Abstract
Children with congenital focal deficiency of the proximal femur present many problems that are but rarely encountered by the individual surgeon who is thus unable to accumulate a wide experience. This paper reviews the literature and analyses the treatment of twenty-three cases at the Royal Children's Hospital, Melbourne, with the object of producing a rational plan of treatment. The management of instability of the hip, malrotation, inadequate proximal musculature and leg length inequality are separately considered for five grades of deficiency. Milder forms are amenable to subtrochanteric osteotomy to correct varus deformity. Exploration and grafting of the pseudarthrosis is indicated where progressive deformity develops. In the more severe deficiency, conservative management of the proximal bony defect provides a better result with an operation only rarely indicated. The gross leg length inequality may be most successfully overcome by Syme's amputation with subsequent fusion of the knee to create an above-knee amputee with an end-bearing stump allowing ready fitting of a prosthesis.
