Child Population and Translational Health Research, Children's Hospital at Westmead Clinical School, University of Sydney, Sydney, Australia.
The Western Australian Register of Developmental Anomalies, Department of Health, Government of Western Australia, Subiaco, Australia.
Birth Defects Res. 2021 Jul 15;113(12):945-957. doi: 10.1002/bdr2.1891. Epub 2021 Mar 18.
Esophageal atresia (EA) affects around 2.3-2.6 per 10,000 births world-wide. Infants born with this condition require surgical correction soon after birth. Most survival studies of infants with EA are locally or regionally based. We aimed to describe survival across multiple world regions.
We included infants diagnosed with EA between 1980 and 2015 from 24 birth defects surveillance programs that are members of the International Clearinghouse for Birth Defects Surveillance and Research. We calculated survival as the proportion of liveborn infants alive at 1 month, 1- and 5-years, among all infants with EA, those with isolated EA, those with EA and additional anomalies or EA and a chromosomal anomaly or genetic syndrome. We also investigated trends in survival over the decades, 1980s-2010s.
We included 6,466 liveborn infants with EA. Survival was 89.4% (95% CI 88.1-90.5) at 1-month, 84.5% (95% CI 83.0-85.9) at 1-year and 82.7% (95% CI 81.2-84.2) at 5-years. One-month survival for infants with isolated EA (97.1%) was higher than for infants with additional anomalies (89.7%) or infants with chromosomal or genetic syndrome diagnoses (57.3%) with little change at 1- and 5-years. Survival at 1 month improved from the 1980s to the 2010s, by 6.5% for infants with isolated EA and by 21.5% for infants with EA and additional anomalies.
Almost all infants with isolated EA survived to 5 years. Mortality was higher for infants with EA and an additional anomaly, including chromosomal or genetic syndromes. Survival improved from the 1980s, particularly for those with additional anomalies.
全世界范围内，食管闭锁（EA）的发病率约为每 10000 名活产婴儿中有 2.3-2.6 名。患有这种疾病的婴儿需要在出生后不久接受手术矫正。大多数关于 EA 婴儿的生存研究都是基于局部或区域性的。我们旨在描述多个世界区域的生存情况。
我们纳入了 24 个出生缺陷监测项目的诊断为 EA 的婴儿，这些项目均为国际出生缺陷监测和研究信息交换所的成员，时间范围为 1980 年至 2015 年。我们计算了所有 EA 婴儿、单纯 EA 婴儿、EA 伴额外异常或 EA 伴染色体异常或遗传综合征婴儿的活产婴儿在 1 个月、1 岁和 5 岁时的生存比例。我们还研究了几十年来（20 世纪 80 年代至 21 世纪 10 年代）的生存趋势。
我们纳入了 6466 例活产 EA 婴儿。1 个月时的生存率为 89.4%（95%CI 88.1-90.5），1 岁时为 84.5%（95%CI 83.0-85.9），5 岁时为 82.7%（95%CI 81.2-84.2）。单纯 EA 婴儿的 1 个月生存率（97.1%）高于伴有额外异常（89.7%）或伴有染色体或遗传综合征诊断的婴儿（57.3%），1 岁和 5 岁时几乎没有变化。1 个月时的生存率从 20 世纪 80 年代到 21 世纪 10 年代有所提高，单纯 EA 婴儿提高了 6.5%，伴有额外异常的婴儿提高了 21.5%。
几乎所有单纯 EA 婴儿都能存活到 5 岁。伴有额外异常，包括染色体或遗传综合征的 EA 婴儿死亡率更高。从 20 世纪 80 年代开始，生存率有所提高，特别是对于伴有额外异常的婴儿。
