.9 kb
and specimens of potential forensic science interest. X and Y length as a hybridization probe. The DNA samples were taken
sequences could be coamplified under some of the PCR from 1- and 4-week-old bloodstains of males and females,
conditions employed. X and Y sequence amplification can respectively. Strong signals with male DNA were observed by
provide information about the sex of origin. Amplification of Y-probe, while faint signals with female DNA were detected. In
the X, H, and D17Z1 sequences was found to be primate- addition, clear signals were observed in the extract samples from
specific among the common animals tested and can thus male bloodstains (16-week-old) on paper. Dot hybridization of
provide species of origin information about a specimen. The the Y-probe would be widely applicable to studies on sex
authors suggest that amplification of X and D17Z1 or H determination of medicolegal materials such as blood,
sequences might provide “relaxed” and “stringent” controls for bloodstains, teeth, and cadaverous parts.30
appropriate PCR amplification tests on forensic science
specimens. Testing was carried out using PCR protocols that AmpFLPs and STRs
employed Thermophilus aquaticus (Taq) and Thermus flavis
Dinucleotide repeats are not generally used in forensic
(Replinase) thermostable DNA polymerases.29
laboratories due to the artifactual production of so-called
“shadow” and “stutter” bands.
Dot/Blots
The shorter STR fragments are generally preferable for a
Sequence information can be obtained through the use of DNA variety of technical reasons. These STR systems work well
probes. A DNA probe is a small piece of single stranded DNA despite significant degradation and are quite amenable to
(oligonucleotide) which will bind to another single-stranded automation. Sufficient numbers of STR systems can be
DNA with the complementary sequence. A sequence specific performed to achieve discriminatory powers similar to current
oligonucleotide (SSO) probe, also known as an allele-specific RFLP testing. The British and Canadian crime labs are moving
oligonucleotide (ASO) probe, is a single-stranded DNA towards using STR systems exclusively.26
Forensic Odontology in Children 419

TYPES OF DNA In relatively fresh cadavers, unclotted blood is the

preferable source of DNA. Although heme is an inhibitor
Pretty and Sweet have pointed out the use of two types of DNA.
of PCR, laboratories are accustomed to blood as a DNA
The first is called genomic or nuclear DNA. This is located in
specimen and although only white blood cells carry the
the nucleus of the cells and is commonly used in forensic cases. DNA, ample DNA is present for testing. Due to the settling
The second, known as mitochondrial DNA (mt DNA), is out of white blood cells, clotted blood may not be a good
present in the mitochondria of cells. Bender and associates source of DNA. Blood is a good culture medium and
point out that a major advantage of mt DNA is that each cell bacterial growth may render blood samples useless. Any
has a high copy number of mt DNA, e.g.: epithelial cells contain tissue can be used successfully for DNA typing purposes.
5000 mt DNA molecules. Also, mt DNA is exclusively inherited Brain tissue is said to be a particularly good source of DNA
from the mother.31 in intermediate postmortem time periods. Hard tissues (bone
and teeth) are the best source of DNA in cases of advanced
Mitochondrial DNA (mt DNA) decomposition.
DNA is not only present in chromosomes in the nuclei of the
cells, but also is present in mitochondria of cells. Mitochondria PRESERVATION
are known as the powerhouses of the cells as they are the The specimens should be kept cold or preferably frozen
primary machinery for accomplishing oxidative mechanism. (repeated freezing and thawing is not good). Desiccation, even
Tens, hundreds or even thousands of mitochondria are present simple air-drying, may be adequate for storage of some DNA
within a single cell and each mitochondrion contains thousands specimens, e.g. bloodstains and bone.
of copies of mitochondrial ‘DNA particles’ of 16000 bp (base Formalin fixed tissues are not optimal, but can be used for
pairs) mt DNA sequence can be obtained when the nuclear PCR-based DNA testing. No tissues or biologic fluids should
DNA type cannot. be discarded as inadequate without first attempting DNA
No significant regions of repetitive DNA exist in mt DNA, testing. Great care should be taken to prevent specimen
only sequence polymorphisms are typed. The region of mt contamination. Specimens should be collected with gloves and
DNA which is analyzed for human identification is the pristine instruments. Fresh tissues should be collected by an
noncoding region known as the displacement loop (D-loop) incisional biopsy technique where possible.27
or control region. A unique feature of mt DNA is its mode of
inheritance-one half of the nuclear DNA is from the mother TEETH AS EXCELLENT SOURCE OF DNA
and one-half from the father. Mitochondria are inherited in a Since teeth can resist extreme conditions, Pretty and Sweet state
strictly mother-to-child manner; there is no paternal contri- that teeth are an excellent source of DNA. One may doubt
bution. Accordingly, mt DNA can be traced through a family ability of the teeth to yield sufficient quantities of DNA for
via maternal lineages for many generations. It has great analysis, particularly under the circumstances when the post-
application to severely decomposed or skeletonized remains. mortem interval ranges from a few months to years. However,
Very few laboratories are performing this kind of testing at a routinely applied technique in forensic investigations,
this point in time.26 polymerase chain reaction (PCR), allows amplification of even
highly degraded DNA.31
SPECIMEN SELECTION, COLLECTION AND DNA is present in the vascular pulp of the tooth, but it is
PRESERVATION also found throughout the tooth in varying levels, particularly
in the odontoblastic processes, accessory canals and cellular
DNA can be isolated and tested from virtually any postmortem
cementum. Most information necessary for traditional dental
tissue, although after death DNA, will undergo fragmentation identification is present in the crown (enamel and dentin) of
by autolytic and bacterial enzymes, specifically DNases. the tooth. Consequently, a tooth can be sectioned horizontally
Nevertheless, the sequence information is still present within through the cervical root subjacent to the CEJ, preserving most
the DNA fragments and therefore, the information is not restorations for traditional dental comparison purposes.26
completely lost despite the fairly extensive fragmentation, Sweet and Hildebrand have advocated a method known
which occurs from decomposition. Traditional RFLP testing as cryogenic grinding for extracting DNA. This involves
will require non-degraded high molecular weight DNA, cooling the whole tooth to extremely low temperatures using
whereas PCR-based analysis can be performed on degraded liquid nitrogen and then mechanically grinding it to fine
samples and mt DNA can be obtained from skeletal remains powder. The major drawback of cryogenic grinding is that the
when nuclear DNA cannot. tooth needs to be completely crushed.32
420 Essentials of Pediatric Oral Pathology

REFERENCE SAMPLES/DATABASES group substances or antigens have been found on the red cells.
Some of these antigens frequently occur whereas others are
Reference specimens for DNA testing are generally available
rare, e.g. H antigen is present in 99.9 percent of the population
from family members. Specimens from the spouse and children
whereas B3 is present in less that 0.1 percent.33
will permit ‘reverse paternity’ testing using nuclear DNA
The most important system is the ABO system, which is of
probes. Specimens from parents and siblings will permit
major importance in blood transfusion and tissue transplan-
identification, particularly in closed populations. Mt DNA
tation. The well known Rhesus (Rh) blood group system is
analysis must be performed on maternal kindred (mothers,
important because of its role in hemolytic diseases of the
siblings, children only in the case of a female); it can be
newborn (erythroblastosis fetalis). Other well known systems
performed even in distant relatives (maternal aunts and uncles,
are MN, Lewis, Kell, P, I, Kidd, Lutheran and Duffy systems.33
children of sisters).
Primary DNA specimens of individuals may be available
INHERITANCE OF BLOOD GROUP SYSTEMS
from toothbrushes, biopsies or tissue slides archived in a
hospital’s pathology department, from stored blood donations, Within a few years after the ABO system was described, it was
from licked envelopes and stamps, or in case of mt DNA from established that blood groups are inherited in accordance with
locks of baby hair or clippings from an electric shaver.10 Mendelian laws and are generally inherited as simple
Future DNA testing technologies will permit high-volume, Mendelian dominant characters.
low-cost testing, significant in mass disaster. The laws of inheritance of red blood antigens have been
applied extensively to medicolegal examinations. This type of
IMPORTANCE OF BLOOD GROUP application has been useful in resolving claims of parentage in
DETERMINATION estate, immigration problems, mixed babies in hospitals, in
cases of kidnapped children when kidnapper claims the baby
In 1900, Landsteiner noted that blood from one person mixed
as her own. Greatest numbers of cases involve disputed
with the blood from another produced visible clumping of red
paternity.
blood cells. This observation led to discovery of the ABO
A child cannot possess a red cell antigen unless it is present
groups and opened a new and complex field of study (Fig.
in one or both of his parents. The mathematical chances of
15.7).
proving exclusion have been calculated and the chances for
The use of blood group substances in medicolegal
exclusion increase as more blood systems are used.
examinations is based on the fact that once a group is estab-
lished in an individual it remains unchanged throughout his life. DEMONSTRATION OF BLOOD GROUP
SUBSTANCES33
BLOOD GROUP SYSTEMS
All blood-grouping tests are dependent on an observable
The term “blood groups” is applied to inherited antigens reaction between a blood group substance and an antibody to
detected on the red blood cell surface by specific antibodies. this substance in serum. The type of antigen-antibody reaction
Blood groups within a blood group system are inherited by used most commonly in blood group determination is
single or multiple allelic genes. At present more than 250 blood agglutination, which results in clumping of red blood cells if
the test is positive. Precipitation reactions are seldom used
routinely, but are of value in forensic cases in differentiation
between human blood and that of other species. The sera used
for these reactions are produced by injecting human red cells
in animals or human volunteers.

Agglutination or Hemagglutination Test

This test consists of demonstrating the agglutination of red cells
after the cells have been mixed with appropriate antiserum in
normal saline. Antibodies producing this change are called
complete antibodies and represent immunoglobulins of the IgM
class. The incomplete antibodies coat the cells but will not
produce agglutination. These are of the IgG class of
FIGURE 15.7: Karl Landsteiner, the discoverer immunoglobulins and agglutination with these antibodies is
of various blood groups produced by manipulating the system. In forensic cases, fresh
Forensic Odontology in Children 421

blood may not be available. Since, the agglutination test descri- the interpretation of the tests requires accurate notation as to the
bed above cannot be used on dried blood stains, secretions or degree of contamination. Take swab from normal adjacent site
tissues, one of several modifications of the antigen-antibody on victim’s skin that would not have been exposed to saliva to
reaction must be substituted. The two often used methods are serve as control for the blood grouping tests. Saliva should be
agglutination-inhibition and mixed cell agglutination tests: collected in the same manner from floor, ground, or on various
objects that cannot be moved from the site of crime. Since, ABH
Agglutination-Inhibition Test substances are widely distributed in nature, as part of the plant
or animal world, the saliva sample may give a false-positive test
This test consists of demonstrating inhibition of agglutination
due to contamination from the substrate. Similarly, the substrate
between red blood cells with a known antigen and antisera to
may also contain inhibitors that interfere with the test for blood
this antigen. Extracts of dried blood, tissues and saliva can be
substances and thus be responsible for a false-negative test.
used in this manner for testing, but relatively large amounts of
materials are needed for this test.
DENTAL TISSUES AND THEIR ROLE IN
Mixed Cell Agglutination FORENSIC SCIENCE

This requires smaller amounts of materials since it does not TEETH AND THE BLACK DEATH
depend on preparation of tissue extracts. Dried red blood cells Teeth have been used to answer historical questions as well as
or other tissue cells are treated with a known antiserum. If the identify victims.
cells tested have antigens that react with antiserum, antibody For years, scientists and historians have sought to discover
will bind to cells. The reaction can be further adapted to test if the bubonic plague outbreaks during the Middle Ages were
materials that have been soaked with human blood or actually caused by the Yersinia pestis bacteria. Pulp was
secretions, e.g.: fibers stained with dry saliva from a group A extracted from the teeth of people who had allegedly died of
individual and treated with anti-A antiserum will show the plague, and the DNA tested for the presence of Y. pestis.
adherence of group A blood cells that can be visualized under Although it was found in some of the teeth, not all of the alleged
the microscope. plague victims’ teeth contained the bacteria. Some of these
people died of other diseases and not bubonic plague after all.
DETERMINATION OF BLOOD GROUP SUBSTANCES
IN SECRETIONS AND TISSUES33 TYPES OF TEETH
Identification of blood substances in secretions and tissues • When teeth grow in, or erupt, they do so differently in each
found at the scene of a crime is more complex than it is with person.
blood. Even in the fresh state, the only antigens that can be • Teeth grow an average of four micrometers per day, so it
identified are A, B and H and these require more complicated is possible to give a rough age estimate based on teeth.
techniques. The examination of secretions and tissues may be • It can also be possible to distinguish ethnicity from the
useful in corroborating findings from blood samples and may teeth. Some Asians and Native Americans have incisors
be particularly valuable in the absence of blood. with scooped-out backs.
• The patterns of tooth wear also vary and can change over
BLOOD GROUP SUBSTANCES IN SALIVA time. Not only can people be identified by their teeth, you
The use of saliva in forensic science is based on the presence can also learn a lot about their lifestyles and habits by the
in the saliva of secretors of ABH blood group substances in state of their teeth.
fairly high concentration. All secretors show some H activity
TOOTH IDENTIFICATION
in their saliva, but larger amounts are usually present in the
saliva of group O individuals than in those possessing A and B Tooth enamel is harder than any other substance in the human
substances. Since saliva may be found on various objects at body, which is why teeth remain long after all other parts have
the scene of a crime, care must be taken that this evidence is decayed.
not neglected or handled improperly. • There is no database of teeth that corresponds with
In cases of human bites, saliva should be collected before databases of fingerprints, so dental records are how forensic
making impressions. Swabs should be taken from different areas dentists identify the dead.
of the bite with clear records kept as to where the swabs were • Victims of fires are often identified by their teeth, which
taken in relationship to the individual tooth marks. Since the can withstand temperatures of more than 2,000 degrees
victim’s blood or tissue may be mixed with the attacker’s saliva, Fahrenheit (1,093 degrees Celsius).
422 Essentials of Pediatric Oral Pathology

• Teeth that have been through especially intense heat are • Contusion—A bruise
very fragile and may shrink, but they can be preserved with • Hemorrhage—A profusely bleeding bite
lacquer and used for identification as long as they are • Incision—A clean, neat wound
handled very carefully. • Laceration—A puncture wound.
• Dental work, such as a partial or full coverage gold crown, Several different types of impressions that can be left by
will be distorted by fire but can still aid in identification. teeth, depending on the pressure applied by the biter can be
• To identify a person from his or her teeth, a forensic dentist identified:
must have a dental record or records from the deceased • Clear impression means that there was significant pressure
person’s dentist. • Obvious bite signifies medium pressure
• Even if only a few teeth are available, a forensic dentist • Noticeable impression means that the biter used violent
can still make a positive identification. The best pressure to bite down.
comparisons come from X-rays.
• In addition to the dental records, forensic investigators can BITE-MARK ANALYSIS CONTROVERSY
retrieve DNA samples by extracting the pulp from the
Forensic dentists may be giving juries the impression that bite
center of the tooth.
marks are as unique as fingerprints or DNA but they are not as
• Unlike the enamel, pulp can be damaged by fire and other
unique. Bite marks cannot be used as the only thing linking
conditions, but it can also last for hundreds of years.
the suspect to the crime.
• Dental erosion will show as enamel being lost on the palatal
surfaces which could be caused by a number of different
BOLD
conditions such as anorexia, chronic alcoholism and gastric
problems. Each causes repeated vomiting, which causes BOLD is a forensic odontology laboratory at the University
acid erosion of the teeth. of British Columbia. It is the first and only laboratory in North
• Staining visible on teeth could be due to fluorosis or America that is dedicated to full-time forensic dentistry
tetracycline administration during tooth development or a research, casework and graduate teaching.
number of causes resulting in intrinsic or extrinsic stains. It is the place where laboratory discoveries and modern
• Children who hold pencils or pins between their teeth may forensic methods are applied to dental evidence to assist in the
have wear facets on incisors. resolution of legal issues.
• Dental identification is often the last resort, but it isn’t BOLD is a center of excellence that aims to act as a
always possible—some people simply can’t be identified. resource for odontologists and other forensic scientists who
deal with teeth, bones, saliva, DNA and dental records.
BITE-MARK ANALYSIS Experts here can provide assistance with:
• Investigation
Bite-mark analysis is extremely complex, with many factors
• Identification
involved in a forensic dentist’s ability to determine the identity
• Analysis
of the perpetrator.
• Testimony.
The movement of a person’s jaw and tongue when he or
she bites contributes to the type of mark that is left.
CHILD ABUSE
Depending on the location of the bite, it is not typical to
find bite marks where both the upper and lower teeth leave Selwyn et al 1985, have defined child abuse as a non-accidental
clear impressions—usually one or the other is more visible. physical injury, minimal or fatal, inflicted upon children by
If the victim is moving while being bitten, the bite would persons caring for them.
look different from that inflicted on a still victim. “The battered child syndrome” is a term, which was
Bite marks are tricky because they’re about more than just introduced by Kempe in 1962. He pointed out that guilty
the teeth. Time can affect bite marks, and so can movement parents were not confined to one socioeconomic class, and that
and pressure. whilst the syndrome might occur at any age, in general affected
Forensic dentists use several different terms to describe the children were under the age of three years.34
type of bite mark: Reasons for reluctance to acknowledge child abuse by
• Abrasion—A scrape on the skin dentist:35
• Artifact—When a piece of the body, such as an ear lobe, • Lack of adequate histories.
is removed through biting • Lack of knowledge about the problem of child abuse and
• Avulsion—A bite resulting in the removal of skin their role and responsibilities in reporting it.
Forensic Odontology in Children 423

