异常形式的食道的诊断和手术挑战:一个案例系列
Nikolay F Shchapov1, Ekaterina V Ekimovskaya2, Denis V Kulikov3
1Veramed Medical Center, 12, Moskovskaya St., Zvenigorod, Moscow Region, Russia.
Pediatrics and neonatology
|December 19, 2025
概括
非典型食道缩症 (EA) 变种需要个性化管理. 通过详细的成像和内镜来识别异常解剖是成功治疗和改善这些罕见病例的结果的关键.
科学领域:
- 儿科手术 儿科手术
- 胃肠病学 胃肠病学
- 医学遗传学 医学遗传学
背景情况:
- 食道缩 (EA) 是一种罕见的先天性形,大约在2500-3000名活产中影响1名.
- 典型的EA涉及盲目上袋和远端气管食管 (TEF),约有86%的病例存在.
- 非典型的形式,包括重复,血管环或功能障碍,不常见,并可能使诊断和治疗复杂化.
研究的目的:
- 审查和分析非典型食道缩症 (EA) 的病例,以强调个性化管理策略的重要性.
- 强调先进的成像和内镜技术在诊断罕见EA变体中的作用.
- 证明量身定制的手术和非手术方法的有效性,以改善复杂EA病例的结果.
主要方法:
- 在2018年至2021年期间治疗的43例食道缩 (EA) 病例的回顾性审查.
- 鉴定和详细分析三例呈现异常解剖或功能特征的病例.
- 评估临床,放射和内镜发现,以指导诊断和手术规划.
主要成果:
- 一个病例涉及长期食管与隔膜的重复,经过手术修复但因吸入性肺炎而复杂.
- 另一个病例呈现出右大动脉门,需要在门后面道化胸腔镜解剖,然后进行狭窄扩张.
- 第三个案例最初被怀疑是EA,通过内镜诊断出由于运动障碍而缩小了食道,并通过输液管养和避免手术成功治疗.
- 队列中的两名婴儿在良好的养结果下幸存下来.
结论:
- 非典型食道缩症 (EA) 变体,约占队列的7%,需要个性化诊断和管理计划.
- 详细的成像和内镜对于识别异常解剖和指导治疗决策至关重要.
- 量身定制的手术技术,如隔膜切除和逆弓解剖,以及对运动障碍的识别,可以显著改善复杂EA病例的结果.
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