Although the survival rate of esophageal atresia (EA) has increased to over 90%, the risk of functional long-term neurodevelopmental deficits is uncertain.

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.

Esophageal atresia (EA) is a congenital deformity in which the upper esophagus does not connect to the lower esophagus and the stomach, which occurs in 2.43 per 10,000 live births.1 After correction of the defect, >90% of the children born with EA survive nowadays.2 Therefore, long-term outcome requires growing attention. The evaluation of long-term outcome in children born with EA focuses on several aspects, such as gastroesophageal reflux, dysphagia, respiratory problems, weight, growth, quality of life (QOL), psychological status, social behavior, and neurodevelopment.2–4 Most research on long-term outcome of EA has focused on physical impairments or QOL, both in children and young adults.5,6 A recent elaborate review on health related QOL (HrQOL) of patients born with EA concluded that clinical subgroups of children with EA present with impaired HrQOL, and
that digestive symptomology negatively influences the HrQOL.6 Neurodevelopment has been less well studied, and available studies reported conflicting results.
Furthermore, the variety of used test instruments and cohorts make it difficult for clinicians to interpret these results, and a comparative study is lacking.