膝窩翼状片症候群の1例

Popliteal Pterygium Syndrome. Case Report

　本邦で初めての膝窩翼状片症候群(popliteal pterygium syndrome)の1例を報告した．症例は女児で，妊娠週数38週の正常分娩にて出生したが，右下肢に翼状片を伴う変形を認め，心房中隔欠損症と膣低形成，外反扁平足を合併していた．翼状片によって－90°の膝関節伸展制限を認めたが，生後6カ月から3歳までに右下肢アキレス腱延長術と皮膚形成術を3回施行した．8歳の現在，膝関節の屈曲140°，伸展－20°で約4cmの脚長差を認めるが，走行，跳躍は可能である．

　We report the case of popliteal pterygium syndrome ever reported in Japan.　The patient is an 8-year-old girl who was the product of a normal delivery at 38 weeks of gestation.　A popliteal pterygium of the right leg that limited extension of the knee to approximately －90°, a hypoplastic vagina, and talipes planovalgus were noted at birth.　Between 6 months and 3 years of age three surgeries were performed to lengthen the Achilles tendon and skin of the right leg.　At present the range of motion of her right knee is 140° of flexion and －20° of extension.　Although the right leg is approximately 4cm shorter than the left, the patient is able to run and jump.