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Closed Gastroschisis With Jejunal Atresia - A Case Report

Radu-Iulian Spataru, Dan-Alexandru Iozsa

Pediatric Surgery Unit, „Marie Sklodowska Curie” Emergency Clinical Hospital for Children, Bucharest, Romania



Radu-Iulian Spataru

Pediatric Surgery Unit

„Marie Sklodowska Curie” Emergency Clinical Hospital for Children

20 C-tin Brâncoveanu Blvd., 041451, Bucharest, Romania

Phone: 0040788372478

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.



Spontaneous closure of the abdominal wall defect is a rare occurrence in the antenatal evolution of gastroschisis. This can compromise mesenteric blood supply to the extra-abdominal gut affecting the bowel in different forms from ischemia to necrosis. Intestinal atresia in a variable range is also known to coexist. We report the case of a newborn male admitted in our unit for neonatal bowel obstruction and a “mummified” tissue mass attached on the right side to the umbilical cord. Laparotomy revealed short length jejunal atresia and jejuno-ileal anastomosis has been performed. Despite the fact closed gastroschisis is frequently reported in association with short-bowel syndrome due to a vanishing midgut, our case had uneventful outcome.

Keywords: closed gastroschisis, intestinal atresia, neonatal bowel obstruction




Gastroschisis (GS) is a congenital malformation in which the bowel (and occasionally other viscera) prolapses outside the abdominal cavity via a fullthickness wall defect, without a covering, most of the times right-sided to the umbilical cord which appears to be normal [1,2]. Gastroschisis occurs in very early gestation. Young mothers who do not follow proper prenatal care represent a group at risk. Development of early antenatal diagnosis together with improvement of postnatal treatment and increased survival in the last decades seems to have increased incidence of GS to 4-5:10,000 live births. This could be explained by a drop in the number of abortions, since certain risk factors aren’t clearly known [2,3]. Intestinal atresia and malrotation are well-documented associated conditions in GS [4].

In 6% of the GS cases antenatal closure of the abdominal wall may happen, condition being called closing/closed GS. This can lead to a “tourniquet effect” around the prolapsed bowel and a variable degree of vascular compromise of adjacent mesenteric vessels depending on the tightness of the abdominal ring. In exceptional cases, the abdominal wall may be entirely closed in conjunction with intestinal reabsorption due to severe chronic ischemia, situation known as vanishing midgut [5,6]. Nevertheless, intestinal atresia with consecutive short-bowel syndrome complications and sepsis remain the main risk factors for a poor outcome in GS [7].

Case Report

A full-term three-hour new-born male was admitted to our intensive care unit for neonatal bowel obstruction. No prenatal monitoring has been done and the baby was born via natural delivery in a secondary care center.

Clinical examination revealed abdominal distension and high output on the nasogastric tube. A „mummified” anhydrous aspect tissue, attached to a small circular scar through a short-narrow stalk was recognized just right to the umbilical cord (Fig. 1). No passage of meconium was noted so far.

Figure 1. Preoperative abdominal aspect

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Urgent midline laparotomy has been performed. A considerable dilated jejunum continued by 9 cm of type II atretic segment adjacent to a superior mesenteric artery branch vanishing into the abdominal wall could be observed (Fig. 2A and 2B). The distal bowel had a significantly reduced diameter. Exploration of the peritoneal cavity revealed approximately 80 cm of ileum and a normal length colon. Intestinal malrotation was noted. Maximum preservation of the small bowel in order to prevent intestinal failure was attempted with a jejuno-ileal anastomosis being done after resection of the atretic segment. The patient had an uneventful postoperative course tolerating early enteral feeds and no parenteral nutrition was needed (TPN). At 2 years follow-up, the patient has a normal height and weight gain.

Figure 2. Intraoperative aspect of the bowel illustrating complete jejunal atresia. Mesenteric artery branch vanishing into the abdominal wall.

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The mechanisms of pathogenesis in GS are not clear and its relationship with intestinal atresia is not fully-understood [8]. Many assumptions are made in order to find a link between intestinal atresia and closing GS and the mainstay is the idea of a vascular accident in utero. This could be a spontaneous closure of the abdominal wall around the gut and mesenteric artery resulting into strangulation. Also, incarceration or volvulus within the closing defect and consecutive atrophy of the bowel are also postulated hypotheses [1,5,8].

During prenatal monitoring, closing GS main sign is a progressive and persistent dilatation of an intraabdominal bowel loop. This requires close observation and a good compliance of the mother since early delivery in order to salvage the bowel may be indicated when a high index of suspicion is considered [1].

Contrary to our situation, in many cases of closing/closed GS massive intestinal length is lost. Short-bowel syndrome demands further lengthening procedures and TPN with subsequent complications such as sepsis or intestinal failure associated liver disease [9]. However, in some cases partial compression can lead to “an amorphous nonviable appearing extra-corporeal mass of matted bowel” which some surgeons opt to resect considering it non-functional. Estrada JJ, et al. suggested reintegrating it into the abdominal cavity if a patent vascular pedicle is attached since a considerable segment development can surprise the surgeon in a second-look further intervention, so morbidity and mortality related to short-bowel syndrome may be reduced [3].

Completely closed abdominal wall defect in GS is extremely rare [8]. In 1999, Fowler CL, et al. reported an unique presentation of an antenatal closed gastroschisis with apparently normal abdominal wall outlined by a colo-cutaneous fistula which was proven by a barium enema performed for neonatal bowel obstruction differential diagnosis [10].

In our case, considering the narrow-stalk attaching the mummified bowel to the abdomen, the absence of antenatal monitoring of the pregnancy and the secondary care centre where our patient was delivered, if the necrotic external bowel would have been purposely or accidentally removed during or after birth, we simulated how the abdomen would have looked using computer assisted picture manipulation we illustrated the scenario (Fig. 3). We think that signs of neonatal bowel obstruction coexisting with a paraumbilical scar should be considered a suggestive sign for closed gastroschisis. In our case the circumstances that brought the most favourable outcomes were: early diagnosis and intervention, short bowel segment affected and localization to the jejunum, since this part is naturally shorter and exhibits less adaptive and absorptive capacity than the ileum [11].

Figure 3. Extra-abdominal mummified midgut removed using computer assisted picture manipulation

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10. Fowler CL, Rothman EA, Congenital colocutaneous fistula as presenting sign of prenatally closed gastroschisis. Pediatr Radiol 1999; 29: 811-2.

11. Quiros-Tejeira, Ament ME, Reyen L, et al. Long-term parenteral nutritional support and intestinal adaptation in children with short bowel syndrome : a 25-year experience. J Pediatr 2004; 145: 157- 63.