Background: The major problems associated with
severe congenital deficiency of the femur are an unstable hip joint
and a femur that is more than 50% shorter than the contralateral,
normal femur. The usual treatment of these extreme cases of congenital
femoral deficiency is a Syme or Boyd amputation when the child begins
to walk. A knee fusion is done when the child is older, and the
patient functions as an above-the-knee amputee. Rotationplasty has
been described as an alternative treatment that allows the patient
to function as a below-the-knee amputee. None of the currently described
types of rotationplasty address the problem of the unstable hip.
Methods: Three patients with severe congenital femoral
deficiency underwent a unique single-stage reconstruction. In this
procedure, the limb is completely detached except for the sciatic
nerve and the femoral vessels. The proximal part of the dysplastic femur
and some muscles are resected. The residual limb is externally rotated
180° and the rotated distal part of the femur is fused to the pelvis.
All of the muscles distal to the knee remain undisturbed.
Results: The anatomical knee in its rotated position
functioned as a hip with flexion and extension, and the femoropelvic
arthrodesis provided a more stable support. The rotated ankle acted
as a knee, and the patients functioned as below-the-knee amputees. The
duration of follow-up of these three patients was eight, six, and
four years. Active hip flexion was 10° to 70°, 10° to 90°, and 0°
to 80°, and active knee flexion was 90° and 95°. The patients' gait continued
to improve as they matured.
Conclusions: The femoropelvic arthrodesis provides
a stable hip. Since the muscles distal to the knee are not disturbed,
the problem of derotation of the limb, which is often seen following
the Van Nes rotationplasty, is not seen after this operation.