Polyostotic fibrous dysplasia - a rare case report

INTRODUCTION Fibrous dysplasia, is an anomaly characterised by widening of the affected bone with cortical thinning and presence of fibro-osseous tissue inside the bone. It may present under a monostotic or polyostotic form. The main orthopaedic problems include coxa vara (shepherds crook deformities of the proximal femur), pathologic fractures, bone pain, deformities of other bones, and subsequent major problems with walking. Treatment of symptomatic patients should have a long-term perspective and should minimise the risk of recurrence and fracture or deformity which may occur secondarily. MATERIALS AND METHODS Our patient is a 23 yrs old male patient , presented to us with pain in left hip and thigh and was subsequently diagnosed as a case of polyostotic fibrous dysplasia. Considering Mirels criteria, prophylactic fixation was done for proximal femur with reconstruction nailing. Post operatively patient was followed up closely. RESULTS Though patient sustained a pathological fracture after fixation , fracture healed well and no additional procedures were done for the same, thus minimising the morbidity. Patient at his one and half years follow up, has full functional range of movements without pain or progression of lesion. CONCLUSION Polyostotic fibrous dysplasia is a rare disease constituting only one fifth of fibrous dysplasia. Pain at functional range of movements, deformities and pathological fracture are relatively common in these patients. Prophylactic fixation of lesion as per Mirels criteria will avoid problems that may occur following pathological fractures and morbidity.