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Chen 2012

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Available Formats
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This issue of CDR will qualify for 1 ABR Self-Assessment Module (SAM) credit.

See page 6 for more information.

Volume 35 • Number 17
August 15, 2012

Periosteal Reaction: Review of Various Patterns Associated


With Specific Pathology
Eric M. Chen, MD, Sulabha Masih, MD, Kira Chow, MD, George Matcuk, MD, and
Dakshesh Patel, MD
Qualified by the American Board of Radiology (ABR) in meeting the criteria for self-assessment toward the purpose
of fulfilling requirements in the ABR Maintenance of Certification (MOC) Program. Dated: June 11, 2012.
Please note that in addition to the SAM credit, subscribers completing the activity will receive the usual ACCME credit.
After participating in this activity, the radiologist should be better able to distinguish the various patterns of periosteal
reaction and to use this information to help formulate a concise differential diagnosis.

Aggressive patterns typically are interrupted patterns and result


CME Category: General Radiology
Subcategory: Musculoskeletal from faster growing processes, which deny the periosteum time
to form new bone. Types of aggressive patterns include lami-
nated (onionskin), spiculated, sunburst (“hair-on-end”), disor-
The periosteum is a thin membrane surrounding every bone ganized, and Codman triangle.1
except surfaces covered by cartilage. In response to injury or
other stimuli, the periosteum forms new bone, in what is known Differential Diagnosis of Periosteal Reaction
as periosteal reaction. This is synonymous with other terms, The differential diagnosis for periosteal reaction is broad, as
such as periostitis, periosteal new bone formation, and reac- it results from any type of insult to the underlying cortical bone.
tive bone formation. Understanding the patterns of periosteal Categorization of aggressive versus nonaggressive patterns
reaction can be useful for assessing the aggressiveness of the helps, although there is some overlap between benign and
underlying lesion and formulating a differential diagnosis. malignant etiologies. Etiologies include genetic, drug-related,
systemic inflammatory disease, metabolic disease, trauma,
Periosteal reactions can be categorized as either nonaggres- tumor, infection, and vasculopathy. Systemic processes should
sive or aggressive. Nonaggressive patterns result from slower be considered in cases where there is bilateral distribution.
growing processes, after healing or treatment, and generally are Sometimes, age of the patient also can be helpful, as periosteal
an uninterrupted pattern. Types of nonaggressive patterns include reaction in infants younger than 6 months usually is due to
thin, solid, thick, irregular, and septated periosteal reactions. physiologic periostitis of the newborn (i.e., Caffey disease) or
periostitis related to prostaglandin use.
Dr. Chen is Resident, Department of Radiology, Ronald Reagan UCLA Medical
Center, 757 Westwood Plaza Drive, Los Angeles, CA 90095; E-mail: erchen@mednet. Osteogenesis Imperfecta
[Link]; Dr. Masih is Associate Clinical Professor and Dr. Chow is Associate Osteogenesis imperfecta, also known as “brittle bone dis-
Clinical Professor, Department of Radiology, West Los Angeles Veteran Affairs
Medical Center, Los Angeles, California; and Dr. Matcuk is Assistant Professor of ease,” is a genetic disorder (almost always autosomal domi-
Clinical Radiology and Dr. Patel is Assistant Professor of Clinical Radiology, nant) of type I collagen formation leading to brittle bone. It is
Department of Radiology, University of Southern California, Los Angeles, California. associated with blue sclera, early hearing loss, thin skin, joint
All authors and staff in a position to control the content of this CME activity and laxity, and respiratory and cardiac problems. Radiographic
their spouses/life partners (if any) have disclosed that they have no relationships
with, or financial interests in, any commercial organizations pertaining to this edu- findings include cortical thinning, multiple fractures that can
cational activity. have exuberant callus formation, bowing deformities of the
Lippincott Continuing Medical Education Institute, Inc., is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical
education for physicians. Lippincott Continuing Medical Education Institute, Inc., designates this enduring material for a maximum of 1.5 AMA PRA Category 1
Credits™. Physicians should only claim credit commensurate with the extent of their participation in the activity. To earn CME credit, you must read the CME
article and complete the quiz and evaluation on the enclosed answer form, answering at least seven of the 10 quiz questions correctly. This continuing medical education
activity expires on October 19, 2012.
1
pain/weakness, and joint pain. Hypervitaminosis A presents
radiographically with a thick, dense periosteal reaction that
may be painful.
Fluorosis
Fluorosis is caused by excessive intake of fluoride, most com-
monly in drinking water. Moderate amounts lead to dental effects,
but long-term, large amounts can lead to skeletal manifestations.
Radiographic findings of systemic involvement include sacro-
spinous or sacrotuberous ligament calcification, exuberant
periosteal reaction commonly in the tubular bones in a sym-
metric distribution, and dense metaphyseal lines (Figure 2).
Psoriatic Arthritis
Psoriatic arthritis is an inflammatory arthritis with envi-
Figure 1. Osteogenesis imper- ronmental and hereditary factors that occurs in 10% to 15%
fecta. Frontal radiograph of the
left femur shows exuberant solid Figure 2. Fluorosis. Lateral radio-
of patients with psoriasis.2 Radiographically, in the hands it
periosteal reaction (arrow) in the graph of the knee demonstrates is characterized by a lack of juxta-articular osteopenia (in con-
diaphysis of the femoral shaft solid periosteal reaction involv- trast with rheumatoid arthritis), generalized asymmetric involve-
from recurrent fractures. ing the tibia and fibula (arrow). A ment, solid periosteal reaction along the shaft of the phalanges
radiograph of the contralateral
knee (not shown) demonstrated
symmetric periosteal reaction.

