Short bowel syndrome
- Overview
- Theory
- Diagnosis
- Management
- Follow up
- Resources
Treatment algorithm
Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer
jejunoileocolic anastomosis
oral diet and re-hydration
When thirsty, oral re-hydration solution (ORS) should be encouraged in all patients instead of water, juice, or sports drinks.[16]Buchman AL. Short bowel syndrome. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's gastrointestinal and liver disease. 11th ed. Philadelphia, PA: Saunders; 2020.[38]Pfeiffer A, Schmidt T, Kaess H. The role of osmolality in the absorption of a nutrient solution. Aliment Pharmacol Ther. 1998 Mar;12(3):281-6. http://onlinelibrary.wiley.com/doi/10.1046/j.1365-2036.1998.00301.x/full http://www.ncbi.nlm.nih.gov/pubmed/9570264?tool=bestpractice.com [39]Buchman AL. Etiology and initial management of short bowel syndrome. Gastroenterology. 2006 Feb;130(2 suppl 1):S5-S15. http://www.ncbi.nlm.nih.gov/pubmed/16473072?tool=bestpractice.com
Patients must be encouraged to eat 2 to 3 times more calories than they did prior to acquiring SBS.
Patients should be encouraged to eat a diet rich in complex carbohydrates, starch, and soluble fibre, and low in oxalate.[4]Massironi S, Cavalcoli F, Rausa E, et al. Understanding short bowel syndrome: current status and future perspectives. Dig Liver Dis. 2020 Mar;52(3):253-61. https://www.doi.org/10.1016/j.dld.2019.11.013 http://www.ncbi.nlm.nih.gov/pubmed/31892505?tool=bestpractice.com [28]Nordgaard I, Hansen BS, Mortensen PB. Colon as a digestive organ in patients with short bowel. Lancet. 1994 Feb 12;343(8894):373-6. http://www.ncbi.nlm.nih.gov/pubmed/7905549?tool=bestpractice.com Foods containing lactose are a rich source of calcium and should be encouraged unless the patient is known to be lactose intolerant, or a massive jejunal resection has been performed.[40]Marteau P, Messing B, Arrigoni E, et al. Do patients with short-bowel syndrome need a lactose-free diet? Nutrition. 1997 Jan;13(1):13-6. http://www.ncbi.nlm.nih.gov/pubmed/9058441?tool=bestpractice.com
Patients with bile salt malabsorption may benefit from a diet rich in medium-chain triglycerides (MCT) or may use MCT supplements.
anti-diarrhoeal treatment
Treatment recommended for ALL patients in selected patient group
Anti-diarrhoeal medicine is required to control fluid losses.[4]Massironi S, Cavalcoli F, Rausa E, et al. Understanding short bowel syndrome: current status and future perspectives. Dig Liver Dis. 2020 Mar;52(3):253-61. https://www.doi.org/10.1016/j.dld.2019.11.013 http://www.ncbi.nlm.nih.gov/pubmed/31892505?tool=bestpractice.com
Options include loperamide, diphenoxylate/atropine, codeine, tincture of opium, octreotide, or clonidine.
Clonidine produces side effects of hypotension and central effects such as drowsiness.
Octreotide slows intestinal transit and inhibits intestinal adaptation, and must be administered subcutaneously or intravenously. It should therefore be used only if other agents fail to control diarrhoea because of potential adverse effects on intestinal adaptation and gallbladder motility.
Primary options
loperamide: 2 mg orally four times daily initially, increase according to response, maximum 16 mg/day
OR
diphenoxylate/atropine: 2.5 mg orally four times daily initially, increase according to response, maximum 20 mg/day
More diphenoxylate/atropineDose refers to diphenoxylate component.
