胸郭不全症候群を呈した先天性後側弯症の治療経験

Surgical Treatment of Congenital Kyphoscoliosis in Thoracic Insufficiency Syndrome. Case Report

　胸郭不全症候群とは，胸郭が正常な呼吸と肺の成長をサポートできない状態であり，早期発症の脊柱変形に付随した胸郭の変形により，高度の呼吸機能障害を引き起こす疾患である．われわれは，術前％VCが19.8％の高度拘束性障害を認め，100°を超える先天性後側弯に対し手術加療を施行したので報告する．症例は14歳の男児である．胸椎カーブはX線正面像で152°，側面像で89°の後側弯を認めた．手術は後方矯正固定術を施行した．胸郭不全症候群に対する治療は，脊柱変形の矯正と，脊椎の成長の維持だけでなく，胸郭の矯正による肺機能の改善を図る必要がある．

　Thoracic insufficiency syndrome (TIS) is a condition in which the thorax is unable to support normal respiration or lung growth, and the early onset of scoliosis may cause rib cage deformity and severe respiratory dysfunction.　We report a case of TIS in which it was very difficult to treat severe congenital kyphoscoliosis and restrictive ventilatory impairment.　The patient was a 14 year-old male with 152 degrees of scoliosis and 87 degrees of kyphosis.　Posterior fusion with osteotomy was performed.　The scoliosis was corrected from 152 degrees to 77 degrees.　Treatment for TIS requires not only correction of the spinal deformity but relative correction of the rib cage deformity and pulmonary function.