Bone scintigraphy in Ollier's disease: A rare case report
A 35-year-old male patient presented with 1 year history of intermittent headache and giddiness. This was sometimes associated with loss of consciousness and involuntary movements in hands. There was occasional loss of bowel and bladder control. In the past patient gave history of hard swellings in the right hand and lower legs from the age of 9 years. The swellings were biopsied and reported as enchondroma. On and off he sustained fractures in hand swellings after trivial trauma. They were managed conservatively with splints and analgesics. Patient had no other symptoms until 1 year back. There was no family history of similar illness. Systemic examination of the patient was unremarkable. Local examination revealed marked bony deformities with hard swellings in both the hands. Hard swellings were also palpable in the right fore arm and distal ends of both lower limbs. The laboratory profile revealed a normal hemogram, normal kidney function tests and normal serum electrolytes. The fasting blood sugar, serum uric acid, calcium and phosphorus were within the normal limits. Serial serum liver function tests revealed isolated elevation of bilirubin ranging from 2.51 mg/dL to 2.58 mg/dL. Total serum proteins were 6.50 g/dL with albumin fraction of 4.5 g/dL. The coagulogram parameters were within the normal limits. The electrocardiogram was normal. Abdominal ultra-sonogram revealed a Grade I prostatomegaly. X-ray examination showed lytic lesions with sclerosed margins in the metacarpals and phalanges of both hands []. Similar lesions were noted in both the tibia and fibula []. There were two exostosis (osteochondroma) one each at the medial aspects of upper ends of both the tibia []. X-ray skull was reported to be normal. In order to see the extent of skeletal involvement patient underwent a whole body technetium-99m methylene diphosphonate scan on a dual head gamma camera fitted with a low-energy all-purpose collimator. The bone scan revealed multiple foci of intense tracer uptake in sternum, right humerus, right radius, right ulna, right hand, right femur, right tibia, right fibula, left hand, left femur, left tibia, left foot []. The lesions predominantly involved the tubular limb bones sparing the axial skeleton. Bone scan revealed extra sites of involvement at right humerus, sternum and both femoral shafts. Bone scan appearance was reported to be consistent with multiple enchondroma seen in Ollier's disease. A non-contrast CT head showed a well-defined hypo-dense lesion with no evidence of perilesional edema in left insular cortex. MRI revealed a well-defined lesion with no perilesional edema in left insular area that was hypo-intense on T1-W image [] and hyper-intense on T2-W image and flair sequences []. Radiological features suggested a glioma in the region of left insular cortex. There was moderate enhancement on contrast imaging. At surgery a grayish mass was seen in the left insular area of brain, which on histo-pathological examination was reported as low grade astrocytoma. A final diagnosis of Ollier's disease associated with osteochondroma, Gilbert's syndrome and insular glioma of brain was made.