Shprintzen-Goldberg症候群に伴った側弯症の4例

Shprintzen-Goldberg Syndrome. Report of 4 Cases

　Shprintzen-Goldberg症候群に合併した側弯症に対し手術を施行した4例を報告する．手術時年齢は平均7.3歳，主弯曲Cobb角は平均103.5°であり，全例で胸腰椎部の後弯変形を伴っていた．手術方法は，growing rod法(2例)および後方矯正固定(2例)であった．インプラントの脱転・創感染をそれぞれ2例に認めた．本症候群に伴う脊柱変形は進行性であり早期治療が望ましいが，手術には多くの困難が伴う．特にgrowing rod法を施行時には，骨の脆弱性のため，インプラントの脱転の可能性が高く十分な注意が必要である．

　Shprintzen-Goldberg syndrome (SGS) is characterized by marfanoid habitus and craniosynostosis, and patients have been found to present with scoliosis.　We report four cases (2 male, 2 female) of SGS with severe scoliosis treated by correction surgery.　Average age at operation was 7.3 years old.　The average Cobb angle of the major curve was 103.5°.　Kyphosis was observed in the thoracolumbar area in all four patients.　Posterior correction surgery was performed with a growing rod in the two juvenile patients, and posterior correction and fusion surgery was performed in the two adolescent patients.　Implant dislodgement and deep wound infection occurred as a postoperative complication in one patient each.　The scoliosis associated with SGS often progresses rapidly and becomes severe.　Although early surgical intervention is necessary, meticulous preoperative planning and careful postoperative patient management are mandatory to prevent complications, including wound infection and implant failure.