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Heterotaxy Syndrome and Intestinal Rotation Abnormalities
Lindsay M Ryerson1, Scott Pharis2, Charissa Pockett2
1Pediatric Cardiac Intensive Care Unit, Stollery Children's Hospital, Edmonton, Alberta, Canada; ryerson@ualberta.ca.
Pediatrics
|July 28, 2018
Summary
Intestinal rotation abnormalities (IRAs) are common in infants with heterotaxy syndrome (HS). Expectant management showed no cases of midgut volvulus during follow-up.
Area of Science:
- Pediatric Surgery
- Congenital Abnormalities
- Gastrointestinal Motility Disorders
Background:
- Heterotaxy syndrome (HS) involves abnormal organ arrangement along the body's right-left axis.
- Intestinal rotation abnormalities (IRAs) are a significant cause of morbidity and mortality in infants with HS.
- Understanding the incidence and natural history of IRAs in HS is crucial.
Purpose of the Study:
- To prospectively observe a cohort of infants with HS.
- To determine the incidence of IRAs in infants with HS.
- To describe the natural history of IRAs in this population.
Main Methods:
- Prospective observational study of infants ≤6 months with HS.
- Exclusion of infants with congenital abnormalities requiring abdominal surgery.
- HS defined by non-situs solitus/inversus organ arrangement with congenital heart disease.
Main Results:
- 38 infants (22 right isomerism, 16 left isomerism) were included.
- 76% of infants were evaluated for IRAs, with 72% showing abnormalities.
- No cases of acute midgut volvulus occurred during a median follow-up of 1.6 years.
Conclusions:
- IRAs are frequently observed in infants with HS.
- Symptomatic infants presented before 6 months of age.
- Expectant management was not associated with midgut volvulus in this cohort.
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