extremities, vertebral fractures (kyphoscoliosis), and absent


secondary trabecula with sparse primary trabecula (Figure 1).

Infantile Cortical Hyperostosis


Infantile cortical hyperostosis, also known as Caffey disease,
occurs in 2 forms. The more common infantile form is benign
and presents in infants younger than 6 months. The rare severe
prenatal form is likely autosomal recessive and presents with
major angulation of long bones, symmetric involvement, and
polyhydramnios. Patients may present with the clinical triad of
fever, soft tissue swelling, and hyperirritability. On radiographs,
there is solid, thick periosteal reaction, most commonly involving
the mandible, long bone diaphyses, clavicles, ribs, and scapulae.

Vitaminosis (Scurvy and Hypervitaminosis A)


Scurvy now is a rare nutritional disorder caused by vitamin C
deficiency. It presents in elderly persons, alcoholic persons, or
infants and children on diets devoid of fresh fruits or vegetables.
Radiographic findings include large subperiosteal hemorrhage
causing periosteal elevation and physeal separation.
Figure 3. Psoriatic arthritis. Frontal radiograph of the left hand demon-
Excessive vitamin A intake results in a wide clinical pre- strates solid periosteal reaction along the phalangeal shafts (arrow).
sentation (differing between acute and chronic toxicity), but Additional radiographic findings include lack of juxta-articular osteope-
symptoms include nausea, vomiting, osteoporosis, muscle nia, and involvement of the distal interphalangeal joints.
The continuing education activity in Contemporary Diagnostic Radiology is intended for radiologists. EDITOR: Robert E. Campbell, M.D., Clinical Professor of Radiology,
University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania
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2
Figure 6. Stress fracture. Frontal
radiograph of the left foot demon-
strates solid periosteal reaction
in the diaphysis of the second
metatarsal (arrow).

Hypertrophic Osteoarthropathy
Hypertrophic osteoarthropathy occurs as both a rare genetic
primary form, known as pachydermoperiostosis, and a secondary,
much more common (⬎95%) form, known as hypertrophic (pul-
monary) osteoarthropathy. Patients with primary hypertrophic
osteoarthropathy may also later on develop conditions that cause
the second form.3 The secondary form has no genetic associa-
tion but classically is associated with underlying thoracic con-
ditions, such as bronchogenic carcinoma or pulmonary metastases,
and congenital heart disease, although extrathoracic causes (e.g.,
Figure 4. Hypertrophic osteoarthropathy. Frontal radiograph of the cirrhosis, ulcerative colitis, and Crohn disease) also have been
right hand demonstrates thick irregular periosteal reaction at the
distal radius (arrow). reported. Patients present with skin thickening, digital clubbing,

often with soft tissue swelling, osteolysis and erosions of the


phalanges with “pencil-in-cup” deformity, and involvement
of the distal interphalangeal joints (Figure 3). Reactive arthri-
tis may present with a similar pattern of arthritic changes and
periosteal reaction but with a predilection for the feet.

