Zhang Yan,Bao Kun,Chen Bochang.Correction of genu valgum in Ellis-van Creveld Syndrome:one case report[J].Journal of Clinical Pediatric Surgery,2024,(07):685-688.[doi:10.3760/cma.j.cn101785-202203041-015]
Ellis-van Creveld综合征源性膝外翻的矫形治疗1例
- Title:
- Correction of genu valgum in Ellis-van Creveld Syndrome:one case report
- 关键词:
- 膝外翻; Ilizarov技术; 截骨矫形; 外科手术; 儿童
- 摘要:
- 埃利伟氏 (Ellis-van Creveld syndrome,EVC)综合征即软骨外胚层发育不良,是一种常染色体隐性遗传性疾病,发病率约为1/1 000 0000,以软骨及外胚层发育不良、先天性心脏病为主要特征。就骨科病症而言,进行性膝外翻伴髌骨脱位对患儿下肢行走能力及外观的影响最大,而膝外翻矫正术后复发率高是骨科医师面临的主要问题。本文介绍上海交通大学医学院附属第六人民医院收治的1例EVC源性膝外翻病例,在回顾相关文献的基础上,利用跨关节外固定支架逐渐矫正下肢轴线,为后续彻底矫正关节内塌陷提供必要的条件与支撑,再分侧分期逐步全面矫正畸形,患儿术后随访8年余,矫形效果维持良好。
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备注/Memo
收稿日期:2022-3-12。
通讯作者:鲍琨,Email:dr_baokun@163.com