Paeds · gastroenterology-hepatology-and-nutrition
Short-bowel syndrome and intestinal failure
Also known as Short bowel syndrome · Intestinal failure · Short gut syndrome · Parenteral nutrition dependence · Intestinal rehabilitation · Teduglutide therapy · Serial transverse enteroplasty · Intestinal failure-associated liver disease · IFALD · Intestinal transplant
Fellowship guide to short-bowel syndrome and intestinal failure in children, built around the rule that the remaining anatomy matters more than the residual length: an intact colon and ileocaecal valve is the strongest predictor that a child will one day feed entirely by mouth. The page covers how massive small-bowel resection from necrotising enterocolitis, volvulus or atresia produces malabsorption, how the remnant gut adapts under the drive of enteral feeding and glucagon-like peptide 2, how to manage parenteral nutrition, advance feeds and balance the sodium-hungry high-output stoma, when to add teduglutide at 0.05 milligrams per kilogram subcutaneously once daily, when to offer serial transverse enteroplasty, and how to recognise and prevent intestinal failure-associated liver disease and central-line bloodstream infection.
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- A jejunostomy with under about 50 centimetres of residual small bowel and no colon almost never achieves enteral autonomy on its own and usually needs an intestinal rehabilitation programme and often transplant
- Persistent or rising conjugated bilirubin in an infant on parenteral nutrition is intestinal failure-associated liver disease until proven otherwise, and lipid overload plus recurrent sepsis are the two modifiable drivers
- A high-output stoma losing more than about 40 to 50 millilitres per kilogram per day quietly drains sodium and water and causes growth failure even when the calorie prescription looks adequate
- Recurrent central-line bloodstream infection, loss of venous access sites and irreversible cholestasis are the three classical indications that escalate a child toward intestinal or liver-intestine transplant
- An intercurrent gastroenteritis in a child with a short bowel can precipitate rapid dehydration and decompensation because the remnant gut cannot compensate for the extra losses
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Clinical exam formats
Board mappings
- Definition of short-bowel syndrome and intestinal failure
- Common causes of short bowel in neonates and children
- Complications of long-term parenteral nutrition
- Intestinal adaptation physiology and the role of enteral nutrition and glucagon-like peptide 2
- Indications, dose and monitoring of teduglutide in paediatric intestinal failure
- Role of serial transverse enteroplasty and intestinal transplant
- The neonate with a high-output stoma after necrotising enterocolitis resection
- Intestinal failure-associated liver disease: recognition and lipid strategy
- Central-line bloodstream infection prevention in the child on parenteral nutrition
- Long case: a child with short bowel syndrome on home parenteral nutrition
- Short case: assessing growth, hydration and stoma output in the intestinal-failure child
- Gastroenterology: intestinal failure and the short-bowel child
- Nutrition: parenteral nutrition dependence and weaning
- Causes and classification of short-bowel syndrome
- Complications of long-term parenteral nutrition including IFALD
- Principles of intestinal rehabilitation and teduglutide
- Assessment and long-case discussion of a child with short bowel on parenteral nutrition
- Short-bowel syndrome and intestinal failure
- Parenteral nutrition complications and IFALD
- Intestinal rehabilitation and transplant
- Patient Care: management of the paediatric intestinal-failure patient on parenteral nutrition
- Medical Expert: short-bowel syndrome and intestinal failure in children
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]
References12ShowHide
- [1]Duggan CP; Jaksic T Pediatric Intestinal Failure. N Engl J Med, 2017.PMID 28813225
- [2]Premkumar MH Nutritional Management of Short Bowel Syndrome. Clin Perinatol, 2022.PMID 35659103
- [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]Rosete BE; de Paula CB; Sperber LF; et al Teduglutide for pediatric short bowel syndrome patients. Expert Rev Gastroenterol Hepatol, 2021.PMID 33798402
- [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]Fundora J; Lopez M; Saps M; et al Intestinal Failure-Associated Liver Disease in Neonates. Neoreviews, 2020.PMID 32873652
- [7]Goulet OJ Lipid Emulsion Use in Pediatric Patients Requiring Long-Term Parenteral Nutrition. JPEN J Parenter Enteral Nutr, 2020.PMID 32049395
- [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]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]Lee EJ; Iyer KR; Horslen S Pediatric intestinal transplantation. Semin Pediatr Surg, 2022.PMID 35725057
- [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]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