Department of Pediatric Surgery, Istanbul Medeniyet University Faculty of Medicine, Istanbul, Turkey.
Department of Pediatric Surgery, Goztepe Prof. Dr. Suleyman Yalcin City Hospital, Istanbul, Turkey.
Eur J Pediatr Surg. 2024 Feb;34(1):44-49. doi: 10.1055/a-2123-5026. Epub 2023 Jul 5.
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.
A database search was done for the years 2015 to 2022.
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 ( = 3) and major cardiac malformations ( = 3).
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.
先天性十二指肠梗阻和食管闭锁（EA）并存的情况，其发病率和死亡率均较高。目前，针对这种关联，其治疗策略尚未明确。本研究评估了土耳其 EA 注册处的数据。
对 2015 年至 2022 年的数据进行了数据库检索。
在 857 例 EA 患者中，31 例（3.6%）合并先天性十二指肠梗阻。平均出生体重为 2104（±457）g，6 例患儿体重低于 1500g。26 例（84%）为 C 型 EA。15 例患儿存在完全性十二指肠梗阻，16 例存在不完全性十二指肠梗阻。27 例（87%）患儿存在其他畸形。15 例（48%）患儿存在 VACTERL-H 畸形，10 例（32%）存在肛门直肠畸形，6 例（19%）存在重大心脏畸形，3 例（10%）存在 21 三体综合征。10 例（32%）患儿的十二指肠梗阻诊断延迟，中位时间为 7.5（1-109）天。食管病变的诊断也存在延迟，2 例患儿的食管病变诊断延迟。在同时存在两种疾病的 19 例患儿中，1 例患儿未接受手术治疗死亡，6 例行三切口修复术（TEF、EA 和十二指肠梗阻），3 例患儿在同一次手术中同时行 TEF 和十二指肠梗阻修复术。其余 9 例患儿计划分期修复。共有 15 例（48%）患儿接受了胃造口术，8 例患儿因长段 EA 行胃造口术。25 例（77%）患儿存活。死亡原因分别为脓毒症（ = 3）和重大心脏畸形（ = 3）。
先天性十二指肠梗阻合并 EA 是一个复杂的问题，其诊断常存在延迟。关于单阶段修复或胃造口术插入的治疗策略，因患儿特征和机构偏好的不同而存在显著差异。
