A Case of Proximal Focal Femoral Deficit Managed with Uniplanar External Fixator Application (Limb Reconstruction System)

ABSTRACT

Limb elongation surgeries have been described as an important modality for the management of proximal focal femoral deficiencies. Limb reconstruction systems are available to perform these limb lengthening procedures.Congenital focal femoral deficiency is a clinical condition that was first described by Aitken. They are congenital disfigurements of the lower extremities, mostly involving proximal femur. However, they are clearly distinguishable from isolated coxa vara1. It has clinical presentations varying from short femur in mild cases to coxa-vara deformity seen in extreme cases. Other presentations include pseudoarthrosis in the proximal femur, hypoplasia of the lateral femoral condyle, absent cruciate ligaments of knees, muscle hypoplasia, and sponge like network of vessels in the proximal femoral plate.1,2Limb lengthening is performed in patients with Paley’s type 1a and 1b congenital femoral deficiency. In cases with Paley Type 2a congenital femoral deficiency, treatment options include knee arthrodesis along with extension prosthesis fitting. Van des rotationplasty, Symes amputation or ablative techniques are the other surgical modalities available.3,4Current literature does not provide us with just a single treatment of choice which is completely safe and successful for limb lengthening procedure in congenital proximal focal femoral deficiency patients. Limb reconstruction system is an excellent method for femoral lengthening by external fixation technique.5 Here we present a case of a female child, aged seven with unilateral proximal femoral focal deficiency managed with LRS technique.