Secondary options
codeine phosphate: 15-60 mg orally every 6-8 hours when required, maximum 240 mg/day
OR
opium tincture: 15 drops orally four times daily, increase gradually according to response, maximum 20 mL/day
Tertiary options
clonidine transdermal: 0.1 mg/24 hour patch once weekly
or
clonidine: 0.1 to 0.6 mg orally twice daily
OR
octreotide: 100-1500 micrograms/day subcutaneously given in 2-4 divided doses
calcium supplementation
Treatment recommended for ALL patients in selected patient group
Indicated in all patients to maintain normal calcium status and protect against osteoporosis.
Primary options
calcium carbonate: 1-2 g/day orally given in 3-4 divided doses
parenteral nutrition (PN) support with subsequent weaning
Additional treatment recommended for SOME patients in selected patient group
Patients require PN support if adequate nutrition is not achieved with oral diet alone. This is more common in patients with ≤100 cm residual bowel (or ≤150 cm if colon partially resected). Patients with a jejunoileocolic anastomosis have the greatest likelihood of weaning from PN.[4]Massironi S, Cavalcoli F, Rausa E, et al. Understanding short bowel syndrome: current status and future perspectives. Dig Liver Dis. 2020 Mar;52(3):253-61. https://www.doi.org/10.1016/j.dld.2019.11.013 http://www.ncbi.nlm.nih.gov/pubmed/31892505?tool=bestpractice.com
PN is initially administered over a 24-hour period and the feeding time is gradually condensed to a 10- to 12-hour night-time feeding schedule (usually by 2-hour increments whereby the infusion rate is increased and infusion duration is decreased).
Catheter safety instructions are critical for the prevention of complications such as catheter sepsis and venous thrombosis.
Weaning is achieved by gradually increasing oral intake in small, frequent feedings as tolerated. The aim is to consume 2 to 3 times the normal oral caloric intake. This is accompanied by: a corresponding adjustment to the frequency and content of PN; monitoring of fluid and nutrition status; diarrhoea control; and use of oral rehydration solutions.
proton-pump inhibitor
Additional treatment recommended for SOME patients in selected patient group
Required for 6 months after the initial surgery to control gastric hyper-secretion.[2]Cuerda C, Pironi L, Arends J, et al. ESPEN practical guideline: clinical nutrition in chronic intestinal failure. Clin Nutr. 2021 Sep;40(9):5196-220. https://www.doi.org/10.1016/j.clnu.2021.07.002 http://www.ncbi.nlm.nih.gov/pubmed/34479179?tool=bestpractice.com [4]Massironi S, Cavalcoli F, Rausa E, et al. Understanding short bowel syndrome: current status and future perspectives. Dig Liver Dis. 2020 Mar;52(3):253-61. https://www.doi.org/10.1016/j.dld.2019.11.013 http://www.ncbi.nlm.nih.gov/pubmed/31892505?tool=bestpractice.com [43]Windsor CW, Fejfar J, Woodward DA. Gastric secretion after massive small bowel resection. Gut. 1969 Oct;10(10):779-86. http://www.ncbi.nlm.nih.gov/pubmed/4186939?tool=bestpractice.com [44]Williams NS, Evans P, King RF. Gastric acid secretion and gastrin production in the short bowel syndrome. Gut. 1985 Sep;26(9):914-9. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1432854 http://www.ncbi.nlm.nih.gov/pubmed/4029719?tool=bestpractice.com [45]Jeppesen PB, Staun M, Tjellesen L, et al. Effect of intravenous ranitidine and omeprazole on intestinal absorption of water, sodium, and macronutrients in patients with intestinal resection. Gut. 1998 Dec;43(6):763-9. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1727363 http://www.ncbi.nlm.nih.gov/pubmed/9824602?tool=bestpractice.com [46]Jacobsen O, Ladefoged K, Stage JG, et al. Effects of cimetidine on jejunostomy effluents in patients with severe short-bowel syndrome. Scand J Gastroenterol. 1986 Sep;21(7):824-8. http://www.ncbi.nlm.nih.gov/pubmed/3095911?tool=bestpractice.com [47]Nightingale JM, Walker ER, Farthing MJ, et al. Effect of omeprazole on intestinal output in the short bowel syndrome. Aliment Pharmacol Ther. 1991 Aug;5(4):405-12. http://www.ncbi.nlm.nih.gov/pubmed/1777549?tool=bestpractice.com
Less-extensive surgery, such as small segmental resections for Crohn's disease, may not cause gastric hyper-secretion, and therapy may not be not required for these cases.
