The objective of this study was to describe prevalence and epidemiologic data on esophageal atresia from 23 well-defined European regions and to compare prevalences between these regions. We wanted to characterize the cases as syndromic, multiple malformed or isolated. Furthermore we wanted to characterize the cases with regards to gender, birth type, gestational age, birth weight and survival.
The study is based on data from 23 European registries of congenital malformations (EUROCAT). For further details see www.eurocat.ulster.ac.uk/ memberreg/. All cases with ICD9 and ICD10 code with EA born 1987-2006 were included. The total number of births was 5,027,181.
1222 cases of EA were recorded giving an overall prevalence of 2.43 per 10,000 births. There where large regional differences in the prevalence. Also the prenatal detection rates showed large regional differences.
57.3% of cases were boys. 70.5% had a tracheoesophageal fistula along with the atresia.
88.7% of cases were live births, 3.5% were still births and 7.8% were termination of pregnancy. Mean birth weight in the liveborn cases was 2462±766 grams and median gestational age was 38 (35-39) weeks.
The cases were classified in three groups: 44.7% had isolated esophageal malformation, 31.6% were multiple malformed, 23.7% had an association or syndrome.
During the study period the prenatal detection rates increased.
There are large regional differences in the prevalence and in the prenatal detection rate.
After diagnosis of EA, it is important to evaluate the infant for associated malformations or syndromes, as these are present in more than half of all cases.
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Pedersen, R., Husby, S. & Garne, E. 443 Epidemiological Characteristics of Esophageal Atresia in Europe, 1222 Cases. Pediatr Res 68, 227–228 (2010). https://doi.org/10.1203/00006450-201011001-00443