Gorham-Stout综合征合并脊柱侧后凸一例  

A Case Report of Gorham⁃Stout Syndrome with Kyphoscoliosis

作　　者：李晨恺 王升儒 李子全 吴南 仉建国 LI Chenkai;WANG Shengru;LI Ziquan;WU Nan;ZHANG Jianguo(Department of Orthopaedic Surgery,Beijing Key Laboratory for Genetic Research of Skeletal Deformity,Key Laboratory of Big Data for Spinal Deformities,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;State Key Laboratory of Complex Severe and Rare Diseases,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医院骨科,骨骼畸形遗传学研究北京市重点实验室,中国医学科学院脊柱畸形大数据研究与应用重点实验室,北京100730 [2]中国医学科学院北京协和医院疑难重症及罕见病全国重点实验室,北京100730

出　　处：《罕见病研究》2023年第4期602-606,共5页Journal of Rare Diseases

基　　金：国家自然科学基金(82172382,81972037);中央高水平医院临床科研业务费(2022⁃PUMCH⁃B⁃122,2022⁃PUMCH⁃A⁃045);北京市自然科学基金(7222133,L222096)。

摘　　要：本文报道1例Gorham-Stout综合征(GSS)合并脊柱侧后凸患者的治疗过程。患者男性,11岁,发现脊柱侧后凸4年,加重伴双下肢无力1年。患者同时合并乳糜胸、通气功能障碍和骨质疏松。患者曾于外院接受颅骨牵引术治疗7月余,效果不佳;最终,患者于我院接受后路脊柱侧后凸矫形、内固定、植骨融合术(C4-T8),术后临床疗效良好,矫形满意,双下肢不全瘫明显改善。本文通过典型病例报道及既往文献回顾,以期提高临床医师对该疾病的认识,并为该疾病的诊治提供借鉴。This article reported the treatment of Gorham Stout syndrome(GSS)with kyphoscoliosis.The patient was an 11-year-old male who was presented with kyphoscoliosis,first developed 4 years ago,accompa-nied by incomplete paralysis of both lower limbs.The syndrome worsened in the previous year.He had a history of chylothorax,ventilation dysfunction,and osteoporosis.The patient underwent skull traction in another hospital for 7 months,which was ineffective.Finally,he underwent posterior correction,internal fixation,and bone graft fusion(C4-T8)in our hospital.The postoperative clinical outcomes and the posterior correction were satisfactory,with significant improvement in incomplete paralysis of both lower limbs.This article aims to im-prove the understanding of GSS and provided reference for its diagnosis and treatment through a typical case re-port and review of previous literature.

关 键 词：Gorham-Stout综合征 脊柱侧后凸 乳糜胸 

分 类 号：R682.13[医药卫生—骨科学]