At birth, a baby girl was found to have central-type eight-toed polydactyly of the left foot associated with ipsilateral nonvisualization of the kidney and atrophy of the gluteal maximum muscle. X-ray examination showed eight completely developed metatarsal and digit bones, and a chromosome study showed a normal 46,XX karyotype. She received ray amputation of the extra toes when she was 1 year old. Follow-up examination at age 9 showed unchanged renogluteal anomalies and a slightly smaller left foot. In addition, a bony defect over the posterior iliac spine and a wide femoral neck-shaft angle were found on the radiograph. Her left leg was found to be 1 cm shorter than the right, but there was no evidence of valgus or varus. Presently she can walk and run quite well, and there is no callus formation over her sole. The timing and options for excision of the supernumerary digits are discussed and this rare disease reviewed.
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