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

Giuseppe Lauriti1, Maria Enrica Miscia2, Valentina Cascini2

  • 1Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, ON, Canada; Department of Pediatric Surgery, "Spirito Santo" Hospital, Pescara, and "G. d'Annunzio" University, Chieti-Pescara, Italy.

Journal of Pediatric Surgery
|October 13, 2018
PubMed

Insights

Investigating intestinal malrotation in infants with omphalocele is not recommended. This meta-analysis found a low incidence of malrotation, with no clear link to omphalocele size or benefit from routine screening.

Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Neonatal Care

Background:

  • Intestinal malrotation is a congenital anomaly requiring prompt diagnosis and management.
  • Omphalocele, a defect of the anterior abdominal wall, is often associated with other congenital anomalies, including malrotation.
  • Current surgical approaches for managing intestinal malrotation in infants with omphalocele lack a consensus, prompting investigation into optimal diagnostic strategies.

Purpose of the Study:

  • To determine the incidence of intestinal malrotation in infants diagnosed with omphalocele.
  • To assess the correlation between the size of the omphalocele defect and the presence of malrotation.
  • To evaluate the risks of volvulus and adhesive bowel obstruction in infants with omphalocele concerning malrotation investigation and surgical management.

Main Methods:

  • A systematic literature search was conducted to identify studies reporting on omphalocele and malrotation.
  • A meta-analysis was performed on 12 selected articles, encompassing 3888 children, following PRISMA guidelines.
  • Key outcome measures included the incidence of malrotation, its association with omphalocele size, and the risks of volvulus and adhesive bowel obstruction.

Main Results:

  • Malrotation was identified in 3.5% of infants with omphalocele (136 out of 3888).
  • The incidence of malrotation was similar in both major (15.2%) and minor (13.6%) omphalocele cases.
  • Volvulus occurred more frequently in children who underwent Ladd's procedure (8%) compared to those who did not (1%), while adhesive bowel obstruction rates were comparable.

Conclusions:

  • The incidence of malrotation in infants with omphalocele is low, though potentially underreported, and is not influenced by the defect's size.
  • Current evidence does not support routine investigations for malrotation in all infants with omphalocele.
  • Further research may be needed to refine management strategies for this patient population.
Abstract

Keywords:
Adhesive bowel obstructionExomphalosInfantsLaddVolvulus

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