治療に難渋した先天性多発性関節拘縮症に伴う脊柱後側弯症の1例

A Severe Progressive Kyphoscoliosis Secondary to Arthrogryposis Multiplex Congenita. Case Report

　患者は14歳の女児で，先天性多発性関節拘縮症に併発した側弯111°，後弯90°の進行性脊柱変形を認めた．術前矯正目的でhalo gravity牽引を予定した．Haloリング装着時に，2度の心肺停止を来したが蘇生した．手術は椎弓根スクリューを用いた後方矯正固定術を行ったが，カーブの可撓性が低いこと，骨が脆弱であったことから側弯は86°，後弯は75°までの矯正にとどまった．本疾患による側弯症は進行性で可撓性に乏しいため，早期の手術を考慮する必要がある．また，拘束性肺機能障害，顎関節硬化と頚椎可動域低下があるため，術前に呼吸機能の十分な評価を要する．

　We report a case of arthrogryposis multiplex congenita (AMC) complicated by severe progressive kyphoscoliosis.　A 14-year-old girl with AMC presented with a severe progressive trunk deformity and was referred to our clinic.　Radiography revealed 96° scoliosisand 58° kyphosis in the thoracic spine.　The patient also exhibited severe restrictive pulmonary dysfunction (%VC：18%).　We attempted halo-gravity traction prior to corrective surgery.　During the halo ring attachment, cardiopulmonary arrest occurred twice because of difficulty in intubation, but resuscitation was successful.　Following halo traction for six weeks, posterior correction surgery with a pedicle-screw-only construct was performed, but the extensive rigid curvature and fragility of the vertebrae hampered optimal correction of the deformity.　Since the scoliosis in AMC patients is often extremely rigid and progressive, early surgical intervention should be considered, and meticulous preoperative evaluation of both pulmonary function and the feasibility of intubation is mandatory.