Figure 5. Thyroid acropachy. Frontal radiograph of both hands Figure 7. Osteosarcoma. Frontal radiograph of the left knee demon-
demonstrates solid periosteal reaction in the diaphysis of the pha- strates sunburst pattern of periosteal reaction (arrow) at the meta-
langes and metacarpal bones (arrows). physis of the knee with extension into the epiphysis.
3
Figure 9. Lymphoma. Sagittal T1-weighted MR image of the knee
demonstrates hypointense homogeneous tumor mass (white arrows)
with extensive soft tissue component (black arrow) and thin periosteal
reaction (arrowhead ).

histologic subtypes, each with different clinical and radiographic


Figure 8. Ewing sarcoma. Frontal radiograph of the left knee demon-
patterns.5 The most common form, conventional intramedullary
strates ill-defined intramedullary lesion with permeative bone destruc- osteosarcoma, presents with progressive pain and soft tissue
tion and onionskin periosteal reaction (arrow). mass/swelling, commonly in the second to third decades of life,
with a second peak after the sixth decade of life. The classic
and periosteal reaction of tubular bones. Radiographically, there origin is in the metaphysis of the long bones (66% around
are findings of symmetric periosteal thickening along the shafts knees) with associated bone destruction (osteosclerosis and/or
of the tubular bones, most commonly seen in the tibia, radius, osteolysis), aggressive periosteal reaction, and an adjacent
ulna, and fibula (Figure 4). soft tissue mass. The most common form of periosteal reac-
tion is a sunburst pattern (Figure 7), although hair-on-end or
Thyroid Acropachy Codman triangle appearance also may be seen.
Thyroid acropachy is an extreme manifestation of autoim-
mune thyroid disease, which almost always presents with Graves Ewing Sarcoma
dermopathy (pretibial myxedema) and ophthalmopathy.4 It also Ewing sarcoma is the second most common primary bone
may present with digital clubbing and swelling of digits and toes. tumor in the pediatric population after osteosarcoma.6
Patients usually have a long history of active Graves disease but Approximately 20% to 30% of patients have metastatic disease
usually are euthyroid or hypothyroid on presentation. On radi-
ography, there is lacy, thick subperiosteal new bone in the dia-
physes of short tubular bones of the hands and feet (Figure 5).
The first, second, and fifth metacarpal bones, and proximal and
middle phalanges of the fingers are most commonly involved.

Stress Fracture
Stress fractures may be due to abnormal or repetitive stress
on normal bone (fatigue fractures) or due to normal stress
on weakened or osteopenic bone (insufficiency fractures).
Clinically, they may present with pain, swelling, and erythema.
Typical radiographic findings include subperiosteal new bone
at various stages, most commonly in the tibia, calcaneus, talus,
cuboid, and metatarsals (Figure 6).

