Diagnosis and management of intestinal rotational abnormalities with or without volvulus in the pediatric population

Intestinal malrotation is a congenital anomaly that can be associated with midgut volvulus, requiring an emergent operation in order to maintain blood supply to the compromised intestine. It results from an abnormal rotation of the intestines, leading to three types of malrotation. Non-rotation is complete failure of the intestines to rotate, while the most common configuration is associated with the cecum in the mid-upper abdomen, close to a malpositioned duodenal-jejunal (DJ) flexure. This configuration has a narrow mesentery that has a high risk of volvulus. The final form of malrotation is incomplete rotation, where the DJ flexure and cecum are malpositioned, but the mesentery is not narrowed. The benefits of performing a Ladd's procedure for these individuals is controversial. Workup for malrotation should be considered in all patients who present with abdominal pain/distention and bilious emesis. An upper gastrointestinal contrast study is 93-100% sensitive and will show a corkscrew appearance when a volvulus is present. While the basic tenets of the Ladd's procedure have not changed and include detorsion of a volvulus, adhesiolysis of Ladd's bands and broadening of the mesentery, how this is accomplished and in whom are controversial. Laparoscopic Ladd's is associated with shorter hospital stays but also has a higher incidence of recurrent volvulus compared to an open approach. Patients with heterotaxy syndrome also represent a controversial group with some studies showing no difference in post-operative complications despite a higher mortality due to underlying cardiac disease, while other studies show a low incidence of volvulus and question the need for Ladd's in those who are asymptomatic. This review highlights the major aspects of diagnosing and treating malrotation, including the pathophysiology, workup, surgical options and areas of controversy.