How is intestinal malrotation treated in patients with heterotaxy syndromes?

Updated: Dec 19, 2018
  • Author: Denis D Bensard, MD, FACS, FAAP; Chief Editor: Carmen Cuffari, MD  more...
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Patients with heterotaxy syndromes have increased rates of abnormalities of intestinal rotation. The approach to the management of these rotational abnormalities is controversial. [48]

Some authors advocate for screening of all children with an upper GI study and elective correction of any rotational abnormality. [48] However, a retrospective review of 29 children who underwent Ladd procedure revealed that the complication rate was 57% among patients with heterotaxy who were otherwise asymptomatic, compared with only 9% in the symptomatic, nonheterotaxy group. [49] These authors recommend that asymptomatic children be managed conservatively with operative intervention reserved for those children who become symptomatic. A systematic review by Cullis et al found that out of 414 asymptomatic patients that had no screening for intestinal rotation, one patient developed malrotation symptoms which was confirmed by laparotomy. [50]

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