Can induce diarrhoea in some patients, therefore monitoring the volume of diarrhoea before and after the commencement of treatment may be useful.
Primary options
omeprazole: 40 mg orally twice daily
OR
lansoprazole: 15-30 mg orally once or twice daily
electrolyte, vitamin, and micro-nutrient replacement
Additional treatment recommended for SOME patients in selected patient group
Patients are tested regularly for electrolyte and vitamin deficiencies to guide supplementation, including vitamins A, pyridoxine (B6), B12, D, E, and K, and copper, zinc, and selenium.
Deficiencies of fat-soluble vitamins (A, D, E, K) are the most common vitamin deficiencies encountered; absorption of water-soluble vitamins is preserved.
Potassium, magnesium, bicarbonate, and phosphorus supplementation should also be provided as needed.
Caution must be used with oral magnesium supplements as they can induce diarrhoea and worsen absorption. Magnesium gluconate is preferred as it may result in less diarrhoea than other formulations.
Patients with greater than 60 cm terminal ileum resection usually develop vitamin B12 deficiency.
jejunoileal anastomosis with fully resected colon
parenteral nutrition (PN) with oral re-hydration
PN is initially administered over a 24-hour period and the feeding time is gradually condensed to a 10- to 12-hour night-time feeding schedule (usually by 2-hour increments whereby the infusion rate is increased and infusion duration is decreased) to enable the patient to be pump free during the day.
Instruction in proper catheter care is essential to avoid complications such as catheter sepsis and venous thrombosis.
When thirsty, oral re-hydration solution should be encouraged in all patients instead of water, juice, or sports drinks.[16]Buchman AL. Short bowel syndrome. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's gastrointestinal and liver disease. 11th ed. Philadelphia, PA: Saunders; 2020.[38]Pfeiffer A, Schmidt T, Kaess H. The role of osmolality in the absorption of a nutrient solution. Aliment Pharmacol Ther. 1998 Mar;12(3):281-6. http://onlinelibrary.wiley.com/doi/10.1046/j.1365-2036.1998.00301.x/full http://www.ncbi.nlm.nih.gov/pubmed/9570264?tool=bestpractice.com [39]Buchman AL. Etiology and initial management of short bowel syndrome. Gastroenterology. 2006 Feb;130(2 suppl 1):S5-S15. http://www.ncbi.nlm.nih.gov/pubmed/16473072?tool=bestpractice.com
anti-diarrhoeal treatment
Treatment recommended for ALL patients in selected patient group
Anti-diarrhoeal medicine is required to control fluid losses.[4]Massironi S, Cavalcoli F, Rausa E, et al. Understanding short bowel syndrome: current status and future perspectives. Dig Liver Dis. 2020 Mar;52(3):253-61. https://www.doi.org/10.1016/j.dld.2019.11.013 http://www.ncbi.nlm.nih.gov/pubmed/31892505?tool=bestpractice.com
Options include loperamide, diphenoxylate/atropine, codeine, tincture of opium, or octreotide.
Clonidine produces side effects of hypotension and central effects such as drowsiness.
Octreotide slows intestinal transit and inhibits intestinal adaptation, and must be administered subcutaneously or intravenously. It should therefore be used only if other agents fail to control diarrhoea because of potential adverse effects on intestinal adaptation and gallbladder motility.
