Van Nes Rotationplasty and Hip Reconstruction in the Treatment of Proximal Femoral Focal Deficiency (PFFD)

We present a case of a 3 year old, otherwise healthy, child born with proximal femoral focal deficiency (PFFD) Aitken type B. The child has severe leg length discrepancy with the foot on the affected side at level just distal to the contralateral knee. The hip was dysplastic with large ossification defect of the proximal femur and marked varus alignment. Her knee was also dysplastic with significant flexion contracture. She was felt not to be a candidate for leg equalization procedures, and since she had relatively normal ankle and foot, modified Van Nes rotationplasty was recommended as the best treatment option. Her treatment consisted firstly of modified Van Nes rotationplasty followed by hip reconstruction osteotomy as staged procedures 1 year apart. She has become functional rotationplasty prosthesis user with below the knee amputation-like function and a stable hip.