Polyostotic Fibrous Dysplasia- a case analysis
Background: Fibrous dysplasia of bone is a congenital non heritable disorder that was first reliably reported by von Recklinghausen, when he first described patients with pathologic condition of bone characterized by deformity and fibrotic changes that he termed as osteitis fibrosa generalisata. Fibrous dysplasia may involve one bone(monostotic) or multiple bones(polystotic) and occurs throughout the skeleton with predilection for long bones, ribs and cranio-facial bones. 70% of the lesions are monostotic, asymptomatic and identified incidentally. The polyostotic form of disease is often deforming and devastating with multiple skeletal complications like repeated fractures, limb length discrepancies and bone pains. The bone lesion of unknown origin is characterized by slow progressive replacement of normal bone by abnormal proliferative, isomorphic fibrous tissue.
Case Report: This case report documents a 40 yr old male with severe polyostotic fibrous dysplasia that involved most of the skeleton including long bones of all extremities, pelvis, facial bones and skull base. Initial evaluation consisted of physical examination, plain radiographs which was followed by CT scan, SPECT scan, bio-chemical and hematological investigations and histologic examination. This poster stresses on the clinical implications and management of this rare debilitating disease.