Omphalocele minor in a neonate with fistulation: A Case Report and insights on management from a systematic review of case reports

Background Minor exomphalos is a rare congenital abdominal wall defect<5 cm. The condition is characterized by protrusion of abdominal organs covered with amnion, Wharton’s jelly and parietal peritoneum. Its association with fistulation is exceedingly uncommon with only 30 cases reported in literature. Methods We present a unique case report and a systematic review of similar existing case reports. A systematic review was conducted from inception to April 2025, adhering to PRISMA guidelines to include case reports of neonates with minor exomphalos and fistulation. The search was done on Pubmed, BMJ case reports, International Journal of Surgery Case Reports, Journal of surgical case reports, Taylor&Francis, and web of science. Results A preterm male neonate (34+4 weeks, 2.2 Kg) born to a diabetic mother presented with a minor omphalocele exhibiting fistulation. Surgical exploration on day two of admission revealed a Meckel diverticulum with a patent vitellointestinal duct (PVID). Resection of the Meckel diverticulum with end-to-end ileo-ileal anastomosis was performed. The postoperative course was uneventful, and the patient was discharged on the 14th day. Our initial search yielded 673 studies, with 20 case reports (25 patients) meeting the inclusion criteria. The mean gestational age was 38.18 ± 1.53 weeks, and the mean birth weight was 2.84 ± 0.65 Kg. Meckel diverticulum was associated in 40% of cases, and other system anomalies in 44%. Standard treatment was surgical fistulectomy with end-to-end anastomosis (44%) or diverticulectomy with fistulectomy and anastomosis (36%). One case was managed conservatively initially. Postoperative outcomes were generally favorable (80% uneventful), with one mortality reported. Conclusion Our case report and systematic review highlight the demographics, symptomatology, and management of exomphalos minor with fistulation. It can be concluded from our review that most cases are males from the southeast countries and that prompt surgical management is necessary.