New Developments in the Management of Short Bowel Syndrome

US Gastroenterology & Hepatology Review, 2008;4(1):56-8

Short bowel syndrome (SBS) is a malabsorption syndrome resulting from extensive intestinal resection.1 Although the diagnosis of SBS relies less on an anatomical definition and more on a functional definition, for practical purposes in adults SBS can be defined as the presence of <200cm of remaining small intestine. In infants, necrotizing enterocolitis and congenital intestinal anomalies are frequently responsible. In older children and adults, multiple resections for Crohn’s disease and massive resections due to catastrophic mesenteric vascular events, radiation enteritis, adhesive obstruction, and trauma represent the more common causes of SBS.2 These patients frequently experience chronic diarrhea, dehydration, and macro- and micronutrient deficiencies often requiring enteral or parenteral nutrition support at home.

While SBS is uncommon, it remains an important clinical problem due to its effect on the quality and duration of life of these patients, the high rate of associated complications, and the subsequent high costs involved in their care.3 Survival studies from France and the US have demonstrated two-year and five-year survival rates for SBS at over 80 and 70%, respectively.4,5 Furthermore, the study from France reported parenteral nutrition (PN) dependency at two years of 49%, and 45% at five years.5 Survival rates were lowest in the end-jejunostomy and ultra-short small bowel groups. Other factors affecting survival include the patient’s age, primary disease process, comorbid diseases, presence of chronic intestinal obstruction, and the experience of the team managing the patient.6 Knowledge of the small bowel length can be useful for predicting the clinical outcome in SBS patients. The large range of small bowel length in humans (300–800cm) underscores the importance of being aware of the small bowel length remaining following a resection rather than the length of small bowel removed. When an operative report is unavailable or incomplete, a barium contrast small bowel series may provide an estimate of bowel length and is useful to delineate other structural features, such as the presence of bowel dilatation.7

The region of the remaining small intestine and the presence of the colon also affect the outcome of the SBS patient. In general, an SBS patient will have one of the following bowel anatomies: jejuno-colic anastomosis, end-jejunostomy, or jejuno-ileocolonic anastomosis. Patients with a jejuno-ileal anastomosis have the best prognosis; however, this anatomy is the least common. Patients with an end-jejunostomy are the most difficult to manage and are the most likely to require permanent parenteral support.6 A jejunal resection is generally better tolerated than an ileal resection because the ileum is capable of both structural and functional adaptation, while the jejunum mainly adapts functionally.6 Intestinal adaptation refers to a process following intestinal resection in which the remaining bowel undergoes a variety of macroscopic and microscopic changes in response to a number of stimuli in order to increase its ability to absorb fluid and nutrients.8,9 This stage may last for up to two years and it is during this time that most PN weaning occurs. The presence of the colon is beneficial in SBS patients given its ability to absorb water, electrolytes, and short-chain fatty acids (as an additional energy source), slow intestinal transit, and stimulate intestinal adaptation.10 It has been suggested that, in terms of need for PN, the presence of at least half of the colon is equivalent to about 50cm of small bowel.11

