Abstract
BACKGROUND: Congenital intestinal malrotation (CIM) is a common malformation in neonates. Early diagnosis and surgical intervention can improve the prognosis. CIM combined with congenital gastric wall defect is a potentially fatal condition. We present a severe case of CIM with gastric wall defect causing extensive gut necrosis and short gut syndrome. After three operations, the neonate survived and subsequently showed normal growth and development during infancy. CASE SUMMARY: A male neonate (age: 4 d) was hospitalized due to bloody stools and vomiting for 2 d, and abdominal distention for 1 d. Emergent exploratory laparotomy revealed black purplish discoloration of the bowel loops. Bowel alignment was abnormal with congestion and dilatation of the entire intestine, and clockwise mesentery volvulus (720°). The posterior wall of the gastric body near the greater curvature showed a defect in the muscularis layer (approximately 5.5 cm), and a circular perforation (approximately 3 cm diameter) at the center of this defect. Ladd's procedure was performed and gastric wall defect was repaired. Third operation performed 53 d after birth revealed extensive adherence of small intestine and peritoneum, and adhesion angulated between many small intestinal loops. We performed intestinal adhesiolysis, resection of necrotic intestine, and small bowel anastomosis. CONCLUSION: This case highlights that prolonged medical treatment may help improve intestinal salvage after surgical removal of necrotic intestines, and improve patient prognosis.