• Concern about the effects on their practices if they report EXAMINATION OF ABUSED AND
cases. NEGLECTED CHILD
• Fear of confronting the parents.
• Lack of literature about dentists’ experiences with cases of Common points to be observed and examined:
child abuse. 1. Frozen watchfulness—Staring constantly. There are no
spontaneous smiles and almost no eye contact.
INCIDENCE OF OROFACIAL LESIONS 2. Lack of cleanliness and indications of malnutrition.
3. Overdressed/underdressed children.
Cameron et al found that facial trauma is commonly found in 4. Fractured anterior teeth or torn frenum.
children. Becker et al found that orofacial trauma in 49 percent 5. Multiple injuries in various stages of healing.
of 260 documented cases was child abuse. Analysis of types
of injuries that dentists may see showed that 33 percent were DEFINITIVE EXAMINATION FOR
head injuries, 61 percent were facial injuries and 6 percent were CHILD ABUSE/NEGLECT
intraoral injuries.34,35 • It requires a keen observation and detailed documentation
when suspicion exists. A systematic approach should be
DETECTING CHILD ABUSE IN followed and to protect the examiner legally, the dental
THE DENTAL OFFICE36 assistant should be present in the room and aware of the
When a child presents for examination, particularly if there is dentist’s suspicion, to verify and record the findings.
an injury involved, the history may alert the dental team to the • Detailed examination and palpation of the skull looking for
possibility of child abuse. subgaleal hematomas and cephalomatomas.
The possibility of child abuse or neglect should be • Positive sign of any battle like laceration, scar, and bruise.
• Body surfaces that are covered should be examined by
considered whenever history reveals the following:
lifting up the clothes to the limit they allow.
• The present injury is one of a series of injuries that the child
has experienced. PARENT CONSULTATION
• The family offers an explanation that is not compatible with
the nature of the injury. Once the suspicion is confirmed, the parent should be informed
• There has been an extraordinary delay in seeking care for that an injury has been noticed.
injury. The explanation of the cause of the injury should be
• The family does not want to discuss the circumstances of understood fully by the dentist. Any inconsistencies in the
injury. narration or change in attitude of the parent or lack of
correlation between the injury and its cause should be duly
TYPES OF CHILD ABUSE37 noted down. Explanation of the cause of injury should be
obtained from both the parent and child to reveal any
Physical abuse 31.8% discrepancies.
Educational abuse 26.3%
Emotional abuse 23.3% COLOR CHANGES OF BRUISES
Sexual abuse 6.8% 0–2 days Swollen, tender.
Failure to thrive 4.0% 0–5 days Red, blue, purple.
Intentional drugging or poisoning not specified 5–7 days Green.
Munchausen syndrome by proxy not specified 7–10 days Yellow.
10–14 days Brown.
CHILD NEGLECT37 2–4 weeks Cleared.
Child neglect can be defined as an act of omission or the failure OVERDIAGNOSIS OF CHILD ABUSE36
to provide food, shelter, clothing, health care, safety need,
dental care and supervision. While the importance of reporting suspected cases of child
abuse and neglect cannot be overemphasized, the thoughtful
Types of Child Neglect practitioner should also consider the other side of the coin.
Kaplan reviewed 15 cases that were misdiagnosed as child
Emotional neglect 27.8% abuse. They included a child whose generalized bruises were
Health care neglect including dental neglect 8.7% later found to be related to cystic fibrosis. McClain et al
Physical neglect 7.8% reported on another supposedly abused child who was found
424 Essentials of Pediatric Oral Pathology

to have acute lymphoblastic leukemia of childhood. Kaplan • A frictional burn or rope burn will result due to a piece or
concluded that overdiagnosing the battered child syndrome can strap of sheeting used to restrain the child, presenting a
be as harmful as failing to consider it. large blister that encircles the extremity.
• Gagging abrasion is due to restraining of mouth to stop
Human Hand Marks
crying or yelling of children.
• Grab marks or finger-tip bruises: Oval shaped bruises that
resemble finger tips. Facial Injuries Include
• Linear grab marks: Pressure of entire finger when capillaries (in order of decreasing frequency)
at the edge of the injury are stretched enough to rupture.
• Slap marks: Two to three parallel linear bruises at a finger • Contusions and ecchymosis
width. • Abrasions and laceration
• Crescent shaped bruises: Due to pinching. • Burns
• Bone fractures
Strap Marks • Bite marks.
• These are one to two inches wide, sharp border rectangular
bruises of various lengths. Injuries of Dentition Include
• Often lash marks are narrow, straight edged bruises or
• Traumatized or avulsed teeth indicating blunt trauma or
scratches caused by a thrashing with a tree branch.
• Loop marks are due to rope commonly breaking the skin pattern injury from instruments.
and loop shaped scars because of force of the distal end. • Tears of the labial or lingual frenula.
• Oral mucosa torn from gingiva.
Bizarre Marks • Loosened or fractured teeth.
• Root fractures.
• Bizarre-shaped bruises with borders are nearly always
inflicted when a blunt instrument is used resulting in a welt • Darkened/discolored and/or nonvital teeth indicating
or a bruise (Fig. 15.8). repeated trauma.
• The wide assortment of instruments used to abuse children • Previously missing teeth.
suggests that the caretaker who loses temper, grabs • Trauma to the lip.
whatever objects are handy. • Trauma to the tongue.
• Circumferential tie marks on ankle or wrist can be caused • Other spot tissue injuries.
when the child is restrained. • Fractures of jaws and associated structures.
• Circumferential cuts are due to a narrow rope or cord. • General neglect of the mouth.

BITE MARKS
The first man on earth “Adam” committed the sin of biting an
apple and was punished by God. Despite this fact, man on earth
did not realize the importance of bite marks and its proper
utilization until late.
As no two fingers are identical, neither two mouths nor two
teeth are exactly identical.38
Biting is considered to be a primitive type of assault and
results when teeth are employed as a weapon in an act of
dominance or desperation.
Bite mark may be defined as a mark caused by teeth alone
or in combination with other oral parts or as consisting of teeth
marks produced by the antagonist teeth, which can present as
two opposing arch marks.
MacDonald (1974) has defined a bite mark as a mark
FIGURE 15.8: Bizarre shaped marks on the back of a child caused by the teeth either alone or in combination with other
indicative of physical child abuse mouth parts.39
Forensic Odontology in Children 425

Other definitions of bite mark are: series of arches where the tissues are sucked into the mouth
• “A pattern left in an object or tissue by the dental structures and pressed against the back of the teeth with the tongue.
of an animal or human”.40 Aggressive bite marks: These marks show evidence of
• “A bite mark may be defined as a pattern produced by scraping, tearing and avulsion of tissues. Usually involves ears,
human or animal dentitions and associated structures in any nose, or nipples. Such bites may be difficult to interpret.
substance capable of being marked by those means”. 41
• “A bite mark is the registration of tooth cutting edges on a WEBSTER’S CLASSIFICATION 43
substance caused by jaw closure”.42
It is not uncommon to note bite marks in foodstuffs, especially
• “A bite mark is a mark made by the teeth either alone or in
in cases of theft or robbery, where the involved may
combination with other mouth parts”.
conveniently grab a bite from the kitchen refrigerator or a
— As has been discussed in a previous section, bite marks
supermarket food shelf.
are produced in flesh, foodstuffs or a number of other
Type I: Food item readily fractures with limited depth of
materials by teeth and the surrounding soft tissue.
— Marks in human body are often contributed by pressure tooth penetration, e.g. hard chocolate.
from lips and tongue and from teeth themselves. Type II: Fracture of fragment of food item with considerable
— Dental evidence has become increasingly important in penetration of teeth, e.g. bite marks in apples and other firm fruits.
the investigation of non-accidental injuries to children. Type III: Complete or near complete penetration of food
— The most common areas to be bitten in children are: item with slide marks, e.g. cheese.
head, neck, limbs and trunk.
In female: Breast, arms and legs. MECHANISM OF BITE MARKS
In males: Arms and shoulders.
Tooth Pressure
CLASSIFICATION OF BITE MARKS • Marks caused by a direct application of incisal edges of
Depending on biting agent anterior teeth or occlusal surfaces of posterior teeth.
Human: • Marks depend on force applied and duration of force.
Children • A pale area represents incisal edges and bruising represents
Adults margin of incisal edge.
Animals: • Shape of marks may be useful in the identification of
Mammals specific tooth.
Reptiles • Tooth mark pattern as an attack or defensive bite mark.
Fish
Mechanical: Tongue Pressure
Full denture It is caused when material is taken into the mouth and pressed
Saw blade tooth marks by tongue against the teeth or palatal rugae.
Bicycle chain and others like electric cord.
Bite marks of sadistic origin exhibit a central ecchymotic
Depending on material bitten
area or a suck mark with a radiating linear abrasion pattern
Skin:
surrounding the central area and resembling a sunburst.
Human
Animal
Perishable items: Tooth Scrape
Food items like cheese, apple. • Caused by teeth scraping across the surface of the skin.
Non Perishable • These marks are usually inflicted by anterior teeth.
Unanimated objects such as pipes, pens, pencils. • May appear as scratches or abrasions.
Depending on degree of biting
Definitive bite marks: Tooth pressure marks are formed when
FACTORS AFFECTING BITE MARK INJURIES
a direct application of pressure by the biting edges has caused
tissue damage. 1. Inherent skin factor.
Amorous bite marks: These marks are made slowly with 2. Age.
absence of movement between teeth and tissues. Lower teeth 3. Sex.
marks are formed when teeth are pressed into tissue with 4. Time.
gradually increasingly pressure. Marks of upper teeth form a 5. Vascularity.
426 Essentials of Pediatric Oral Pathology

CHARACTERISTICS OF HUMAN BITE MARKS 3. Impressions

FOR IDENTIFICATION 4. Tissue sample.
Human bite marks include an elliptical or ovoid pattern
containing tooth and arch marks. Photography (Fig. 15.9)
An arch mark may indicate the presence of four to five teeth Obtain or produce photographs which meet the following
marks reflecting the shape of their incisal or occlusal surfaces. guidelines:
Other significant findings may include: • Orientation and close-up photographs should be taken.
• Presence or absence of any tooth • Photographic resolution should be of high quality.
• Peculiar shape of any tooth • Both color and black and white photographs should be
• Mesiodistal dimensions taken.
• Arch form and size • If color film is used, accuracy of color balance should be
• Relationship between upper and lower jaws assured.
• Any rotation, fractured teeth, microdontia, diastema, etc. • It is recommended to start with the broader orientation
photographs then move towards close-up photographs,
BITE MARK ANALYSIS
which are used in detailed documentation of the injuries
Description of the bite mark: themselves.
Demographics • Photographs of the mark should be taken with and without
• Name of victim a scale in place.
• Case number • When the scale is used, it should be on the same plane and
• Date of examination adjacent to the bite mark. It presently appears desirable to
• Referring agency include a circular reference in addition to a linear scale.
• Person to contact • The American Board of Forensic Odontology (ABFO) no. 2
• Age of victim scale is widely used and recommended, but many other
• Race of victim scales can be used, including rulers, coins or any other
• Sex of victim objects that can be used as a size reference when the photo-
• Name of examiner(s). graphs are enlarged to life size. Such scales or reference
Location of bite mark items become part of the evidence and should be
• Anatomical location
maintained with rest of the case evidence.44
• Surface contour: Flat, curved or irregular
• The most critical photographs should be taken in a manner
• Tissue characteristics:
that will eliminate distortion.
a. Underlying structure—bone, cartilage, muscle, fat.
• In the case of a living victim, it may be beneficial to obtain
b. Skin—relatively fixed or mobile.
serial photographs of the bite mark.
Shape
Round, ovoid, crescent or irregular.
Color
The color should be noted, e.g. red, purple, etc.
Size
Vertical and horizontal dimensions of the bite marks, preferably
in metric system.
Type of injury
• Petechial hemorrhage
• Contusion (ecchymosis)
• Abrasion
• Laceration
• Incision
• Avulsion
• Artifact.

COLLECTION OF EVIDENCE FROM VICTIM

1. Photography
2. Salivary swabbing FIGURE 15.9: Bite marks on thigh of a child
Forensic Odontology in Children 427

• Good quality extraoral photographs—both full face and • Wearing gloves is strongly recommended during swabbing
profile. because that prevents contamination of evidence. We each
• Intraoral—frontal view, two lateral views, occlusal view secrete the ABH blood groups in saliva, seminal fluid, tears
of each arch and any additional photograph that may and perspiration. In 80 to 85% of most individuals, the
provide useful information. levels are high enough for routine testing to be effective.41
• Maximum interincisal opening with scale in place. • Swabbings of bite injury are done to recover trace evidence.
• If inanimate materials, such as food stuffs, are used for test It is recommended that such evidence be collected
bites, the results should be preserved photographically. whenever possible because it is not possible to inflict a bite
• The camera lens should be positioned at 90º to the surface injury without leaving biologic trace evidence. Harvey
of the skin that bears the wound. Several exposures should estimated that 0.3 ml of saliva is deposited when making a
be made at varying magnifications. bite mark. Ideally, the site should not be washed or
• The position of light source should be varied to illuminate contaminated by improper handling. Saliva swabbings or
the wound from multiple angles and to be certain that washings can be performed using a single cotton swab
highlights of any surface irregularities are documented in technique for recovering salivary amylase. If amylase is
relief. present in sample, ABO/ABH blood group classification
• All photographs must bear a legend containing the is undertaken. Another unbitten site must be swabbed for
necessary identifying information. a control sample.
• At least one photograph of the entire injured area should • Swabbings are done to collect DNA present in salivary trace
be taken with no scale in place to show that no injuries evidence. The double swab technique involves moistening
were purposefully obscured by the scale.38,45 the site with a swab moistened with sterile saline, then
• Additional visible light technique used to photo-document removing moisture with a second dry swab. Both swabs
bite mark injuries is referred to as alternative light imaging are sent for analysis. Research has shown recovery of biter’s
or visible light fluorescent photography. This technique is exfoliated epithelial cells from salivary trace evidence. The
used in specialized situations. cells can provide DNA for analysis.46
• Digital photography has many technologic improvements for
visible light documentation of bite mark injuries. Digital Impressions (Fig. 15.10)
camera captures image on a charge-coupled device (CCD) that
• Whenever required, make impressions of the bite mark.
can be transferred to a computer for processing and printing.
• Use ADA specification impression materials and make note
One advantage of this is that the image can be changed to
of it in report.
black and white, eliminating the step of taking separate black • Support should be provided for the impression material to
and white photographs, as must be done with film. accurately reproduce body contour.
• In addition to visible light photographic documentation, • Material used to produce cast should produce accurate
nonvisible light can be used to record bite mark injury. details in impression.
Shorter-wavelength UV light and longer wavelength • Master casts should be prepared using ADA specified Type
infrared (IR) light can be used. These techniques present IV stone.
the forensic dentist with another means to document and • Additional models when required, should be duplicated
preserve details of bite mark injury. from master casts using accepted duplication procedures.
• Epiluminescence microscopy is a dermatologic technique • Teeth and adjacent soft tissue areas of master cast should not
developed for evaluation of pigmented skin lesions. The be altered by carving, trimming, marking or other alterations.
application to bite mark analysis was discussed at the AAFS • One of the better impression materials to use is a vinyl
meeting in 1999. This technique, through rendering the polysiloxane (VPS) or similar low to medium viscosity
stratum corneum translucent, can aid in visualization and material. VPS and polythers or polysulfide impression
photographic documentation of bite marks.46 materials have been found experimentally to be extremely
accurate.47,48 The use of stiffer mixtures, such as heavy body
Salivary Swabbing or more firm type materials should be avoided as these distort
the injured area due to pressure used to apply the materials.
• After photographs are taken, salivary swabs should be done. • Alginate impressions usually are enough and packs of
• Whenever possible, salivary trace evidence should be powder, measuring cylinders, rubber bowls and spatulae
collected according to recommendations of the testing with full and part mouth trays, disposable syringes, etc. are
laboratory. required.
428 Essentials of Pediatric Oral Pathology

to the ring with cyanoacrylate adhesive, sutured to ring and

excised.
• The excised tissue is preserved in a tissue fixative, placed
in a sealed plastic bag and stored.
• The excised tissue can be transilluminated by shining a light
from the dermal side or inner aspect of tissue. Successful
transillumination studies can provide additional evidence
for use in bite mark analysis.46

COLLECTION OF EVIDENCE FROM SUSPECT

• Before collecting evidence from suspect, the dentist should
ascertain that the necessary search warrant, court order or
legal consent has been obtained and should make a copy
of this document as part of his/her records.
• At least two sets of dental casts should be fabricated. All
necessary information should be noted on the base of each
cast and a virgin set should be placed aside for safe-
keeping.46
• Test Bites or Sample Bites: It is useful to collect sample or
test bites of victim and suspect. Sample bites usually are
FIGURE 15.10: Making impressions of bite marks and
assembling casts for identification made in wax medium, such as Aluwax, pink baseplate wax
or Coprwax. Wax is bitten or indented with incisal edges
• Technique: Use the premixed materials in syringe with a of teeth because bite mark analysis involves position, shape
small orifice tip; they are injected directly onto the injured and alignment of incisal edges of biter’s teeth as they relate
site. Small tips used on the conventional impression to injury, care must be exercised to avoid biting all the way
syringes by dentists work very well. Material is injected through the wax.
slowly so that it flows into lower areas of the mark and • Styrofoam has been used as a test bite medium for the
does not trap air that will create a bubble or void in finished demonstration of the pattern of the incisal edges of teeth.
negative impression. • Test bites can be done with the stone models of the biter’s
• VPS materials are flexible when done in a thin sheet as teeth into the skin of a volunteer.46
would be used in a bite mark impression. So, the material
should be supported and reinforced by dental laboratory Recent Advances for Collecting Evidences
stone, acrylic dental impression tray material, thermoplastic
dental tray or fracture material and an orthopedic 1. Xeroradiography
thermoplastic mesh material (Hexcelite). 2. Transillumination
• To ensure a mechanical locking of stone and impression 3. Videotape analysis
material, staples are dipped into extra impression material 4. Superimposition technique
and placed on end in impression material on injury site. 5. Scanning microscopy
6. DNA fingerprinting.
Tissue Samples
ANALYSIS OF BITE MARKS
• In some instances, it may be necessary to excise the bite
mark from a deceased victim to facilitate the preservation Distortion arises from three factors:
of the evidence as well as to aid investigations relating a 1. Inherent distortion within the mark itself due to the
possible biter to the injury. mechanics of biting and physical and biological properties
• The bite mark and adjacent tissues are attached to a rigid of skin and underlying structures.
ring of plastic before excising to preserve orientation of 2. Distortion produced by trying to represent a three-
injured tissue. dimensional mark on a two-dimensional photograph.
• Plastic material used to stabilize tissue usually is cold-cure 3. Distortion produced in printing the negative to a life-size
acrylic or any thermoplastic material. The tissue is attached or preset magnification.49
Forensic Odontology in Children 429

