TAR Syndrome

A 3 year old child presented with bilateral complete absence of the radius, presence of both thumbs and bilateral genu varum, coxa valga, and severe internal rotation deformity. The diagnosis was TAR syndrome: thrombocytopenia absent radius syndrome. The complaint of the family was the awkward gait. The patient was treated with guided growth using a stainless steel two-hole plate applied laterally to the physis of the lower femur and upper tibia on both sides. Follow-up every 2 months with standing long film of both lower limbs was carried out. Gradual correction of the deformities occurred over a period of 1 year until overcorrection was obtained. However, there was residual internal rotation deformity of the right side. Removal of the plates was performed under general anesthesia and acute de-rotation through osteotomy of the lower right tibia and fixation with k-wire and above-knee cast for one and half months. At follow-up the gait was markedly improved and normal functional activities were regained. Early application of guided growth principle in children with TAR syndrome can treat the severe knee deformities. Residual internal rotation can be corrected by osteotomy and de-rotation.