Abstract
Purpose: Omphaloceles often coexist with chromosomal abnormalities, cardiac malformations, and other complications. Although isolated cases of omphaloceles complicated by gastrointestinal anomalies, such as omphalomesenteric fistulas and Meckel’s diverticula, have been reported, systematic studies are lacking. In this study, we investigated a series of cases of omphaloceles complicated by omphalomesenteric fistulas, focusing on their clinical characteristics.
Methods: Data were collected from children with omphaloceles complicated by omphalomesenteric fistulas treated at Osaka Women and Children’s Hospital and Osaka University Hospital between 1981 and 2021. Their perinatal data and clinical course were retrospectively reviewed.
Results: Fifteen patients were included in this analysis; 14 (93.3%) exhibited herniation into the umbilical cord, predominantly involving the intestine, and one showed involvement of the intestine and liver. Chromosomal anomalies were identified in eight patients (53.3%), all with trisomy 13. Cardiac malformations were present in seven patients (46.7%), six of which had trisomy 13. Nine patients survived until discharge, whereas six did not, all of which had trisomy 13 and cardiac malformations.
Conclusion: Chromosomal abnormalities and concurrent cardiac malformations significantly affected the prognosis of omphaloceles complicated by omphalomesenteric fistulas. Surgical indications should be assessed, taking into account individual patient’s goals and risk factors.
