Management of proximal femoral shaft fractures in osteopetrosis: a case series using internal fixation.

Abstract

Osteopetrosis is a group of rare sclerosing bone dysplasias. Orthopedic concerns in osteopetrosis are principally related to the characteristic brittle "marble bone" in which fractures may be easily induced by relatively low-energy mechanisms. Femoral fractures are common in this patient population, and management presents a unique technical challenge. While osteopetrotic bone may be penetrated with a drill bit, the drill bit flutes are immediately filled with bone. This renders the drill ineffective, and generation of significant frictional heat can result in breakage of the drill. This case series describes the long-term management of nine osteopetrotic femoral fractures in three patients. The difficulties encountered in these cases prompted the development of a safe and efficacious technique for intramedullary fixation of these fractures.