References:
1. Buchman AL, et al.,AGA technical review on short bowel syndrome and intestinal transplantation, Gastroenterology, 2003;124:1111–34. 2. Dabney A, Thompson J, DiBaise J, et al., Short bowel syndrome after trauma, Am J Surg, 2004;188:792–5. 3. Howard L, Ament M, Fleming CR, et al., Current use and clinical outcome of home parenteral and enteral nutrition therapies in the United States, Gastroenterology, 1995;109:355–65. 4. Scolapio JS, Fleming CR, Kelly DG, et al., Survival of home parenteral nutrition-treated patients: 20 years of experience at the Mayo Clinic, Mayo Clin Proc, 1999;74:217–22. 5. Messing B, Lemann M, Landais P, et al., Prognosis of patients with nonmalignant chronic intestinal failure receiving long-term home parenteral nutrition, Gastroenterology, 1995;108:1005–10. 6. Carbonnel F, Cosnes J, Chevret S, et al., The role of anatomic factors in nutritional autonomy after extensive small bowel resection, JPEN J Parenter Enteral Nutr, 1996;20:275–80. 7. Nightingale JM, et al., Length of residual small bowel after partial resection: correlation between radiographic and surgical measurements, Gastrointest Radiol, 1991;16:305–6. 8. Williamson RCN, Chir M, Intestinal adaptation: mechanisms of control, N Engl J Med, 1978;298:1444–50. 9. Williamson RCN, Chir M, Intestinal adaptation: structural, functional and cytokinetic changes, N Engl J Med, 1978;298:1393–1402. 10. Nordgaard I, Hansen BS, Mortensen PB, Importance of colonic support for energy absorption as small bowel failure proceeds, Am J Clin Nutr, 1996;64:222–31. 11. Nightingale JMD, Lennard-Jones JE, et al., Colonic preservation reduces the need for parenteral therapy, increases the incidence of renal stones but does not change the high prevalence of gallstones in patients with a short bowel, Gut, 1992;33:1493–7. 12. DiBaise JK, et al., Intestinal rehabilitation and the short bowel syndrome: part 2, Am J Gastroenterol, 2004;99:1823–32. 13. McIntyre PB, Fitchew M, et al., Patients with a high jejunostomy do not need a special diet, Gastroenterology, 1986;91:25–33. 14. Norgaard I, Hansen BS, Mortensen PB, Colon as a digestive organ in patients with short bowel, Lancet, 1994;343:373–6. 15. Woolf GM, Miller C, Kurian R, Jeejeebhoy KN, Nutritional absorption in short bowel syndrome. Evaluation of fluid, calorie, and divalent cation requirements, Dig Dis Sci, 1987;32:8–15. 16. Rodrigues Ca, Lennard-Jones JE, Thompson DG, Farthing MJG, What is the ideal sodium concentration of oral rehydration solutions for short bowel patients, Clin Sci, 1988;74:69. 17. Nightingale JM, Lennard-Jones JE, et al., Oral salt supplements to compensate for jejunostomy losses: comparison of sodium chloride capsules, glucose electrolyte solution, and glucose polymer electrolyte solution, Gut, 1992;33:759–61. 18. Marteau P, Messing B, Arrigoni E, et al., Do short bowel patients need a lactose-free diet?, Nutrition, 1997;13:13–16. 19. Byrne TA, Veglia L, Camelio M, et al., Beyond the prescription: optimizing the diet of patients with short bowel syndrome, Nutr Clin Pract, 2000;15:306–11. 20. Williams NS, Evans P, King RFGJ, Gastric acid secretion and gastrin production in the short bowel syndrome, Gut, 1985;26:914–19. 21. Cortot A, Fleming CR, Malagelada JR, Improved nutrient absorption after cimetidine in short-bowel syndrome with gastric hypersecretion, N Engl J Med, 1979;11;300:79–80. 22. Nehra V, Camilleri M, Burton D, et al., An open trial of octreotide long-acting release in the management of short bowel syndrome, Am J Gastroenterol, 2001;96:1494–8. 23. O’Keefe SJD, Haymond MW, Bennet WM, et al., Long-acting somatostatin analogue therapy and protein metabolism in patients with jejunostomies, Gastroenterology, 1994;107:379–88. 24. King RFGJ, Norton T, Hill GL, A double-blind crossover study of the effect of loperamide hydrochloride and codeine phosphate on ileostomy output, Aust N Z J Surg, 1982;52:121–4. 