Management of proximal femur varum deformity in skeletal dysplasia: an illustrative case report

Abstract

<p>Metaphyseal chondrodysplasia is skeletal dysplasia involving long tubular bones at metaphyseal regions, sparing the epiphysis, predominantly involving lower limbs, hence hindering normal mobilization activity of the individual. Individuals present with pain and deformity due to pathological fracture at the deformity site which necessitates deformity correction and surgical fixation. This is a case of skeletal metaphyseal dysplasia in 47-year female, short statured with bilateral proximal femoral Vara with pathological fracture, treated with deformity correction, surgical fixation. Patient initially presented at 36 years of age with sudden onset left hip pain. She was diagnosed with left femoral neck fracture and coxa Vara deformity. The femoral neck had spontaneously fractured due to increased bending forces acting on the femoral neck due to the coxa Vara deformity. She underwent valgus osteotomy with dynamic hip screw fixation. Further advised for prophylactic corrective osteotomy of contralateral proximal femur however patient was lost to follow-up. At 47 years she presented with pain in the right thigh. She was diagnosed to have right proximal femur pathological fracture due to progressive femur varus deformity for which she underwent right proximal femur valgus closing wedge subtrochanteric osteotomy and fixation using dynamic condylar screw. In our case left proximal femur which was operated did not have any complications. However, right proximal femur which was operated, at four months follow-up revealed no complications except for the foot drop which she developed post-surgery. Hence main aim of the study is to prove that the early prophylactic surgical management of the Vara help to provide successful outcomes.</p>