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

Area of Science:

• Pediatric Gastroenterology
• Intestinal Rehabilitation
• Neonatal Surgery

Background:

• Neonatal onset short bowel syndrome (SBS) presents a significant challenge in pediatric care.
• Long-term outcomes and disease burden in adolescents with SBS are not well-defined.
• Multidisciplinary intestinal rehabilitation centers play a crucial role in managing SBS.

Purpose of the Study:

• To describe the long-term outcomes of adolescents with neonatal onset short bowel syndrome (SBS).
• To evaluate the disease burden in this patient population.
• To assess the feasibility of achieving enteral autonomy through multidisciplinary care.

Main Methods:

• A prognosis study (Level II evidence) analyzed 70 adolescents (10-19 years) with neonatal onset SBS requiring parenteral nutrition (PN) for over 90 days.
• Patients were followed at a multidisciplinary intestinal rehabilitation center between 2009 and 2018.
• WHO criteria for adolescence were utilized for patient selection.

Main Results:

• Zero mortality and 94% transplant-free survival were observed in the cohort.
• Seventy-six percent (53/70) of adolescents achieved enteral autonomy.
• Patients dependent on PN experienced higher rates of hospitalizations, procedures, clinic visits, and medication use compared to those with enteral autonomy.

Conclusions:

• Adolescents with neonatal onset SBS demonstrate excellent survival rates.
• Achieving enteral autonomy is feasible for a majority of patients through long-term multidisciplinary rehabilitation.
• Disease burden remains substantial for those requiring ongoing parenteral nutrition.