eMedicine Specialties > General Surgery > Abdomen

Short-Bowel Syndrome: Follow-up

Author: Burt Cagir, MD, FACS, Assistant Professor of Surgery, State University of New York, Upstate Medical Center; Consulting Staff, Director of Surgical Research, Robert Packer Hospital; Associate Program Director, Department of Surgery, Guthrie Clinic
Coauthor(s): Michael AJ Sawyer, MD, Consulting Staff, Department of Surgery, Southwestern Medical Center; Consulting Staff, Department of Surgery, Comanche County Memorial Hospital; Consulting Staff, Great Plains Surgical Clinic, Inc
Contributor Information and Disclosures

Updated: Sep 8, 2009

Outcome and Prognosis

No reliable cure currently exists for short-bowel syndrome. Patients who are maintained on parenteral nutrition at home have reasonably good short-term outcomes. Data from Howard and colleagues and Ladefoged and coworkers revealed that the 4-year survival rate in patients who depend on parenteral nutrition is about 70%.7,45  Eventually, many of these patients run out of venous access or have severe septic complications. Cost also is a major factor. Home parenteral nutrition costs range from about $50,000 to more than $200,000 per year. As mentioned before, the most common cause of death in these patients is liver failure.

The authors have reviewed the use and results of pharmacologic bowel compensation, including growth hormone, glutamine, and a high-carbohydrate diet. This may allow additional patients to be liberated from parenteral nutrition. The clinical results have been favorable as described in the studies by Wilmore, Byrne, and colleagues, but these results have not been reproduced at numerous medical centers.17

Nontransplant surgical procedures have been applied to short-bowel syndrome. Early results were mixed, but many of the procedures being performed then involved segment reversal. The most recent series described herein have demonstrated clinical improvement in greater than 80% of patients. The most common operations performed in these series were intestinal tapering, intestinal lengthening, and strictureplasty. Even in these series, segment reversal and creation of artificial valves produced dubious results.

Organ transplantation is a promising therapeutic option but continues to be fraught with problems. Early postoperative mortality rates can be as high as 30%. Data from leading transplant centers have shown that the 1-year survival rates can be as high as 80-90%, and approximately 60% of patients are alive at 4 years.

Future and Controversies

Future developments in the therapy of short-bowel syndrome will consist of finding ways to maximize bowel adaptation and of refining techniques of transplantation and immune modulation.

Pharmacologic bowel compensation is a novel idea and has had some good results to date. Better understanding of small bowel trophic signals and the interaction among foodstuffs, enteric hormones, and the intestinal mucosa might lead to improved bowel adaptation.

Transplantation techniques are improving, and factors that negatively impact the success of these operations have been described and discussed. The introduction of tacrolimus was associated with increasing 1-year graft survival rates from 19-57%. Problems still exist. Rates of sepsis and immunosuppression-related malignancies are still high.

The search for the transplantation "holy grail" of donor-specific immunologic tolerance continues. Animal studies by Gorczynski and others have suggested that introduction of donor antigens via the portal vein before implantation might help induce a state of tolerance.46 So far, this has not been demonstrated in humans.

The identification of the gamma-delta receptor–positive T (GDT) cell was an exciting discovery. GDT cells are a source of type 2 cytokines, including interleukin (IL)-4, IL-10, and transforming growth factor (TGF) beta. These type 2 cytokines are associated with enhanced graft survival, making the GDT cell an intense research topic.

 


More on Short-Bowel Syndrome

Overview: Short-Bowel Syndrome
Workup: Short-Bowel Syndrome
Treatment: Short-Bowel Syndrome
Follow-up: Short-Bowel Syndrome
References
Further Reading
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References

  1. Weser E. Nutritional aspects of malabsorption: short gut adaptation. Clin Gastroenterol. May 1983;12(2):443-61. [Medline].

  2. Wilmore DW, Dudrick SJ, Daly JM, et al. The role of nutrition in the adaptation of the small intestine after massive resection. Surg Gynecol Obstet. Apr 1971;132(4):673-80. [Medline].

  3. Wilmore DW, Dudrick SJ. Growth and development of an infant receiving all nutrients exclusively by vein. JAMA. Mar 4 1968;203(10):860-4. [Medline].

