Journal of Clinical and Diagnostic Research (Feb 2012)
Failure of treatment of Coxa Vara in Gauchers Disease: A Case Report
Abstract
Gaucher’s disease (GD) is an autosomal recessive storage disorder which occurs due to the deficient functioning of the lysosomal hydrolase enzyme [1, 2]. In this case report, coxa vara occurred after the union of a stress fracture in the basicervical and the intertrochanteric region of the left femur of a male child who had Gaucher’s disease. Femoral Valgus osteotomy and fixation with cannulated screws was done. The disease showed a progressive course even after the surgery, resulting in refracture, increase in the coxa vara and shortening of the limb. An incidental association of Perthe’s disease in the contralateral hip was seen.