Evaluating a management strategy for malrotation in heterotaxy patients

BackgroundThere is disagreement over the management of malrotation in children with heterotaxy and congenital heart disease (CHD). We sought to evaluate the outcomes of management with a Ladd procedure compared to observation in this cohort of patients.MethodsWe performed a retrospective review of CHD children with heterotaxy and malrotation identified on radiographs treated from 8/2002 until 4/2014. Primary outcomes evaluated were readmission for small bowel obstruction (SBO) or volvulus.ResultsWe identified 88 patients with cardiac heterotaxy and malrotation. Sixty-eight (77%) had a Ladd procedure. Eighteen (26%) of the 68 had abdominal symptoms, but only one had an underlying volvulus without ischemia. Twenty (23%) patients died of cardiopulmonary complications, 8 before and 12 after the Ladd procedure. Sixty-eight patients survived to the review date (median: 5 years): 56 in the Ladd cohort and 12 observed. Six of the 56 (11%) surviving Ladd patients were readmitted to hospital with an SBO, and 2 required surgical intervention. None of the 12 surviving nonoperative patients developed a volvulus.ConclusionEleven percent of patients developed SBO after their Ladd procedure. Conversely, no observed patients developed a volvulus. This suggests that complications from a Ladd procedure occur with higher frequency than complications from observing heterotaxy patients with malrotation.