SurgeryHeterotaxia syndromes and their abdominal manifestationsBorenstein, Steven H.a; Langer, Jacob C.bAuthor Information aDepartment of Surgery, McMaster Children's Hospital, Hamilton, Ontario, Canada bDepartment of Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada Correspondence to Jacob C. Langer MD, Head, Division of General Surgery, Room 1526, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8; E-mail: [email protected] Current Opinion in Pediatrics: June 2006 - Volume 18 - Issue 3 - p 294-297 doi: 10.1097/01.mop.0000193313.75827.e9 Buy Metrics Abstract Purpose of review Children with heterotaxia often have abnormalities of intestinal rotation, some of which may predispose to midgut volvulus. This review summarizes the literature and proposes an individualized approach to the patient based on symptoms and findings on gastrointestinal imaging. Recent findings This report reviews the types of heterotaxia syndromes and the debate surrounding the optimal management of the associated intestinal rotational abnormalities. Recent publications suggest that not all rotational abnormalities predispose to volvulus and that the natural history of rotational abnormalities in asymptomatic patients with heterotaxia is relatively benign. On the basis of these findings, an algorithm for the management of intestinal rotational abnormalities is proposed. Summary Malrotation and midgut volvulus is a life-threatening complication. Children with heterotaxia and symptoms suggestive of a proximal intestinal obstruction require urgent investigation and surgical treatment. Asymptomatic children require close clinical follow-up and urgent investigation of gastrointestinal symptoms. © 2006 Lippincott Williams & Wilkins, Inc.