COMPARISON TECHNIQUES Assisted Comparisons

Once all relevant data are assembled, comparison of the mark It should be noted that these methods are best used as
with the suspect’s dentition can begin. Comparison involves confirmation of the result rather than as the sole assessment.
not only use of superimposition techniques but also, the • Tsutsumi and Furukawa, 1984, described the use of a
collection of all evidence, including physical features as well measuring instrument called Vectron which is similar to a
as dynamics of the bite and the compatibility of the features dental surveyor and measures distances between fixed
with suspect’s teeth. points, angles and radii.
• Bang, 1976, used stereometric graphic analysis in mark
Life-size Comparisons investigation and produced a contour map of the features
Direct method: The models can be placed directly over the of the mark and suspect dentition. A visual representation
photographs and corresponding points can be demonstrated, is then available which is compared electronically with the
e.g. fit of the incisal edges. Advantage of this method is that mark in terms of longitudinal contours and topographical
models can be moved to demonstrate the dynamics of the bite features.
by showing slippage and scraping. • Light, electron and split-image microscopes can be used.
West and Friar, 1989, used direct model-to-victim Bang, 1976, and David, 1986, both described the use of
comparisons to demonstrate marks caused by slippage of the scanning electron microscope to good effect. Ligthelm
teeth, by placing the models directly over the breast of a et al 1987, have added the reflex microscope to the range
deceased victim and dragging the model across the skin to of microscopes in use. Various electronic techniques such
demonstrate how marks were produced in vivo. The entire as splitting the image, image stacking, bite edge enhance-
procedure was photographed, videotaped and produced as ment, etc. can be used.49
evidence. • Quantitative forensic evaluation of bite marks with the aid
Furness, 1968, described a method similar to that used by of a shape analysis computer program (“SCIP”—Shape
fingerprint officers, where lines are used linking various points Comparison Interactive Program) has been employed to
of correspondence between models and teeth. Advantage of this quantify the comparison in the form of Similarity Index (SI)
technique is that fine detail such as cervical margin indentations between offenders teeth and the bite marks produced on a
can be seen and compared. Disadvantage is that the technique standard flat wax form.51,52
cannot be used on grossly distorted marks.49 • Xeroradiography and transillumination, as described by
Indirect methods49: Indirect comparisons are made using Rawson et al and Dorion, 1987, respectively, are specialized
transparent overlays on which biting surfaces of the teeth are techniques that have been used in bite mark analysis. Both
recorded; these are placed directly over the marks on the these techniques require removal of bitten tissue. In
photograph. This method was first used by Sorup, 1924, and Xeroradiography, a layer of iodine contrast material is used
cited by Strom, 1963. Morgen, 1943, used photographs of and radiographs of the mark are taken. This is only applicable
models to produce overlays. The photographic production of when indentations are present. Transillumination utilizes the
overlays by various methods is the most reliable way of changed hemorrhagic structure of the tissue which is viewed
producing true reproductions of dentitions. under a light source that enhances the areas of varying
Use of photographic tracings (reverse negatives) can be hemorrhagic density. This is a useful technique when the
enhanced by using oblique lighting on model to highlight mark is very diffuse.
specific features. This model can be photographed with a scale • Biopsy and histological examination of bite marks is
at the occlusal surface and reverse negative produced, which confined to the deceased. Whittaker, 1989, refers to
shows not only biting surfaces but other anatomical details as methods of staining for iron, other blood products, elastic
well. fibers and collagen. He also established, using histology,
Other methods such as pressing the teeth into wax and whether mark was inflicted ante- or post-mortem. Glass et
photographing the indentations can produce accurate overlays. al 1980, used histology to demonstrate the presence of
The indentations can be enhanced if sprinkled with radiopaque micro-organisms and calculus within the lesion, thereby
powder and radiographed. Farrell et al 1987, used computed confirming that the mark was caused by teeth.49
axial tomography (CAT) scanning to produce overlays of • Experimental marks: The use of experimental marks to
dentition at varying depths, so that teeth not involved initially analyze a bite mark has been used as an aid to comparisons.
in the bite were shown at precise depth at which they began to They can be produced under varying circumstances.
be involved in the mark.50 Jakobsen and Keiser-Nielsen, 1981, stipulated that three
430 Essentials of Pediatric Oral Pathology

criteria should be met before undertaking experimental bite A suggested method for analyzing multiple cusp marks is
marks:53 as follows:
1. The mark has been established as having been made • Isolate and identify the shapes of marks, i.e. triangular
by human teeth. (canines and premolars), rectangular (incisors) or round
2. A reliable reproduction, e.g. a photograph, is available. (molar cusps).
3. The circumstances under which the bite was inflicted • Look for multiple involvement of same cusp.
are known. • Check for corresponding upper and lower tooth cusp marks.
Vale et al 1976, made a rubber model of the part that was • Check for the orientation of the object in the mouth.
bitten and produced similar marks using the suspect’s dentition • Enlarge photographs of cusp marks to two or three times
on the model.54 Various attempts have been made to produce life-size.
skin-like substance, such as baker’s dough (Buhtz and Erhardt, Histopathologic and clinical changes used to monitor the time
1938, cited by Jakobsen and Keiser-Nielsen, 1981) 53 or elapsed (aging) in skin injuries associated with bite marks:24
pigskin (Whittaker, 1975).55 • At 4–8 hours – PMNs (polymorphonuclear leuckocytes)
with peripheral front predominate. Clinical color is red-
BITE MARKS IN INANIMATE OBJECTS blue-purple.
Inanimate objects in which tooth marks can be made fall into • At 12 hours – PMNs predominate
three broad categories: • At 16–24 hours – Macrophages peak
1. Edible substances. • At 24–36 hours – PMNs peak and peripheral fibroblasts
2. Objects that are habitually chewed. are seen
3. Substances making contact with teeth in falls or skirmishes. • At 1–3 days – Central necrosis is seen
Whatever the substance, the overriding priority is • At 3+ days – Hemosiderin pigment is seen. Clinical color
preservation of the evidence.49 of the bite mark is green-blue.
• At 4 days – Collagen fibres predominate
Perishable Substances49 • At 4–5 days – Capillary growth predominates. Clinical
color of the bite mark is brown-yellow-green
1. Saliva swabbing.
• At 6 days – Lymphocytes peak at periphery
2. Object should be examined for fingerprints.
• At 10–14 days – Granulation tissue predominates. Clinical
Preservation of foodstuff is an urgent priority as all food
deteriorates once exposed to air. This is done by placing the color of the bite mark is tan-yellow.
substance in an airtight bag in a refrigerator or immersing in a
medium of 5 percent glacial acetic acid, 40 percent formal- FORENSIC ANTHROPOLOGY (FIG. 15.11)
dehyde solution and 70 percent ethanol in the ratio of 5:5:90 Forensic odontologists often work with specialists in other fields
(FAA solution which has preserved apples for 10 years). of forensic science. Besides the forensic pathologist, the forensic
anthropologist is perhaps the next most common collaborating
Non-Perishable Substances colleague. Forensic anthropology was not widely practiced on a
Non-perishable objects are dimensionally stable and may regular basis until after World War-II. The last 20 years have
reproduce marks. These objects are bullet, pipe stems, pencils seen rapid growth of this field in developed countries.
and soap.56 Forensic odontologists and anthropologists are both
Long-term preservation can be made by photography and primarily interested in the hard tissues of the body. The forensic
model preparation. Stoddart57 described a method for making anthropologist usually gives more attention to the osseous
models of perishable substances. For other inanimate objects, material rather than dental evidence. This does not mean that
modern two-stage crown and bridge impression materials give the anthropologist does not study the evolution and variation
satisfactory results. Models can be made using plaster, acrylic of human dentition nor does it imply that the dentist is unaware
or composite restorative materials. of the anatomy of the skull.

HABITUAL CHEWING MARKS49 ROLE OF FORENSIC ANTHROPOLOGIST

These are usually found on pipe stems, pencils and key bows. Forensic anthropologists may be asked to give very specific
Often cusp marks are present. Cusp marks made by human information such as an estimation of age at death or they may
canines, premolars and molars can be similar to those produced be asked to give all information that can be determined from
by dentition of dogs and cats. skeletal remains.
Forensic Odontology in Children 431

TECHNIQUES
Various techniques are used by the forensic anthropologist. It
is wise to discuss only those techniques that are useful to the
forensic dentist.
Remember, no single technique is always the best indicator
of age, sex or race. Multiple indicators are the key—not single
indicators or techniques.
First step is to confirm that whether remains are human and
how many individuals are present. Incorrect conclusions at this
point lead to embarrassment or worse.

AGE ESTIMATION FROM POINT OF VIEW OF

ODONTOLOGIST AND ANTHROPOLOGIST
Dental aging techniques based on formation of crown, eruption
and root tip completion are commonly used by dentists, but
FIGURE 15.11: The Department of Anthropology at occasionally age estimates derived from dental information may
the US National Museum in 1904
be in conflict with skeletal age determinations.
It is now common practice to derive age estimates using
When remains of deceased cannot be fully evaluated during one’s experience and judgment to arrive at the best estimate.
a normal postmortem examination by the pathologist, a forensic There is no agreement of anthropological age estimate and the
anthropologist is called for. These remains become unidentified dental age estimate especially in remains of teenaged victims.
due to partial or complete decomposition of soft tissues, burned Increased caution is indicated when very poor oral health
or mutilated by intent or accident. is present. In cases of very poor oral health, such as in transient
In cases where the body is intact, the specialist in human people, dental age may appear to be greater than normal.
osteology may be helpful in determining weapon characteristics Indeed, transients and migrant workers often show increased
from damage caused to the skeletal system. periarticular lipping in the joint and osteophytic lipping in the
The anthropologist may be able to establish identification vertebrae.58
by a number of means, including comparison of antemortem
and postmortem radiographs. The anthropologist must remove DEVELOPMENT OF HUMAN DENTITION BY
remaining soft tissue from the skeletal evidence before any SCHOUR AND MASSLER
analysis can be done. In a mass disaster this is not always The surveys of Schour and Massler, 1941,59 have their origin
possible because of the time factor, so the technique should be in the work of Logan and Kronfield, 1933,60 and are often
modified or limited to those that can be used with soft tissue cited (Figs 15.12A and B). In 1935, Schour and Massler
remaining. More radiography is necessary then. published a numerical development chart for the deciduous and
Soft tissue removal is accomplished after radiography by permanent teeth. The ‘Schour and Massler Development of the
boiling, preferably under an odor hood. Boiling of remains Human Dentition’ chart is periodically updated and is published
reduces the biohazard exposure for the staff of the life-size by the American Dental Association. Its appeal is
anthropology laboratory as well as those handling the remains obvious: both developing dentitions are displayed in situ, and
afterwards, such as forensic dentist, prosecutors and other it includes root resorption sequences for the deciduous teeth.
attorneys. As the drawings are life-size, it is easy to make direct
comparisons with either radiographs or individually removed
developing teeth. Criticisms are that the chart does not have
FORENSIC LABORATORY EQUIPMENTS
separate surveys for males and females, and the range about
Forensic anthropology laboratories that are properly equipped the mean ages from 2 years to 15 years is put at 6 months and
have odor hoods over stainless steel sinks, an X-ray machine, is thus too narrow.
radiograph dry processing and duplicating equipment, Ciapparelli, 1985, compared the Schour and Massler data
osteometric instruments, video superimposition equipment with a sample of schoolchildren.61 The mean ages from 4 years
and ample macrophotographic capabilities. Adequate space to 16 years corresponded well in the males, the females
for layout and storage of skeletal remains must be available.58 developing, on average 3 to 6 months earlier. The variation
432 Essentials of Pediatric Oral Pathology

FIGURES 15.12A and B: Developmental pattern of the child as reflected in the calcification pattern of teeth
A: Deciduous dentition, B: Permanent dentition. (Massler and Schour and Poncher)
Forensic Odontology in Children 433

was comparable in children 4 to 6 years old, but by the age of racial group in the world. The fact that a forensic skeleton has
12 years the male variation was double, and at 16 years treble, shovel-shaped incisors may suggest the possibility of Asian
that of the Schour and Massler variation. origins, but does not exclude Eastern Europeans, Africans or
In spite of these shortcomings, these surveys do have a even Polynesians. Similarly, large mesiodistal incisor diameters
useful role to play in forensic investigations. suggest African or Oceanian ancestry, but the range of variation
in all races precludes any absolute conclusion.
SEX DETERMINATION OF SKELETAL REMAINS The shape of the upper dental row (V-shaped in Whites,
U-shaped in Blacks and horseshoe-shaped in Mongoloids), the
Most experienced forensic anthropologists of their time, Wilton width and shape of the nasal aperture, the development of the
Krogman, could determine the sex of skeletal remains correctly nasal spine and the shape of the lower margin of the nasal
in about 80 percent of the cases when using the skull only, but
about 90 percent correctly when the pelvis was also used.62
Forensic dentists consider only skulls or jaws for sex
determination. The forensic anthropologist can usually bring
greater precision to gender determination by using the entire
skeleton remains.

COMPARATIVE FEATURES OF MALE AND

FEMALE SKULL
The male skull is larger than the female skull, has better marked
muscle attachment areas (nuchal and temporal lines); larger and
blunter mastoid processes, more superciliary arch development,
much blunter superior orbital margins, heavier zygomatic
arches, larger jaws and more sloping foreheads.
Males may show everted or neutral gonial angles, while
females usually show inverted or neutral gonial angles. The
anterior mandible (chin) may be squared or rounded in males,
but is usually pointed or rounded in females.58,63
Determination of sex in prepubescent remains is very
difficult. Puberty begins development of many secondary sexual
characteristics. One usually does not count the female and male
features in the immature skeleton to find which predominate.
If such a skeleton shows any male features, it is probably male.
There is less gender variation in the age of dental
development than there is in skeletal development. Males lag
behind females in skeletal maturation.58,63

RACE DETERMINATION (FIGS 15.13A TO C)

Race is a population concept. Races are “populations which
differ in the frequency of some genes” (Dobzhansky, 1950).64
Since individuals within that population vary considerably
in their genes, it is very difficult to assign any individual to a
particular race with any reliability. The racial identity that we
carry with us throughout our lives is a sociological label, not a
biological reality.
Obviously, there is considerable racial information in the
teeth, but individual teeth are seldom diagnostic. Shovel-shaped
maxillary incisors (trace, semi or full), for example are
considered a Mongoloid trait and are found in 100 percent of FIGURES 15.13A to C: Race determination by skull bones:
Aleuts studied, but they may be found in individuals from any A: Caucasoid; B: Negroid, C: Mongoloid
434 Essentials of Pediatric Oral Pathology

aperture, orbital and supraorbital shapes, relative length and Fragmented skulls and sometimes other bones must be
height of the braincase and the shape of the occipital bone are reconstructed to visualize perforations from gunshot wounds.
some of the cranial features that are useful in the identification Fractures must be recorded by photographs and diagrams.
of racial affinity. Alveolar prognathism, defined by Patterns of radiating fractures can demonstrate the type of
anthropologists as the anterior projection of the jaws, is also a weapon used, the site or sites of impact, and the number of
good trait for race determination.63,58 wounds or the sequence of wounds. Bevelling on gunshot
wounds will frequently show direction of the projectile.
HEIGHT DETERMINATION Firearm caliber cannot be accurately determined from the
wounds. Skulls fractured in blunt trauma may show patterns
Height can be estimated after age, gender and race of the
characteristic of the weapon (Maples, 1986).66 Hammers, pry
remains are determined. For adults, the Trotter and Gleser
bars, jack handles, handgun butts and muzzles, beverage
(1952) formulae are probably the best. Separate formulae are
bottles, axe handles and many other common tools and weapons
used for each of six major long bones.65 In case of the humerus,
often leave very distinctive patterns. The damage or perforation
radius, ulna, femur and fibula, the measurement used is the
maximum length. The maximum length, not the bicondylar is the result of the maximum cross section of the weapon that
length, of the femur is used. The tibia is measured from the has passed through the surface of the bone.
most superior point on the lateral condyle to the most inferior Incised wounds (cuts or stabs) are commonly found on
point on the medial malleolus, parallel to the shaft of the bone. bones. Scalpels should not be used to open the body near any
Always use the formula, which has lowest standard error of skin perforated by decomposition or wounds. During
the estimate for the best estimate of stature. macerations, no metal instruments should be used that may
After age, sex, race and height are determined, possible damage the bone.
identifications are usually proposed by investigators assigned In examining bones for incised marks, every bone surface
to the case. The identification must be proven biologically; by should be examined with magnification (X2 to X3 is ideal).
fingerprints, a good dental record showing multiple restorations Anterior surfaces of cervical vertebrae, inferior margins of the
or extractions, antemortem/postmortem radiograph mandible, ribs, sternum, clavicles and posterior portions of
comparisons, complex medical histories involving the skeleton thoracic and lumbar vertebrae should receive particularly
or teeth or sometimes, by superimposition. careful attention. When bones are found disarticulated, it is
The investigators must secure all useful records on the important to place damaged bones back into articulated
deceased. Obviously, dental records and radiographs must be positions with adjacent bone when trauma is considered. If a
sought, but medical radiographs, medical records, DNA stab wound severed a transverse process of a thoracic vertebra,
reference samples from appropriate relatives and photographs one would expect damage to the rib at that location if injury
(especially “mug shots”) must be secured. If any ridge detail occurred around the time of death with bones in their proper
remains in soft tissue, fingerprints may still be useful. If hair anatomical positions.
is still present in the remains, reference samples may be Dismemberment or decapitation will inevitably leave
obtained from the combs, hairbrushes or handbags of the evidence on skeletal remains. The type of saw used for
missing person. dismemberment is important. The grooves made from the saw
At some point during the analysis of the remains, trauma teeth may travel in straight parallel lines (band or other power
analysis is necessary. While skeletal damage may be obvious, saw), straight but overlapping lines (hand saws), curved lines
sometimes skeletal evidence of perimortem injury is very (oscillating or rotating circular blade) or the very coarse cuts
subtle, such as fractures of the alveolar margins, chipped teeth, of a chainsaw. Chainsaws may leave chainsaw oil on the bones
longitudinal enamel fractures or damage to the very thin cortex and hacksaw blades may leave distinctively colored paint on
of articular bone, such as the mandibular condyles. The remains the cut bone surfaces.
where skin is no longer intact should be radiographed prior to Bodies are cremated with various internal accruements such
maceration to locate metal fragments from knife tips or debris as prosthetic joints, surgical staples, vascular clips, surgical wire
from gunshot wounds. Lead debris may still be found on bones and wire catheters, dental prosthesis, orthopedic pins, nails,
that have remained exposed to the surface environment for plates and screws. So before beginning analysis, the expert may
more than a decade. separate the remains by particle size using proper analytic
If it becomes necessary to prove the metal debris located sieves.
radiographically on bones is in fact lead, proton induced X- The skeletal evidence is rich in information. The forensic
ray emission (PIXE) analysis may be used. Physics and Nuclear anthropologist, working as part of a team of forensic
Departments at universities with an accelerator can do this pathologists, odontologists, and other forensic scientists, can
nondestructive analysis. greatly add to the results of the analysis. Indeed, the results of
Forensic Odontology in Children 435

the scientists working as a team and discussing the case at all rag or even in the folds of plastic bags that have been used to
stages of the investigation greatly exceed what each expert can wrap the corpse. Pieces may be identifiable and reconstruction
do individually.58 may be possible, but a single cusp from the deciduous second
molar can be extraordinarily difficult to distinguish from the
THE FETAL SKELETON (FIG. 15.14) single cusp of a deciduous canine.
In practice, dental evidence is unlikely to be complete
A few circumstances come to forensic odontologists when he enough for accurate age determination of the fetus for the
has to work on a fetal skeleton, particularly calcified tooth caps following reasons:
of facial skeleton, so this topic is included here. • Tooth caps are small and fragile.
Calcified tooth caps can be retrieved from the whole fetus • They may be difficult (or impossible) to gather or identify.
throughout the second and third trimesters of pregnancy. These • This problem is often made worse by poor scene-of-crime
tooth caps can be examined, identified and compared with technique.
standard charts or the whole dentition can be collected, dried
to constant weight and from the mass of calcified tissue present
ASSESSMENT OF SKELETAL REMAINS 67
and reference to appropriate charts, the age of the fetus can be
estimated. This dental evidence that is often missing is firstly In the absence of teeth, what can be inferred or deduced from
because the anatomists rarely wait for their specimens to putrefy the bony part of the skeleton?
before examination. Putrefaction is important because it First thing is to identify whether the bones are of a human
liberates the tooth caps from the forming alveoli of the jaws. or not. If cranial bones are present, the answer is obvious.
It is often assumed that the deciduous teeth remain safe in their When the head is missing then it becomes more difficult. The
crypts even after decay of the soft tissue; unfortunately, this is bones of small animals (rat, rabbits) or large birds such as
not so. Consequently, when fetus putrefies, the tooth caps fall chickens are often mistaken by the ordinary person for fetal
from the jaws.67 or neonatal human remains. It is worth remembering that bird
These fragments are small; for example, the calcified part bones have to be extremely light to be compatible with flight
of the first permanent molar is only about the size of a pinhead and therefore have large medullary cavities, whereas the
at birth and weighs about 1 mg. Such pieces can be easily be bones of small mammals are usually mature and have
lost, to the soil or lost in investing materials such as clothing, completely fused epiphyses. Human bones of similar size
really only comprise the diaphysis and therefore have no
articular appendages.
All bones collected should be identified as far as possible,
preferably by comparison with a reference collection of
prepared skeleton of known sex and maturity. The bones should
then be laid out in their approximate anatomical relationships
on black card or filter paper together with a measuring scale.
Particular care should be taken to assign a side or establish
the handedness of bones, since to find two left zygomatic bones
or clavicles even of the same size has obvious implications. If
there is a body of a single fetus wrapped in a plastic bag and
discovered later after putrefaction has destroyed the soft tissues
all that may be necessary to put the contents of the bag through
a sieve aided by copious quantities of water. The sieve mesh
size should not be too fine or too coarse. It is better to use a
medium sized domestic kitchen sieve.
If the soft tissues are intact, the bones can be examined in
two ways. The first method is radiographically. If the object-
film distance is no closer than one meter, the inevitable
magnification inherent in all radiographic techniques is
negligible and can be discounted when bone images are
measured to determine bone size and hence fetal maturity. It
is not always possible to lay all bones flat against the film.
FIGURE 15.14: Fetal skeleton Articulated cranial bones are impossible to see individually
436 Essentials of Pediatric Oral Pathology

