Long-term neurodevelopment in children born with esophageal atresia: a systematic review

Abstract

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.

The risk of autism spectrum disorder and intellectual disability but not attention deficit/hyperactivity disorder is increased in individuals with esophageal atresia

“…The rates of ID may change with longer follow-up, as found in a recent systematic review and meta-analysis of long-term neurodevelopment in children born with EA. 39 Infants up to 12 months of age had cognitive function within the normal limits in most studies, whereas two of four studies in school-aged children (6–18 years) found impaired cognitive performance with lower IQ levels. 17 , 38 …”

Systematic review of long term follow-up and transitional care in adolescents and adults with esophageal atresia - why is transitional care mandatory?

“…In a recent systematic review [33], secondary scoliosis after thoracotomy repair in EA children is reported in 13% of cases; according to a meta-analysis by Drevin et al [34], musculoskeletal complications are less common with the thoracoscopic approach. Considering only adult patients, a study by Sistonen et al [35] reported a 13-fold risk of scoliosis compared to the general population, with rib fusions post-thoracotomy being the strongest predictive factor; however, spinal surgery was never needed. As further studies are needed to define technique-related differences in musculoskeletal outcomes, efforts must be put also in defining the orthopedic follow-up in adult EA patients.…”

Clinical outcome, quality of life, and mental health in long-gap esophageal atresia: comparison of gastric sleeve pull-up and delayed primary anastomosis

“…It has been reported that complex surgeries and long anesthesia may lead to neurodevelopmental delays in cognition, learning, and behavior [ 48 ]. Recently, long-term neurodevelopment impairment in children with EA have been found; especially in motor function and in cognitive performance [ 49 ].…”