  4. Lennard-Jones JE. Indications and need for long-term parenteral nutrition: implications for intestinal transplantation. Transplant Proc. Dec 1990;22(6):2427-9. [Medline].

  5. 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. Sep 1995;222(3):243-54; discussion 254-5. [Medline].

  6. Moreno JM, Planas M, Lecha M, et al. [The year 2002 national register on home-based parenteral nutrition]. Nutr Hosp. Jul-Aug 2005;20(4):249-53. [Medline].

  7. Ladefoged K, Hessov I, Jarnum S. Nutrition in short-bowel syndrome. Scand J Gastroenterol Suppl. 1996;216:122-31. [Medline].

  8. Nightingale JM, Lennard-Jones JE. The short bowel syndrome: what's new and old?. Dig Dis. 1993;11(1):12-31. [Medline].

  9. Stollman TH, de Blaauw I, Wijnen MH, et al. Decreased mortality but increased morbidity in neonates with jejunoileal atresia; a study of 114 cases over a 34-year period. J Pediatr Surg. Jan 2009;44(1):217-21. [Medline].

  10. Sellin JH. Intestinal electrolyte absorption and secretion. In: Feldman M, Scharschmidt BF, Sleisinger MH. Sleisinger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management. 6th ed. Philadelphia, Pa: WB Saunders Co; 1998:1451-1471.

  11. Clarke RM. Mucosal architecture and epithelial cell production rate in the small intestine of the albino rat. J Anat. Nov 1970;107:519-29. [Medline].

  12. Borgstrom B, Dahlquist A, Lundh G, et al. Studies of intestinal digestion and absorption in the human. J Clin Invest. 1957;36:1521-1536.

  13. Johansson C. Studies of gastrointestinal interactions. VII. Characteristics of the absorption pattern of sugar, fat and protein from composite meals in man. A quantitative study. Scand J Gastroenterol. 1975;10(1):33-42. [Medline].

  14. Phillips SF, Giller J. The contribution of the colon to electrolyte and water conservation in man. J Lab Clin Med. May 1973;81(5):733-46. [Medline].

  15. Pomare EW, Branch WJ, Cummings JH. Carbohydrate fermentation in the human colon and its relation to acetate concentrations in venous blood. J Clin Invest. May 1985;75(5):1448-54. [Medline].

  16. Sundaram A, Koutkia P, Apovian CM. Nutritional management of short bowel syndrome in adults. J Clin Gastroenterol. Mar 2002;34(3):207-20. [Medline].

  17. Wilmore DW, Byrne TA, Persinger RL. Short bowel syndrome: new therapeutic approaches. Curr Probl Surg. May 1997;34(5):389-444. [Medline].

  18. Vanderhoof JA, Langnas AN. Short-bowel syndrome in children and adults. Gastroenterology. Nov 1997;113(5):1767-78. [Medline].

  19. Cavicchi M, Beau P, Crenn P, et al. Prevalence of liver disease and contributing factors in patients receiving home parenteral nutrition for permanent intestinal failure. Ann Intern Med. Apr 4 2000;132(7):525-32. [Medline].

  20. Klein S, Nealon WH. Hepatobiliary abnormalities associated with total parenteral nutrition. Semin Liver Dis. Aug 1988;8(3):237-46. [Medline].

  21. Quigley EM, Marsh MN, Shaffer JL, et al. Hepatobiliary complications of total parenteral nutrition. Gastroenterology. Jan 1993;104(1):286-301. [Medline].

  22. Puder M, Valim C, Meisel JA, et al. Parenteral fish oil improves outcomes in patients with parenteral nutrition-associated liver injury. Ann Surg. Aug 5 2009;[Medline].

  23. Pansky B. Duodenum-jejunum-ileum, duodenal fossae, and superior mesenteric artery. In: Review of Gross Anatomy. 4th ed. New York, NY: Macmillan Publishing Co; 1979:334-335.

  24. Starzl TE, Todo S, Tzakis A, et al. The many faces of multivisceral transplantation. Surg Gynecol Obstet. May 1991;172(5):335-44. [Medline].

  25. Cosnes J, Gendre JP, Evard D, et al. Compensatory enteral hyperalimentation for management of patients with severe short bowel syndrome. Am J Clin Nutr. May 1985;41(5):1002-9. [Medline].

