In neonatal-onset surgical short bowel syndrome survival is high, and enteral autonomy is related to residual bowel length.

ABSTRACT

BACKGROUND: In an era of improved management and treatment options, this study aims to describe the long-term outcomes and factors predictive of outcomes of neonatal-onset intestinal failure (IF) due to surgical short bowel syndrome (SBS). METHODS: Retrospective, single-center cohort study of infants born between January 2011 and December 2018 with inclusion criteria <44 weeks postmenstrual age at SBS diagnosis, <28 days on admission, parenteral nutrition dependence >60 days, and documented intestinal resection. Primary outcomes included survival and achievement of enteral autonomy (EA). Data analysis utilized Fisher.s exact test, Kruskal-Wallis test, survival analysis methods, Cox proportional hazards regression, linear regression and logistic regression. RESULTS: Ninety-five patients (males 56%) were studied with median follow-up of 38 months (IQR 19, 59). Survival at last follow-up was 96%, and EA was achieved in 85%. Forty-eight patients had documented residual bowel length (RBL) with median length of 49 cm (IQR 36, 80). Survival in patients with RBL of <30cm (n = 8), 30-59cm (n = 19), and >60cm (n = 21) was 100%, 95%, and 95% respectively. Shorter RBL was associated with longer time to achieve EA (p = 0.007), but not with survival (p = 0.81). Delay in achieving EA was associated with absence of ileocecal valve (p = 0.002) and bloodstream infections (p < 0.001). Peak conjugated bilirubin correlated with increased mortality (p = 0.002). CONCLUSION: Overall high rate of survival and achievement of EA was found in neonatal onset IF due to SBS. EA but not survival was correlated with RBL. Ileocecal valve, bloodstream infections, and conjugated bilirubin levels were the other predictive factors of outcomes.