A patient with type I Gaucher's disease had avascular necrosis of the right
femoral head that resulted in an altered bony pelvis and marked restrictio
n of right hip abduction. Enzyme replacement therapy with alglucerase preve
nted further deterioration and improved thrombocytopenia. Vaginal delivery
was achieved with the patient in the left lateral position with exaggerated
flexion at the contralateral hip.