先天性多発性関節拘縮症に合併した脊柱側弯症の3例

Surgical Treatment for Scoliosis Associated with Arthrogryposis Multiplex Congenita. Report of Three Cases

　背景：先天性多発性関節拘縮症に合併した脊柱側弯症の手術成績は不良と考えられてきた．

　対象と方法：本症に合併した側弯症に対し後方矯正固定術を行った3例を対象に，手術治療成績を検討した．

　結果：術前主カーブの可撓性は17％，12％，22％と著しく低下していた．さらに全例に拘束性換気障害を認めた．術後の矯正率は72％，74％，23％であった．周術期に腸閉塞，心肺停止を各1例経験した．

　結語：本症に合併する側弯症は可撓性が低く矯正が困難である．また，拘束性換気障害を伴うため，周術期の呼吸管理が重要である．

　Background：The results of corrective surgery for spinal scoliosis associated with arthrogryposis multiplex congenita (AMC) have been reported to be poor.

　Materials ＆ methods：We report the clinical results of corrective surgery for severe scoliosis in three patients with AMC.

　Results：The preoperative flexibility of the main curves of the spine in the three patients was 17%, 12%, and 22%, respectively.　Restrictive pulmonary dysfunction was diagnosed in all three patients.　After posterior corrective surgery with segmental pedicle screw systems, Smith-Petersen osteotomy, and a double bender technique, the main curves were corrected to 17°, 22°, and 86°, respectively, and the correction rate was 72%, 74%, and 23%, respectively.

　Conclusion：Extremely rigid spinal curves associated with AMC were one of the reasons for the poor clinical results described in previous reports.　Our surgical techniques were effective in two of our three patients.　However, intensive care should be taken to prevent perioperative respiratory complications because of restrictive pulmonary dysfunction that is always associated with scoliosis in patients with AMC.