Introduction: Important changes in the management of pediatric intestinal failure (IF) have been implemented over the last 15 years.

Objective: To assess the impact of those changes on the outcome of a contemporary cohort of pediatric IF patients from 6 IF programs, 5 of which offer intestinal transplantation.

Methods: Retrospective analysis of a multicenter IF cohort. Entry criteria included patients < 18 years of age receiving PN for more than 42 consecutive days, primary gastroenterological disorder and diagnosis of IF between 2010 and 2015. Primary outcome was death, transplant or enteral autonomy. Kaplan-Meier analysis was used for time-to-event and competing risk analysis for cumulative incidence rates. Comparisons were conducted with a log rank test and Cox regression analysis.

Results: 443 patients (male 61%) with a median gestational age of 34 weeks (29-37) and birth weight of 2.1 kg (1.2-2.8) constituted the study group. Patients were followed for 3.8 years (2.3-5.3). Median time on PN was 384 days (110-947). Patients had 40% (16-100) of expected small bowel length for age remaining and 250 (56.4%) patients had intact full colon in continuity. Main etiologies included necrotizing enterocolitis (29.3%), abdominal wall defects (30.5%), dysmotility disorders (7.2%), and mucosal enteropathies (6.8%). Eighty-one patients (18.3%) developed progressive intestinal failure associated liver disease (IFALD) with a bilirubin >75mmol/l. All patients were managed with hepatoprotective nutritional strategies (fish oil based lipids or lipid lowering strategies). The presence of IFALD impacted negatively on transplant free survival (P<0.001) and achievement of enteral autonomy (P<0.01). Overall, 213 (48.1%) patients achieved enteral autonomy, 137 (30.9%) remained on PN, 53 (12%) were transplanted and 40 (9%) patients died. Achievement of enteral autonomy was associated with short bowel syndrome (P<0.01), NEC (P=0.026), intact colon (P<0.01), and ileocecal valve (P<0.01).

Conclusions: This contemporary cohort achieved a significantly lower incidence of death and transplantation compared with previous multicenter reports. However, the number of children achieving enteral autonomy has not changed while a larger number of patients remain PN dependent. Although this cohort may represent a more complex group of patients due to potential referral bias, these findings suggests a shift in outcomes and a need for new strategies for the achievement of enteral autonomy.