Abstract

Introduction: According to the Intestinal Transplant Registry report 2018, gastroschisis was the most frequent indication (21%) for pediatric intestinal transplantation. However, in Japan, none of the pediatric intestinal transplant candidates has gastroschisis. The purpose of this study was to analyze gastrointestinal outcomes of gastroschisis in and around Osaka, Japan. Methods: One hundred and nine patients with gastroschisis were treated between January 1982 and December 2018 at our three hospitals. The patients’ gender, gestational age at birth, birth weight, Apgar Score, delivery route, fetal diagnosis, gastrointestinal complications of gastroschisis, surgical treatment, and outcomes were analyzed. Results: Mean gestational age at birth of the 109 patients (males: 57, 52%) was 36.3 ± 2.1 weeks. Mean birth weight was 2159 ± 441 g, mean Apgar Score (after 1 minute / after 5 minute) was 6.9 ± 2.3 / 8.4 ± 1.2, and 38 patients were delivered by Cesarean section. Fetal diagnosis of gastroschisis was made in 78 (72%) cases. All 109 patients were operated for gastroschisis, with primary repair in 63, silo repair in 23, and silo placement with delayed abdominal closure in 23. Eight patients (7%) had the gastrointestinal complications: seven had atresia of the intestine or colon, one had intestinal perforation. Closed gastroschisis was detected in three patients after birth. The patients with gastrointestinal complications were treated concurrently by intestinal anastomosis or enterostomy at the initial operation. Three of the eight patients underwent massive intestinal resection, resulting in short bowel syndrome (SBS), and required total parenteral nutrition (TPN) for about a year. Two of the three patients with SBS survived weaning off TPN. However, the other baby with SBS died of intestinal failure-associated liver disease. The other two patients without gastrointestinal complications died, one was an extremely low birth weight infant and the other with thrombosis in the lower extremities. Eventually, the remaining 106 (97.2 %) patients with gastroschisis have been surviving without TPN. Conclusion: Gastrointestinal complications are rare in Japanese patients with gastroschisis. One of the factors contributing to this might be the low incidence of closed gastroschisis and low prevalence of SBS and complications secondary to intestinal failure after repair of gastroschisis. Ethical approval: The hospital research ethics committee (reference number H30-36).

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