P1.56: Colonic continuity and Incidence of weaning from PN by 5 years of age in children with onset of intestinal failure(IF) related to short bowel syndrome(SBS) by 3 months of age with chronic persistent dependence on parenteral nutrition(PN) at one year of age

Objectives: To review 5-year outcome of children presenting in infancy with chronic intestinal failure (IF) associated with short bowel syndrome (SBS) who were still on parenteral nutrition (PN) treatment at 12 months of age. Method: Medical records of patients with SBS related IF presenting in neonatal period and up to 3 months of age from 2007–2017 who were still on PN at 12 months of age were reviewed. Data obtained included small intestinal length at time of resection, aetiology, presence/absence of ileo-caecal (I-C) valve and/or colon and number of patients still on PN at 5 years. Results: Twenty patients were identified. Eleven were male and 9 female. 14/20 were born prematurely. Diagnoses were necrotising enterocolitis (NEC) in 9, volvulus in 4, long segment Hirschsprung’s disease in 3, gastroschisis in 2, and intestinal atresia in 2. The remaining small intestinal length at time of surgery ranged from 4.5cm - 120cm (median 18cm) in 19 cases (not measured in 1). Four patients had ultra SBS (length <10cm). Eleven patients weaned from PN by 5 years of age and 9 were still on PN. The median age at time of weaning (corrected for gestational age in premature infants) was 2 years 4 months (range 1 year 6 months - 5 years 9 months). Small intestinal length ranged from 7–120 cm (median 30cm) in the weaned group and from 4–90 cm (median 20cm) in the persistent IF group. Seven/11 patients who weaned and 2/9 still on PN were female (p=0.09 not significant). 8/11 who weaned and 2/9 still on PN had NEC (p=0.068, not significant). 7/11 who weaned had an intact I-C valve compared to 3/9 on PN (p=0.6 not significant). All 11 children who weaned had a colon present compared to 5/9 who remained on PN (p=0.026, significant). 11/16 or 68% with colon in continuity weaned. Conclusion: Over 50% (11/20) infants with SBS and PN dependence for >12 months weaned from PN by or at 5 years of age. There was a statistically significant association with presence of colon with 68% with a colon weaning and even with some cases of ultra-SBS. Larger studies are needed to gain other predictors of weaning in this diverse group of patients.