Management of giant omphalocele with intestinal perforation

Abstract Case of a male infant delivered at 36 weeks of gestational age and found to have a giant omphalocele, later complicated by intestinal perforation. A giant omphalocele is an omphalocele with an opening greater than five centimeters in diameter and containing the liver. It represents a therapeutic challenge secondary to viscero-abdominal disproportion, limiting the feasibility of a primary closure. Nowadays, the standard of care for giant omphaloceles is to adopt the “paint and wait” approach with delayed closure. Intestinal perforation is a rare complication of omphaloceles. Prior reports in the literature describe intestinal perforation in the setting of small to medium size omphalocele treated with operative exploratory laparotomy to address the intestinal perforation, followed by either primary or staged closure of the abdominal wall. We report the case of an infant with giant omphalocele who developed intestinal perforation at 4 weeks of life while undergoing “paint and wait”. He was managed non-operatively with a peritoneal drain, antibiotics, and supportive care, while continuing with the “paint and wait” approach until he was optimized for operative repair of his giant omphalocele.