Primary options
loperamide: 2 mg orally four times daily initially, increase according to response, maximum 16 mg/day
OR
diphenoxylate/atropine: 2.5 mg orally four times daily initially, increase according to response, maximum 20 mg/day
More diphenoxylate/atropineDose refers to diphenoxylate component.
Secondary options
codeine phosphate: 15-60 mg orally every 6-8 hours when required, maximum 240 mg/day
OR
opium tincture: 15 drops orally four times daily, increase gradually according to response, maximum 20 mL/day
Tertiary options
octreotide: 100-1500 micrograms/day administered subcutaneously in 2-4 divided doses
calcium supplementation
Treatment recommended for ALL patients in selected patient group
Indicated in all patients to maintain normal calcium status and protect against osteoporosis.
Primary options
calcium carbonate: 1-2 g/day orally given in 3-4 divided doses
proton-pump inhibitors
Additional treatment recommended for SOME patients in selected patient group
Required for 6 months after the initial surgery to control gastric hypersecretion.[2]Cuerda C, Pironi L, Arends J, et al. ESPEN practical guideline: clinical nutrition in chronic intestinal failure. Clin Nutr. 2021 Sep;40(9):5196-220. https://www.doi.org/10.1016/j.clnu.2021.07.002 http://www.ncbi.nlm.nih.gov/pubmed/34479179?tool=bestpractice.com [4]Massironi S, Cavalcoli F, Rausa E, et al. Understanding short bowel syndrome: current status and future perspectives. Dig Liver Dis. 2020 Mar;52(3):253-61. https://www.doi.org/10.1016/j.dld.2019.11.013 http://www.ncbi.nlm.nih.gov/pubmed/31892505?tool=bestpractice.com [43]Windsor CW, Fejfar J, Woodward DA. Gastric secretion after massive small bowel resection. Gut. 1969 Oct;10(10):779-86. http://www.ncbi.nlm.nih.gov/pubmed/4186939?tool=bestpractice.com [44]Williams NS, Evans P, King RF. Gastric acid secretion and gastrin production in the short bowel syndrome. Gut. 1985 Sep;26(9):914-9. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1432854 http://www.ncbi.nlm.nih.gov/pubmed/4029719?tool=bestpractice.com [45]Jeppesen PB, Staun M, Tjellesen L, et al. Effect of intravenous ranitidine and omeprazole on intestinal absorption of water, sodium, and macronutrients in patients with intestinal resection. Gut. 1998 Dec;43(6):763-9. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1727363 http://www.ncbi.nlm.nih.gov/pubmed/9824602?tool=bestpractice.com [46]Jacobsen O, Ladefoged K, Stage JG, et al. Effects of cimetidine on jejunostomy effluents in patients with severe short-bowel syndrome. Scand J Gastroenterol. 1986 Sep;21(7):824-8. http://www.ncbi.nlm.nih.gov/pubmed/3095911?tool=bestpractice.com [47]Nightingale JM, Walker ER, Farthing MJ, et al. Effect of omeprazole on intestinal output in the short bowel syndrome. Aliment Pharmacol Ther. 1991 Aug;5(4):405-12. http://www.ncbi.nlm.nih.gov/pubmed/1777549?tool=bestpractice.com
Less-extensive surgery, such as small segmental resections for Crohn's disease, may not cause gastric hyper-secretion, and therapy is not required for these cases.
Can induce diarrhoea in some patients, therefore monitoring the volume of diarrhoea before and after the commencement of treatment may be useful.
Primary options
omeprazole: 40 mg orally twice daily
OR
lansoprazole: 15-30 mg orally once or twice daily
electrolyte, vitamin, and micro-nutrient replacement
Additional treatment recommended for SOME patients in selected patient group
Patients are tested regularly for electrolyte and vitamin deficiencies to guide supplementation, including vitamins A, pyridoxine (B6), B12, D, E, and K, and copper, zinc, and selenium.