without the superimposition of other structures. The dimensions The Temporal Bone
of head also introduce an unacceptable degree of distortion into
It is a very useful predictor of age too, even without measure-
any radiographic images for accurate measurements to be made.
ment. It develops from three separate elements; (a) squamous
This leads to the second method of examination: the freeing
part of vault of skull, (b) petrous part of base of skull; and
of the bones from their investing soft tissues and their (c) the tympanic ring (delicate circular bone). At seven lunar
subsequent measurement directly using vernier calipers. months of gestation, all three elements are still separate bones.
Determination of fetal age is made from estimates of fetal The fusion of the squamous part with the tympanic ring occurs
size. The parameter of size of the fetus that most closely very soon after this date and may be taken as a morphological
correlates with gestational age is length measured from the crown sign indicating the viability of the fetus. The fusion of all three
of the head to the heel of the foot with the body laid out straight. elements is complete by 10 lunar months and may be taken as
The relationship is summarized by Haase’s rule.68 Until the fifth a sign of a full-term fetus.
lunar month of gestation, the fetal body length in centimeters
can be obtained by squaring the number of months of pregnancy Mandible
and after this time by multiplying the months by five.
Fazekas and Kosa, 1978, found that all bones of the fetus This is perhaps the easiest bone for dentists to identify. This is
could be measured and by the construction of regression curves represented by a separate bone on each side of the face until
for each bone, the size of the bone could be related to the size well into the first year of postnatal life. In fetal period each
—and hence age—of the corpse as a whole.69 This should not half of the mandible is always a separate bone. In the early
be misinterpreted to mean that all bones are of similar value stages of its formation, the mandible is a remarkably straight
to the forensic investigator. Consider ribs or cranial vault, these bone. It is easy to measure from the articular head of the
are important but are very fragile and destroyed by burial or condyle to the symphyseal face at the anterior extremity of the
even drying from water. bone. This length in millimeters is always equal to the total
crown-heel body length in centimeters: for example, when the
WHICH ARE THE BEST BONES TO LOOK FOR? mandible is 50 mm in length the fetus is 50 cm long and that
occurs at full term. The length of the mandible is one-tenth of
The requirements are that the bones should be unequivocally the body length during the whole period of intrauterine life.
identifiable, robust, easily and reproducibly measurable The same relationship holds for radius. Another useful bone
between anatomical landmarks that are easy to find. with direct relationship is the maxilla, which is one-twentieth
the length of the body.
Zygomatic Bones One avenue of research is to examine bone microstructure
and chemistry in addition to overall bone and organ
This fulfills all the criteria but are probably not ideal because
morphology. Preliminary work in these areas has already shown
the relationship between their size and that of the entire fetus
age-related differences.
is not a simple one.
CHRONOLOGY OF DENTAL DEVELOPMENT AND
Basilar Part of the Occipital Bone AGE ASSESSMENT
An excellent bone for this purpose is the basilar part of the In forensic dentistry, age estimation is not only one of the
occipital bone. There is only one of these in the body; it looks standard requests upon the discovery of a dead body, but is
like nothing else; it is robust and easily measured; moreover, also critical if identity is in question in living individuals.
its proportions change with age. If the bone is measured simply Age calculation by means of tooth development and
in the midline from the front (spheno-occipital synchondrosis) particularly the root formation of third molars has already often
to the notch at the back (foramen magnum) and then at right proved to be effective in determining an individual’s chrono-
angles to this between the lateral tubercles to determine its logic age. Other methods for age calculation using skeletal
greatest width, then the relationship between these two radiology (hand-wrist, sternoclavicular joints, long bones and
measurements alone has great significance. At the age of vertebrae) or secondary sex characteristics have at least
7 to 7½ lunar months after conception, the width exceeds the comparable limitations.70
sagittal length; prior to this the relationship is the reverse. In Dental age is estimated by comparing the dental develop-
many countries, seven lunar months (28 weeks) is considered ment status of a person of unknown age with published dental
in law to be the age at which a fetus becomes incipiently (or development surveys. By doing so, a likely chronologic age
potentially) viable in its own right. for that individual can be deduced. There are also more subtle
Forensic Odontology in Children 437

ways in which dental development data can be applied. The membrane, the stratum intermedium and stellate reticulum. By
premineralization and mineralization stages in dental 12 weeks, the incisors and canines consist of a single conical
development are well established. The incremental pattern of soft tissue cusp. During the 12th week, the first and second
mineralization is subject to periodic disturbances, which affects primary maxillary and mandibular molars initiate their soft
the developing teeth in a unique way. Birth, diseases, drug tissue crown development. Kraus and Jordan summarize the
intake, dietary changes and the uptake of certain chemical early dental developmental horizons for the primary molars.72
elements can all cause changes in the incremental pattern. These At 12½ weeks a distinct bulge occurs on the mesiobuccal
changes can be detected and, in some instances, be retrospec- portion of the occlusal aspect of the soft tissue crown. This
tively linked to a specific time or event during the individual’s marks the initial appearance of the mesiobuccal cusp. Initial
life.71 calcification of the first mesiobuccal cusp occurs at 14½ weeks
prior to distal cusp development.
BIOLOGICAL AND CHRONOLOGICAL AGE The chronologic specificity for the appearance of the
successional permanent tooth anlage ranges from about the 20th
Chronologic age: This is the time from birth to the present
week in utero for permanent incisors to the 10th postnatal
for a living individual as measured in units of time. However,
month for the second premolars. Proliferative growth by distal
in the majority of cases in forensic, an individual’s biological
extensions from the second primary molar dental laminae
age is mostly determined.
begins about the fourth month of fetal life. The permanent
Biological age: This is an age estimate based on the state of molars arise directly from these extensions.
development of the remains quoted in years and months. The first permanent molars begin their soft tissue
Radiographic technique is unable to address the development during the fourth intrauterine month; the first
discrepancy between biological and chronological age. As a postnatal year for the second permanent molars; and the fourth
result radiographs reflect only chronological age. They cannot or fifth year for the third permanent molars.73
take into account any acceleration or retardation in the rate of
growth or maturation. Histology is the more sensitive technique, Calcification
early mineralization being detectable up to 12 weeks before it
There is usually an orderly sequence of cusp calcification for
becomes apparent on a radiograph. As such, throughout
primary molars with variations occuring primarily with the
development, the radiographic appearance of a tooth will be
distal and distolingual cusps of second primary molars.
largely behind its anatomical appearance by several months. A
The calcification sequences for the primary and permanent
radiograph of the first permanent molar will not show
cusps, crowns and roots are presented by Schour and Massler.
significant calcification until approximately six months of age
This chart is based on radiographic data. Necessarily, the
despite beginning of calcification prenatally.
prenatal timing, in many instances, conflicts with the vital
But, studies are conducted with both methods, histologic
staining data of Kraus and Jordan, which give earlier times for
and radiographic and errorless techniques are being discovered
the onset of calcification. (Alizarin red detects initial calcifica-
with the help of newer technologies like advanced computer
tion prior to radiographic observations). Prenatal crown age
softwares.
assessments, based on this chart, must be used with caution.
Age estimation in forensic dentistry by using important dental
According to the chart, the primary teeth cusp tips begin to
developmental milestones
calcify during the 15th week in utero. In spite of the early
Dentition’s maturation is well documented and broadly
aforementioned problems in initial crown age assessment, the
categorized by three basic processes:
crown completion and root formation data in the Schour and
1. Proliferative growth
Massler chart are remarkably accurate. Accordingly, coronal
2. Calcification of crown and root
calcification is not completed until the end of the first year and
3. Eruption.
root calcification for primary teeth begins about the third to fourth
postnatal month and does not end until about the third year.
Proliferative Growth
Permanent tooth crowns (first molars) initiate calcification
The primary dentition is initiated at approximately 10 weeks during the ninth fetal month (Alizarin red). Radiographic
in utero by a downgrowth of epithelial cells from the oral evidence shows calcification initiating at birth. Coronal
epithelium in the anterior regions of the jaws to form dental calcification for permanent teeth continues to about 15 to 16
lamina. During the 11th intrauterine week, the differentiation years when third molar crowns complete calcification.
of the tooth bud tissues occurs, i.e. the dental papilla, the outer Permanent root development begins about the fourth year and
enamel epithelium, the inner enamel epithelium, the performed continues to about 21 to 23 years.73
438 Essentials of Pediatric Oral Pathology

Eruption visual assessment of teeth present in the mouth and requires

little expertise or equipment. The emergence of deciduous teeth
The third phase of dental development is eruption. Akin to
is under genetic control and is relatively regular, commencing
eruption is the shedding of primary teeth during the mixed
approximately at six months and completing by 2½ years. On
dentition period. Shortly after the primary roots are completely
the other hand, emergence patterns of permanent teeth are under
formed primary root resorption begins. It is important to the influence of the intraoral environment, being affected by
distinguish between incompletely formed roots and resorbed infection, arch space, and premature tooth loss.74
roots, indeed a difficult task at times. Therefore, evaluation of radiographs to assess tooth
Primary teeth without successors may or may not show root calcification is a much better alternative, since:
resorption. If roots are partially resorbed, it is likely that Calcification of teeth can be observed from the radiographs
ankylosis will occur. Ankylosed teeth do not maintain capacity for a period of several years.
for vertical development. Consequently adjacent teeth continue It is not altered by local factors such as lack of space,
developing vertically leaving the ankylosed teeth in a relatively infection, etc., and the study of tooth calcification also lets us
submerged position. assess age at periods when no emergence takes place (2½–6
From the aforementioned discussion, it should be apparent years and 12 years). 74
that age can be assessed with a high degree of accuracy up to
20 years of age using events in the developing dentition. INCREMENTAL LINES OF CEMENTUM
Caution must be exercised when using third molar data from
the Schour and Massler chart. Little information is provided Evaluation of annual incremental lines of cementum is one of
after 15 years of age. the potentially valuable methods for biological age estimation
More importantly, the developmental events may be in forensic anthropology. Biological age was estimated by
influenced by racial and/or socioeconomic criteria. Therefore, pooling the number of lines counted and the average age of
each forensic dentist should have a reference data base peculiar tooth eruption. It was found that number of lines strongly
to the population.73 correlates with chronologic age. Factor of sex has no significant
influence on the number of lines. This method is suitable for
Age estimation is an important subspeciality of forensic
forensic anthropology and digitalized system enhances the
sciences. It also has application in living individuals whose
count and provides better result.75
chronologic age is under dispute. Dental age estimation makes
There is no known reason for this pattern of incremental
use of morphologic, radiographic, histologic and biochemical
lines. There is some speculation regarding changes in quantities
methods to examine age dependent changes in teeth.
of mineral salts laid down and/or differences in growth rates
of cemental tissues at different times of the year, resulting in
AGE ESTIMATION IN PRENATAL, NEONATAL
appearance of concentric lines in cementum which can be
AND EARLY POSTNATAL CHILD
equated with years.75
The primary tooth germ begins to form at seven weeks in utero A reduced rate of cementum apposition was observed in
(IU), and the enamel formation of all deciduous teeth is usually the elderly. Also, maxillary teeth had more cementum on the
complete by the first year. Among the permanent teeth, the first lingual than on the vestibular surfaces. A tendency was noted
molar shows germ formation first at about 3½ to 4 months IU. for less cementum to occur in women than in men and on teeth
The age estimation in this group of individuals can be very removed from deceased persons or extracted for pathologic
accurate. It makes use of histologic techniques, which enable reasons. The cementum thickness might give a significant
observation of tooth mineralization up to 12 weeks before it is contribution to statistical methods of age assessment.76
actually apparent on radiographs. The neonatal ‘line’ is
considered as an indicator of birth. Bowers attributes its AGE ESTIMATION FROM DENTIN
formation to the slowing down of enamel prism growth rate,
Bang and Ramm were the first to use dentine translucency alone
thus “creating an apparent line of demarcation”. Estimating age
for estimating age and reported significant increase in root
in this age group may have legal implications in cases that
translucency with age. Root dentine starts to become translucent
involve feticide and infanticide.
during the third decade of life beginning at the apex and
advancing coronally. The alteration is believed to be due to
AGE ESTIMATION IN CHILDREN AND
decreased diameter of dentinal tubules caused by increased
ADOLESCENTS
intratubular calcification. Hence, difference in refractive indices
Nystrom and colleagues consider the estimation of age by study between intratubular organic and extratubular inorganic
of tooth emergence as a convenient clinical method. It involves material is equalized, resulting in increased translucency of the
Forensic Odontology in Children 439

affected dentine. For example, Solheim suggested translucency, Moorees, Fanning and Hunt81
length (mm) or area (mm2) may be measured on intact or
This method used and tabulated data thus making the survey
sectioned teeth.6
of Moorees et al 1963, a useful development standard for the
The amount of secondary dentin in a tooth has been used
forensic dentist. Another study by Anderson et al. 1976, using
as one of several parameters in methods for age estimation.77
a different sample and radiographic view but the same
Dentin deposition has been measured according to various
mineralization criteria, allows a useful comparison between two
scoring systems and the Pearson correlation coefficient with geographically close but different population groups.
age has been found to be approximately 0.6. A tendency was
also observed towards reduced speed of secondary dentin Advantage: Between these two studies, development data are
formation in the elderly and in women.78 available for each individual developing permanent tooth.
The amount of sclerotic root dentine increases with age, Moorees et al defined 14 stages of mineralization for
proceeding from the apex towards the crown. There are obvious developing single and multirooted permanent teeth. The results
optical changes in the tissue, which becomes translucent (dentin are expressed as the mean age of attainment for each of the 14
is normally opaque). Therefore, sclerosis of root dentin could stages for developing teeth studied, ± 2 standard deviations.
be a reliable indicator of age in anthropological studies of The crown formation stages show less variation when compared
human remains. Qualitative analysis was performed with with root formation stages; this should be kept in mind when
polarized light microscopy and measurements were made with accuracy is of prime importance. The earliest age in surveys is
a quote 2D, x, y viewer and on digital images.79 6 months, and the data include development of the third
Age related changes in tooth color have been described mandibular molar.81
previously. Age can be estimated by objective measurement Points of forensic interest from this study are:
of dental color using spectroradiometry. The determination of • Sex difference in crown formation stages is minimal.
dentin color by spectroradiometry is a potentially useful Differences of development in sexes are apparent in root
objective method to estimate age in forensic studies in formation, where females developed ahead of males.
combination with other methods.80 • The teeth emerge clinically at R ¾ stage.
• Greatest sexual dimorphism is expressed in the mandibular
REVIEW OF THE VARIOUS DEVELOPMENT canine, females being up to 11 months in advance of males
SURVEYS with this particular tooth.71
Dental developmental surveys are packaged in different ways; Anderson, Thompson and Popovich82
they give us two types of information:
• The sequence of developmental events; Anderson et al 1976, applied the criteria of Moorees et al in a
• The timing at which these events are said to occur. longitudinal study using cephalometric radiographs. All
Dental age assessment may be described as an ‘educated developing teeth were rated including third molars.
approximation’ based on developmental surveys that are According to Fanning, 1961, interobserver variation is
designed to solve clinical rather than forensic problems. Some found in as many as 27 percent of sample ratings, but is usually
of these surveys, which can be used for forensic purposes, are confined to plus or minus one stage. This fact highlights one
reviewed below. of the difficulties in using a rating system with so many stages,
which might lead to a debate in court as to where one stage
COMMONLY USED SURVEYS begins and another ends. A modified presentation package using
the 14 stage mineralization criteria is given.71
Schour and Massler
The surveys of Schour and Massler, 1941, have their origin Demirjian, Goldstein and Tanner83
in the work of Logan and Kronfield, 1933. In 1935, Schour In this method of Demirjian et al (1973), each stage of
and Massler published a numerical development chart for the mineralization is given a score, which provides an estimate
deciduous and permanent teeth. The Schour and Massler of dental maturity on a scale 0 to 100. The mathematics and
surveys appeal is obvious; both developing dentitions are rationale used to calculate the scores are those of Tanner et
displayed in situ, and it includes root resorption sequences for al 1983.84 Eight stages of dental development are depicted
the deciduous teeth. As the drawings are life-size, it is easy to by published radiographic surveys, supplemented with a
make direct comparisons with either radiographs or individually wri tten descri ption of th e lim its of each stage of
removed developing teeth. This has been discussed earlier. mineralization which are clearly defined and do not require
440 Essentials of Pediatric Oral Pathology

