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症例は4歳の男児で,Freeman-Sheldon症候群にみられる口笛を吹くような特異的顔貌,両内反足を認め,手指に拘縮はないが右短合指症と右大胸筋欠損,右乳頭形成不全を伴い,Poland症候群を合併していた.他医により両内反足に対し,生後3カ月時と6カ月時に両側アキレス腱切腱術,後方解離術が施行されたが変形は再発した.2歳8カ月時に当科にて後内側解離術,足底腱膜切離,母趾外転筋切離を施行し,足底接地可能で経過は良好である.本症の内反足は難治性で,初回からの広範囲な軟部組織解離術が必要と思われる.
We report a case of bilateral congenital clubfoot in Freeman-Sheldon syndome associated with Poland syndrome.
A 4-year-old boy had a whistling-face and bilateral congenital clubfoot, which are common features of Freeman-Sheldon syndome, and also had absence of the right pectoralis muscles and hypoplasia and syndactyly of the right hand, which are characteristic of Poland syndome. Tenotomy of the Achilles tendon and posterior release were performed twice, once at 3months of age and again at 6months of age. But neither of these operations was sucessful. At a 2 years 8 months of age we performed bilateral posteromedial release, plantar fascia release, and abductor hallucis tendon release to achieve plantigrade feet, and at the most recent follow-up examination, at four years of age, there was no evidence of any tendency to recur. Congenital clubfoot in Freeman-Sheldon syndrome is difficult to treat, and wide soft tissue release is necessary to correct the deformity at the time of the initial surgery.
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