Intestinal transplantation for short gut syndrome attributable to necrotizing enterocolitis

Background. Necrotizing enterocolitis (NEC) is a life-threatening condition of the neonatal age, which frequently requires surgical intervention. Afte r extensive bowel resection, a small proportion of these patients may devel op chronic short gut syndrome (SGS) and require chronic total parenteral nu trition (TPN) use. Intestinal transplantation has been performed in these p atients as a life-saving option. This study reviews our experience with intestinal transplantation for SGS a ttributable to NEC emphasizing the mode of presentation, natural history, t iming, and outcome. Methods. A retrospective chart review was performed for all pediatric patie nts who underwent small bowel transplantation for NEC at the University of Miami between August 1994 and March 1999. Results. Eleven transplants were performed for 10 patients with NEC (8 male and 2 female; median age: 1.75 years [range: 10 months to 10.1 years]). Pr ocedures performed were isolated intestinal transplants (n = 2), combined l iver-intestinal transplants (n = 6), and multivisceral transplants (n = 3). All patients were born prematurely with median birth weight of 1.640 kg (r ange: 810 g to 2.730 kg). They developed NEC in the first few days of life and subsequently underwent an average of 5 surgeries per patient before tra nsplant. Transplant was indicated for liver failure in 8 patients and recur rent central line sepsis in 2 others. At present, 6 patients are alive with an overall 1-year and 3-year actuaria l survival of 60% and a median follow-up of 29 months (range: 9-46 months). Six children have been weaned off TPN after a median time of 71 days (rang e: 19-131) from transplantation. All survivors are at home with functional grafts. Conclusion. Intestinal transplantation provided a reasonable outcome in pat ients with NEC-associated SGS who had already developed life-threatening co mplications related to TPN. Intestinal transplantation replaced the disease d intestine and liver, enfranchised patients from TPN, and conferred improv ed quality of life. These patients should be actively considered for intest inal transplantation and referred to a transplant center as soon as possibl e.