Management of children with congenital hypoplasia - defficient femur

Abstract

Congenital femoral hypoplasia is an extremely rare and severe dysplasia. The main characteristic is the severe leg length discrepancy. It covers a wide spectrum of femoral deformities ranging from hypoplasia to severe proximal deficiency. The aim of our study is to describe the management of the femoral deficiency. We used distraction histogenesis, with a circular Ilizarov frame, for the children that have prospective to equalize the discrepancy. A series of 6 children (5 girls and 1 boy) were treated over a period of 10 years. Patients were classified with the Pappas method. During the first 5-6 years of age, using appropriate orthotic devices, all of them had achieved a reasonable independence for their daily and school activities. In 3 of our patients treatment provided was distraction histogenesis using Ilizarov frame. For 1 patient, with untreatable dis-crepancy, we continue with special orthotic device. The increase in length was 5 cm to 6 cm, with an increase of 32% of the initial length (ranging from 33% up to 42%). The mean healing index was 17 days/cm. Severe complications presented during the procedures. Twice a child had fractures of the regenerated bone, soon after the removal of the device. They were treated with reapplication of the frame until union of the fractured bone. One child had knee subluxation that is partly corrected with realignment of the device at the knee joint. Treating children with deficient femur is a great challenge for the surgeon. It is almost impossible to correct in one procedure the whole discrepancy. The active life of the surgeon is not sufficient to correct all the deformities of the growing child.