呼吸機能障害を来したKlippel-Feil症候群に合併した高度側弯症の1例

Severe Scoliosis with Respiratory Failure Associated with Klippel-Feil Syndrome. Case Report

　症例はKlippel-Feil症候群に伴った高度側弯症により，重度の呼吸機能障害を生じた患者である．Cobb角143°で，％VC 14％と呼吸不全状態であり，すぐに手術を行うには危険であった．そこで術前halo牽引を施行した．牽引で側弯と呼吸機能の改善を認めたため手術を施行し，Cobb角110°，％VC 24％に改善した．側弯矯正により横隔膜の機能障害が解消し，呼吸機能が改善したために救命することができた．

　We treated a patient with severe scoliosis and respiratory failure associated with Klippel-Feil syndrome. The Cobb angle was 143°, and the vital capacity was 14％of predicted. Surgery was indicated, but could not be performed because of the risk of respiratory failure. We therefore applied halo-traction, and when the scoliosis and respiratory function improved, we were able to proceed with corrective spinal surgery. Postoperatively, the Cobb angle had improved to 110°, and vital capacity had increased to 24％of predicted. The corrective spinal surgery reduced the obstruction of diaphragm movement and has prolonged the patient's life.