Vitelline fistula associated with omphalocele: Diagnostic dilemma?

INTRODUCTION: Combination vitelline fistula (VF) and omphalocele at birth is a rare congenital anomaly as a result disturbance in organogenesis with failure of normal return of intestines into the abdominal cavity and failed obliteration of the vitelline duct. CASE PRESENTATION: A newborn presented with omphalocele sac with visible intestine, stoma like lesion with prolapsing mucosa just lateral to the umbilical cord and passage of meconium stool. Operative surgery was confirmed an intact omphalocele sac and vitelline fistula. Fistulectomy, using wedge resection of the small bowel and primary closure abdominal wall defect. DISCUSSION: In our review of literature, VF associated with omphalocele had not been reported. Combination of anomaly maybe misleading, however, can be easily diagnosed the location of VF opening on the omphalocele sac, which is adjacent to the umbilical cord and luminal passage of meconium stool after birth. A fistulogram may be the best initial diagnostic imaging approach for identifying and confirmation of a fistula tract. CONCLUSION: VF associated with omphalocele is rare. Post-natal diagnosis is easily by gross appearance stoma like lesion, which is located just lateral of the umbilical cord, an intact omphalocele sac and post-natal meconium stool passage.