Navigating the Twist: An Atypical Presentation of Malrotation

Intestinal malrotation is a congenital anomaly resulting from abnormal midgut rotation and fixation and occurs in approximately 1 in 500 live births. Malrotation results in a narrow mesenteric root, predisposing to midgut volvulus and potentially life-threatening bowel ischemia. Symptoms develop in about 1 in 6,000 individuals, over 75% of cases presenting in the early neonatal period. Bilious vomiting and abdominal distension are common signs of presentation. We report a case of a healthy term male neonate who was breastfeeding with formula supplementation until day of life 3, when he passed two bloody stools. He was transferred to our neonatal intensive care unit for evaluation and management. Abdominal ultrasound demonstrated pathognomonic reversal of the relationship between the superior mesenteric artery and superior mesenteric vein and an upper gastrointestinal (GI) contrast study confirmed intestinal malrotation. He underwent an urgent exploratory laparotomy and corrective Ladd procedure. There was no intraoperative evidence of volvulus or bowel ischemia. He had an uncomplicated recovery and was discharged several days later. GI bleeding is a rare initial presentation of malrotation, particularly in the absence of bilious emesis. This case emphasizes the importance of considering malrotation in neonates with hematochezia to enable early diagnosis and prevent life-threatening complications.