Deficiencies of fat-soluble vitamins (A, D, E, K) are the most common vitamin deficiencies encountered in this patient group; absorption of water-soluble vitamins is preserved. The risk of vitamin K deficiency is further increased because the colonic bacterial flora, which can synthesise vitamin K, have been lost.
Potassium, magnesium, bicarbonate, and phosphorus supplementation should also be provided as needed.
Caution must be used with oral magnesium supplements as they can induce diarrhoea and worsen absorption. Magnesium gluconate is preferred as it may result in less diarrhoea than other formulations.
Patients with greater than 60 cm of terminal ileum resection usually develop vitamin B12 deficiency.
oral diet with weaning of parenteral nutrition (PN)
Additional treatment recommended for SOME patients in selected patient group
Weaning from PN may be possible in patients with >100 cm of residual small bowel, and is achieved by gradually increasing oral intake in small, frequent feedings as tolerated. The aim is to consume 2 to 3 times the normal, pre-resection oral caloric intake. This is accompanied by: a corresponding adjustment to the frequency and content of PN; monitoring of fluid and nutrition status; diarrhoea control; and use of oral rehydration solutions.
end jejunostomy or duodenostomy
re-anastomosis of colon
Primary re-anastomosis of small bowel with colon must be performed in all patients to enable the intestine to take advantage of the colon's role in fluid and nutrient absorption. This greatly improves the patient's chances of weaning from parenteral nutrition (PN).
Patients will likely be dependent on PN if re-anastomosis of the colon is not possible.
parenteral nutrition (PN) support and oral re-hydration
Treatment recommended for ALL patients in selected patient group
Patients require total PN support for the first 7 to 10 days after surgery, after which partial PN support in combination with oral diet should be commenced. Patients with >100 cm residual small bowel (or >150 cm if the colon is partially resected) can be transitioned to oral diet relatively quickly, while those with ≤100 cm residual small bowel (or ≤150 cm if the colon is partially resected) may require PN for longer. Dietary intake may need to be decreased to control faecal losses.
Instruction in proper catheter care is essential to avoid complications such as catheter sepsis and venous thrombosis.
When thirsty, oral re-hydration solution (ORS) should be encouraged in all patients instead of water, juice, or sports drinks.[16]Buchman AL. Short bowel syndrome. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's gastrointestinal and liver disease. 11th ed. Philadelphia, PA: Saunders; 2020.[38]Pfeiffer A, Schmidt T, Kaess H. The role of osmolality in the absorption of a nutrient solution. Aliment Pharmacol Ther. 1998 Mar;12(3):281-6. http://onlinelibrary.wiley.com/doi/10.1046/j.1365-2036.1998.00301.x/full http://www.ncbi.nlm.nih.gov/pubmed/9570264?tool=bestpractice.com [39]Buchman AL. Etiology and initial management of short bowel syndrome. Gastroenterology. 2006 Feb;130(2 suppl 1):S5-S15. http://www.ncbi.nlm.nih.gov/pubmed/16473072?tool=bestpractice.com
anti-diarrhoeal treatment
Treatment recommended for ALL patients in selected patient group
Anti-diarrhoeal medicine is required to control fluid losses.[4]Massironi S, Cavalcoli F, Rausa E, et al. Understanding short bowel syndrome: current status and future perspectives. Dig Liver Dis. 2020 Mar;52(3):253-61. https://www.doi.org/10.1016/j.dld.2019.11.013 http://www.ncbi.nlm.nih.gov/pubmed/31892505?tool=bestpractice.com
Options include loperamide, diphenoxylate/atropine, codeine, tincture of opium, or octreotide.
Octreotide slows intestinal transit and inhibits intestinal adaptation, and must be administered subcutaneously or intravenously. It should therefore be used only if other agents fail to control diarrhoea because of potential adverse effects on intestinal adaptation and gallbladder motility.
