Long term outcomes of no gut syndrome in children - A multicentre study.

Abstract

Background: Children with extreme short bowel syndrome, including those who have undergone resection of the entire jejunum and ileum-termed "No Gut Syndrome" (NGS) in the adult literature-are now surviving due to advancements in parenteral nutrition (PN) and medical care. This study aims to describe the population characteristics and long-term outcomes of children with NGS.

Material and methods: A questionnaire was distributed to the paediatric intestinal failure (IF) centres in the UK to identify patients with NGS discharged home on PN. Inclusion criteria: infants/children < 18 years with duodenum plus up to 5 cm jejunum and colon (total/partial). Follow-up data was collected two years after the initial recruitment. A multicentre Health Research Authority approval was obtained.

Results: 19/20 (95 %) IF centres managing 379 HPN patients responded - 27/379 (7 %) had ultrashort-bowel syndrome (U-SBS) and an additional 14/379 (4 %) NGS. Aetiology of NGS was - midgut volvulus (64 %), bowel ischaemia (22 %), necrotising enterocolitis (7 %), jejuno-ileal atresia (7 %).Nine patients had a colonic remnant in continuity. Thirteen/14 patients (93 %) remained stable on PN with one weaned off following small bowel transplant. Nine/13 (69 %) patients received > 80 % of EAR as PN and 4/13 (31 %), 30-80 %. Eight/13 (61 %) tolerated some form of enteral nutrition. There were no deaths during the follow-up period. Educational outcomes were positive showing that 11/14 (79 %) are attending school, 2/14 (14 %) nurseries with 1 awaiting enrollment (7 %).

Conclusion: Advances in PN and health care have improved the long-term survival of patients with NGS. In addition, these children are attending school and participating in daily activities without significant limitation.