Short bowel syndrome and intestinal failure in infants and children

Abstract

Children with short bowel syndrome (SBS), and resultant intestinal failure (IF), represent a unique and challenging patient population for paediatric health professionals. The number of infants requiring prolonged parenteral nutrition (PN) for SBS continues to increase. The need for often complex PN and enteral nutrition (EN) regimens requires the formation of a multidisciplinary nutrition support team (NST) with specialist expertise. The achievement of enteral adaptation and weaning from PN can take up to several years, necessitating delivery of PN at home (HPN). The complications of PN, namely recurrent sepsis and intestinal failure associated liver disease (IFALD) limit survival in this group. While medical and nutritional therapies for SBS and IF remain limited, advancing surgical techniques may help improve adaptation in the long-term SBS. Intestinal transplantation also continues to play a role for patients with irreversible SBS, IF and advanced IFALD. The optimum design of regional services for SBS in children remains controversial; however the standardization of care and dissemination of skills through managed clinical networks will enable a cohesive approach to patient management.