AB021. SOH24AB_125. Outcomes of children with intestinal failure secondary to gastrointestinal dysmotility

Background: The primary objective of our study is to determine the characteristics and outcomes of pediatric patients with intestinal failure (IF) secondary to dysmotility. Methods: This is a retrospective cohort study of all patients managed by a multidisciplinary intestinal rehabilitation program with a diagnosis of IF with gastrointestinal (GI) dysmotility between January 1, 2006 and December 31, 2020 with a minimum one year follow up. Student's t-test, Wilcoxon rank sum test, and X2 were used where appropriate. Results: Fifty-one patients with intestinal dysmotility [median gestational age of 35 weeks, interquartile range (IQR): 31–37 weeks; 22 males (43%)] were studied. Median birthweight was 2,160 g (IQR: 1,635–2,586 g). Eighty-eight percent achieved enteral autonomy (EA) after a median parenteral nutrition duration of 150.5 days (IQR: 93.0–206.0 days). Advanced liver disease [conjugated bilirubin (cBili) >100 μmol/L] was seen in 2/51 patients (3.9%), with no patients receiving an intestinal or liver transplant. The case fatality rate was 3/51 (5.9%) but was related to complications of prematurity. Median follow up was 747 days (IQR: 420.5–1,691.5 days). Patients with a congenital short bowel syndrome (SBS) diagnosis (i.e., gastroschisis, necrotizing enterocolitis) had an earlier referral age [75 (IQR: 48–109) vs. 868 (IQR: 61–1,286) days] and were more likely to achieve EA (97% vs. 64%, P<0.01) compared to those with primary dysmotility (i.e., chronic intestinal pseudo-obstruction). Conclusions: This cohort study demonstrates significant long-term survival for patients with dysmotility. There were two distinct populations-neonates with a congenital SBS diagnosis and those presenting later with a primary dysmotility. Patients with a congenital diagnosis had transient symptoms and were likely to achieve EA by 6 months.