Surgical Treatment of Osteopetrosis-Related Femoral Fractures: Two Case Reports and Literature Review

Abstract

Osteopetrosis is a rare hereditary disease which is characterized by increased bone density. Bone resorption is insufficient or fails due to the osteoclast defect in osteopetrosis. Half of the patients are asymptomatic and diagnosed incidentally or based on the presence of fracture. Adult onset osteopetrosis usually presents with hip and proximal femoral fractures. Internal fixation can be performed; however, technical challenges may be experienced due to increased bone density. As in other fractures, nonunion or varus malunion of these fractures may occur. Although rare, osteopetrosis may complicate treatment of fractures in such patients. In this study, we aimed to present two new cases of ADO type II with an osteopetrotic femoral fracture along with the clinical and radiological findings in the light of a comprehensive literature review. Orthopaedics surgeons should be aware of intraoperative technical difficulties and possible postoperative complications during the follow-up period. Investigation would be beneficial for the diagnosis of osteopetrosis such the patient with fractures who has minor trauma history and increased bone density in radiography.

Treatment of Osteopetrosis-Related Subtrochanteric Fracture Using the Ilizarov Compression-Distraction Device: A Case Report

“…Despite literature showing non-operative treatment as a feasible option [4,5], we opted against controlled-traction and casting. As the patient was young, we wanted her to start mobilizing early to prevent joint contractures and other complications associated with prolonged traction-pressure sores, orthostatic pneumonia.…”

“…We reviewed literature [3][4][5][6][7] on the intraoperative and post-operative complications associated with the various surgical techniques, and the subsequent outcomes. Hardware failure and breakage-due to the dense bone are the main intraoperative complications, which in turn lead to prolonged operative time, excessive bleeding, periosteum and tissue damage.…”

“…Hardware failure and breakage-due to the dense bone are the main intraoperative complications, which in turn lead to prolonged operative time, excessive bleeding, periosteum and tissue damage. These issues either directly or indirectly lead to an array of post-operative complications such as delayed union, non-union, infection, osteomyelitis, hardware failure-breakage, cut-out; and also there complications experienced during removal of implant and revision surgery, if required [4][5][6][7].…”

“…Osteopetrosis is a rare hereditary bone disease characterized by increased bone density [1][2][3][4]. There is a failure of bone remodeling due to disease of the osteoclasts..…”

“…ADO 2 patients are usually asymptomatic and are diagnosed either incidentally or while presenting with a fracture as although the density increases, the mechanical strength is decreased [5]. Literature has documented various treatment options-surgical and conservative, with their respective outcomes [3][4][5][6][7]. The common feature noted is the difficulty faced intra-operatively due to the hard, dense bone resulting in equipment failure, prolonged surgery, suboptimal fixations and complications to the patient-blood loss, tissue and bone damage.…”