Osteosarcoma
Figure 10. Eosinophilic granuloma. Frontal radiograph of the humerus
Osteosarcoma is the most common malignant, primary demonstrates a well-defined diaphyseal lytic lesion without a sclerotic
bone tumor in young adults and children. There are multiple rim and intermediate smooth periosteal reaction (arrow ).
4
mass with cartilaginous calcifications, scalloping of the under-
lying cortex, and subjacent sclerosis.
Eosinophilic Granuloma
Eosinophilic granuloma is a benign form of Langerhans cell
histiocytosis. There are monostotic and polyostotic forms.
Although any bone can be involved, the skull is affected in half
of the patients. It most commonly presents as a well-defined lytic
lesion without a sclerotic rim. Periosteal reaction occurs in 10%
of lesions as a thin, intermediate, or solid reaction, usually in a
circumferential orientation around the lesion8 (Figure 10).
Osteoid Osteoma
Osteoid osteoma is a benign lesion with a nidus less than
2 cm of osteoid and woven bone in vascular tissue. Classically,
Figure 11. Osteoid osteoma. Axial noncontrast CT of the femur demon- there is focal bone pain, which is relieved with aspirin, and
strates a lucent central nidus surrounded by periosteal reaction (arrow). worsens at night or with increased activity. Periosteal reac-
tion is typically thick and dense (Figure 11). A central lucent
nidus may be difficult to identify on radiographs.
at presentation (most commonly to the lungs and bones). These
patients present with progressive pain and soft tissue mass/ Osteomyelitis
swelling. Ninety percent of the patients are younger than 20 years Osteomyelitis is an infection of the bone, most commonly
of age. The radiographic appearance is variable but it classi- due to direct extension from an ulcer, especially in patients with
cally presents as an ill-defined intramedullary lytic or perme- diabetes. However, hematogenous spread is more common in
ative lesion with aggressive periosteal reaction and an adjacent children and young adults. Children more commonly get infec-
soft tissue mass, commonly in the diaphysis of long bones of tion in the metaphysis, whereas adults commonly have infec-
the lower extremity (Figure 8). tion spreading to the joint space. Acutely, osseous abnormalities
Lymphoma
are not seen on plain radiographs. Many types of periosteal reac-
tion can be seen, including disorganized, thin, lamellated, or
Primary lymphoma of bone is defined as lymphoma within spiculated forms.
the medullary cavity of a single bone without evidence of con-
current lymph node or visceral involvement for 6 months after Venous Stasis
diagnosis. It is most common in the long bones (femur, tibia, and Venous stasis leads to soft tissue edema, dermatitis with shal-
humerus) but also occurs in low skin ulcers, and thick undulating or nodular periosteal reac-
the pelvis, spine, scapula, and tion. It typically presents in the lower extremities bilaterally
ribs.7 Lesions typically pre- and can be associated with phleboliths and superficial vari-
sent as solitary lytic lesions cosities. Radiographically, venous stasis usually causes smooth,
(70%) near the end of a long solid symmetrical periosteal reaction; however, irregular sym-
bone with a mottled perme- metrical periosteal reaction is seen occasionally (Figure 12).
ative pattern, large soft tissue
component, and aggressive Conclusion
periosteal reaction (Figure 9). Periosteal reaction occurs as a result of insult to the under-
lying bone cortex. This CME activity emphasizes that recog-
Periosteal Chondroma nizing the different patterns of periosteal reaction will help to
Periosteal chondroma, also guide the formulation of an appropriate differential diagnosis
known as juxtacortical chon- by the radiologist.
droma, is most commonly
seen in children and young References
adults. It is a slow-growing 1. Rana R, Wu J, Eisenberg R. Periosteal reaction. AJR Am J Roentgenol. 2009;193(4):
W259-W272.
neoplasm that develops with- 2. Klecker R, Weissman B. Imaging features of psoriatic arthritis and Reiter’s
in periosteal connective tis- syndrome. Semin Musculoskelet Radiol. 2003;7(2):115-127.
sue of tubular bones. The 3. Martinez-Lavin M, Vargas A, Rivera-Vinas M. Hypertrophic osteoarthropathy: a
palindrome with a pathogenic connotation. Curr Opin Rheumatol. 2008;20:88-91.
usual radiographic appear- 4. Fatourechi V, Ahmed D, Schwartz K. Thyroid acropachy: report of 40 patients
ance is a localized soft tissue treated at a single institution in a 26-year period. J Clin Endocrinol Metab.
2002;87(12):5435-5441.
5. Spina V, Montanari N, Romagnoli R. Malignant tumors of the osteogenic matrix.
Figure [Link] stasis. Frontal Eur J Radiol. 1998;27:S98-S109.
radiograph of the right tibia and 6. Mar W, Taljanovic S, Bagatell R, et al. Update on imaging and treatment of
Ewing sarcoma family tumors: what the radiologist needs to know. J Comput
fibula demonstrates irregular
Assist Tomogr. 2008;30:108-118.
periosteal reaction (arrow ). A 7. Mulligan M, McRae G, Murphey M. Imaging features of primary lymphoma
radiograph of the contralateral of bone. AJR Am J Roentgenol. 1999;173:1691-1697.
leg demonstrates symmetric 8. David R, Oria R, Kumar R, et al. Radiologic features of eosinophilic granuloma
involvement (not shown). of bone. AJR Am J Roentgenol. 1989;153:102-126.
5
CME QUIZ: VOLUME 35, NUMBER 17
To earn CME credit, you must read the CME article and complete the quiz and evaluation on the enclosed answer form, answering at least
seven of the 10 quiz questions correctly. Select the best answer and use a blue or black pen to completely fill in the corresponding
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address do not appear on the answer form, please print that information in the blank space at the top left of the page. Make a photocopy
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Time on the due date.

