Abstract
Popliteal pterygium syndrome is a rare disorder, usually showing autosomal dominant inheritance. A four-year-old girl presented with a popliteal web on the left extending from the buttock to the heel. She also had an incomplete cleft lip, hypoplastic labia majora and foot/digit/nail anomalies. The popliteal web was released by Z-plasty procedures. However, the patient dropped out of further treatment. Ten years later she was brought again with severe deformity in her left lower extremity, tibial shortening of approximately 15 cm and foot deformity. Nearly-full knee extension was obtained and below-knee amputation was performed at the patient’s request. Plans of inserting a prosthesis was prevented by the loss of contact with the patient and her family.
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