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

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 < 0.01), procedures (p = 0.01), clinic visits (p < 0.01), and number of prescribed medications (p < 0.01). Conclusion: Survival for adolescents with neonatal onset SBS is excellent. Of the cohort studied, there was no mortality, and more than 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.