Questions marked with an asterisk are ABR Self-Assessment Module (SAM) questions. Participants can claim credit for the SAM regardless
of the test outcome. Notify the ABR of the SAM completion, or visit the ABR website at [Link] to set up or log in to your personal
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wish to include the ID number in your ABR database, contact a MOC Specialist at the ABR office for instruction by calling 520-519-2152.
*1. All of the following are features usually associated with thyroid *6. Radiographs of the hands of a 45-year-old patient reveal asym-
acropachy, except metric osteolysis and erosions of bones of the distal interphalangeal
A. pretibial myxedema joints with a pencil-in-cup deformity. The most likely diagnosis is
B. long history of active Graves disease A. reactive arthritis
C. periosteal reaction of long bones of the extremities B. hypertrophic osteoarthropathy
D. thick, subperiosteal new bone in the diaphyses of short C. hypervitaminosis A
tubular bones of the hands and feet D. skeletal fluorosis
E. digital clubbing E. psoriatic arthritis
See Reference 4 for further study. See Reference 2 for further study.
*2. Secondary hypertrophic osteoarthropathy is associated with all 7. Primary lymphoma commonly presents in all of the following bones,
of the following conditions, except except
A. renal lymphoma A. metatarsals
B. bronchogenic carcinoma B. femur
C. pulmonary metastasis C. humerus
D. congenital heart disease D. tibia
E. ulcerative colitis E. scapula
See Reference 3 for further study. 8. Which one of the following is the most likely pattern of periosteal
*3. Which one of the following statements regarding eosinophilic reaction in a patient with fluorosis?
granuloma of bone is true? A. Onionskin
A. It is a malignant form of Langerhans cell histiocytosis. B. Sunburst
B. It never involves the skull. C. Disorganized
C. It presents radiographically as a lytic lesion with a thick scle- D. Solid
rotic rim. E. Codman triangle
D. The pattern of associated periosteal reaction is nonaggressive. 9. Which one of the following is the most common location of an
E. It always presents with multiple lesions. osteosarcoma in a young adult?
See Reference 8 for further study. A. Scapula
B. Skull
*4. A 16-year-old boy presents with multiple nodular lung lesions on
C. Rib
chest radiography and an ill-defined, intramedullary, permeative
D. Spine
lesion with aggressive periosteal reaction in the diaphysis of the
E. Knee
right femur with an associated soft tissue mass. The most likely
diagnosis is 10. A 4-month-old child presents clinically to his pediatrician with fever,
A. osteoid osteoma soft tissue swelling about the mandible, and hyperirritability.
B. Ewing sarcoma Nonaggressive, solid, thick periosteal reaction is noted about
C. osteosarcoma the mandible radiographically. The most likely diagnosis is
D. eosinophilic granuloma A. osteosarcoma
E. osteomyelitis B. Caffey disease
See Reference 6 for further study. C. Ewing sarcoma
D. eosinophilic granuloma
*5. Bilateral symmetric periosteal reaction is seen radiographically in E. lymphoma
all of the following conditions, except
A. venous stasis
B. fluorosis
C. primary lymphoma
D. hypertrophic osteoarthropathy
See Reference 1 for further study.

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