absolute measurements. This system is most highly developed • Root resorption at the apex (R).
of all dental age surveys. • Dentine translucency (T).
There are two options when using this method, one For each of these regressive changes or variables, different
involving the rating of seven mandibular teeth (Demirjian, scores ranging from 0 to 3 were assigned. This meant attrition
1978) and the second using four mandibular teeth (Demirjian could have any one of four scores (A0, A1, A2, or A3) and
and Goldstein, 1976). Missing teeth from one side can be similar one of the four scores for other variables. Adding the
substituted by those from the other side. If first molar is absent, allotted score for each variable (e.g. A3 + S2 + P2 + C1 + R2
the central incisor can be substituted (Demirjian, 1978). Griffin + T1 = X), a total score was obtained. It was found that an
and Malan (1987) reviewed this system and produced a pocket increase in the total score (X) corresponds to an increase in
version of the system, which can be used in field. age. Age was estimated using the formula
Data obtained using Demirjian system indicates that dental
Age = 11.43 + 4.56X.
developmental differences between males and females are not
usually apparent until age of 5 years. Inter-observer variation Maples and Rice found that there was a miscalculation in
with this system is as high as 20 to 25 percent.71 the above formula, and proposed a correction:
Age = 13.45 + 4.26X.
Disadvantages: It does not include the developing third
molars. The reliance on mandibular tooth rating can be a However, the improvements made by Johanson are widely
problem in skeletonized remains where often the mandible accepted. Instead of the original four grades (0–3), he proposed
disarticulates and is lost. Griffin and Malan, 1987, concluded seven grades (0, 0.5, 1, 1.5, 2, 2.5, and 3). Using these seven
that eight-stage system was less prone to examiner error.71 grades, the formula:
This method is also applicable for Indian Population as in Age = 11.02 + (5.14A) + (2.3S) + (4.14P) + (3.71C) +
agreement with Nanda and Chawla, 1966 and Demirjian, 1971, (5.57R) + (8.98T) was suggested.
who reported that dental formation of French-Canadian children
closely correlated with Lucknow (India) children.85 Van der Linden and Duterloo
Demirjian and coworkers developed an age estimation
method that made use of a scoring system.86 The development Moving away from radiographic surveys, van der Linden and
of seven mandibular teeth on the left side was divided into eight Duterloo, 1976, produced an atlas of development of human
stages each. These stages were named ‘A’ to ‘H’. While the dentition spanning the period from birth to completion, at yearly
third molars were not used in the original method, a recent study intervals. The atlas is a photographic presentation supplemented
by Chaillet and Demirjian accommodates them. Each tooth is with diagrams.71
assigned a ‘maturity score’ that corresponds to its develop-
mental stage. The maturity score assigned for each tooth is Third Molar Development
added and a total maturity score obtained. This total maturity
The tooth most commonly missing in man is the third
score is then plotted on a chronological ‘age conversion table’.
permanent molar.40
Bearing in mind the differences in dental development between
After the age of approximately 14 years, the third molar is
boys and girls, the authors provided separate maturity scores
the only developing tooth and assumes great forensic
and age conversion tables for both sexes.
significance. At or after this age, whether clinically visible or
not, its status should always be investigated. Its morphology
Gustafson
and clinical presentation make it the most variable of the
Gustafson, 1966, developed a comprehensive compact chart permanent teeth. Its development is prone to wide temporal
of dental development for forensic use, using four stages of fluctuations. The data of Moorees et al 1963, indicate that the
development, the data being derived from a comprehensive crown formation stages of this tooth display less variation than
review of the literature. The chart does not differentiate between the root formation stages.81 Anderson et al 1976, held an
males and females.71 opposite view.82 Ciapparelli, 1985, found an almost constant
In 1950, Gosta Gustafson developed a method for age variation of about ± 1 year for both root development and
estimation based on morphological and histological changes crown formation stages.61 Therefore, it cannot be assumed that
of the teeth.87 This assessed regressive changes such as: variation during development of this tooth is greater than that
• Amount of occlusal attrition (A). of other developing teeth. Johanson, 1971, Harris and Nortje,
• Coronal secondary dentine deposition (S). 1984, and van Heerden, 1985, all used a similar five-stage
• Loss of periodontal attachment (P). system of root development in their studies of root formation
• Cementum apposition at the root apex (C). for the third molar. Their findings indicated insignificant
Forensic Odontology in Children 441

differences in third molar development between the four • Consistency and reproducibility of techniques and results
quadrants and no sexual difference.71 are more important than artistic composition.
Gleiser and Hunt method consists of a 10-stage develop- • The use of Kodak processing laboratories for color film is
ment scale, with three stages for crown formation and seven an assurance of quality control, standardization and legal
for root development. When Mesotten et al used Gleiser and acceptability.
Hunt method, the results revealed standard deviations similar
to those reported in comparable publications and even to those PHOTOGRAPHY IN DENTAL IDENTIFICATION 45
calculated with other skeletal age calculation techniques.88
Types of Photography
FORENSIC PHOTOGRAPHY • Visible light photography:
Accurate photography is crucial to forensic investigation as a — Visible light color photography
means of documenting evidence. The need to photographically — Visible light black and white photography.
record injury patterns on skin is paramount to the odontologists • Alternate light imaging and fluorescent techniques:
and pathologists.
The process of photographically recording images on film, • Nonvisible light photography:
videotape or other media occurs through the capture of — Reflective long-wavelength ultraviolet (UV)
electromagnetic radiation (light) of specific wavelengths. photography
Photographic films are sensitive to light wavelengths in the — Infrared photography.
range of 250 to 900 nm. Visible light comprises the range of
electromagnetic radiation from 400 to 760 nm. Most modern Suggested Photographs to be Taken89
camera equipment and film is specifically designed to record In situ full body photographs: These views could be important
images seen in the visible range of light. since they provide a version of the untampered evidence and
It is also possible to record images specifically illuminated ensure that any subsequent manipulation has not concealed or
in the shorter ultraviolet range (210 to 400 nm), and longer destroyed information. These photographs are of greatest
infrared range (750 to 900 nm). Wavelengths of lights that are concern when one attempts to reconstruct mass disasters and
outside visible range of electromagnetic radiation are commonly bite mark cases.
referred to as ‘nonvisible light’. Photography using nonvisible
light requires special techniques to record the injury. It also Full face and profile photographs: These photographs would
requires minor focusing adjustments called as ‘focus shifts’. be useful in instances in which body is well preserved and is
When light strikes skin, four basic events occur (1) reflec- identifiable visually.
tion (2) absorption (3) transmission and (4) fluorescence. These Photographs of anterior teeth: Close-up photographs of the
events occur simultaneously when light strikes human skin. exposed anterior teeth are important in case the only ante-
Depending on the wavelength of the source of the incident light mortem record that materializes is a photograph of the victim
and the configuration of the camera, it is possible to record, smiling. Also, postmortem photographs are useful when
individually, any of the four reactions of skin to light energy.45 carbonization of anterior teeth threatens their preservation.
Photographs of resected jaw specimens or skeletonized
GENERAL CONSIDERATIONS jaws: If jaws are removed, following series should be made:
In almost all photographic work intended for legal purposes, • View of all recovered portions of maxilla and mandible;
the following considerations apply: • Close-up occlusal view of mandible;
• Photographs should be made in duplicate, anticipating that • Close-up palatal view of maxilla;
one set may have to be surrendered as permanent courtroom • Anterior view of articulated jaws (if possible);
evidence. • Left and right lateral views of articulated jaws (if possible);
• Bracketing the exposures is prudent and will buffer slight • Any additional photographs necessary to demonstrate a
exposure errors due to mechanical failure or human peculiarity not seen to advantage in other views.
miscalculation.
• Using varied perspectives to make photographs is advisable, Photographs of antemortem radiographs: These photographs
particularly because the electronic flash may produce are helpful when radiographic duplicates are unavailable or
unpredictable and often unpleasant reflections. when originals cannot be retained by the forensic dentist.
• The recording of data (date, time, location, case number, Photographs of antemortem and postmortem radiographs
camera, lens, aperture, film, light source, subject distance) mounted in tandem on same exposure provide an effective tool
helps reconstruct cases. to demonstrate concordance in court.
442 Essentials of Pediatric Oral Pathology

to each other and placed close to and in plane of the bite mark
ensuring that they are not obscuring any part of mark.90

Camera Positioning
Flat surface photography: The film plane is made parallel to
the scale by placing the angle setter on the scale and rotating
the adjustable vial until the bubble is centered. The angle setter
is then moved back to the camera and the camera adjusted
laterally until the bubble is centered—the film plane is now
FIGURE 15.15: A single lens reflex 35 mm camera with 100 mm
parallel to the scale. To position the lens perpendicular to the
focal length lens, exchangeable extension rings and ring-flash is scale, the angle setter is turned through 90 degrees and replaced
usually adequate on the scale.
Curved surface photography: Each dental arch is photographed
Photographic Equipment:90 individually. When photographing curved surfaces, the above
procedures are followed, in addition to which a 5-degree
1. 35 mm SLR cameras (Nikon 35 mm or digital cameras)
adjustment is made, using the angle setter graduations, so that
(Fig. 15.15)
the lens is aimed from outside of the arch at a point in the central
2. Electronic flash gun
part of the curve. It is important to note that the overlays are
3. Cable release
life-size and distortion-free and that mismatches with
4. Metal measuring tape
photographs are due to photographically induced errors where
5. Angle setter
they are not due to tissue behavior.
6. Beanbags
7. Grey scale card/color/monochrome patches
Recording the Images
8. Framing attachment/lens
9. Tripod Films: The light sensitive materials used in forensic dental
10. Film (color and monochrome) photography are a matter of personal preference.90
11. Lens hood Film manufacturers have designed photographic films that
12. Rigid scales (photographic)/ABFO #2 record light wavelengths from 250 to 700 nm. Special infrared
13. Disc (photographic) films are available that can record photographs taken in light from
14. Writing materials. 250 to 900 nm. Correct film speed must be determined. Films
come with a rating, referred to as the ASA/ISO number, which
Procedure serves as an indicator of the amount of light energy necessary to
Consent: It is of vital importance that consent, in writing, is properly expose the film. The higher the ASA/ISO number, faster
obtained from the subject prior to the commencement of any the film, in other words less light is needed to expose an image.
photography. Digital photography utilizes a special computer hard disk
in the camera that stores the images as digital information.
Method These images can be later written to a CD-ROM for storage.
Advantage is that the image can then be immediately viewed
A comfortable working position for both victim and on a computer monitor or printed on a color printer.45
photographer is achieved by supporting the part being
photographed on strategically placed beanbags. The camera Light Source Placing
must be mounted on a sturdy tripod with pan and tilt facility
and lateral arm. The flashgun is connected to the camera such To achieve optimum reproduction of bite marks, which leave
that the shutter speed is set at appropriate synchronization. very slight impressions, the light source must be positioned at
For identification purposes a general photograph is taken 45-degree angle to the mark. The aim is to achieve oblique
of the entire subject, this includes facial features and gray scale illumination of the irregularities in the tissue surface.
card with color or monochrome patches attached.
Bite mark photographs without any scale present to prove Bite Marks in Food and Other Material
that nothing of evidential value will be obscured when scales Photography of bite marks on inanimate objects is taken with
are applied. Rigid self-adhesive scales are introduced for a rigid scale. It is important to obtain a control sample of food
remaining photographs. These are assembled at right angles and to swab the bitten food for saliva traces. It is also important
Forensic Odontology in Children 443

that food be removed from the refrigerator early enough, prior necessary to competently document these injuries with visible
to photography, to prevent condensation of water vapor spoiling and nonvisible light is one of the great challenges in forensic
the detail in the picture. dentistry.

Photographic Reproduction of Overlays ROLE OF FORENSIC DENTISTRY IN

MASS DISASTERS
The photographic transparency technique is the most accurate
method of producing overlays for comparison purposes. The The world has experienced a plethora of mass disasters in
production of the transparency involves emphasizing the incisal recent years—hurricanes, earthquakes, floods, typhoons, mud
and cuspal edges of the teeth of a duplicate set of dental models slides, transportation mishaps, aircraft mishaps, fires, volcanic
with a fine, indelible black marker pen. In order to produce high- eruptions, industrial accidents, terrorist acts and armed
contrast line transparency, the models are placed on an conflicts. In addition to naturally occurring disasters, as the
illuminated light box and a photograph taken using a lithographic world population increases, technology expands and life
emulsion. A rigid scale must be placed in the plane of the teeth becomes more complicated. As a result, we may anticipate more
to ensure accurate 1:1 reproduction.90 untoward events generated by people. The role that forensic
dentistry and the forensic science community play in such
Photography of Faces, Dental Models, disasters varies with jurisdiction throughout the world.91
Dentures and Dissected Jaws In five mass disasters handled by the British team between
1985 and 1989, involving over 1000 victims, dentistry
Photographs of faces for record purposes should be taken prior
contributed to identification in just over 80 percent.42
to the commencement of any postmortem dissections. It may
Recently, 26 December 2004 saw devastation and loss of
be necessary to photograph faces, dental models, dentures or
life around the Indian ocean. Till date 1,474 deceased have
dissected jaws as part of the evidential photography to be
been identified. Dental comparison has been the primary
produced in court. A rigid scale is fixed in plane of the teeth
identifier in 79 percent of cases and a contributor in another 8
when taking photographs. Color photographs show the colors
percent, a total of 87 percent.92
of the teeth, gums and restorations, often assisting with
The identification of human remains in mass disasters
identification of the victim. Photographs of dentures, especially
allows surviving family members to go through the grieving
those that show laboratory or identification marks are most
process; place legal, business and personal affairs in order, and
useful and can be included in the professional publications
continue with the processes of life.
when requesting information as to the identity of the owner.
DISASTER SITE MANAGEMENT91
Photographs of Teeth
In the recovery phase of the disaster, a 500 to 2000-ft security
For record purposes it may be necessary to photograph anterior
cordon should be established outward from the disaster site.
teeth or intraoral pictures. Use of ring flash is more convenient
An entry control point for the cordon is placed. An access list
in these cases and for intraoral photography a set of front
is developed and all personnel within the cordon must wear
reflecting mouth mirrors is necessary. A conventional 35 mm
identification badges. These actions are necessary to protect
camera may be used for this purpose.90
disaster evidence, staff, and bystanders. The team should
carefully search the site for human remains or fragments (Fig.
Handling of Photographic Evidence
15.16). Fragmentation and commingling of remains will be a
The photographs documenting a victim’s injuries may become major problem with high impact force mishaps. A body recovery
part of the legal system and, as such, are subject to ‘chain of tag should be placed on each specimen. The numbering system
evidence’ rules. This requires accountability as to what should be as simple as possible. The use of computers and bar
individuals had possession of the evidence from the time it was code readers can be most helpful from the beginning in remains/
collected until it is marked and introduced into the legal system. fragments tracking, documentation and management.
Categorizing system usually consists of numbers or letters Once the body has been prepared, it is placed in a recovery
which include the case number, as well as an identifying mark bag for transport to the identification center. The remains should
of the forensic photographer. This can be his or her initials or be removed to the Identification Center and refrigerated to
signature, so that photographs can be identified as originals approximately 37º F as soon as possible. Once all the remains
and the chain of evidence maintained. Photography is one of have been removed from the site, it is wise to repeat the
the important protocols of forensic dentistry. Developing skills overlapping ground search. Surprisingly, large volume of body
444 Essentials of Pediatric Oral Pathology

Section’ has found a name in an article of clothing or a military

identification tag on a particular body. The status of human
remains may be thought to be in three categories:
(1) positive identification; (2) findings consistent between
antemortem and postmortem records; and (3) unidentified.
Registrar, computer services, communication services, public
affairs, mortuary service, security and support services are
critical sections supporting the Identification Center chief and
the various forensic science sections. The source of information
emanating from the Identification Center must be either the
Identification Center chief or the Public Affairs officer.

Forensic Dentistry Section

A Forensic Dentistry Section should be an integral part of the
organization of the disaster Identification Center. The Forensic
FIGURE 15.16: Collection of evidence
at the site of mass disaster Dentistry Section should be divided into three subsections and
should be headed by a team chief responsible to the Identifica-
tion Center chief. The role of Forensic Dentistry Section chief
fragments and personal effects will be located with the second is that of manager, facilitator, coordinator and spokesperson
effort. for the section.
Postmortem dental examination and dental radiology
DISASTER MANAGEMENT
subsection:91 The role of this section is straightforward and
In most jurisdictions, there are three or four legally admissible uncomplicated of the three subsections. A forensic photo-
methodologies used to identify human remains: grapher should be available to provide photographic support
1. Visual identification. during postmortem examinations. The first part of dental team,
2. Fingerprints or footprint identification. made up of oral surgeons, accomplishes the necessary facial
3. Dental identification. dissection to allow the oral cavity to be visualized and
4. DNA evidence. radiographed. After facial dissection has been accomplished,
Among these methods visual identification is least reliable the remains are moved to the next area within the postmortem
due to subjective factors and stressful situation in which a dental examination subsection for dental radiographs.
relative or friend is placed. Fingerprint mode is long respected, A full-mouth postmortem dental radiographic series is
but is subject to availability of antemortem prints on file. Dental accomplished utilizing a portable dental X-ray unit. A 50-kVP
identification is also subject to available antemortem dental dental radiographic instrument is quite adequate since normal
records and radiographs. DNA comparison is legally admissible exposure factors can generally be reduced by one quarter to
in a growing number of jurisdictions. If immediate relatives of one half when working with severely burned victims because
the victim are available, the genetic makeup may be established of water loss from the body.
without an antemortem DNA record of the victim being on file. The use of automatic dental X-ray film processors with
daylight loading hoods is recommended. Dental radiographs
FORENSIC IDENTIFICATION CENTER provide objective evidence that is essential within the scientific
ORGANIZATION93 community and in court. Flat plate radiographs of the entire
Past disaster experiences have shown that a multidisciplinary body, although expensive, provide significant medical and
disaster Identification Center with a forensic sciences anthropological evidence and are an excellent tool for locating
processing line is highly effective in the identification of large personal effects and dental fragments in commingled, charred
numbers of human remains. The success of this organization remains.
is keyed to preplanning, cooperation and smooth communica- After a thorough cleaning of dental structures with a dilute
tions between all sections. Data flow between all sections must bleach solution, a team of three dentists, or two dentists and a
be orderly to enable each section to capitalize on what other dental hygienist or assistant, charts all dental evidence on a
sections have learned. For example, time can be saved if the postmortem dental record form. The universal numbering
Forensic Dentistry Section knows that the ‘Personal Effects system is preferred because it is simple in nature and is easily
Forensic Odontology in Children 445

computerized. The use of a fiber-optic light is invaluable in out as positive identification. After the case has been signed
the examination process. out as positive identification, the antemortem and postmortem
The examiner begins by evaluating both tooth #1 and dental records and associated evidence should be combined
associated radiographs. The second dentist on the examination with the summary sheet into a single completed file.
team evaluates tooth #1 and confirms the findings of the tooth A Computer-Assisted Postmortem Identification System
#1 and all three team members confirm the charting. Errors (CAPMI) designed by Colonel Lewis Lorton and Mr. William
are corrected in a legally acceptable fashion. H Langley, represents latest development of an identification
Confusion should be avoided on extracted or congenitally system utilizing today’s technology.94 The system provides
missing teeth. In some cases, prosthetic appliances may have efficient management of data. It permits comparing statistically
been specifically marked for identification. It is wise to solicit, significant and related antemortem and postmortem records.
from the victim’s dentist or family, study models or extra Due to its inherent high selectivity, it can also overcome many
prosthetic appliances, which may be available. Such evidence human errors in the database.
is important in providing antemortem data regarding ridge The CAPMI System, Northwestern University system and
shape/size, rugae patterns and general oral anatomy. the Mertz and Purtilo system are examples of available
computer programs in forensic dentistry.
Antemortem record subsection:91 Dentists, hygienists, skilled
dental assistants and dental investigators can effectively operate CONCLUSION
this subsection. The task of this section will always be most
difficult in the entire forensic dentistry arena. Each practitioner has a responsibility to understand the forensic
It is clearly necessary to reduce all antemortem dental implications associated with the practice of his or her
evidence to a single antemortem dental record form in order profession. This understanding should include more than ethics
to provide a composite antemortem picture. Photographs of and jurisprudence, which were traditionally the only aspects
of a dentist’s knowledge of the law. Appreciation of forensic
possible fatalities are often received by this subsection. They
dental problems permits clinicians to maintain legally
may be of value in demonstrating malocclusions and other
acceptable records and assist legal authorities in the
facial and dental anatomy. The anthropologist and forensic artist
identification of victims of disasters and crimes.
will also find these photographs of value. Carefully mark the
Dental surgeons around the world can contribute
reverse of these photographs with the name and address of the
significantly in solving crime, identifying victims of mass
provider.
disaster and understanding of prehistoric man by giving due
Postmortem dental records/computers and comparison importance to the subject of Forensic Odontology. The science
subsection:91 This is the third part of the Forensic Dentistry needs necessary inputs in the form of formal training, job
Section. Postmortem dental records and completed antemortem allocation and research by the profession if it is to play an active
composite dental records are forwarded to this section. The role in criminology and allied fields. Utilization of computers
task of this section is comparison of antemortem and and newer technology hold promise for the advancement of
postmortem examination and radiographic findings. This Forensic Odontology as an area of specialization that needs to
section must also keep abreast of the findings of all forensic be taken up on a priority basis.
sections within the Identification Center and apply their findings
in dental comparison process. REFERENCES
If the subsection operates without a computer, the size of
1. Oscar Amoedo. Dental Cosmos 1897;39(11):905-12.
the section is dependent on the number of fatalities, since there
2. American Board of Forensic Odontology, Inc. Diplomates
is a requirement to place all postmortem dental records face- Reference Manual, Oct 2009.
up on tables in numerical order for a comparison with the 3. Kempe CH, Silverman FN, Steele BF, Droegemueller W, Sweet
antemortem composite dental records as they are received with HK. The battered child syndrome. JAMA 1962;181:17-24.
the postmortem dental records placed on the table. Once 4. Standish SM, Stimson PG. The scope of forensic dentistry. Dent
significant points of comparison are noted, radiographs of the Clin North Am 1977;21(1):3-5.
respective records can be reviewed and a possible identification 5. Definition of Forensic Odontology, http:11 encyclopedia
established. Time is a major consideration. A dental laburlawtalk.com/Forensic Odontology.
identification form which summarizes the identification data 6. Luntz LL. History of forensic dentistry. Dent Clin North Am
can be completed at this time. Only after all sections have 1977;21(1):7-17.
presented their evidence and all inconsistencies have been 7. Stimson PG. Oral Autopsy Protocol. Dent Clin North Am 1977;
explained should the Identification Center chief sign the case 21(1):177-80.
446 Essentials of Pediatric Oral Pathology

