骨形成不全症における病的大転子下骨折の2例の管理

Osteopetrosis is a rare genetic disorder characterized by increased bone density due to defective osteoclast function, leading to brittle bones prone to pathological fractures. The management of fractures in osteopetrotic patients presents unique challenges due to extreme bone hardness, narrow medullary canals, and increased risk of non-union or implant failure.

We present two cases of pathological subtrochanteric femoral fractures in young female patients with osteopetrosis. The first case involved a 30-year-old female with bilateral subtrochanteric fractures, managed surgically with open reduction and internal fixation (ORIF) using a distal femur locking plate for the complete right-sided fracture, while the left-sided unicortical fracture was treated conservatively. The second case involved a 29-year-old female with a history of prior femoral fractures, presenting with a subtrochanteric fracture of the left femur and a broken plate, which was also managed by Open reduction internal fixation by an anatomical proximal femur plate. Due to the dense sclerotic bone, modifications to standard surgical techniques were necessary, including the use of low-speed drilling with continuous cooling, pre-tapping for screw insertion, and careful implant selection.

The management of osteopetrotic fractures requires a multidisciplinary approach and specialized surgical techniques. Our cases highlight the challenges and successful strategies for treating subtrochanteric fractures in osteopetrosis, contributing valuable insights to the orthopedic management of this rare condition.