Background: Recent advances in the management of children with chronic intestinal failure have presented new therapeutic options for treatment in this complex patient group. To help inform future therapeutic strategies, we assessed the outcome of children with chronic intestinal failure managed by a multidisciplinary intestinal rehabilitation program over a 27-year period.

Methods: A retrospective longitudinal review of children with chronic intestinal failure assessed for home parenteral nutrition (PN) therapy at the Royal Children’s Hospital Melbourne from 1991 to 2018.

Results: A total of 64 children with chronic intestinal failure due to short bowel syndrome [SBS: median bowel length 33.5cm] (n=51, 80%), chronic intestinal pseudo-obstruction (n=5), congenital enteropathy (n=4) and other genetic abnormalities (cystic fibrosis n=1, megacystitis microcolon syndrome n=1, immune deficiency n=2) were included. The overall survival was 57 (88%), with no deaths attributed to intestinal failure or its management in home PN patients in the past decade. Weaning from PN was successful in 31/45 (69%) of SBS survivors after a median of 18.5 months. Longer residual small bowel length and the presence of the ileocaecal valve (ICV) and colon were predictors of success. The frequency of central line associated blood stream infections has fallen dramatically from ~7.3 episodes/1000 line days in 1991 to 2011, to ~0.5 episodes/1000 line days in 2018. Eight patients transitioned to an adult Home PN program and two patients have undergone successful liver-intestinal transplantation.

Conclusion: Advances in the management of children with chronic intestinal failure have had a significant impact resulting in improved outcomes. Children with SBS, even in the presence of small residual bowel length have a good prospect of weaning from PN, particularly if the ICV and colon are preserved. The survival rate and frequency of central line associated blood stream infections have dramatically improved. Targeted individualized therapy and care by a multidisciplinary team expert in intestinal rehabilitation and nutrition can optimize outcomes in children with chronic intestinal failure.