8. Burgman G. Examination of the identified body: mortuary 27. Schwartz TR, Schwartz EA, Mieszerski L, McNally L,
procedures. In: Clark DH (Ed). Practical Forensic Odontology. Kobilinsky L. Characterization of deoxyribonucleic acid (DNA)
Oxford, Butterworth-Heinemann, 1992;53-66. obtained from teeth subjected to various environmental
9. Acharya AB, Sivapathasundharam B. Forensic Odontology. conditions. J Forensic Sci 1991;36(4):979-90.
Shafer’s Textbook of Oral Pathology. 5th Edn., Elsevier, 2006; 28. Adams DE, Presley LA, Baumstark AL, Hensley KW, Hill AL,
1199-1227. Anoe KS, et al. Deoxyribonucleic acid (DNA) analysis by
10. Wagner GN. Scientific methods of investigation. In: Stimson restriction fragment length polymorphisms of blood and other
PG, Mertz CA (Eds). Forensic Dentistry. Boca Raton: CRC body fluid stains subjected to contamination and environmental
Press, 1997.p.1-36. insults. J Forensic Sci 1991;36(5):1284-98.
11. Acharya AB, Sivapathasundharam B. Chptr. 21. Forensic 29. Gaensslen RE, Berka KM, Grosso DA, Ruano G, Pagliaro EM,
odontology. In: Rajendran, Sivapathasundharam (Eds). Shafer’s Messina D, et al. A polymerase chain reaction (PCR) method
textbook of oral pathology. Elsevier. 5th edn, 2007.p. 1211. for sex and species determination with novel controls for
deoxyribonucleic acid (DNA) template length. J Forensic Sci
12. Fairgrieve SI. “SEM analysis of incinerated teeth as an aid to
1992;37(1):6-20.
positive identification.” Journal of Forensic Sciences. 1994;39
(2):557-65. 30. Fukushima H, Hasekura H, Nagai K. Identification of Male
Bloodstains by dot hybridization of human Y chromosome-
13. Smith BC. A preliminary report: proximal facet analysis and
specific deoxyribonucleic acid(DNA) probe. J Forensic Sci
the recovery of trace restorative materials from unrestored teeth.
1988;33(3):621-7.
J Forensic Sci. 1990;35(4):873-80.
31. Pretty IA, Sweet D. A look at forensic dentistry-part 1: The role
14. Fulton BA, Meloan CE, Finnegan M. Reassembling scattered
of teeth in the determination of human identity. Br Dent J 2001;
and mixed human bones by trace element ratios. J Forensic Sci.
190(7):359-66.
1986;31(4):1455-62.
32. Sweet D, Hildebrand D. Recovery of DNA from human teeth
15. Kido A, Kimura Y, Oya M. Transferrin subtyping in dental
by cryogenic grinding. J Forensic Sci 1998;43(6):1199-1202.
pulps. J Forensic Sci 1993;38(5):1063-7.
33. Neiders ME, Standish SM. Blood group determinations in
16. Lopez-Abadia, Ruiz de la Cuesta. A simplified method for forensic dentistry. Dent Clin North Am 1977;21(1):99-111.
phenotyping alpha-2-HS-glycoprotein. J Forensic Sci
34. Tate RJ. Facial injuries associated with the battered child
1993;38(5):1183-6.
syndrome. Br J Oral Surg 1971;9(1):41-5.
17. Smith FP. The detection of drugs in bloodstains. In: Lee HC,
35. Becker DB, Needleman HL, Kotelchuck M. Child abuse and
Gaennslen RE, editors. Advances in forensic science.
dentistry: Orofacial trauma and its recognition by dentists. J Am
Biomedical Pubs, Foster city, CA, 1985:235-50.
Dent Assoc 1978;97(1):24-8.
18. Lysell L. Plicae palatinae transversae and papilla incisive in man: 36. Vale GL. Dentistry’s role in detecting and preventing child
a morphologic and genetic study. Acta Odontologica abuse. In: Stimson PG, Mertz CA (Eds). Forensic Dentistry.
Scandinavia 1955;13(Suppl 18). Boca Raton: CRC Press, 1997.p.161-84.
19. Thomas CJ, Kotze TJvW. The palatal ruga pattern: a new 37. Rao NG, Tandon S. Child Abuse. In: Shobha Tandon, editor.
classification. J Dent Assoc S Afr 1983;38:153-7. Section 15, Chapter 15.2, Forensic Paedodontics, Textbook of
20. Kapali S, Townsend G, Richards L, Parish T. Palatal rugae Paedodontics. Paras Medical Publisher, Hyderabad, AP, 1st
patterns in Australian aborigines and Caucasians. Aust Dent J Edn., 2001.p.706-12.
1997;42(2):129-33. 38. Barsley RE. Forensic and legal issues in oral diagnosis. Dent
21. Limson KS, Julian R. Computerized recording of the palatal Clin North Am 1993;37(1):133-56.
rugae pattern and an evaluation of its application in forensic 39. MacDonald DG. Bitemark recognition and interpretation. J
identification. J Forensic Odontostomatol 2004;22(1):1-4. Forensic Sci Soc 1974;14(3):229-33.
22. Ball J. The current status of lip prints and their use for 40. Gravely JF. A radiographic survey of third molar development.
identification. J Forensic Odontostomatol 2002;20(2):43-6. Br Dent J 1965;119(9):397-401.
23. Tsuchihashi Y. Studies on personal identification by means of 41. Stimson PG, Mertz CA. Bite mark technique and terminology.
lip prints. Forensic Sci 1974;3:233-48. In: Stimson PG, Mertz CA, editors. Forensic Dentistry. Boca
24. Herschaft EE. Forensic Dentistry. Neville’s textbook of oral and Raton: CRC Press, 1997.p.137-60.
maxillofacial pathology. 2nd edn., Elsevier Saunders, 42. Clark DH. Bite mark examination procedures: victims and
2002.p.763-83. suspects. In: Clark DH (Ed). Practical Forensic Odontology.
25. Ubelaker DH, Bubniak E, O’Donnell G. Computer assisted Oxford, Butterworth-Heinemann, 1992.p.128-37.
photographic superimposition. J Forensic Sci 1992;37(3): 43. Webster G. A suggested classification of bite marks in food stuffs
750-62. in forensic dental analysis. Forensic Sci Int 1982;20(1):
26. Weedn VW. DNA identification. In: Stimson PG, Mertz CA, 45-52.
editors. Forensic dentistry. Boca Raton: CRC Press, 1997.p. 44. American Board of Forensic Odontology. In: Guidelines for bite
37-46. mark analysis. J Am Dent Association 1986;112:383-6.
Forensic Odontology in Children 447

45. Wright FD, Golden GS. Forensic photography. In: Stimson PG, 64. Dobzhansky Theodosius, Dunn LC. Heredity, Race and Society
Mertz CA (Eds). Forensic dentistry. Boca Raton: CRC Press, (Hardcover-Jan 1, 1950).
1997.p.101-36. 65. Trotter M, Gleser GC. Estimation of stature from long bones.
46. Wright FD, Dailey JC. Human bite marks in forensic dentistry. American Journal of Physical Anthropology 1952;10:463-514.
Dental Clinic North Am 2001;45(2):365-97. 66. Maples WR. Trauma analysis by the forensic anthropologist.
47. Ciesco JN, Malone WF, Sandrik JL, Mazur B. Comparison of Radiology 1986;54:314-39.
elastomeric impression materials used in fixed prosthodontics. 67. Clement JG, Kosa F. The Fetal skeleton. In: Clark DH, editor.
J Prosthet Dent 1981;45(1):89-94. Practical forensic odontology. Oxford: Butterworth-Heinemann
48. Lacy AM, Fukui H, Bellman T, Jendrensen MD. Time- 1992.p.43-52.
dependent accuracy of elastomer impression materials. Part II: 68. Biggerstaff RH. Craniofacial characteristics as determinants of
Polyether, polysulfides and polyvinylsiloxane. J Prosthet Dent age, sex, and race in forensic dentistry. Dent Clin North Am
1981;45(3):329-33. 1977;21(1):85-97.
49. Ciapparelli L, Hughes P. Bite marks in tissue and in inanimate 69. Fazekas I, Kosa K. Forensic fetal osteology. 1st Budapest
objects: analysis and comparison. In: Clark DH. Ed. Practical Hungary Akademiai Kiado Publishers, 1978.p.232-77.
Forensic Odontology. Oxford: Butterworth-Heinemann,
70. Mesotten K, Gunst K, Carbonez A, Willems G. Chronological
1992;149-77.
age determination based on the root development of a single
50. Farrell WL, Rawson RD, Steffens RS, Stephens D. Computeri- third molar: A retrospective study based on 2513 OPGs. J
zed axial tomography as an aid in bite mark analysis: a case Forensic Odontostomatol 2003;21(2):31-5.
report, 1987;32(1):266-72
71. Ciapparelli L. The chronology of dental development and age
51. Nambiar P, Bridges TE, Brown KA. Quantitative forensic
assessment. In: Clark DH (Eds). Practical Forensic Odontology.
evaluation of bite marks with the aid of a shape analysis
Oxford: Butterworth-Heinemann, 1992:p,22-42.
computer program: Part 1; The development of “SCIP” and the
72. Kraus BS, Jordan RE. The human dentition before birth. Lea
similarity index. J Forensic Odontostomatol 1995;13(2):18-25.
and Febiger, Philadelphia 1965.
52. Nambiar P, Bridges TE, Brown KA. Quantitative forensic
evaluation of bite marks with the aid of a shape analysis 73. Biggerstaff RH. Forensic dentistry and the human dentition in
computer program: Part 2; “SCIP” and bite marks in skin and individual age estimations. Dent Clin North Am 1977;21(1):
foodstuffs. J Forensic Odontostomatol 1995;13(2):26-32. 167-74.
53. Jakobsen JR, Keiser-Nielsen S. Bitemark lesions in human skin. 74. Nystrom M, Peck l, Kleemola-Kujala E, Evalahti, Kataja M.
Forensic Sci Int 1981;18(1):41-55. Age estimation in small children: Reference values based on
54. Vale GL, et al. Unusual three-dimensional bite mark evidence counts of deciduous teeth in Finns. Forensic Sci Int 2000;110:
in a homicide. Case, J Forensic Sci 1976;21:642. 179-88.
55. Whittaker DK. Some Laboratory studies on the accuracy of bite 75. Bojarun R, Garmus A, Jankauskas R. Microstructure of dental
mark comparison. Int Dent J 1975;25:166-71. cementum and individual biological age estimation. Medicina
(Kaunas) 2003;39(10):960-4.
56. Corbett ME, Spence D. A forensic investigation of teeth marks
in soap. Br Dent J 1984;157(8):270-1. 76. Solheim T. Dental cementum apposition as an indicator of age.
Scand J Dent Res 1990;98(6):510-9.
57. Stoddart TJ. Bite marks in perishable substances. A method of
producing accurate permanent models. Br Dent J 1973;135(6): 77. Luntz LL. History of forensic dentistry. Dent Clin North Am
285-7. 1977;21(1):7-17.
58. Maples WR. Forensic anthropology. In: Stimson PG, Mertz CA, 78. Solheim T. Amount of secondary dentin as an indicator of age.
editors. Forensic dentistry. Boca Raton: CRC Press, 1997.p. 65- Scand J Dent Res 1992;100(4):193-9.
82. 79. Micheletti Cremasco M. Dental histology: Study of aging
59. Schour, Isaac, Massler M. The development of the human processes in root dentine. Boll Soc Ital Biol Sper 1998;74
dentition. J Am Dent A 1941;28:1153-60. (3-4):19-28.
60. Logan WHG, Kronfeld R. Development of the human jaws and 80. Martin-de las Heras S, Valenzuela A, Bellini R, Salas C, Rubino
surrounding structures from birth to the age of fifteen years. J M, Garcia JA. Objective measurement of dental color for age
Amer Dent Ass 1933;20:379. estimation by spectroradiometry. Forensic Sci Int 2003;132(1):
61. Ciapparelli L. An assessment of dental age in Essex school 57-62.
children using panoral radiographs with forensic application. 81. Moorrees CFA, Fanning EA, Hunt EE, Jr. Age variation of
Diploma in Forensic Odontology, London Hospital Medical formation stages for ten permanent teeth. Journal of Dental
College, London, 1985. Research 1963;42(6):1490-1502.
62. Krogman WM. The human skeleton in forensic medicine. 82. Anderson DL, Thompson GW, Popovich F. Interrelationships
Postgraduate Medicine 1955;17:A48-A62. of dental maturity, height, and weight from 4 to 14 years. Aust
63. Biggerstaff RH. Craniofacial characteristics as determinants of Orthodont J 1976;4:87-104.
age, sex, and race in forensic dentistry. Dent Clin North Am 83. Demirjian A, Goldstein H, Tanner JM. A new system of dental
1977;21(1):85-97. age assessment. Hum Biol 1973;45:211-27, 6.
448 Essentials of Pediatric Oral Pathology

84. Tanner, et al. Assessment of skeletal maturity and prediction of 89. Bernstein ML. The application of photography in forensic
adult height (TW2 method). Academic Press (London, New dentistry. Dent Clin North Am 1983;27(1):151-70.
York), 2nd edn, 1983. 90. Summers R, Lewin D. Forensic Dental Photography. In: Clark
85. Hegde RJ, Sood PB. Dental maturity as an indicator of DH, (Ed). Practical forensic odontology. Oxford: Butterworth-
chronological age: Radiographic evaluation of dental age in 6 Heinemann, 1992:188-205.
to 13 years children of Belgaum using Demirjian methods. J 91. Morlang-II WM. Mass disaster management. In: Stimson PG,
Indian Soc Pedod Prev Dent 2002;20(4):132-8. Mertz CA, (Eds). Forensic dentistry. Boca Raton: CRC Press,
86. Demirjian A, Goldstein H. New systems for dental maturity based 1997:185-216.
on seven and four teeth. Ann Hum Biol 1976;3(5):411-21. 92. James H. Thai tsunami victim identification overview to date.
87. Gustafson G. Age determination on teeth. J Am Dent Assoc J Forensic Odontostomatol 2005;23(1):1-18.
1950;41:45-54. 93. Vale GL, Noguchi TT. The role of the forensic dentist in mass
88. Mesotten K, Gunst K, Carbonez A, Willems G. Chronological disasters. Dent Clin North Am 1977;21(1):123-35.
age determination based on the root development of a single 94. Lorton L, Rethman M, Friedman R. The Computer-Assisted
third molar: A retrospective study based on 2513 OPGs. J Postmortem Identification (CAPMI) System: A computer-based
Forensic Odontostomatol 2003;21(2):31-5. identification program. J Forensic Sci 1988;33(4):977-84.
Forensic Odontology in Children 449

Annexure 1: Postmortem dental examination

DENTAL EXAMINATION OF DECEASED

To be completed for each deceased aircrew member and where indicated for deceased passengers:

Rank Name Number

Crew Duty (or passenger seating) Date

Body number Location after crash

Time (a) of crash (b) death (c) autopsy

Dentures

Soft tissue examination

Remarks and identification criteria

Australian armed forces form for

the dental examination
of deceased aircrew and passengers
450 Essentials of Pediatric Oral Pathology

Annexure 2: Antemortem and Postmortem Dental Records

Health commission of New South Wales Dental Identification Record

A. Case History:
B. Examination Date: –––––––––/–––––––––––/––––––––––– Site of Examination:............................................................................
Requested by:...........................................................
Examiner’s Name and Position: ................................................................................................................................................................
Post-mortem dental Post-mortem dental
Yes No Yes No
photographs: X-rays

Part 1 of the Healt commision of NSW

dental identification record
D.

