Management of gastroschisis in an extremely low birth weight infant: report of a case

A 21-year-old primigravida woman with a history of smoking was referred to our institution at 17 weeks of gestation after a prenatal screening revealed fetal gastroschisis. The pregnancy was further complicated by intrauterine growth restriction, oligohydramnios, and non-reassuring fetal status, ultimately necessitating an emergency cesarean section at 29 weeks of gestation.

The female infant was born weighing 768 g, with Apgar scores of 5 and 8 at 1 and 5 min, respectively. She required immediate intubation and transfer to the neonatal intensive care unit. An abdominal wall defect measuring 10 mm in diameter was located to the right of the umbilical cord, with a herniated small bowel and colon (Fig. 1). Initially, the exposed bowel appeared viable; however, it showed signs of rapid swelling and ischemia shortly after birth.

Fig. 1figure 1

Abdominal wall defect at birth. a Female infant was born weighing 768 g, with Apgar scores of 5 and 8 at 1 and 5 min, respectively. b Abdominal wall defect measuring 10 mm in diameter was located to the right of the umbilical cord, with herniated small bowel and colon. The arrowhead indicates the defect

An emergency surgery was performed to relieve the pressure at the defect in the abdominal wall, which was constricting the pedicle of the herniated intestine. The abdominal defect was enlarged through an upper midline incision extending 1 cm superiorly, allowing for relaxation of the skin and fascia. No other vascular or intestinal complications were found (Fig. 2a). The "silo placement" technique was employed according to the Allen–Wrenn method [7], using expanded polytetrafluoroethylene (ePTFE) sheets that were sutured to the abdominal wall (Fig. 2b). The herniated intestines were gradually reduced into the peritoneal cavity via gentle compression and gravity.

Fig. 2figure 2

Emergency surgery and silo placement. a Emergency surgery was performed to relieve the pressure at the abdominal wall defect, which was constricting the pedicle of the herniated intestine. The arrowhead indicates the umbilicus, and the asterisk indicates the cranial direction. b Abdominal defect was enlarged through an upper midline incision extending 1 cm superiorly, and the “silo placement” technique was employed using expanded polytetrafluoroethylene (ePTFE) sheets, which were sutured to the abdominal wall

Postoperative care initially progressed well, with half segments of the bowel successfully reduced. However, on day 5, bile-stained ascites leaked from the suture line between the silo and the abdominal wall, indicating bowel perforation (Fig. 3a).

Fig. 3figure 3

Bowel perforation with necrotizing enterocolitis. a On day 5 of life, bile-stained ascites leaked from the suture line between the silo and the abdominal wall, indicating bowel perforation. b, c, d Emergency laparotomy revealed necrotizing enterocolitis with localized bowel perforation in the exposed ileum in the silo, necessitating the excision of a 10 cm segment of the ileum and the creation of an ileostomy. The abdominal defect could be closed without increasing intra-abdominal pressure. The arrowhead indicates the umbilicus, and the asterisk indicates the cranial direction

An emergency laparotomy revealed necrotizing enterocolitis (NEC) with localized bowel perforation in the exposed ileum within the silo (Fig. 3b,c), necessitating the excision of a 10 cm segment of the ileum and the creation of an ileostomy. The abdominal defect could be closed without increasing intra-abdominal pressure (Fig. 3d). Pathological findings revealed transmural necrosis at the site of the perforation, accompanied by surrounding transmural inflammation and hemorrhage, consistent with NEC.

Postoperative peritonitis and septicemia developed, both of which were successfully treated with antibiotics. The infant required ventilation support by day 23 of life, transitioning from intubation to continuous positive airway pressure management. Nasogastric milk feeding was initiated on day 13 of life, but progressed slowly because of the patient’s gastrointestinal dysmotility. High-calorie parenteral nutrition was administered to support growth, but elevated serum bilirubin levels and transaminases, suggestive of liver dysfunction, were observed beginning on day 50 and peaking on day 97 (total bilirubin, 9.4 mg/dL; direct bilirubin, 6.7 mg/dL; AST, 120 IU/L; ALT, 69 IU/L). The patient's liver function subsequently improved and normalized by day 140, when enteral feeding was achieved. Ileostomy closure was performed on day 83 when the infant's weight exceeded 2000 g.

Although insufficient body weight gain was observed during the postoperative course, the infant was discharged on day 142 following birth, weighing 2774 g. At the time of discharge, she was on oral feeding and showed no signs of significant complications. By 18 months of age, the patient has shown acceptable growth and remains free of abdominal symptoms.

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