Short-bowel syndrome and intestinal failure

Overview & Definition

Picture a baby born at 28 weeks who develops necrotising enterocolitis, loses most of the small bowel, and comes back to the ward with a high jejunostomy and a central line dripping parenteral nutrition. Or picture a four-year-old who had a mid-gut volvulus as a newborn and now feeds almost entirely by mouth, the gut having reshaped itself over years. These two children are the same diagnosis at opposite ends of its course, and they hold the central idea of this topic: when a child loses most of the small intestine, the body fights to make the remnant do more work, and the job of the paediatric team is to keep that child alive, growing and uninfected long enough for the gut to adapt — or to plan a transplant when it cannot. [1]

The vocabulary matters because examiners use the terms precisely. Short-bowel syndrome is the malabsorption that follows a massive resection of small intestine; it is an anatomical event with a functional consequence. Intestinal failure is the broader state in which the gut cannot sustain growth, hydration and electrolyte balance on its own, so the child needs parenteral support. Every short-bowel child who still needs parenteral nutrition has intestinal failure, but a child can have intestinal failure from mucosal disease or a dysmotile gut with a normal-length bowel. Holding that distinction stops the common viva slip of conflating the two. [1] [2]

The governing principle, then, is to classify the child by what remains, protect the remnant, and buy time for adaptation while preventing the two complications that end the race early — intestinal failure-associated liver disease and central-line bloodstream infection. Almost everything else in management flows from that single strategy. [1]

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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. [4]Rosete BE; de Paula CB; Sperber LF; et al Teduglutide for pediatric short bowel syndrome patients. Expert Rev Gastroenterol Hepatol, 2021.PMID 33798402
5. [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
6. [6]Fundora J; Lopez M; Saps M; et al Intestinal Failure-Associated Liver Disease in Neonates. Neoreviews, 2020.PMID 32873652
7. [7]Goulet OJ Lipid Emulsion Use in Pediatric Patients Requiring Long-Term Parenteral Nutrition. JPEN J Parenter Enteral Nutr, 2020.PMID 32049395
8. [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
9. [9]Mercer DF; Sutton DG; Pinschewer DD; et al Repeat serial transverse enteroplasty leads to reduction in parenteral nutrition in children with short bowel syndrome. J Pediatr Surg, 2021.PMID 32736789
10. [10]Lee EJ; Iyer KR; Horslen S Pediatric intestinal transplantation. Semin Pediatr Surg, 2022.PMID 35725057
11. [11]Crenn P; Messing B; Cynober L Citrulline as a biomarker of intestinal failure due to enterocyte mass reduction. Clin Nutr, 2008.PMID 18440672
12. [12]Fallon EM; Le HD; Puder M Prevention of parenteral nutrition-associated liver disease: role of omega-3 fish oil. Curr Opin Organ Transplant, 2010.PMID 20503524