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


ULUKAYA DURAKBAŞA Ç., SOYER T., İLHAN H., Oztan M. O., Uzunlu O., FIRINCI B., ...Daha Fazla

European Journal of Pediatric Surgery, cilt.34, sa.1, ss.44-49, 2023 (SCI-Expanded) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 34 Sayı: 1
  • Basım Tarihi: 2023
  • Doi Numarası: 10.1055/a-2123-5026
  • Dergi Adı: European Journal of Pediatric Surgery
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, CAB Abstracts, CINAHL, EMBASE, MEDLINE, Veterinary Science Database
  • Sayfa Sayıları: ss.44-49
  • Anahtar Kelimeler: duodenal atresia, esophageal atresia, management, outcome, surgery
  • Hacettepe Üniversitesi Adresli: Evet

Özet

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.