Primary options
loperamide: 4 mg orally four times daily
OR
diphenoxylate/atropine: 5 mg orally four times daily
More diphenoxylate/atropineDose refers to diphenoxylate component.
Secondary options
codeine phosphate: 15-60 mg orally every 6-8 hours when required, maximum 240 mg/day
OR
opium tincture: 15 drops orally four times daily; increase gradually according to response, maximum 20 mL/day
Tertiary options
octreotide: 100-1500 micrograms/day subcutaneously given in 2-4 divided doses
calcium supplementation
Treatment recommended for ALL patients in selected patient group
Indicated in all patients to maintain normal calcium status and protect against osteoporosis.
Primary options
calcium carbonate: 1-2 g/day orally given in 3-4 divided doses
proton-pump inhibitors
Additional treatment recommended for SOME patients in selected patient group
Required for 6 months after the initial surgery to control gastric hyper-secretion.[2]Cuerda C, Pironi L, Arends J, et al. ESPEN practical guideline: clinical nutrition in chronic intestinal failure. Clin Nutr. 2021 Sep;40(9):5196-220. https://www.doi.org/10.1016/j.clnu.2021.07.002 http://www.ncbi.nlm.nih.gov/pubmed/34479179?tool=bestpractice.com [4]Massironi S, Cavalcoli F, Rausa E, et al. Understanding short bowel syndrome: current status and future perspectives. Dig Liver Dis. 2020 Mar;52(3):253-61. https://www.doi.org/10.1016/j.dld.2019.11.013 http://www.ncbi.nlm.nih.gov/pubmed/31892505?tool=bestpractice.com [43]Windsor CW, Fejfar J, Woodward DA. Gastric secretion after massive small bowel resection. Gut. 1969 Oct;10(10):779-86. http://www.ncbi.nlm.nih.gov/pubmed/4186939?tool=bestpractice.com [44]Williams NS, Evans P, King RF. Gastric acid secretion and gastrin production in the short bowel syndrome. Gut. 1985 Sep;26(9):914-9. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1432854 http://www.ncbi.nlm.nih.gov/pubmed/4029719?tool=bestpractice.com [45]Jeppesen PB, Staun M, Tjellesen L, et al. Effect of intravenous ranitidine and omeprazole on intestinal absorption of water, sodium, and macronutrients in patients with intestinal resection. Gut. 1998 Dec;43(6):763-9. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1727363 http://www.ncbi.nlm.nih.gov/pubmed/9824602?tool=bestpractice.com [46]Jacobsen O, Ladefoged K, Stage JG, et al. Effects of cimetidine on jejunostomy effluents in patients with severe short-bowel syndrome. Scand J Gastroenterol. 1986 Sep;21(7):824-8. http://www.ncbi.nlm.nih.gov/pubmed/3095911?tool=bestpractice.com [47]Nightingale JM, Walker ER, Farthing MJ, et al. Effect of omeprazole on intestinal output in the short bowel syndrome. Aliment Pharmacol Ther. 1991 Aug;5(4):405-12. http://www.ncbi.nlm.nih.gov/pubmed/1777549?tool=bestpractice.com
Can induce diarrhoea in some patients, therefore monitoring the volume of diarrhoea before and after the commencement of treatment may be useful.
Primary options
omeprazole: 40 mg orally twice daily
OR
lansoprazole: 15-30 mg orally once or twice daily
electrolyte, vitamin, and micro-nutrient replacement
Additional treatment recommended for SOME patients in selected patient group
Patients are tested regularly for electrolyte and vitamin deficiencies to guide supplementation, including vitamins A, thiamine (B1), pyridoxine (B6), folate (B9), B12, C, D, E, and K, and copper, zinc, and selenium.
This patient group can develop deficiencies of both fat- and water-soluble vitamins.
Iron, potassium, magnesium, bicarbonate, and phosphorus supplementation should also be provided as needed.
