Esophageal Atresia Associated with Congenital Duodenal Obstruction: Turkish Esophageal Atresia Registry (TEAR) Evaluation

Abstract

Introduction: Coexistent congenital duodenal obstruction and esophageal atresia (EA) is known to have significant morbidity and mortality. Management strategies are not well-defined for this association. The data from Turkish EA registry is evaluated. Materials and Methods: A database search was done for the years 2015-2022. Results: Among 857 EA patients, 31 (3.6%) had congenital duodenal obstruction. The mean birth weight was 2104 (±457) g with 6 babies <1500 g. Twenty-six (84%) had Type C EA. The duodenal obstruction was complete in 15 patients and partial in 16. Other anomalies were detected in 27 (87%) patients. VACTERL-H was present in 15 (48%), anorectal malformation in 10 (32%), a major cardiac malformation in six (19%) and Trisomy-21 in three (10%). Duodenal obstruction diagnosis was delayed in 10 (32%) babies for a median of 7.5 (1-109) days. Diagnosis for esophageal pathologies was delayed in 2. Among 19 babies with a simultaneous diagnosis, one died without surgery, 6 underwent triple repair for TEF, EA and duodenal obstruction and 3 for TEF and duodenal obstruction in the same session. A staged repair was planned in the remaining 9 patients. In total, 15 (48%) patients received a gastrostomy, the indication was long-gap EA in 8. Twenty-five (77%) patients survived. The cause of mortality was sepsis (n=3) and major cardiac malformations (n=3). Conclusions: Congenital duodenal obstruction associated with EA is a complex problem. Delayed diagnosis is common. Management strategies regarding single stage repairs or gastrostomy insertions vary notably depending on the patient characteristics and institutional preferences. © 2023 Georg Thieme Verlag. All rights reserved.