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Heterotaxy syndrome and intestinal rotation abnormalities: a survey of institutional practice

Charissa R Pockett1, Bryan J Dicken, Ivan M Rebeyka

  • 1Department of Pediatrics, University of Alberta, Edmonton, AB, Canada.

Journal of Pediatric Surgery
|October 8, 2013
PubMed

Insights

Heterotaxy syndrome (HS) management in infants with intestinal rotation abnormalities (IRA) varies. While most physicians screen for IRA, there

Area of Science:

  • Pediatric Surgery
  • Neonatal Care
  • Congenital Abnormalities

Background:

  • Intestinal rotation abnormalities (IRA) frequently occur with heterotaxy syndrome (HS).
  • Clinical practice is divided on screening asymptomatic infants with HS for IRA and performing prophylactic Ladd procedures.

Purpose of the Study:

  • To investigate North American institutional practices for managing asymptomatic infants with HS and IRA.
  • To identify variations in screening and surgical management approaches.

Main Methods:

  • An international, multi-institutional web-based survey was conducted.
  • The survey assessed current practices and expert opinions on IRA management in HS patients.

Main Results:

  • 84% of surveyed physicians advocate for neonatal screening for IRA in HS patients.
  • Significant divergence exists regarding prophylactic Ladd procedures for asymptomatic IRA in HS, with 61% of general surgeons favoring it, while 55% of all physicians are comfortable with conservative management.

Conclusions:

  • Consideration of midgut volvulus risk, procedural morbidity/mortality, and cardiovascular prognosis is crucial before elective Ladd procedures in asymptomatic HS patients.
  • Varied practices among sub-specialists, lack of consensus, and limited evidence highlight the need for further research in IRA management for HS.
Abstract

Keywords:
HSHeterotaxy SyndromeHeterotaxy syndromeIRAIntestinal Rotation AbnormalityIsomerismLadd procedureMalrotation

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