Pagets disease
It is also called as Osteitis deformans. It was discovered in 1877 by Sir
James Paget. It fondly refers to as Collage of matrix madness. There is abnormal
resorption and opposition of bone in one or more bones. The disease is initiated by
an intense wave of osteolytic activity with resorption of normal bone resulting in
irregularly shaped resorption cavity followed by vigorous osteoblastic activity
forming woven bone after variable period.
Etiology:
Inflammatory: Initially it is thought to be inflammatory disease but later on
this cause is ruled out.
Circulating disturbance: The bone in Pagets disease is excessively vascular
and it has been suggested that vessels are similar to artrivenos aneurysms.
Slow Virus Theory: Slow viruses are those viruses which take a long time for
incubation. The possibility of an infective viral etiology for Pagets disease is
suggested by ultra-structural demonstration of intranuclear inclusions in abnormal
osteoclast.
Genetic and Environmental factors:
Genetic and Environmental factors appear to
be important. It is transmitted as auto somal dominant trait with genetic
heterogenecity.
Connective tissue factors: It may be a disorder of connective tissue biosynthesis.
Others:
Vasculities, trauma, hormonal imbalance, degenerative neurologic disorder.
Clinical Features:
Geographical Prevalence: It is seen most frequently in Britain and less
frequently in North America and Western Europe.
Age and Sex: Predominantly in patients over 40 years of age with a slight
predilection for men. Male to female ratio is 2:1.
Sites: It is prone to occur in the axial skeleton especially the skull, femur,
Sacrum and Pelvis. It is usually polyostotic.
Skull enlargement: First complaint is that patient needs to buy a hat of larger
size because of Skull enlargement.
Pain: Bone pain is a consistent symptom and most often directed towards
weight bearing areas. The patients may direct have ill-defined neuralgic pain as a
result of restriction of foramina and canals, which occurs due to pressure created
by mass on structures passing through the foramina.
Deafness: Deafness due to involvement of the petrous portion of temporal
bone with compression of cochlea have complication.
Skeletal deformities, fracture of limbs, compression of spine and occurrence
of giant cell lesion. Another complication includes renal Calculi, Salivary calculi
and hypercalcemia.
Oral Manifestations:
Sites: Maxilla is involved three times more commonly than mandible. It is
bilaterally symmetrical in the involved Jaw.
Symptoms:
Movement and migration of affected teeth occurs. Due to migration, patient
may notice malocclusion. In case of edentulous as alveolar ridge becomes enlarged
patient complaint of poor fit of denture.
Neurological Symptoms: There may be mental disturbance and dizziness.
Signs: Bowing of legs, curvature of Spine and enlargement of skull. The involved
bones are warn to toud because of increased vascularity and are prone to fracture.
Similar appearance (like an ape or monkey: Due to grotesque facial appearance
along with waddling gait and short stature patient has got simian appearance.
Waddling (Short step) gait: Broadening and flattening of the chest and spinal
curvature, the patient assumes waddling gait.
Signs: Increase in aleveolar width associated with flattening of palate when maxilla
is involved.
Appearance: As the disease progresses, the mouth may remain open exposing the
teeth as the lips are too small to cover the enlarged Jaws.
Extraction Sites: Extraction sites heal slowly, and incidences of osteomyelitis are
higher. Extraction may be further complicated by excessive leeding from highly
vascular abnormal bones in the lytic phase of disease.
Complications: Osteogenic Sacroma, Osteomyelitis Pathological fracture and
facial Paralysis. Sacroma is suspected and in case that experience a marked
increase in intensity of bone pain or other symptoms and show marked increase in
alkaline Phosphatase.
Prognosis: It is poor in patients showing development of osteogenic Sacroma in
Pagets disease.
Radiographic features:
Early radilucent stage:
Appearance: Inferior cortex of mandible may appear osteoporotic and possess a
laminated structure
Trabecular arrangement: Bone pattern in which trabecular though reduced in
number, run linearly in the direction of length of bone and have few intersections
between them. This appearance is most commonly seen posterior to bicuspid. In
the anterior region the bony trabeculae are coarse and relatively straighten than
normal but they intersect producing bone spaces that are larger than normal.
