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

Abstract 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 30 days 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 5.1 years. 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.