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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.
Insights
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.
Background:
Infants with heterotaxy syndrome (HS) have abnormal lateralization of organs along the right-left body axis. Intestinal rotation abnormalities (IRAs) are a potential source of morbidity and mortality. For this study, our objective was to prospectively observe a cohort of infants with HS and determine the incidence and natural history of IRA.
Methods:
Infants ≤6 months of age with HS were enrolled in this prospective observational study. Exclusion criteria were other congenital abnormalities that necessitated abdominal surgery. HS was defined as any arrangement of organs that was not situs solitus or situs inversus along with associated congenital heart disease. The investigation for IRA was at the discretion of each participating center.
Results:
Infants were recruited from January 2012 to December 2016. Thirty-eight infants from 7 institutions were included; 22 infants had right isomerism and 16 infants had left isomerism. Twenty-nine infants (76%) were evaluated for IRAs; 21 of 29 evaluations (72%) were abnormal. Eight infants were investigated because of symptoms, and 21 infants were evaluated routinely. The median age at symptom presentation was 46 days (range: 5-171 days). Seven infants had a Ladd procedure; 4 were prophylactic, with 3 as part of a combined procedure, and 3 were emergent. No child suffered acute midgut volvulus over a median follow-up of 1.6 years (range: 0.06-4.93 years).
Conclusions:
IRAs are common in infants with HS. Infants with symptoms presented by 6 months of age. There was no failure of expectant management resulting in midgut volvulus during a median follow-up of 1.6 years.
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