Coarse and Sparse trabeculae, sometimes tend to converge toward the midline of
mandible which is highly suggestive of pagets disease. In this stage, inferior
border of the mandible may appear osteoporotic and have a laminated structure.
Effect on Root: Root resorption is common.
Osteoporosis circumscripta: In the Skull, early lytic lesion may be seen as discrete
radiolucent areas termed as osteoporosis circumscripta. The margins are somewhat
irregular. There is appearance of denser bone around the radiolucency.
Lincolns sign or black beard: This sign is present in bone scintigraphy. Bone scan
may demonstrate marked uptake throughout the entire mandible. This is called as
Lincolns sign or black beard.
Granular or ground glass appearance:
Radiopaque Patches: There are rounded radiopaque patches of abnormal bone of
greater density within the radiolucent bone in which it is not possible to see any
actual bone structure.
Size: These densities may be a centimeter larger or only a few millimeters in
diameter.
A dense, more radiopaque stage:
Cotton wool appearance: In later stages, rounded radiopaque patches of abnormal
bone are often seen, giving an impression of cotton wool. As the fully opacified
area becomes more numerous and enlarged, they tend to coalesce. The bone is
denser and appears whiter on the radiograph.
Enlargement of bone: It enlarges the affected bone and it may be four times than
their normal thickness on lateral radiographs. In some cases, there is irregular
enlargement of alveolar processes which become prominent and bulge.
Prognathism of mandible: Due to deposition of bone in mandible, it may appear
prognathic.
Effect on maxillary sinus: Lesions in maxillary area may encroach the maxillary
sinus and obliterate it.
Hypercementosis: It may be produced on one or more teeth.
Ankylosis of teeth: It may obliterate the areas of lamina dura and Periodontal
ligament space around both normal and hypercementosed roots resulting in
ankylosis of teeth.
Osteogenic Sarcoma: Development of osteogenic sarcoma may produce frank
dissolution or destruction of bone.
In it, there is loss of continuity of the margins on the one side of the lesion
which is suggestive of the destruction. Also in some cases, soft tissue shadow is
seen.
Diagnosis:
Clinical Diagnosis: In this simian appearance, enlargement of skull bowing of leg
and bone pain may give clue to the diagnosis.
Radiological diagnosis: Cotton wool appearance, Lincoln sign and osteoporosis
circumscripta are diagnostic features of Pagets disease.
Laboratory diagnosis: Serum alkaline Phosphatase level is increased occasionally
attending the level of 200 or more KA units. Serum calcium and Serum
Phosphorus levels are within normal limits. Urinary hydroxyproline levels increase
from normal levels of 440mg/24 hours to 1g/24 hours. Histologically there is
appearance of mosaic bone.
Differential Diagnosis:
Early stage Radiovalent appearance
Giant cell lesions of hyperparathy roidism
Osteoporosis
Osteomalacia
Multiple myeloma
Second stage Mixed radiolucent radiopaque appearance:
Osteogenic sarcoma
Cementifying and ossifying fibroma
Fibrous dysplasia
Osteoblastic metastatic carcinoma
Ossifying sub-periosteal hematoma
Ossifying Post Surgical defect
Chronic Osteomyelitis
Chondroma and chondrosarcoma
Advanced Stage: Purely Radiopque
Florid osseous dysplasia
Osteosclerosis
Tori
Osteoma
Management:
Medical:
Clcitonin: A parathyroid hormone antagonist produced by the thyroid gland,
suppresses bone resorption and also relieves pain and decrease serum alkaline
phosphatase levels.
Sodium Phosphate: It retards bone resorption
Bisphosphonates: They have also been used since they inhibit bone resorption as
well as mineralization. Bisphosphonates which are used as etidronate,
pamodronate, alendronate, tiludronate or risedronate.
Mithyramycin: It is considered as a second line agent due to its toxicity is
cytotoxic to osteoclasts.
Plicamysin: It inhibits osteoclastic activity.
Surgery: Surgical approach for esthetic correction should be carried out.
Radiation: In some cases, radiation therapy can be useful.