Caution must be used with oral magnesium supplements as they can induce diarrhoea and worsen absorption. Magnesium gluconate is preferred as it may result in less diarrhoea than other formulations.
Patients with >60 cm terminal ileum resection usually develop vitamin B12 deficiency.
all patients
electrolyte, vitamin, and micro-nutrient replacement
Patients are tested regularly for electrolyte and vitamin deficiencies to guide supplementation, including vitamins A, thiamine (B1), pyridoxine (B6), folate (B9), B12, C, D, E, and K, and copper, zinc, and selenium.
Calcium supplementation is often indicated in all patients to protect against osteoporosis.
Primary options
calcium carbonate: 1-2 g/day orally given in 3-4 divided doses
long-term PN
Treatment recommended for ALL patients in selected patient group
Patients who cannot be weaned will require long-term PN. They should be carefully monitored for complications such as liver failure.
Patients with a jejunoileocolic anastomosis have the greatest likelihood of weaning from PN. Patients with a jejunoileal anastomosis with a fully resected colon with ≤100 cm of residual bowel will almost certainly be PN-dependent, as will be patients in whom a primary re-anastomosis with the colon is not performed. Instruction in proper catheter care is essential to avoid complications such as catheter sepsis and venous thrombosis.
teduglutide
Additional treatment recommended for SOME patients in selected patient group
A human glucagon-like peptide-2 (GLP-2) analogue that has been shown to improve intestinal absorption and reduce parenteral nutrition (PN) requirement in PN-dependent patients with SBS.[29]Jeppesen PB, Gilroy R, Pertkiewicz M, et al. Randomised placebo-controlled trial of teduglutide in reducing parenteral nutrition and/or intravenous fluid requirements in patients with short bowel syndrome. Gut. 2011 Jul;60(7):902-14. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3112364 http://www.ncbi.nlm.nih.gov/pubmed/21317170?tool=bestpractice.com [30]Jeppesen PB, Pertkiewicz M, Messing B, et al. Teduglutide reduces need for parenteral support among patients with short bowel syndrome with intestinal failure. Gastroenterology. 2012;143:1473-1481;e3. http://www.gastrojournal.org/article/S0016-5085(12)01316-9/fulltext http://www.ncbi.nlm.nih.gov/pubmed/22982184?tool=bestpractice.com
Weaning may be attempted after 4 weeks of treatment with teduglutide, and lifelong continuous treatment is required if weaning is successful. However, there is uncertainty regarding optimal maintenance dosing.
There is a risk of acceleration of neoplastic growth with teduglutide. Small bowel neoplasia has been reported in animal studies and there is a theoretical risk of colorectal cancer in humans.[31]Orhan A, Gögenur I, Kissow H. The intestinotrophic effects of glucagon-like peptide-2 in relation to intestinal neoplasia. J Clin Endocrinol Metab. 2018 Aug 1;103(8):2827-2837. https://academic.oup.com/jcem/article/103/8/2827/4992695 http://www.ncbi.nlm.nih.gov/pubmed/29741675?tool=bestpractice.com Therefore, patients with an intact colon require colonoscopy before starting treatment, and then at regular intervals during treatment (no less frequently than every 5 years). Treatment should be discontinued in patients with gastrointestinal malignancy, and caution should be used in patients with non-gastrointestinal malignancy.
Primary options
teduglutide (recombinant): 0.05 mg/kg subcutaneously once daily to alternating quadrants of the abdomen, the thighs, or the arms
bowel lengthening/tapering surgery
Additional treatment recommended for SOME patients in selected patient group
Bowel lengthening/tapering surgery using the Bianchi procedure or the serial transverse enteroplasty (STEP) procedure can be performed to aid intestinal adaptation and weaning in parenteral nutrition (PN)-dependent patients with dilated bowel segments.
Inclusion criteria include: an intestinal diameter >3 cm; length of residual small bowel >40 cm with ≥20 cm of dilated small bowel; PN dependence; and an absence of liver disease, Crohn's disease, and radiation enteritis.
