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Heterotaxia syndromes and their abdominal manifestations

Steven H Borenstein1, Jacob C Langer

  • 1Department of Surgery, McMaster Children's Hospital, Hamilton, Ontario, Canada.

Current Opinion in Pediatrics
|May 25, 2006
PubMed

Insights

Children with heterotaxia (a condition affecting organ positioning) may have intestinal rotation abnormalities. This review suggests an individualized approach, as not all abnormalities lead to midgut volvulus, a serious complication.

Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Congenital Abnormalities

Background:

  • Heterotaxia is associated with congenital anomalies, including intestinal malrotation.
  • Intestinal malrotation can predispose to midgut volvulus, a surgical emergency.
  • The management of intestinal rotation abnormalities in heterotaxia remains debated.

Purpose of the Study:

  • To review the literature on heterotaxia and associated intestinal rotation abnormalities.
  • To propose an individualized management strategy based on clinical presentation and imaging.
  • To clarify the risk of midgut volvulus in asymptomatic patients with heterotaxia.

Main Methods:

  • Comprehensive literature review of heterotaxia syndromes and intestinal rotation.
  • Analysis of recent publications on the natural history and management of these conditions.
  • Development of a proposed clinical algorithm for patient management.

Main Results:

  • Heterotaxia encompasses various syndromes with diverse intestinal rotation anomalies.
  • Recent evidence indicates that not all rotational abnormalities in heterotaxia carry a high risk of volvulus.
  • Asymptomatic patients with heterotaxia and rotational abnormalities may have a benign natural history.

Conclusions:

  • Malrotation and midgut volvulus are critical, life-threatening complications.
  • Children with heterotaxia and symptoms of proximal intestinal obstruction need prompt evaluation and surgical intervention.
  • Asymptomatic children with heterotaxia require vigilant clinical monitoring and timely investigation of gastrointestinal symptoms.
Abstract

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