Management of femoral fractures in adult onset osteopetrosis: A multicentric retrospective study

Abstract

Background: Osteopetrosis is a relatively rare disease with an incidence of approximately 1 case per 2,00,000 to 5,00,000 population. The clinical features of osteopetrosis includes pathological fractures, severe anemia due to defective erythropoiesis, bone marrow failure, coagulation defects, propensity for developing severe infections. Common fractures seen in these patients include hip and femoral fractures. Management of fractures in these patients is technically difficult and postoperative complications are common. We conducted a retrospective study of 20 patients with osteopetrosis who presented with femoral fractures and were treated in our institute. Materials and Methods: This was a multicentric retrospective study of 20 patients with osteopetrosis who presented with femoral fractures. The patients were included in this study on the basis of predefined inclusion and exclusion criteria. The diagnosis was based upon imaging studies (X-Rays in all the patients and CT in selected cases). The operative procedure post-operative complications, functional outcome and complications during follow up were studied from case papers. The data was tabulated and analyzed using Minitab 17 version software.Results: Out of 20 studied cases there were 16 (80%) males and 4 (20%) females with a M:F ratio of 1: 0.25. The most common affected age group was found to be between 30 – 35 years (35%). Majority of patient sustained fracture following motor vehicular accidents (65%) followed by fall from height (20%) and direct blow or assault (15%). 13 patients were found to be having co-morbidities including anemia, carpal tunnel syndrome and visual disturbances. Open reduction and internal fixation was done in all the cases. Technical difficulties seen during operative procedures included difficult because of obliterated canal, sclerosed medulla and dense swarf. Common complications in these patients included wound infection, hematoma formation and arthritis of hip joint (5%). Repeat surgeries were required in 3 (15%) patients and 1 (5%) patient developed non-union of the fracture ends. Conclusion: Management of femoral fracture in patients with osteopetrosis is challenging for operating orthopedic surgeon. Common problems encountered during open reduction and internal fixation include overheating during drilling, smoke production and increased temperature of both bone and drill bits and production of dense bone swarf. Orthopedic surgeon should be aware of these technical difficulties encountered during operative interventions.