Omphalocele with a patent vitelline duct: intraoperative identification of an uncommon presentation.

backgroundOmphalocele with a concomitant patent vitelline duct is a rare presentation, with very few cases reported in the literature. Most reported cases present with visible bowel prolapse, meconium drainage or mucosal exposure at birth. Presentations without obvious intestinal herniation or mucosal exposure are extremely uncommon and pose diagnostic challenges. CASE PRESENTATION: A male neonate was born to a G3P2 mother at 38 + 6/7 weeks via spontaneous vaginal delivery after a pregnancy notable for a 20-week prenatal ultrasound concerning for omphalocele. Upon delivery, the omphalocele membrane was intact. Shortly after birth, the neonate developed persistent bilious emesis. Abdominal ultrasound demonstrated normal rotation, and abdominal X-ray was reassuring without evidence of obstruction or volvulus. No other congenital anomalies were demonstrated after VACTERL workup, including echocardiogram, abdominal and spinal ultrasounds. Genetic testing for Beckwith-Wiedmann syndrome was found to be normal. Surgical exploration revealed a patent vitelline duct fistula connecting the small bowel to the omphalocele sac. The fistula and adjacent small bowel were resected, followed by primary end-to-end anastomosis and primary fascial closure. The postoperative course was uncomplicated, and the neonate was thriving without any evidence of infection or obstruction at 2-month and 6-month follow-up visits.

conclusionThere have been very few cases of omphalocele with a patent vitelline duct reported in the literature, and those that have been described present with either visible mucosal prolapse or intestinal extrusion at birth. This case demonstrates that a vitelline duct anomaly may remain clinically occult and present only with obstructive symptoms such as bilious emesis. In neonates with omphalocele who develop gastrointestinal obstruction despite unrevealing imaging studies, clinicians may entertain the possibility of a concurrent vitelline duct remnant as a part of their differential diagnosis. Recognition of this association may guide operative planning and improve diagnostic awareness of this uncommon embryologic anomaly.