Observation versus prophylactic Ladd procedure for asymptomatic intestinal rotational abnormalities in heterotaxy syndrome: A systematic review

Background The management of intestinal rotational abnormalities (IRA) in heterotaxy syndrome (HS) remains inconsistent. Because of the high incidence of malrotation in HS, screening of asymptomatic patients is standard of care in some institutions. The Ladd procedure is the treatment for malrotation, and has been reported to have high complication rates in HS patients. Methods We performed a systematic review of publications describing IRA in pediatric HS patients from January 1993 to present. The incidence of volvulus on surgical exploration was determined. Perioperative and long-term outcomes were analyzed to determine complication and mortality rates. Results Eleven retrospective studies describing 649 HS patients were identified. Of all patients with HS, 27% (176/649) underwent Ladd procedure. Only 1.2% (8/649) of HS patients included had volvulus. Postoperative complications occurred in 25 patients (14%), including a 10% incidence of small bowel obstruction. Perioperative and overall mortality rates after Ladd procedure were 3% and 21%, respectively. Six studies described mesenteric width, reporting 43% to have narrow mesentery. Conclusion The Ladd procedure is not without significant morbidity and mortality in heterotaxy patients. Further prospective studies should investigate predictors of mesenteric width to spare the unnecessary morbidity of surgery in patients who are at low risk for volvulus.