An extreme case of vanishing gastroschisis with in utero midgut strangulation

Abstract

Listen

Translate article

Gastroschisis is a congenital ventral abdominal wall defect with subsequent evisceration of intra-abdominal contents. Vanishing gastroschisis is a rare but dreaded complication where closure of the abdominal wall causes strangulation of herniated bowel, with varying degrees of infarction, ischemia or intestinal atresia. An 1856g neonate was delivered at 34 weeks of gestation to a mother with no antenatal care. He was initially referred as an omphalocele. Examination revealed a congested poorly characterized mass (to the right of the umbilical cord) with a narrow base. Xray showed 2 dilated loops of small bowel with no distal pneumatization. Laparotomy confirmed the presumed diagnosis of vanishing gastroschisis. Unfortunately, there was only 13cm of proximal small bowel, with an atretic blind end. Distally, a patent microcolon was seen re-entering the abdomen from the abdominal wall defect. No salvageable bowel was identified within the extra-abdominal mass. Excision of strangulated mid gut, primary anastomosis and closure of abdominal wall was done. Unfortunately, infant succumbed to line related sepsis several weeks post op. Mortality rate for vanishing gastroschisis is high, and survivors usually face short bowel syndrome with long term parenteral nutrition dependence. Several bowel lengthening strategies exist, such as Bianchi, STEP and LILT procedures, if only as temporizing measures until the child is a candidate for transplant. Severe cases are sometimes managed palliatively.