Long-Term Outcomes and Disease Burden of Neonatal Onset Short Bowel Syndrome

Abstract Purpose The study aims to describe long-term outcomes and disease burden of neonatal onset short bowel syndrome (SBS). Methods Utilizing the WHO criteria for adolescence, patients 10–19 years of age with neonatal onset SBS requiring parenteral nutrition (PN) for > 90 days and followed by our multidisciplinary intestinal rehabilitation center between 2009 and 2018 were included for analysis. Results Seventy adolescents with SBS were studied. Median(IQR) age at last follow up in our center was 15 (11,17) years. There was 0% mortality in the cohort, and 94% remained transplant free. Fifty-three patients (76%) achieved enteral autonomy. Three patients were weaned from PN without transplantation after six years of follow-up and another four after ten years of care at our multidisciplinary center. Disease burden remained higher in adolescents receiving PN, including inpatient hospitalizations (p  Conclusion Survival for adolescents with neonatal onset SBS is excellent. Of the cohort studied, there was no mortality, and over 75% achieved enteral autonomy. Disease burden remains high for adolescents who remain dependent on PN. However, achievement of enteral autonomy is feasible with long-term multidisciplinary rehabilitation. Type of Study: Prognosis Study. Level of Evidence: Level II.