Upper right Upper left Lower left Lower right

1.8 2.8 3.8 4.8

1.7 2.7 3.7 4.7
1.6 2.6 3.6 4.6
1.5 2.5 3.5 4.5
1.4 2.4 3.4 4.4
1.3 2.3 3.3 4.3
1.2 2.2 3.2 4.2
1.1 2.1 3.1 4.1

SUBSTITUTE QUADRANT NUMBER FOR DECIDUOUS TEETH

E. Appliances (prosthetic including bridgework, orthodontic, surgical)
Maxilla—
Mandible—
F. Ante-mortem records obtained from:
Name:.................................................................. Address:..........................................................................................................
Describe ante-mortem records:....................................................................................................................................................
Additional Notes on Back: Yes No

Signature of Examiner

Part 2 of the Health Commission of

NSW Dental Identification Record
Appendices 451

Appendices

NORMAL LABORATORY VALUES

FOR CHILDREN

Sr. No. Age range Normal values Sr. No. Age range Normal values

BLOOD CHEMISTRY 14. Iron Newborns 110-270 mcg/dL

Infants 30-70 mcg/dL
1. Albumin 0-1 year 2.0-4.0 g/dL Children 55-120 mcg/dL
1 year to adult 3.5-5.5 g/dL Adults 70-180 mcg/dL
2. Ammonia Newborns 90-150 mcg/dL 15. Iron binding Newborns 59-175 mcg/dL
Children 40-120 mcg/dL Infants 100-400 mcg/dL
Adults 18-54 mcg/dL Adults 250-400 mcg/dL
3. Amylase Newborns 0-60 units/L 16. Lactic acid, lactate 2-20 mg/dL
Adults 30-110 units/L
17. Lead, whole blood <10 mcg/dL
4. Bilirubin, conjugated, Newborns <1.5 mg/dL
direct 18. Lipase Children 20-140 units/L
1 month to adult 0-0.5 mg/dL Adults 0-190 units/L

5. Bilirubin, total 0-3 d 2.0-10.0 mg/dL 19. Magnesium 1.5-2.5 mEq/L

1 month to adult 0-1.5 mg/dL
20. Osmolality, serum 275-296
6. Bilirubin, unconjugated 0.6-10.5 mg/dL mOsm/kg
indirect
21. Osmolality, urine 50-1400
7. Calcium Newborns 7.0-12.0 mg/dL mOsm/kg
0-2 years 8.8-11.2 mg/dL
2 years to adult 9.0-11.0 mg/dL 22. Phosphorus Newborns 4.2-9.0 mg/dL
6 weeks to 3.8-6.7 mg/dL
8. Calcium, ionized, whole 4.4-5.4 mg/dL 19 months
blood 19 months to 2.9-5.9 mg/dL
3 years
9. Carbon dioxide, total 23-33 mEq/L
3-15 years 3.6-5.6 mg/dL
10. Chloride 95-105 mEq/L > 15 years 2.5-5.0 mg/dL

11. Cholesterol Newborns 45-170 mg/dL 23. Potassium, plasma Newborns 4.5-7.2 mEq/L
0-1 year 65-175 mg/dL 2 days to 4.0-6.2 mEq/L
1-20 years 120-230 mg/dL 3 months
3 months to 1 year 3.7-5.6 mEq/L
12. Creatinine 0-1 year 0.6 mg/dL 1-16 years 3.5-5.0 mEq/L
1 year to adult 0.5-1.5 mg/dL
24. Protein, total 0-2 years 4.2-7.4 g/dL
13. Glucose Newborns 30-90 mg/dL > 2 years 6.0-8.0 g/dL
0-2 years 60-105 mg/dL
Children to adults 70-110 mg/dL 25. Sodium 136-145 mEq/L
452 Essentials of Pediatric Oral Pathology

Sr. No. Age range Normal values Males (mg/dL) Females (mg/dL)

26. Triglycerides Infants 0-171 mg/dL 5-9 10-14 15-19 5-9 10-14 15-19
Children 20-130 mg/dL years years years years years years
Adults 30-200 mg/dL
90th 183 191 183 189 191 198
27. Urea nitrogen, blood 0-2 years 4-15 mg/dL percentile
2 years to Adult 5-20 mg/dL
95th 186 201 191 197 205 208
28. Uric acid Male 3.0-7.0 mg/dL percentile
Female 2.0-6.0 mg/dL
TRIGLYCERIDES
ENZYMES
50th 48 58 68 57 68 64
1. Alanine aminotransferase 0-2 months 8-78 units/L
percentile
(ALT) (SGPT) > 2 months 8-36 units/L

2. Alkaline phosphatase Newborns 60-130 units/L 75th 58 74 88 74 85 85

(ALKP) 0-16 years 85-400 units/L percentile
>16 years 30-115 units/L
90th 70 94 125 103 104 112
3. Aspartate Infants 18-74 units/L percentile
aminotranferase (AST)
95th 85 111 143 120 120 126
4. Serum glutamic Children 15-46 units/L percentile
oxaloacetic transaminase Adults 5-35 units/L
(SGOT) LDL-C

5. Creatine kinase (CK) Infants 20-200 units/L 50th 90 94 93 98 94 93

Children 10-90 units/L percentile
Adult male 0-206 units/L
Adult female 0-175 units/L 75th 103 109 109 115 110 110
percentile
6. Lactate dehydrogenase Newborns 290-501 units/L
(LDH) 1 month to 110-144 units/L 90th 117 123 123 125 126 129
2 years percentile
>16 years 60-170 units/L
95th 129 133 130 140 136 137
percentile

HDL
SERUM LIPID CONCENTRATIONS
5th 38 37 30 36 37 35
BY AGE AND GENDER percentile

Males (mg/dL) Females (mg/dL) 10th 43 40 34 38 40 38

percentile
5-9 10-14 15-19 5-9 10-14 15-19
years years years years years years 25th 49 46 39 48 45 43
percentile
TOTAL CHOLESTEROL
50th 55 55 46 52 52 51
50th 153 161 152 164 159 157
percentile
percentile
Adapted from American Academy of Pediatrics Committee on
75th 168 173 168 177 171 176
Nutrition, “Lipid Screening and Cardiovascular Health in Childhood”.
percentile
Pediatrics, 2008;122:(1)198-208.
Appendices 453

THYROID FUNCTION TESTS contd...

Sr. Hormone Age Concentration

Sr. Hormone Age Concentration No.
No.
5. TSH Cord 3-22 micro international units/mL
1. T4 (thyroxine) 1-7 day 10.1-20.9 mcg/dL 1-3 day < 40 micro international units/mL
8-14 day 9.8-16.6 mcg/dL 3-7 day < 25 micro international units/mL
1 month to 1 year 5.5-16.0 mcg/dL > 7 day 0-10 micro international units/mL
> 1 year 4.0-12.0 mcg/dL

2. FTI 1-3 day 9.3-26.6

BLOOD GASES
1-4 week 7.6-20.8
1-4 month 7.4-17.9 Sr. Arterial Capillary Venous
4-12 month 5.1-14.5 No.
1-6 year 5.7-13.3
1. pH 7.35-7.45 7.35-7.45 7.32-7.42
> 6 year 4.8-14.0
2. pCO2 (mm Hg) 35-45 35-45 38-52
3. T3 Newborns 100-470 ng/dL
1-5 year 100-260 ng/dL 3. pO2 (mm Hg) 70-100 60-80 24-48
5-10 year 90-240 ng/dL
10 years to Adult 70-210 ng/dL 4. HCO3 (mEq/L) 19-25 19-25 19-25

4. T3 uptake 35-45% 5. TCO2 (mEq/L) 19-29 19-29 23-33

contd... 6. O2 saturation (%) 90-95 90-95 40-70

7. Base excess (mEq/L) 5 to +5 5 to +5 5 to +5

HEMATOLOGIC VALUES

Sr. Age Hgb Hct RBC RDW MCV MCH MCHC PLTS
No. (g/dL) (%) (mill/mm3 ) (fL) (pg) (%) (x103/mm3 )

1. 0-3 days 15.0-20.0 45-61 4.0-5.9 <18 95-115 31-37 29-37 250-450
2. 1-2 weeks 12.5-18.5 39-57 3.6-5.5 <17 86-110 28-36 28-38 250-450
3. 1-6 months 10.0-13.0 29-42 3.1-4.3 <16.5 74-96 25-35 30-36 300-700
4. 7 months 10.5-13.0 33-38 3.7-4.9 <16 70-84 23-30 31-37 250-600
to 2 years
5. 2-5 years 11.5-13.0 34-39 3.9-5.0 <15 75-87 24-30 31-37 250-550
6. 5-8 years 11.5-14.5 35-42 4.0-4.9 <15 77-95 25-33 31-37 250-550
7. 13-18 years 12.0-15.2 36-47 4.5-5.1 <14.5 78-96 25-35 31-37 150-450
8 Adult male 13.5-16.5 41-50 4.5-5.5 <14.5 80-100 26-34 31-37 150-450
9. Adult female 12.0-15.0 36-44 4.0-4.9 <14.5 80-100 26-34 31-37 150-450

WBC AND DIFFERENTIAL LEUKOCYTE COUNTS

Age WBC Segs Bands Lymphs Monos Eosinophils Basophils Atypical No. of
(x103 mm3) Lymphs NRBCs

0-3 days 9.0-35.0 32-62 10-18 19-29 5-7 0-2 0-1 0-8 0-2
1-2 weeks 5.0-20.0 14-34 6-14 36-45 6-10 0-2 0-1 0-8 0
1-6 months 6.0-17.5 13-33 4-12 41-71 4-7 0-3 0-1 0-8 0
7 months to 6.0-17.0 15-35 5-11 45-76 3-6 0-3 0-1 0-8 0
2 years
2-5 years 5.5-15.5 23-45 5-11 35-65 3-6 0-3 0-1 0-8 0
5-8 years 5.0-14.5 32-54 5-11 28-48 3-6 0-3 0-1 0-8 0
13-18 years 4.5-13.0 34-64 5-11 25-45 3-6 0-3 0-1 0-8 0
Adults 4.5-11.0 35-66 5-11 24-44 3-6 0-3 0-1 0-8 0

Segs = Segmented neutrophils

Bands = Band neutrophils
Lymphs = Lymphocytes
Monos = Monocytes
454 Essentials of Pediatric Oral Pathology

ERYTHROCYTE SEDIMENTATION RATES AND IMMUNIZATION SCHEDULE IN CHILDREN

RETICULOCYTE COUNTS Recommendations of the INDIAN ACADEMY OF PEDIATRICS (IAP)
Committee on Immunization
Sr. Age Values
No. Age Vaccines Note

1. Sedimentation rate, Children 0-20 mm/hour Birth BCG

Westergren Adult males 0-15 mm/hour OPV zero
Adult females 0-20 mm/hour Hepatitis B-1

2. Sedimentation rate, Children 0-13 mm/hour 6 weeks OPV-1 + IPV-1/ OPV alone if IPV cannot be
W introbe Adult males 0-10 mm/hour OPV-1 given
Adult females 0-15 mm/hour DTPw-1/DTPa-1
Hepatitis B-2
3. Reticulocyte count Newborns 2-6% Hib-1
1-6 months 0-2.8%
Adults 0.5%-1.5% 10 weeks OPV-2 + IPV-2/ OPV alone if IPV cannot be
OPV-2 given
DTPw-2/DTPa-2
Hib-2
CEREBROSPINAL FLUID VALUES
14 weeks OPV-3 + IPV-3/ OPV alone if IPV cannot be
1. Cell count % PMNs OPV-3 given
Preterm mean 9 (0-25.4 57% DTPw-3/DTPa-3
WBC/mm3 ) Hepatitis B-3 Third dose of Hepatitis B can
Term mean 8.2 (0-22.4 61% be given at 6 months of age
WBC/mm3 ) Hib-3
> 1 month 0.7 0
9 months Measles
2. Glucose
15-18 months OPV-4 + IPV-B1/ OPV alone if IPV cannot be
Preterm 24-63 mg/dL mean 50
OPV-4 given
Term 34-119 mg/dL mean 52
DTPw booster -1 or
Children 40-80 mg/dL
DTPa booster -1
3. CSF glucose/blood Hib booster
glucose MMR-1
Preterm 55-105% 2 years Typhoid Revaccination every 3-4 years
Term 44-128%
Children 50% 5 years OPV-5
DTPw booster -2 or
4. Lactic acid 5-30 units/mL mean 20 DTPa booster -2
dehydrogenase units/mL MMR-2 The second dose of MMR
vaccine can be given at any
5. Myelin basic protein < 4 ng/mL
time 8 weeks after the first dose
6. Pressure:
10 years Tdap
Initial LP (mm H2O) HPV Only girls, three doses at
Newborns 80-110 (< 110) 0, 1-2 and 6 months
Infants/children < 200 (lateral
recumbent position) Vaccines that can be given after discussion with parents

7. Respiratory 5-10 More than Pneumococcal 3 primary doses at 6, 10,

movements 6 weeks conjugate and 14 weeks, followed by
a booster at 15-18 months
8. Protein
Preterm 65-150 mg/dL mean 115 More than Rotaviral vaccines 2/3 doses (depending on
Term 20-170 mg/dL mean 90 6 weeks brand) at 4-8 weeks interval

9. Ventricular 5-15 mg/dL After 15 Varicella Age less than 13 years: one
months dose. Age more than 13 years:
10. Cisternal 5-25 mg/dL 2 doses at 4-8 weeks interval

11. Lumbar 5-40 mg/dL After 18 Hepatitis A 2 doses at 6-12 months

months interval
Appendices 455

1. The IAP endorses the continued use of cell pertussis vaccine 4. Combination vaccines can be used to decrease the number of pricks
because of its proven efficacy and safety. Acellular pertussis being given to the baby and to decrease the number of clinic visits.
vaccines may undoubtedly have fewer side-effects (like fever, local The manufacturer’s instructions should be followed strictly whenever
reactions at injection site and irritability), but this minor advantage “mixing” vaccines in the same syringe prior to injection.
does not justify the inordinate cost involved in the routine use of 5. At present, the typhoid vaccine available in our country is the Vi
this vaccine. polysaccharide vaccine. Revaccination may be carried out every
2. If the mother is known to be HBsAg negative, HB vaccine can be three to four years.
given along with DTP at 6, 10, 14 weeks/6 months. If the mother’s 6. Under special circumstances (e.g. epidemics), measles vaccine
HBsAg status is not known, it is advisable to start vaccination soon may be given earlier than 9 months followed by MMR at 12-15
after birth to prevent perinatal transmission of the disease. If the months.
mother is HBsAg positive (and especially HBeAg positive), the 7. During pregnancy, the interval between the two doses of TT should
baby should be given Hepatitis B Immune Globulin (HBIG) within be at least one month.
24 hours of birth, along with HB vaccine. 8. We should continue to use OPV till we achieve polio eradication
3. Varicella, hepatitis A and pneumococcal conjugate vaccines should in India. IPV can be used additionally for individual protection.
be offered only after one to one discussion with parents. Also refer 9. OPV must be given to children less than 5 years of age at the
to the individual vaccines notes for recommendations. time of each supplementary immunization activity.

THE RECOMMENDED IMMUNIZATION SCHEDULES FOR PERSONS AGED 0 THROUGH 18 YEARS ARE
APPROVED BY THE ADVISORY COMMITTEE ON IMMUNIZATION PRACTICES (HTTP://WWW.CDC.GOV/
VACCINES/RECS/ACIP), THE AMERICAN ACADEMY OF PEDIATRICS (http://www.aap.org), AND THE AMERICAN
ACADEMY OF FAMILY PHYSICIANS (http://www.aafp.org).

Vaccine Age

Birth 1 2 4 6 12 15 18 19-23 2-3 4-6

month months months months months months months months years years

Hepatitis B Hep B Hep B Hep B

Rotavirus RV RV RV

Diphtheria, DTaP DTaP DTaP DTaP DTaP

Tetanus,
Pertussis

Hemophilus Hib Hib Hib Hib

influenzae type b

Pneumococcal PCV PCV PCV PCV PPSV

Inactivated IPV IPV IPV IPV

Poliovirus

Influenza Influenza (Yearly)

Measles, MMR MMR

Mumps, Rubella

Varicella Varicella Varicella

Hepatitis A Hep A (2 doses) Hep A series

Meningococcal MCV

Range of recommended ages for all children except certain high-risk groups.

Range of recommended ages for certain high-risk groups.

456 Essentials of Pediatric Oral Pathology

RECOMMENDED IMMUNIZATION SCHEDULE FOR PERSONS AGED 7 THROUGH 18 YEARS.

Vaccine Age

7-10 years 11-12 years 13-18 years

Tetanus, Diphtheria Tdap Tdap

Pertussis

Human Papillomavirus HPV (3 doses) HPV Series

Meningococcal MCV MCV MCV

Influenza Influenza (Yearly)

Pneumococcal PPSV

Hepatitis A Hep A Series

Hepatitis B Hep B Series

Inactivated Poliovirus IPV Series

Measles, Mumps, MMR Series

Rubella

Varicella Varicella Series.

Range of recommended ages for all children except certain high-risk groups

Range of recommended ages for catch-up

Range of recommended ages for certain high-risk groups

Index 457

Index

A Analysis of bite marks 429 Bowing of leg 255

Anemia 373 Burkitt’s lymphoma 405, 407
Abnormal frenum attachment 177 Aneurysmal bone cyst 189, 190
Abscess theory 149 Angular cheilitis 365 C
Absence of Ankyloglossia 20
neutrophils 392 Ankylosed tooth 48 Calcifying
teeth 31 Ankylosis of temporomandibular joint 292 epithelial odontogenic tumor 209-211
Acanthomatous ameloblastoma 205 Anodontia 31 odontogenic cyst 190, 191
Accessory parotid glands 306 Calcium hydroxide 107, 113
Antemortem and postmortem dental records
Achondrogenesis 265 Camera positioning 442
450
Achondroplasia 284 Candida albicans 350
Apert syndrome 279-282
Acinar atrophy 15 Candidal hyphae 22
Apexification 113, 117
Acinic cell adenocarcinoma 315 Capillary hemangioma 242
Apexogenesis 113, 114
Acquired Caries
Apical
immunodeficiency syndrome 361 activity tests 77
periodontal cyst 148
syphilis 343 assessment tool 55, 80
periodontitis 137
Actinomyces 68, 346 detector dyes 88
Aplasia 300 excisional burs 91
Acute of mandibular condyle 292
alveolar abscess 142, 143 in deciduous teeth 72
Aplastic anemia 377, 378 of dentin 73
apical periodontitis 144, 145 Aspartic acid racemization 414 of enamel 71
atrophic candidiasis 166 Assessment of skeletal remains 435 on pulpodentinal organ 103
bacterial infections 167 Atraumatic restorative treatment 98 susceptibility
candidiasis 166 Atresia 304 and caries activity 76
exacerbation of chronic lesion 144 Autoimmune theory 59 testing 76
gingival disease 161 Autopsy methods 412 vaccine 62
leukemias 393 Autosomal dominant chondrodysplasia punctata Cariogenic bacteria 66
lymphoblastic leukemia 393, 395 267 Categories of clefts 8
myeloblastic leukemia 393, 394
Causes of pulp pathology 130
necrotizing ulcerative gingivitis 164, 165 B Cavernous
pseudomembranous candidiasis 166 hemangioma 242
pulpal pain 128 Bacillary epithelioid angiomatosis 368
lymphangioma 246
pulpitis 136, 137 Bacterial
sinus thrombosis/ thrombophlebitis 155
suppurative osteomyelitis 152 alkaline phosphatase theory 61
Cellular stroma 237
Adenomatoid infections of oral cavity 338
Cellulitis 154
hyperplasia of mucous glands 307 Ballooning degeneration in epithelial cells 354
Cellulose granuloma 148
odontogenic tumor 208-210 Basal cell Cemental trabeculae 292
Affecting pulp 133 ameloblastoma 206 Cementoblastoma 291, 292
Agglutination test 420 layer 228 Central
Aglossia 18 Basilar part of occipital bone 436 giant cell granuloma 258, 259
Agnathia 2 Basophilia 390 odontogenic fibroma 217
Agranulocytosis 390 Behçet’s syndrome 164 Chalky white appearance on tooth surface 71
Air abrasion technique 92 Benign Cheilitis
Allergy and gingival inflammation 161 systems 420 glandularis 14, 15
Alveolar soft part sarcoma 251 investigation 375, 376, 382 granulomatosa 15
Ameloblastic Blue sclera 254 Chemical theory 57
carcinoma 219, 220 Bohn’s nodules 185 Chemomechanical caries removal 94
fibrodentinoma 213 Bone marrow 377, 380 Chemoparasitic theory 57
fibroma 212 biopsy in polycythemia vera 387 Cherubism 259, 260
fibro-odontoma 213, 214 film demonstrating erythropoiesis 386 Chewing gum 90
fibrosarcoma 221, 222 Borrelia vincentii 165 Chickenpox 354
Ameloblastomas 204 Bowed Child
Amelogenesis imperfecta 36, 38 hands in child 270 abuse 422
Amorphous calcium phosphate 90 legs in child 270 neglect 423
458 Essentials of Pediatric Oral Pathology

