Introduction: To analyze a single-center outcomes of pediatric-onset intestinal failure (IF).

Methods: All children with IF requiring parenteral nutrition (PN) >2 months or small intestinal resection ≥50% managed by our center were included. Patient characteristics, weaning off PN, survival and their predictors were retrospectively analysed with Kaplan-Meier curves, log-rank test and Cox regression models.

Results: In total, 93 patients with median follow-up of 6.1 (IQR 2.8-12) years and PN-duration of 13 (5.2-40) months were identified. Median gestational age was 35 weeks (28-39) and birth weight 2238 g (870-3380).  PN was started at median age of 0 (0-0) days. Patients had 47 cm (30-95) or 29% (18-80) of age-adjusted small intestine and 86% (41-100) of colon remaining, while 49% had ileocecal valve (ICV) preserved and 18% small intestinal end-ostomy. Etiology of IF included short bowel syndrome (SBS; n=73), primary intestinal motility disorders (PIMD; n=13) and congenital intestinopathies (CI; n=7). Causes of SBS were NEC (n=32), atresia (n=14), volvulus (n=13), Hirschsprung disease (HD, n=10), and gastroschisis (n=4). Twenty-four patients underwent autologous bowel surgery including lengthening/tapering (n=18) to improve function. By end of follow-up 67% had weaned off PN, while Kaplan-Meier 5- and 10-year weaning off estimates were 66% (95%CI 56-77) and 70% (59-81), respectively (Fig. 1). Patients with established intestinal continuity (Hazard Ratio 8.9, 95%CI 2.7-28, p<0.001), SBS-diagnosis (3.2, 1.4-6.9, p=0.004), preserved ICV (2.8, 1.6-4.7, p<0.001), >70% of remaining colon (2.7, 1.5-4.7, p<0.001) and >20% of remaining small bowel (1.8, 1.0-3.3, p=0.045) had increased likelihood to wean off PN. Incidence of culture proven blood stream infections was 1.3/1000 PN-days. Derangements in liver biochemistry was observed in 27%, and 37% (26/69) had Metavir fibrosis stage ≥2 in liver biopsy.  Five patients weaned off after intestinal transplantation and four of them are alive after 3.9 years (1.5-6.5) and two are listed. Eight patients died (8.6%) and overall 10-year survival estimate was 90% (95%CI 84-97, Fig. 2). Among survivors height z-score was -1.5 (-2.5- -0.4) and weight percentile -4.0% (-12-3.3).

Conclusion: Despite reassuring long-term survival, blood stream infections, IF-associated liver disease and weaning off PN in patients with very short remaining small bowel, end-jejunostomy, PIMD and CI are still significant concerns in children with IF.