Simultaneous Bilateral Femoral Osteonecrosis in Gaucher Disease

Gaucher disease (GD) is one of the most common lysosomal storage disorders. Bone complications are the most critical irreversible consequence of GD. Osteonecrosis (ON) of the femoral head inevitably leads to osteoarthritis and may be managed by hip arthroplasty. The introduction and worldwide use of therapeutic agents (specifically enzyme replacement therapies (ERT)) lowered the prevalence of osteonecrosis events per patient. We present the cases of two female patients who sustained simultaneous bilateral femoral head osteonecrosis after receiving ERT for long periods while exposed to concomitant risk factors related to femoral head ON. Both patients suffered severe pain and deterioration of their daily activity capabilities, and thus, were offered bilateral hip arthroplasty. Surgery was performed in both hip joints during the same procedure. The current report highlights several key aspects of femoral head ON in young patients with GD.

Automated summary

Gaucher disease is a common lysosomal storage disorder and its most critical consequence is bone complications. Osteonecrosis of the femoral head leads to osteoarthritis and can be managed through hip arthroplasty. Enzyme replacement therapy has reduced the prevalence of osteonecrosis events in patients.