Intestinal failure
Key points
No consensus for short gut definition in children exists - in general it is either congenital or surgical shortening of the bowel requiring prolonged parenteral nutrition
Normal gut length at birth - 275cm
Average congenital (probably autosomal recessive) short gut length - 50cm
Neonate with 35cm gut - 50% chance of coming off PN
Neonate with 10cm gut survivable with chance of coming off PN
Predictors of enteral autonomy JPS 2015 multicentre US cohort study
Length >41cm best predicts coming off PN
Every 1cm bowel - increase 4% chance of coming off PN
Intact Ileocaecal valve (ICV) improves prognosis
Phases of short bowel syndrome
Acute phase: 3-4 weeks, large gastric fluid losses due to lack of gut feedback inhibitory hormones
Adaptation phase: 1-2 years, bowel increases in length and calibre, all layers hypertrophy and mucosa adapts to absorb more
Gut energy substrates
Small-bowel enterocytes: Glutamine
Colonocytes: Butyrate (Short-chain fatty acid)
Physiology
Breast milk is optimal feed for short bowel syndrome
Ileum is most adaptive - so jejunal resection is best tolerated
Ileal resection disrupts entero-hepatic circulation
Complications
Bacterial overgrowth causes GI bleeding, production of D-lactic acid - causes ataxia, altered mental state, and acidosis
ICV prevents reflux into small bowel and bacterial overgrowth - Proteobacteria overgorwth then causes liver injury
Zinc and essential fatty acid deficiency can cause rashes and failure to thrive
Standard scenario
Patient with short gut
Place Hickman line or port depending on age of child and preference
Manage with gastroenterology MDT, dietician and senior surgeon
Priorities -
Ideally more enteral feeds, less PN
Protect liver
Protect vascular access
Monitor micronutrients
Consider placing gastrostomy for continuous feeds
Ways to protect liver when on PN
Prevent line sepsis
SMOF, cycling timings
Preserve ileum (adapts better) and ICV
Preserve vessels
Vascular access with percutaneous technique
Small calibre catheters
Prevent line sepsis
Indications for STEP
If developing complications of lines/PN
Worsening tolerance of enteral feeds
Should ideally be performed in dilated bowel
Some surgeons will allow bowel to dilate due to an obstruction (not feeding at this time) with the intention of later STEP
Get upper GI contrast follow through to estimate length and calibre of bowel before STEP
Always consult senior surgeon with prior experience of STEP first
An initial STEP can be done, ending in a stoma which can be closed at a later date
STEP can be repeated - but with diminishing returns
Small bowel transplant indications
Failure to gain weight
Failure to maintain fluid balance
Thrombosis of 2 or more central veins
Line infections
Liver failure - may also need simultanoues liver transplant
Glucagon-Like Peptide-2 (GLP-2) Analogues
GLP-2 is a naturally occurring intestinal hormone produced by the L cells in the distal small intestine and colon in response to food intake.
Main functions include:
Stimulating intestinal growth (mucosal hypertrophy)
Enhancing nutrient absorption
Reducing gastric motility (slowing transit)
Increasing mesenteric blood flow
Teduglutide is a synthetic analogue of GLP-2, modified to have a longer half-life (resistant to enzymatic degradation) and used therapeutically in conditions like short bowel syndrome (SBS) and intestinal failure.
FAD approved for children ≥1 year with intestinal failure dependent on PN.
Mechanisms of Action
Teduglutide acts via indirect paracrine stimulation:
Mediators: IGF-1, EGF, VIP
Cellular Effects:
Promotes crypt cell proliferation
Decreases apoptosis
Enhances enzyme activity (e.g., maltase, sucrase)
Increases nutrient absorption
Additional physiological effects:
Slowed intestinal transit time
Increased mesenteric blood flow
These effects collectively lead to improved absorptive function in the remaining intestine.
Impact on Intestinal Failure: 24-Week Phase 3 Trial Findings
Dose: Teduglutide 0.05 mg/kg/day given SC vs. standard care in children:
Outcome:
TPN volume reduction - 42%
≥20% decrease in TPN support 69% of patients
Achieved enteral autonomy -11.5%
Note: Continued therapy is essential to maintain the trophic effect of the drug.
Page edited by Mr Mahmoud Abdelbary MSc, MRCS August 2025
Page edited by Mrs Charnjit Seehra BSc August 2025
References
Van Der Werf CS et al. CLMP is required for intestinal development, and loss-of-function mutations cause congenital short-bowel syndrome. Gastroenterology. 2012 Mar;142(3):453-462.e3. doi: 10.1053/j.gastro.2011.11.038. Epub 2011 Dec 7. PMID: 22155368.
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Khan FA et al; Pediatric Intestinal Failure Consortium. Predictors of Enteral Autonomy in Children with Intestinal Failure: A Multicenter Cohort Study. J Pediatr. 2015 Jul;167(1):29-34.e1. doi: 10.1016/j.jpeds.2015.03.040. Epub 2015 Apr 25. PMID: 25917765; PMCID: PMC4485931.
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Kaufman SS, Atkinson JB, Bianchi A, Goulet OJ, Grant D, Langnas AN, McDiarmid SV, Mittal N, Reyes J, Tzakis AG; American Society of Transplantation. Indications for pediatric intestinal transplantation: a position paper of the American Society of Transplantation. Pediatr Transplant. 2001 Apr;5(2):80-7. doi: 10.1034/j.1399-3046.2001.005002080.x. PMID: 11328544.
Carter BA, Cohran VC, Cole CR, Corkins MR, Dimmitt RA, Duggan C, Hill S, Horslen S, Lim JD, Mercer DF, Merritt RJ, Nichol PF, Sigurdsson L, Teitelbaum DH, Thompson J, Vanderpool C, Vaughan JF, Li B, Youssef NN, Venick RS, Kocoshis SA. Outcomes from a 12-Week, Open-Label, Multicenter Clinical Trial of Teduglutide in Pediatric Short Bowel Syndrome. J Pediatr. 2017 Feb;181:102-111.e5. doi: 10.1016/j.jpeds.2016.10.027. Epub 2016 Nov 15. PMID: 27855998.
Kocoshis SA, Merritt RJ, Hill S, Protheroe S, Carter BA, Horslen S, Hu S, Kaufman SS, Mercer DF, Pakarinen MP, Venick RS, Wales PW, Grimm AA. Safety and Efficacy of Teduglutide in Pediatric Patients With Intestinal Failure due to Short Bowel Syndrome: A 24-Week, Phase III Study. JPEN J Parenter Enteral Nutr. 2020 May;44(4):621-631. doi: 10.1002/jpen.1690. Epub 2019 Sep 8. PMID: 31495952; PMCID: PMC7318247.