Chinese letter pattern 262 Coronal size of teeth 25

Chipmunk facies 382 dens invaginatus 29 structure of teeth 36
Cholesterol ingress of bacteria 131 disturbances of
clefts 188 Corynebacterium diphtheriae 340 gingival 18
crystal 189 Coxsackievirus 357 jaws 2
Chondroblastic osteosarcoma 249 Cracked tooth syndrome, 135 lips and palate 3
Chondrodysplasia punctata 266 Craniofacial oral lymphoid tissue 25
Chondroectodermal dysplasia 286 dysostosis 275 oral mucosa 17
Chondromyxoid fibroma 262 fibrous dysplasia 261, 262 salivary glands 25
Chronic Craniosynostosis syndromes 273 salivary glands in children 300
alveolar abscess 146 Cross section of spinal cord 126 tongue 18
focal sclerosing osteomyelitis 153 Crouzon syndrome 275 lingual salivary gland depression 304
hyperplastic pulpitis 138 Cuneiform appearance 340 Devitalized lamellar bone 152
leukemias 395 Cyclic Diffuse calcification 140
nonspecific gingivitis 167 neutropenia 175 Dilaceration 27
osteomyelitis 154 hygroma 194 of root of maxillary central incisor 28
pulpal pain 129 Cysticercus cellulosae 195 Dilated blood vessels 144, 145
pulpalgia 137 Cysts of salivary glands 196 Diphtheria 339
sialadenitis 309 Cytomegalovirus 370 Diphtheritic membrane 340
Classification of infection 358 Direct
bite marks 425 immunofluorescence 333
cysts 181 D pulp capping 106
dental caries 70 Disaster management 444
diseases of periradicular tissues 142 Deciduous mandibular right second molar 138 Discrete pulp stones 140
lip prints 416 Declaration of eradication of smallpox 355 Disorders of hemostasis 398
non-neoplastic salivary gland diseases 300 Defects of growth of teeth 45 Distortion of face 261
odontogenic tumors 200 Definitions of pain 125 Diverticuli 304
periodontal diseases and conditions 158 Deformity of bones of forearms 271 DNA
platelet disorders 398 Delayed eruption 46 identification 416
pulpal Demonstration of blood group substances 420 methods 417
pain 128 Dendritic cell neoplasms 401 polymorphisms 417
pathoses 130 Dens Dome shaped papule with central umbilication
salivary gland lesions 300 evaginatus 30 369
supernumerary teeth 32 in dente 28 Down syndrome 288
Cleft invaginatus 29 Drumstick appearance 349
lip 5, 11 Dental Ductal ectasia 15
palate 5, 9, 11 caries 58, 71, 79 Dye penetration method 88
tongue 20 considerations 111 Dyes for detection of carious
Cleidocranial dysplasia 257, 258 infections 155 dentin 88
Clostridium tetani 349 pain 128 enamel 88
Commissural lip pits 4 plaque and caries 66 Dyskeratosis congenita 321, 322
Common wart 225 structure identification 414 Dystrophic
Complete cleft lip 11 tissues 421 epidermolysis bullosa 331
Complex odontoma 215 Dentigerous cyst 183 nail beds 321
Compound odontoma 215 Dentin
Conditioned gingival enlargement 168 dysplasia 42, 43 E
Condyloma acuminatum 226 hypocalcification 45
Congenital Dentinal caries 73 Early
epulis of newborn 239 Dentinogenesis imperfecta 41, 42 childhood caries 74
lip pits Depressed nasal bridge 280 onset periodontitis 171
and fistula 3 Dermoid cyst 191 theories of dental caries 56
on lower lip 3 Desmoplastic ameloblastoma 207 Ectodermal dysplasia 318-320
syphilis 345 Desquamative gingivitis 167 Ehlers-Danlos syndrome 325, 326
velopharyngeal incompetence 8 Development of Electrical resistance 83
Connective tissue wall 182, 184, 187 human dentition 431 Ellis-Van Creveld syndrome 286
Conradi-Hünermann syndrome 266 palate 5 Embedded and impacted teeth 47
Conservative operative techniques 98 Developmental Endoscope 87
Contraindications for apexogenesis 114 defects in Endothelial lining 242
Conven tion al p ulpotomy with calcium number of teeth 31 Enlargement of radicular cyst 186
hydroxide 114 shape of teeth 26 Environmental enamel hypoplasia 39
Index 459

Eosinophilia 390 Formocresol pulpotomy 115, 116 Hemophilia 383, 385

Eosinophilic Functional neuroanatomy of pain 125 Hemorrhagic bone cyst 188
granular cytoplasm 239 Fusiform bacillus 165 Herpangina 357
granuloma 296 Fusion 27 Herpes
Epidermal inclusion cyst 191 simplex 162, 353, 366
Epidermoid G infection 161
cells 314 virus infection 161
cyst 191, 192 Gastric secretions 377 zoster 353, 368
Epidermolysis bullosa 330 Gemination 26 Herpetic gingivostomatitis 162
simplex 330 Generalized Herpetiform variant 163
Epithelial cells 211 aggressive periodontitis 172 Heterotropic salivary gland 305
Epithelium 182, 183, 187 microdontia 26 tissue of
Epstein’s pearls 185 Genetic basis of lower neck 305
Eruption 438 nonsyndromic cleft lip and/or palate, 8 middle ear 306
cyst 184 syndromic cleft lip and/or palate, 10 other sites 306
gingivitis 160 Genomic composition of HIV 362 High grade mucoepidermoid carcinoma 314
sequestrum 45 Geographic tongue 22 Hodgkin’s
Erythematous appearance 21 Germ tube formation in serum 351 disease 401
Erythrocyte sedimentation rates 454 German measles 356 lymphoma 401, 402
Euption cyst 185 Ghost Honeycomb appearance 204
Excavators, handpieces and burs 91 cells in epithelial lining 191 Howe’s criteria for pseudocyst 188
External root resorption 150, 151 like teeth 44 Human
Extracted natal tooth 35 Giant cells 190 hand marks 424
Gingiva 158 papillomavirus 370
F Gingival Hydatid cyst 194, 195
cyst of infant 185 Hyperkeratosis of dorsal tongue mucosa 321
Facial injuries 424
fibromatosis 169 Hyperkeratotic epithelium 169, 325
Familial gigantiform cementoma 263
recession 176 Hypermobility of joints 326
Fetal
Globulomaxillary cyst 50 Hyperostosis corticalis generalisata 286
rhabdomyoma 247
Goblet cells 51 Hyperparathyroidism 271
skeleton 435
Gorham’s disease 291 Hyperplasia of
Fiberoptic transillumination 84
Gorlin cyst 190 mandibular condyle 292
Fibrocellular connective tissue 292
Granular palatal salivary glands 303
Fibromatosis 169
cell ameloblastoma 206 Hypertelorism 280
gingivae 18
eosinophilic cytoplasm 251 Hypodontia 31
Fibrosarcoma 247
eosinophils 390 Hypoparathyroidism 272
Fibrous dysplasia 261
Granuloma 147 Hypoplasia of mandibular condyle 292
Fissural cysts of oral region 49
Granulomatous nodule 343 Hypoplastic teeth 44
Fissured tongue 20, 21
Grossman’s classification 130
Focal
Ground glass appearance of bony trabeculae I
epithelial hyperplasia 17, 226, 227
261
infection 156
Grouped polygonal tumor cells 251 Immune mediated theory 149
reversible pulpitis 135
Gumboil 146 Immunoglobulin pemphigus 335
Folic acid deficiency anemia 374
Gustafson 440 Incidence of
Follicular
Gutta percha 113, 148 clefting 7
ameloblastoma 204
GV black’s classification of dental caries 70 orofacial lesions 423
cyst 183
keratocyst 181 Incontinentia pigmenti 322, 324
H Indirect pulp capping 104
Fordyce’s granules 17, 18
Foreign body reactions 148 Habitual chewing marks 430 Infantile
Forensic Hairy tongue 23 cortical hyperostosis 290
anthropology 430 Hamartoma 200 osteomyelitis 153, 256
dentistry section 444 Hand-Schüller-Christian disease 294-296 osteopetrosis 256
laboratory equipments 431 Hansen’s disease 341 Inflamed interdental gingiva 160
medicine 411 Harlequin appearance 2 Inflammation
odontology 411 Heck’s disease 226 in upper anterior region 160
photography 441 Height determination 434 of minor salivary glands 15
science 411, 421 Hemagglutination test 420 Ingle’s classification 130
Formation of Hemangioma 240 Inheritance of sickle cell anemia 379
pulp stones 140 Hemarthrosis 385 Interdental spacing 26
residual cyst 188 Hematoxylin 6 Interstitial fibrosis 15
460 Essentials of Pediatric Oral Pathology

Intraoral lymphoepithelial cysts 193 Macroglossia 18, 19 Natal tooth with Riga Fede disease 35
Intraosseous heterotropic salivary gland tissue Major Necrotizing
306 aphthous ulcers 163 stomatitis 368
Iodoform paste 112 aphthae in labial vestibule 164 ulcerative periodontitis 368
Irreversible pulpitis 139 Male and female skull 433 Neutropenia 391, 392
Management of Neutrophilia 389
J caries 89 Nociceptive afferent neurons 126
deep dental caries in children 102 Non-Hodgkin’s lymphoma 403, 405
Jaw resection methods 413 Mandibular prognathism 280 Non-keratinized squamous epithelium 183
Junctional epidermolysis bullosa 331 Mandibulofacial dysostosis 276 Non-neoplastic salivary gland diseases 300
Juvenile ossifying fibroma 263, 264 Marfan syndrome 263, 264 Nonpainful pulpitis 137
Mass of polyhedral cells 204 Non-setting calcium hydroxide 107
K Massive osteolysis 291 Normal salivary function 301
Mature Numerous
Kaposi’s sarcoma 371 B-cell neoplasms 400 polymorphonuclear leukocytes 144
Keratin filled cystic cavity 192 T-cell and NK-cell neoplasms 401 sebaceous glands 191
Kernahan classification for clefts 8, 9 Maxillary central incisors 26 unerupted and supernumerary teeth 258
Keye’s triad 61 Maxillofacial skeleton 202 Nutritional deficiency theory 60, 149
Koilocytes 226 Measles 356
Median O
L mandibular cyst 51
Odontoameloblastoma 216
palatal cyst 50
Labial Odontogenic
rhomboid glossitis 21
and oral melanotic macule 16 apparatus 202
Melanotic neuroectodermal tumor of infancy
melanotic macule 17 carcinomas 218
238
Lactobacillus 67 cysts 220, 221
Metastasizing ameloblastoma 219
Lamina dura around teeth 271 ectomesenchyme 212
Microdontia 25
Langerhans cell histiocytosis 293, 294 epithelial cells 211
Microglossia 18
Laser light 91 epithelium 212
Micrognathia 2, 3
Lasers fibroma 216
Midface hypoplasia 280
in minimal intervention dentistry 95 ghost cell tumor 190
Midpalatal raphae cyst 185
used for caries removal 95 keratocyst 179, 181, 182
Minor aphthous ulcers 163
Left maxillary lateral incisor 29 myxoma 217
Mitochondrial DNA 419
Lentigo simplex 228 sarcomas 219
Mixed
Leprae bacilli 342 Odontoma 34, 214
cell agglutination 421
Lepromatous leprosy 342 Opalescent dentin 41
salivary gland acini 197
Leprosy 341, 343 Optical caries monitor 84
Modified dye penetration method 88
Leukemia 392 Oral
Molecular basis of disease 326
Leukocytosis 389 and dental management of leukemia 398
Molluscum contagiosum 369
Lichenoid infiltrate 344 autopsy protocol 412
Monocytes 390
Liesegang rings 211 foci of infection 156
Monocytosis 390
Lingual pulse granuloma 148
Monostotic fibrous dysplasia 261
cyst of foregut origin 194 submucous fibrosis 230, 232
Mucocele on lower lip 196
thyroid nodule 24, 25 ulceration 367
Mucoepidermoid carcinoma 313
varices 24 Osler-Weber-Rendu syndrome 326, 327
Mucopolysaccharidosis 267
Lining of stratified squamous epithelium 191 Osmotic pressure theory 149
Multinucleated giant cells 259
Localized aggressive periodontitis 171 Osteoblasts 152
resorbing root surface 151
Low grade mucoepidermoid carcinoma 314 Osteocytes 152
Multiple
Lupus vulgaris 341 Osteogenesis imperfecta 253-255
ulcers on cheek and corner of mouth 352
Lymphangioma 245 Osteomyelitis 151, 152
unerupted teeth 47
Lymphocytes 392 Osteopetrosis 255
vesicles in buttock region 330
and plasma cells 146 Osteosarcoma 248
Mycotic infections of oral cavity 350
Lymphocytosis 392 Outer canthus of eye 192
Myofibroma 235
Lymphoid hamartoma 25 Owl’s eye appearance 361
Lymphoma 372, 400
N P
M Nasal floor 8 Pachyonychia congenital 324, 325
Nasoalveolar cyst 51 Painless
Macrocytosis 376 Nasopalatine duct cyst 49 lobulated mass 237
Macrodontia 26 Nasopharyngeal angiofibroma 244 swelling in maxillary anterior region 184
Index 461

Palatal rugae pattern 415 intraosseous squamous cell carcinoma Rickets 269
Papillon-Lefèvre syndrome 174, 175 219-221 Robinow syndrome 285
Paraneoplastic pemphigus 335 syphilis 343 Role of
Parasitic cysts 194 Principles of calcium hydroxide 107
Parotid gland disease 367 laser/light induced fluorescence 85 forensic
Partial pulpectomy 110 light transmission through teeth 83 anthropologist 431
PCR kit 418 Proliferative periostitis 154 dentistry in mass disasters 443
Peg shaped incisors 320 Prominent bulbous interproximal papillae 168 pedodontist in
Pemphigus 332 Proteolytic theory 59 forensic odontology 412
erythematosus 334 Protrusion of premaxilla 8 management of cleft lip and palate 13
foliaceous 333 Protuberant abdomen 265 Root
vegetans 334 Pseudomacroglossia 19 filling materials 112
vulgaris 332, 333 Puberty gingivitis 168 surface caries 66
Periapical Pulp Rubella 356
cyst 150, 185 hyperemia 135, 136 Rushton bodies 187
granuloma 147 necrosis 141
Periodontal diseases in children 171 reaction to restoration 133
Pulpal S
Periorbital cellulitis 155
Peripheral granuloma 138 Sabouraud’s dextrose agar 351
ameloblastoma 206 pain 128 Salivary
odontogenic fibroma 218 Pulpectomy 109 duct stone 311
ossifying fibroma 237 Pulpotomy 109 gland lesions 299
Permanent dentition 432 Pulsed laser caries detector 85, 86
swabbing 427
Pernicious anemia 376 Pyogenic granuloma 236
Sarcoidosis 309
and vitamin B12 deficiency 376 Scarlatina 338
Peutz-Jegher’s syndrome 15, 16, 328 Q
Scarlet fever 338
Pierre Robbin syndrome 278 Quantitative light Schaumann bodies 310
Pigmented nevi 228 fluorescence device 87 Scorbutic gingivitis 171
Placement of induced fluorescence 86 Scrofula 341
calcium hydroxide in indirect pulp capping Secondary
105 R herpes simplex 162
fiberop tic transillumination probe on syphilis 344
fissure area 64 Raccoon mask sign 268
Race determination 433 Seltzer and Bender’s classification 130
Platelet disorders 398
Radicular cyst 183, 185, 187, 189 Severe mandibular retrognathism 278
Pleomorphic adenoma 312
Radiolucencies in root and adjacent bone 151 Short limbs 265
Plexiform ameloblastoma 205
Ranula 197 Sialadenitis 308
Plunging ranula 197
in floor of mouth 197 Sialolithiasis 310
Polycystic disease of parotid glands 307
Rash with pink macules 357 Sickle
Polycythemia 386
Raspberry tongue 339 cell anemia 378, 380
vera 387
Ray fungus 346 shaped red blood cell 380
Polymerase chain reaction methods 417
Reconstruction of facial tissue 416 Simple gingivitis 159
Polymorphonuclear leukocytes 145, 146
Recording lip prints 416 Single layer of endothelial cells 242
Polyostotic fibrous dysplasia 261
Positive Recurrent Smallpox 354
thumb sign 264 aphthous ulcer 163 Soap bubble appearance 203
wrist sign 264 HSV infection 353 Soft
Postmortem dental examination 449 pulpal pain 129 fluctuant swelling 192
Post-transplant lymphoproliferative disorders Regional odontodysplasia 44 palate 8
401 Replication cycle of virus 363, 364 white, curd-like plaques 351
Precursor lymphoid neoplasms 400 Residual cyst 188 Solid tumor mass 209
Predeciduous dentition 34 Restriction fragment length polymorphisms 417 Solitary bone cyst 188
Predominant epidermoid cells 314 Reticular atrophy of pulp 141 Sparse hair 320
Predominantly erythroid hyperplasia 387 Reticulocyte counts 454 Squamous
Premature Retrocuspid papilla 18 epithelium 211
eruption 45 Reversible pulpitis 134 metaplasia 205
loss of primary teeth 103, 176 RFLP methods 417 odontogenic tumor 211
Prepubertal periodontitis 173 Rhabdomyoma 246 papilloma 224
Prevention of caries 89 Rhabdomyosarcoma 249, 250 Staging of
Primary Rhizomelic lymphoma 402
and permanent teeth 110 chondrodysplasia punctata 266 non-Hodgkin’s lymphoma 404
HSV infection 352 shortening of arms and legs 284 Starry sky appearance 407
462 Essentials of Pediatric Oral Pathology

Stephen’s curve 57 pressure 425 V

Strabismus 280 scrape 425
Strap marks 424 Transmission 384 Van Der Woude syndrome 4
Structure of HIV 361, 362 Treacher Collins Variola viruses 355
Subacute pulpitis 137 Franceschetti syndrome 276 Veillonella 68
Sun-ray appearance 248 Verruca vulgaris 225
syndrome 276
Superficial sebaceous glands 18 Videoscope 87
Treponema pallidum 343
Supernumerary Viral infections of oral cavity 352
Trichodento-osseous syndrome 287, 288
roots 31 Visual observation of dental caries 80
Tubercle bacilli 340
teeth 32 Vital
Tuberculosis 340
Synovial sarcoma 250 pulp therapy 104
Tuberculous lymphadenitis 341
Syphilis 343, 368 theory 57
Types of
Vitamin B deficiency anemia 374
child abuse 423 Von Willebrand’s disease 383
T child neglect 423
Tactile observation 79 dentigerous cysts 183 W
Talon’s cusp 28 DNA 419
Taurodontism 30 leukemia 392 Warthin’s tumor 313
Tennis racket appearance 204 odontogenic keratocyst 179 WBC count 392
Teratoid cyst 192 OKC 181 Webster’s classification 425
Tertiary syphilis 344 pain 126 Well-defined periradicular radiolucency 145
Thalassemia 381 photography 441 White sponge nevus 329
Thanatophoric dysplasia 283 Worm theory 56
teeth 421
Theories of cyst formation 149
Thrombocytopenia 398, 399 X
Thyroglossal duct cyst 193 U
Xeroradiography 82
Thyroid Xerostomia 61, 301, 368
Ultrastructural changes in enamel 72
function tests 453 X-linked recessive chondrodysplasia punctata
Ultraviolet illumination 86
tissue with thyroid follicles 25 266
Tissue samples 428 Unicystic ameloblastoma 207
Uniform spindle-cell appearance 248
Toga virus 356 Y
Tongue Upper and lower gingivae 165
Use of Yellow sulfur granules 68
pressure 425
lasers in pit and fissure caries 96 Yellowish discoloration of sclera 382
tie 20
Tooth ozone in minimally intervention dentistry
composition 64 96 Z
identification 421 polymeric coatings 91 Zinc oxide eugenol 112
morphology 64 xylitol 90 Zygomatic bones 436

Common questions