Vanishing Gastroschisis with a Favorable Outcome after a 3-Year Follow-Up: A Case Report and Literature Review

Abstract

Vanishing gastroschisis (VG) is a severe complication of gastroschisis with a high mortality rate. We report here a case of VG with a favorable outcome after a 3-year follow-up. A 26-year-old primigravida woman was referred to Strasbourg University Hospital because her fetus was diagnosed with an isolated gastroschisis at 13-week gestation. The ultrasound evolution was marked by a progressive closure of the abdominal wall defect from 19-week gestation and the appearance of dilated intra-abdominal loops. The child was born with a closed abdominal wall except a small remnant at the level of the former gastroschisis orifice. Explorative laparotomy revealed extensive midgut atresia with only 50 cm of remaining midgut. A jejunocolic anastomosis was performed. The child is now 3 years old and has a favorable outcome with only 2 nights a week of parenteral nutrition. A total of 39 cases of VG type D from Perrone et al. classification are described in the literature from 1991 to 2019, among which 19 (48.7%) are alive at the time of publication but only 4 cases are described with a long-term follow-up of 3 years or more. This is the fifth case described with a favorable evolution after 3-year follow-up.

A case of vanishing gastroschisis with short bowel syndrome treated by total parenteral nutrition and intestinal lengthening procedures

“…Neonates affected by gastroschisis have a good outcome. 3 , 4 However, 17% of gastroschisis cases are complicated by intestinal atresia, perforation, necrosis, or volvulus, resulting in complex gastroschisis. One of the most feared complications is closed gastroschisis, which is VG that occurs when the abdominal defect is closed in utero in association with atresia of the small intestine and short bowel syndrome (SBS).…”