The Bianchi procedure takes an immotile segment of small bowel, transects the segment, and re-connects the halves end to end, thereby doubling the length of bowel and helping motility.
The STEP procedure involves applying linear staples along the small bowel in alternating and opposite directions in order to incompletely divide the dilated intestine. It has the same effect as the Bianchi procedure but is technically easier to perform, and has the advantage of avoiding postoperative stricture formation.[37]Kim HB, Fauza D, Garza J, et al. Serial transverse enteroplasty (STEP): a novel bowel lengthening procedure. J Pediatr Surg. 2003 Mar;38(3):425-9. http://www.ncbi.nlm.nih.gov/pubmed/12632361?tool=bestpractice.com
No randomised controlled trials have compared the Bianchi and STEP procedures; therefore, it is not clear which procedure is superior.
Patients with SBS and dilated bowel segments may have bacterial overgrowth in the bowel. If persistent, this should be treated before performing bowel lengthening/tapering surgery.
adjust PN composition
Treatment recommended for ALL patients in selected patient group
Patients can be considered to be failing PN therapy if they have 1 or more of the following: impending or overt liver failure, at least 2 thromboses of major central venous channels, frequent central line-related sepsis, or frequent severe volume depletion.[15]Buchman AL, Scolapio J, Fryer J. AGA technical review on short bowel syndrome and intestinal transplantation. Gastroenterology. 2003 Apr;124(4):1111-34. http://www.gastrojournal.org/article/PIIS001650850370064X/fulltext http://www.ncbi.nlm.nih.gov/pubmed/12671904?tool=bestpractice.com
PN composition should be adjusted in patients developing intestinal failure-associated liver disease (IFALD). Dextrose over-feeding (>40 kcal/kg/day) should be avoided and intravenous lipid intake reduced to below 1.0 g/kg/day if possible.[2]Cuerda C, Pironi L, Arends J, et al. ESPEN practical guideline: clinical nutrition in chronic intestinal failure. Clin Nutr. 2021 Sep;40(9):5196-220. https://www.doi.org/10.1016/j.clnu.2021.07.002 http://www.ncbi.nlm.nih.gov/pubmed/34479179?tool=bestpractice.com However, the intravenous soybean oil-based lipid emulsion should still supply at least 4% to 8% of total energy. This usually requires a reduction in overall caloric intake, as over-feeding needs to be avoided. Alternative lipid emulsions, containing fish oil or a combination of fish and olive oils, may be used as an energy source in patients with IFALD.[32]Chang MI, Puder M, Gura KM. The use of fish oil lipid emulsion in the treatment of intestinal failure associated liver disease (IFALD). Nutrients. 2012 Nov 27;4(12):1828-50. https://www.doi.org/10.3390/nu4121828 http://www.ncbi.nlm.nih.gov/pubmed/23363993?tool=bestpractice.com [33]Gura KM, Lee S, Valim C, et al. Safety and efficacy of a fish-oil-based fat emulsion in the treatment of parenteral nutrition-associated liver disease. Pediatrics. 2008 Mar;121(3):e678-86. https://pediatrics.aappublications.org/content/121/3/e678.long http://www.ncbi.nlm.nih.gov/pubmed/18310188?tool=bestpractice.com
intestinal transplantation ± liver transplantation
Additional treatment recommended for SOME patients in selected patient group
Intestinal transplantation can be considered if there is no clinical improvement following parenteral nutrition (PN) adjustment, or PN failure has occurred as a result of recurrent thrombosis of central venous channels or frequent severe volume depletion.
Intestinal transplantation is associated with a risk of morbidity and mortality, and should therefore be regarded as a rescue therapy rather than a therapeutic option.
Patients with end-stage liver failure secondary to long-term intestinal failure will require combined liver and intestinal transplantation.
Choose a patient group to see our recommendations
Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer
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