A meta-analysis of clinical outcome of intestinal transplantation in patients with total intestinal aganglionosis

Total intestinal aganglionosis (TIA) occurs in less than 1% of patients with Hirschsprung disease (HD), and TIA is the most severe form of HD. Survival has improved with the advent of parenteral nutrition and intestinal transplantation (ITx). The field of ITx has rapidly progressed in the last two decades and has now become an established treatment for patients with intestinal failure. The purpose of this meta-analysis was to determine the clinical outcome of ITx in patients with TIA. A systematic literature search for relevant articles was performed in four databases using the combinations of the following terms: total intestinal aganglionosis, intestinal transplantation, and Hirschsprung disease/Hirschsprung's disease for studies published between 2003 and 2016. The relevant cohorts of ITx in patients with TIA were systematically searched for clinical outcomes. Thirteen studies met defined inclusion criteria, reporting a total of 63 patients who underwent ITx for TIA. Majority of patients were males (71.0%), and median age of ITx was 4.3 (range 0.25-17.6) years. Isolated ITx was performed in 37% patients and multivisceral ITx in 63%. Mean follow-up period was 40 months (range 1-154). Overall survival rate was 66%; the longest survivor was 12.8-year-old after ITx. ITx appears promising in the management of TIA. ITx can be considered a feasible treatment option for patients with TIA who suffer from life-threatening complications of intestinal failure.