25. Rovera G, Furukawa H, Reyes J, et al., The use of clonidine for the treatment of high intestinal output following small bowel transplantation, Transplant Proc, 1997;29:1853–4. 26. Buchman AL,Wallin A, Fryer J, Polensky S, Clonidine reduces fecal weight and sodium losses modestly in some patients with proximal jejunostomy, Gastroenterology, 2005;128:A399 (abstract). 27. Little KH, et al., Treatment of severe steatorrhea with ox bile in an ileectomy patient with residual colon, Dig Dis Sci, 1992;37:929–33. 28. Heydorn S, et al., Bile acid replacement therapy with cholylsarcosine for short-bowel syndrome, Scand J Gastroenterol, 1999;34:818–23. 29. Hoffman AF, Poley R, Role of bile acid malabsorption in pathogenesis of diarrhea and steatorrhea in patients with ileal resection, Gastroenterology, 1972;62:918–34. 30. DiBaise JK, Young RJ, Vanderhoof JA, Enteric microbial flora, bacterial overgrowth and short bowel syndrome, Clin Gastroenterol Hepatol, 2006;4:11–20. 31. DiBaise JK, Matarese LE, Messing B, Steiger E, Strategies for weaning parenteral nutrition in adult patients with short bowel syndrome, J Clin Gastroenterol, 2006;40(Suppl.):94–8. 32. Booth CC, The effects of intestinal resection in man, Postgrad Med J, 1961;37:725–39. 33. Byrne TA, Persinger RL, Young LS, et al., A new treatment for patients with short-bowel syndrome: growth hormone, glutamine, and a modified diet, Ann Surg, 1995;222:243–54. 34. Wilmore DW, et al., Factors predicting a successful outcome after pharmacologic bowel compensation, Ann Surg, 1997;226:288–93. 35. Byrne TA, et al., Bowel rehabilitation: an alternative to long-term parenteral nutrition and intestinal transplantation for some patients with short bowel syndrome, Transplant Proc, 200234:887–90. 36. Zhu W, Li N, Ren J, et al., Rehabilitation therapy for short bowel syndrome, Chin Med J, 2002;115:776–8. 37. Byrne TA,Wilmore DW, et al., Growth hormone, glutamine and an optimal diet reduces parenteral nutrition in patients with short bowel syndrome. A prospective, randomized, placebo-controlled, double-blind clinical trial, Ann Surg, 2005;242:665–61. 38. Seguy D, Vahedi K, Kapel N, et al., Low-dose growth hormone in adult home parenteral nutrition-dependent short bowel syndrome patients: a positive study, Gastroenterology, 2003;124:293–302. 39. Scolapio JS, Camilleri M, et al., Effect of growth hormone, glutamine, and diet on adaptation in short bowel syndrome: a randomized, controlled study, Gastroenterology, 1997;113:1074–81. 40. Szkudlarek J, Jeppesen PB, Mortensen PB, Effect of high dose growth hormone with glutamine and no change in diet on intestinal absorption in short bowel patients: a randomized, double-blind, crossover, placebo-controlled study, Gut, 2000;47:199–205. 41. DiBaise JK, Somatropin in short bowel syndrome: opening the door to tropic factor use, Therapy, 2006;3:55–67. 42. Litvak DA, et al., Glucagon-like peptide 2 is a potent growth factor for small intestine and colon, J Gastrointest Surg, 1998;2:146–50. 43. Jeppesen PB, Hartmann B, et al., Glucagon-like peptide 2 improves nutrient absorption and nutritional status in short-bowel patients with no colon, Gastroenterology, 2001;120:806–15. 44. Jeppesen PB, et al., Teduglutide (ALX-0600), a dipeptidyl peptidase IV resistant glucagon-like peptide analogue, improves intestinal function in short bowel syndrome patients, Gut, 2005;54:1224–31. 45. Thompson JS, Strategies for preserving intestinal length in the short bowel syndrome, Dis Col Rectum, 1987;30:208–13. 46. Sudan D, DiBaise J, Torres C, et al., A multidisciplinary approach to the treatment of intestinal failure, J Gastrointest Surg, 2005;9: 165–76; discussion 176–7. 47. Fishbein TM, Gondolesi GE, Kaufman SS, Intestinal transplantation for gut failure, Gastroenterology, 2003;124:1615–28. 48. Grant D, et al., 2003 report of the intestine transplant registry: a new era has dawned, Ann Surg, 2005;241:607–13. 49. Rovera GM, et al., Intestinal and multivisceral transplantation: dynamics of nutritional management and functional autonomy, JPEN J Parenter Enteral Nutr, 2003;27:252–9.