Fetal vanishing gastrochisis and neonatal short bowel syndrome

Purpose: We report a rare case of vanishing gastrochisis. With an incidence of 1:4000 Gastrochisis and Omphalocele are the most common congential abdominal wall defects. Gastrochisis is defined by an paraumbilical defect of the abdominal wall with protrusion of mainly intestine fetal organs.

Material and methods: A 27 year old gravida I was referred at 13+5 week of gestation (WOG) with suspected gastrochisis. Detailed sonography confirmed this finding and revealed no further abnormalites. The patient denied first trimester risk calculation, NIPT and an invasive procedure. During the next examination in 21+4 WOG gastrochisis was diminished. The abdominal wall defect was small and intraabdominal bowel was dilated. At 28+4 WOG the gastrochisis has vanished. The intradominal bowel was dilated. Additionaly, a dilated stomach was visualized at 33+4 WOG. Primary cesarean section was performed at 37+4 WOG because of breech presentation.

Result: A healthy newborn was delivered weighing 2940 g, APGAR 9/10/10 and umbical artery pH 7.37. There was no abdominal wall defect, umbilical cord insertion was normal. Sonography revealed dilated small intestine and stomach with non-propulsive peristalsis. Explorative laparotomy revealed severe dilated small intestine commencing from the duodenojejunal flexure ending blindly after 30 cm. Coecum and appendix were missing. Colon ascendens and transversum were atretic. An end to side anastomosis connected the rest of the small intestine with colon descendens. Because of the short bowel syndrome parenteral alimentation was necessary for six weeks.

Conclusion: Vanishing gastrochisis with dilated intraabdominal bowel is a rare variation of gastrochisis. Antenatal counselling should address this as an severe complication with a worse outcome.