Outcomes of children with short bowel syndrome: experience over two decades in a multidisciplinary intestinal rehabilitation unit [Oral presentation]

Abstract

<p>Introduction<br>Short bowel syndrome (SBS) is a rare but serious form of organ failure, and patients with SBS are dependent on Total Parenteral Nutrition (PN) to maintain growth and development. This study aimed to evaluate the experiences and outcome of children with SBS managed by the multidisciplinary intestinal rehabilitation program in a tertiary paediatric centre.</p><p><br>Methods<br>A retrospective single-centre analysis of all paediatric patients with clinical diagnosis of SBS between 2001 and 2022 was performed. Clinical outcomes and their predictors were extracted and analysed.</p><p><br>Results<br>A total of 64 children were included in the study. Forty-three (67%) had extensive necrotizing enterocolitis. The median bowel length was 45cm (IQR 18 – 65), and 18.9% (IQR 10 – 28.5) of the expected length for age. Over a mean of 8.9 years of follow up, survival was 57 of 64 (89%) and 50 (78%) weaned off PN. Presence of IFALD (OR 6.375, p = 0.02) and patients managed before the introduction of fish-oil based PN in 2007 (OR 5.895, p = 0.001) were significant predictors for mortality. There was overall improvement of survival over time (p = 0.003). Ultrashort bowel length was not associated with significantly higher mortality (OR 1.1, p=0.65), but was a poor prognostic factor for weaning PN (OR 3.57, p = 0.004). Among all patients who were weaned off PN, 2 had bowel lengthening procedures and 1 received GLP-2 analogue.</p><p><br>Conclusion<br>A multidisciplinary intestinal rehabilitation program offers a comprehensive approach for patients with SBS and was shown to be effective with favourable outcomes. Improvement in the choice of PN and development of new treatment strategies potentially improved the survival and enteral autonomy of SBS patients.</p>