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Eur J Pediatr Surg 2024; 34(01): 044-049
DOI: 10.1055/a-2123-5026
Original Article

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

Çiğdem Ulukaya Durakbaşa
1   Department of Pediatric Surgery, Istanbul Medeniyet University Faculty of Medicine, Istanbul, Turkey
2   Department of Pediatric Surgery, Goztepe Prof. Dr. Suleyman Yalcin City Hospital, Istanbul, Turkey
,
Tutku Soyer 
3   Department of Pediatric Surgery, Hacettepe University Faculty of Medicine, Ankara, Turkey
,
Hüseyin İlhan
4   Department of Pediatric Surgery, Eskisehir Osmangazi University Faculty of Medicine, Eskisehir, Turkey
,
Mustafa Onur Oztan 
5   Department of Pediatric Surgery, Izmir Katip Celebi University, Izmir, Turkey
,
Osman Uzunlu 
6   Department of Pediatric Surgery, Pamukkale University, Denizli, Turkey
,
Binali Firinci
7   Department of Pediatric Surgery, Ataturk University School of Medicine, Erzurum, Turkey
,
Rahşan Özcan
8   Department of Pediatric Surgery, Istanbul University-Cerrahpasa Cerrahpasa Faculty of Medicine, Istanbul, Turkey
,
Akgun Oral
9   Department of Pediatric Surgery, Dr. Behcet Uz Child Disease and Surgery Training and Research Hospital, Izmir, Turkey
,
Ilhan Ciftci
10   Department of Pediatric Surgery, Selcuklu Medical Faculty, University of Selcuk, Konya, Turkey
,
Esra Ozcakir
11   Department of Pediatric Surgery, Bursa Yuksek Ihtisas Training and Research Hospital, Bursa, Turkey
,
Ibrahim Akkoyun
12   Department of Pediatric Surgery, Konya Training and Research Hospital, Konya, Turkey
,
Doğuş Güney 
13   Department of Pediatric Surgery, Ankara Yildirim Beyazit University Faculty of Medicine, Ankara, Turkey
,
Onder Ozden
14   Department of Pediatric Surgery, Cukurova University Faculty of Medicine, Adana, Turkey
,
Cengiz Gul
15   Department of Pediatric Surgery, Istanbul Zeynep Kamil Maternity and Children's Diseases Health Training and Research Center, University of Health Sciences, Istanbul, Turkey
,
Coskun Ozcan
16   Department of Pediatric Surgery, Ege University, Izmir, Turkey
,
Ayse Parlak
17   Department of Pediatric Surgery, Bursa Uludag University Medical Faculty, Bursa, Turkey
,
Emrah Aydın 
18   Department of Pediatric Surgery, Tekirdag Namik Kemal Universitesi, Tekirdag, Turkey
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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 the Turkish EA registry is evaluated.

Materials and Methods A database search was done for the years 2015 to 2022.

Results Among 857 EA patients, 31 (3.6%) had congenital duodenal obstruction. The mean birth weight was 2,104 (± 457) g with 6 babies weighing less than 1,500 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 6 (19%), and trisomy-21 in 3 (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, 1 died without surgery, 6 underwent triple repair for tracheoesophageal fistula (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).

Conclusion 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.

Keywords

esophageal atresia - duodenal atresia - management - surgery - outcome


Publication History

Received: 02 May 2023

Accepted: 04 July 2023

Accepted Manuscript online:
05 July 2023

Article published online:
09 August 2023

© 2023. Thieme. All rights reserved.

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