Summary Background Although the survival rate of esophageal atresia (EA) has increased to over 90%, the risk of functional long-term neurodevelopmental deficits is uncertain. Studies on long-term outcomes of children with EA show conflicting results. Therefore, we provide an overview of the current knowledge on the long-term neurodevelopmental outcome of children with EA. Methods We performed a structured literature search in Embase, Medline Ovid, Web of Science, Cochrane CENTRAL, and Google scholar on November 8, 2020 with the keywords ‘esophageal atresia’, ‘long-term outcome’, ‘motor development’, ‘cognitive development’, and ‘neurodevelopment’. Results The initial search identified 945 studies, of which 15 were included. Five of these published outcomes of multiple tests or tested at multiple ages. Regarding infants, one of six studies found impaired neurodevelopment at 1 year of age. Regarding preschoolers, two of five studies found impaired neurodevelopment; the one study assessing cognitive development found normal cognitive outcome. Both studies on motor function reported impairment. Regarding school-agers, the one study on neurodevelopmental outcome reported impairment. Cognitive impairment was found in two out of four studies, and motor function was impaired in both studies studying motor function. Conclusions Long-term neurodevelopment of children born with EA has been assessed with various instruments, with contrasting results. Impairments were mostly found in motor function, but also in cognitive performance. Generally, the long-term outcome of these children is reason for concern. Structured, multidisciplinary long-term follow-up programs for children born with EA would allow to timely detect neurodevelopmental impairments and to intervene, if necessary.

中文翻译：

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食管闭锁患儿的长期神经发育：系统评价

摘要背景 虽然食管闭锁 (EA) 的存活率已增加到 90% 以上，但功能性长期神经发育缺陷的风险尚不确定。对患有 EA 的儿童的长期结果的研究显示出相互矛盾的结果。因此，我们概述了有关 EA 儿童长期神经发育结果的当前知识。方法 我们于 2020 年 11 月 8 日在 Embase、Medline Ovid、Web of Science、Cochrane CENTRAL 和 Google 学者中进行了结构化文献检索，关键词为“食管闭锁”、“长期结果”、“运动发育”、“认知”发育”和“神经发育”。结果 最初的搜索确定了 945 项研究，其中包括 15 项。其中五项已公布的结果是多次测试或在多个年龄进行的测试。关于婴儿，六项研究中的一项发现 1 岁时神经发育受损。关于学龄前儿童，五项研究中有两项发现神经发育受损；一项评估认知发展的研究发现正常的认知结果。两项关于运动功能的研究都报告了损伤。关于学龄儿童，一项关于神经发育结果的研究报告了损伤。四项研究中有两项发现认知障碍，两项研究运动功能的研究均发现运动功能受损。结论 已经使用各种仪器评估了出生时患有 EA 的儿童的长期神经发育，并得出了不同的结果。损伤主要见于运动功能，但也见于认知表现。一般来说，这些孩子的长期结果是值得关注的。结构化的，