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Paeds Casesgastroenterology-hepatology-and-nutrition

Paeds Cases · gastroenterology-hepatology-and-nutrition

Short-bowel syndrome and intestinal failure — structured clinical encounter

Structured encounter testing the approach to a premature neonate after necrotising enterocolitis with a high-output jejunostomy and rising conjugated bilirubin: the definition and classification, the sodium principle, the trophic role of enteral feeding and glucagon-like peptide 2, the prevention of intestinal failure-associated liver disease, and a pivot to an older child on home parenteral nutrition assessed for teduglutide, serial transverse enteroplasty and transplant.

structured clinical encounter3 min readVerification in progress

Target exams

RACP General PaediatricsRACP DCEMRCPCH ClinicalRCPSC Pediatrics
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Target exams

RACP General PaediatricsRACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A baby born at 27 weeks gestation undergoes a massive small-bowel resection for necrotising enterocolitis, leaving a 35-centimetre jejunal remnant ending in a jejunostomy with the colon disconnected. You are the paediatric registrar working through assessment, the sodium principle, intestinal adaptation, IFALD prevention, and a later scenario of an older child on home parenteral nutrition assessed for teduglutide, serial transverse enteroplasty and transplant.

Station 1 — recognition and classification

Asked for my first impression, I explain that a premature baby with a high-output jejunostomy after necrotising enterocolitis resection has intestinal failure from short-bowel syndrome. I would define short-bowel syndrome as the malabsorption following massive small-intestinal resection, and intestinal failure as the broader inability of the gut to sustain growth and hydration. The key prognostic feature here is the end-jejunostomy with the colon disconnected, which carries the poorest outlook of the three anatomical types, in contrast to a jejunoileal configuration with an intact colon and ileocaecal valve which would carry the best. [1]

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References7ShowHide
  1. [1]Duggan CP; Jaksic T Pediatric Intestinal Failure. N Engl J Med, 2017.PMID 28813225
  2. [2]Premkumar MH Nutritional Management of Short Bowel Syndrome. Clin Perinatol, 2022.PMID 35659103
  3. [3]Chiba M; Arnon R; Kori M; et al Efficacy and Safety of Teduglutide in Infants and Children With Short Bowel Syndrome Dependent on Parenteral Support. J Pediatr Gastroenterol Nutr, 2023.PMID 37364133
  4. [5]Lee WS; Teo EH; Chong PY; Poh LK Intestinal failure-associated liver disease (IFALD): insights into pathogenesis and advances in management. Hepatol Int, 2020.PMID 32356227
  5. [8]Dagorno C; Breton A; Lamireau T; et al Serial Transverse Enteroplasty (STEP) for Short Bowel Syndrome (SBS) in Children: A Multicenter Study on Long-term Outcomes. J Pediatr Surg, 2025.PMID 39368852
  6. [10]Lee EJ; Iyer KR; Horslen S Pediatric intestinal transplantation. Semin Pediatr Surg, 2022.PMID 35725057
  7. [11]Crenn P; Messing B; Cynober L Citrulline as a biomarker of intestinal failure due to enterocyte mass reduction. Clin Nutr, 2008.PMID 18440672
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