A rare combination of gastroschisis, jejunal atresia, colonic atresia, and midgut malrotation in a newborn: a case report and literature review

Abstract

Introduction and importance: Gastroschisis is a congenital full-thickness anterior abdominal wall defect that allows bowel evisceration without a covering sac. While most cases are isolated and have good outcomes, complex gastroschisis involves associated intestinal pathology and carries significantly higher morbidity and mortality. The simultaneous occurrence of jejunal atresia, colonic atresia, and midgut malrotation in the setting of gastroschisis is extraordinarily rare. Case presentation: A male infant was delivered at 36 weeks’ gestation following an antenatal diagnosis of gastroschisis. At birth, marked abdominal distension and bilious nasogastric aspirate raised immediate concern for an associated anomaly. Intraoperative assessment revealed type IIIb jejunal atresia, type I right colonic atresia, midgut malrotation, and an unused microcolon. The colonic atresia was only identified after further inspection to ensure distal bowel patency. Single-stage repair was performed, consisting of primary jejunoileal and colocolic anastomoses, Ladd’s procedure, and abdominal wall closure. The early postoperative course was encouraging, with full oral feeding achieved by the second postoperative week. However, by week 6, the infant developed medical necrotizing enterocolitis, septic shock due to an ESBL-producing Klebsiella, and multiorgan failure, and died on day 48 of life. Clinical discussion: Complex gastroschisis with multiple gastrointestinal anomalies severely limits intestinal function, increases susceptibility to infection, and complicates operative and postoperative recovery. Even following a technically successful repair, optimizing nutrition and preventing sepsis remain difficult. Conclusion: Careful intraoperative distal bowel evaluation is essential to avoid missed atresias. Mortality remains notable in this exceedingly rare combination due to the cumulative burden of underlying anomalies and infectious complications.