Purpose: Heterotaxy syndrome (HS) patients often present with congenital heart disease and intestinal malrotation. Controversy exists regarding the management of these patients. Risk of midgut volvulus, morbidity from elective operations, and overall prognosis must be weighed when considering a Ladd procedure on asymptomatic HS/intestinal malrotation patients.
Methods: This is a retrospective review comparing HS and non-heterotaxy syndrome (NHS) patients undergoing a Ladd procedure at Children's Hospital Boston (Mass) from January 1997 to September 2007.
Results: Thirty-one HS and 51 NHS patients were identified. After a Ladd procedure, HS patients remained in the hospital 12.9 days, had a 9.7% risk of small bowel obstruction, and a 9.7% in-hospital mortality. If allowed to develop abdominal symptoms, 27% of HS patients with intestinal malrotation had a midgut volvulus at surgery. Intra-HS group comparison demonstrated longer hospital stays in symptomatic patients (P = .01). Mortality was greater in the HS than NHS patients, but deaths were related to cardiac disease and not to the Ladd procedure.
Conclusions: Elective Ladd procedures are well tolerated by HS patients. Given the risk of midgut volvulus and in light of improved survival beyond infanthood, once identified, HS patients with asymptomatic malrotation should be offered a prophylactic Ladd procedure.