Intestinal malrotation in infants with omphalocele: A systematic review and meta-analysis

Purpose: The management of intestinal malrotation in infants with omphalocele varies among surgeons. Herein, we aimed to determine whether infants with omphalocele should be investigated for malrotation. Methods: Using a defined search strategy, three investigators identified all studies reporting patients with omphalocele and malrotation. Outcome measures included: 1. incidence of malrotation; 2. correlation with the abdominal size defect in patients with omphalocele; 3. risk of volvulus in those not investigated for malrotation; 4. incidence of adhesive bowel obstruction in those who underwent Ladd's procedure. The meta-analysis was conducted according to PRISMA guidelines and using RevMan 5.3. Results: Of 111 articles analyzed, 12 (3888 children) reported malrotation in 136 patients (3.5%). Malrotation was equally found in patients with major (15.2%) and minor (13.6%; p = 0.52) omphalocele. A volvulus was more common in children who had Ladd's procedure (8%) than in those who did not (1%; p = 0.03). Adhesive bowel obstruction rate was similar in both groups (5% vs. 3%; p = 0.21). Conclusion: The incidence of malrotation in infants with omphalocele is low but probably underreported, and is not influenced by the size of the defect. At present, there is no evidence in the literature to support investigations to detect malrotation in infants with omphalocele. Type of study: Therapeutic. Level of evidence: III