The role of screening and prophylactic surgery for malrotation in heterotaxy patients.

PURPOSE: There are no standardized guidelines for screening or management of malrotation in Heterotaxy Syndrome (HS). We sought to review our experience to determine if evidenced based guidelines could be drafted. METHODS: A retrospective chart review was performed at our freestanding children's hospital on all patients under one year of age undergoing a Ladd procedure between 2000 and 2011. In addition, all Heterotaxy patients were reviewed during this period. RESULTS: Twenty-three Heterotaxy patients and seventy-nine Non-Heterotaxy patients underwent a Ladd procedure. Both groups had a high rate of complication. Heterotaxy was associated with significantly higher mortality 30days after Ladd procedure. In our review, we also identified seventy-six HS patients who did not undergo a Ladd procedure. Among these patients, fourteen had normal intestinal anatomy, five had malrotation, and fifty-seven were never evaluated for intestinal malrotation. No patients with intestinal malrotation or unknown intestinal rotation status suffered midgut volvulus. Average follow-up time was 5.1years. CONCLUSIONS: We conclude that prophylactic Ladd procedures in children with Heterotaxy are associated with a high morbidity and mortality. Patients who avoided screening were not exposed to a significant risk of midgut volvulus, and our experience suggests that routine screening of Heterotaxy patients for malrotation should be abandoned.

Heterotaxy syndrome: is a prophylactic Ladd procedure necessary in asymptomatic patients?

Heterotaxy syndrome (HS) is a complex disorder involving thoracic and abdominal asymmetries. Congenital heart disease is often accompanied by an intestinal rotation abnormality (IRA) that may predispose to bowel ischemia and infarction. There is controversy in the literature whether asymptomatic infants with HS must be screened for IRA and, if present, whether a prophylactic Ladd procedure should be performed. We performed a retrospective chart review of all patients who underwent a Ladd procedure from January 2007 to December 2010 at Stollery Children's Hospital, Edmonton, Canada. Twenty-nine patients underwent a Ladd procedure, 22 without HS but with symptomatic malrotation and 7 with HS and asymptomatic malrotation. Asymptomatic HS patients had a complication rate of 57 % after a prophylactic Ladd procedure compared with a complication rate of 9 % in the symptomatic non-HS population. The management of asymptomatic IRA in patients with HS remains controversial. We suggest that HS patients be screened for IRA and that asymptomatic patients be managed conservatively.