Heterotaxia syndromes and their abdominal manifestations.
OBJECTIVE
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.


RESULTS
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.


CONCLUSIONS
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.
Borenstein
Steven H
SH
Department of Surgery, McMaster Children's Hospital, Hamilton, Ontario, Canada.
Langer
Jacob C
JC
D002648:Child; D002675:Child, Preschool; D041981:Gastrointestinal Tract / Q000002:abnormalities; D006325:Heart Atria / Q000002:abnormalities; D006330:Heart Defects, Congenital / Q000150:complications*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007415:Intestinal Obstruction / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D045822:Intestinal Volvulus / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D008099:Liver / Q000002:abnormalities*; D008168:Lung / Q000002:abnormalities*; D013154:Spleen / Q000002:abnormalities*; D013577:Syndrome