Factors impacting the survival of children with intestinal failure referred for intestinal transplantation

Purpose: The aim of this study was to analyze factors impacting on the surv ival of pediatric patients with intestinal failure referred for intestinal transplantation (ITx). Methods: Two hundred fifty-seven children (mean age, 3.4 +/- 0.26 years) wi th intestinal failure were evaluated for ITx between 1990 and 1998. All pat ients were dependent on total parenteral nutrition (TPN) for a mean of 31 /- 2.7 months. The mean follow-up time from the date of evaluation was 9.2 +/- 0.9 months. Results: Eighty-two (32%) children underwent ITx with a mean waiting time o f 10.1 +/- 1.3 months (simultaneous liver-intestinal allograft in 68% insta nces). Of the 175 patients who did not undergo transplantation, 120 died, 2 3 were lost to follow-up, and 32 are alive. Younger patients (less than or equal to 1 year) had poorer survival rates than patients older than 1 year (P < .0001). The patients with the worse prognosis were those with necrotiz ing enterocolitis, and those with the best prognosis were those with Hirsch sprung's disease. Patients with "surgical" causes had poorer survival rates than those with "nonsurgical" causes (P < .04). Patients with bridging fib rosis or established cirrhosis had an earlier mortality than patients with portal fibrosis (P < .003). The worst survival rate was found for patients with bilirubin levels of greater than 3 mg/dl.(P < .0001), platelet counts less than 100.000/mL (P < .0001), prothrombin time greater than 15 seconds (P = .03) or partial thromboplastin time greater than 40 seconds (P < .04). Children who at the time of evaluation needed only an isolated intestinal allograft had a better prognosis than those who required a combined liver-i ntestine allograft (P < .00001). With multivariate analysis independent pro gnosis risk factors of poor outcome were hyperbilirubinemia and severity of histopathologic damage. Conclusions: Early referral for ITx should occur before the development of liver dysfunction, taking into consideration the aforementioned risk factor s that would facilitate the development and ominous evolution to liver fail ure. J Pediatr Surg 34:27-33. Copyright (C) 1999 by W. B. Saunders Company.