  26. Scolapio JS, Fleming CR. Short bowel syndrome. Gastroenterol Clin North Am. Jun 1998;27(2):467-79, viii. [Medline].

  27. Booth IW. Enteral nutrition as primary therapy in short bowel syndrome. Gut. Jan 1994;35(1 Suppl):S69-72. [Medline].

  28. 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. Jul-Aug 1996;20(4):275-80. [Medline].

  29. Nightingale JM, Lennard-Jones JE, Walker ER, et al. Jejunal efflux in short bowel syndrome. Lancet. Sep 29 1990;336(8718):765-8. [Medline].

  30. Woolf GM, Miller C, Kurian R, et al. Nutritional absorption in short bowel syndrome. Evaluation of fluid, calorie, and divalent cation requirements. Dig Dis Sci. Jan 1987;32(1):8-15. [Medline].

  31. Wilmore DW, Lacey JM, Soultanakis RP, et al. Factors predicting a successful outcome after pharmacologic bowel compensation. Ann Surg. Sep 1997;226(3):288-92; discussion 292-3. [Medline].

  32. Van Gossum A, Cabre E, Hebuterne X, et al. ESPEN Guidelines on Parenteral Nutrition: gastroenterology. Clin Nutr. Aug 2009;28(4):415-27. [Medline].

  33. Wallis K, Walters JR, Gabe S. Short bowel syndrome: the role of GLP-2 on improving outcome. Curr Opin Clin Nutr Metab Care. Sep 2009;12(5):526-32. [Medline].

  34. Farthing MJ. Octreotide in dumping and short bowel syndromes. Digestion. 1993;54 Suppl 1:47-52. [Medline].

  35. Thompson JS, Langnas AN, Pinch LW, et al. Surgical approach to short-bowel syndrome. Experience in a population of 160 patients. Ann Surg. Oct 1995;222(4):600-5; discussion 605-7. [Medline].

  36. Thompson JS, Langnas AN. Surgical approaches to improving intestinal function in the short-bowel syndrome. Arch Surg. Jul 1999;134(7):706-9; discussion 709-11. [Medline].

  37. Panis Y, Messing B, Rivet P, et al. Segmental reversal of the small bowel as an alternative to intestinal transplantation in patients with short bowel syndrome. Ann Surg. Apr 1997;225(4):401-7. [Medline].

  38. Javid PJ, Kim HB, Duggan CP, et al. Serial transverse enteroplasty is associated with successful short-term outcomes in infants with short bowel syndrome. J Pediatr Surg. Jun 2005;40(6):1019-23; discussion 1023-4. [Medline].

  39. Todo S, Reyes J, Furukawa H, et al. Outcome analysis of 71 clinical intestinal transplantations. Ann Surg. Sep 1995;222(3):270-80; discussion 280-2. [Medline].

  40. Langnas AN, Shaw BW Jr, Antonson DL, et al. Preliminary experience with intestinal transplantation in infants and children. Pediatrics. Apr 1996;97(4):443-8. [Medline].

  41. Abu-Elmagd K, Reyes J, Todo S, et al. Clinical intestinal transplantation: new perspectives and immunologic considerations. J Am Coll Surg. May 1998;186(5):512-25; discussion 525-7. [Medline].

  42. Sudan D, Thompson J, Botha J, et al. Comparison of intestinal lengthening procedures for patients with short bowel syndrome. Ann Surg. Oct 2007;246(4):593-601; discussion 601-4. [Medline].

  43. Abu-Elmagd K, Fung J, Bueno J, et al. Logistics and technique for procurement of intestinal, pancreatic, and hepatic grafts from the same donor. Ann Surg. Nov 2000;232(5):680-7. [Medline].

  44. Fitzgibbons S, Ching YA, Valim C, et al. Relationship between serum citrulline levels and progression to parenteral nutrition independence in children with short bowel syndrome. J Pediatr Surg. May 2009;44(5):928-32. [Medline].

  45. 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. Aug 1995;109(2):355-65. [Medline].

  46. Gorczynski RM. Immunosuppression induced by hepatic portal venous immunization spares reactivity in IL-4 producing T lymphocytes. Immunol Lett. Jun 1992;33(1):67-77. [Medline].

  47. Andorsky DJ, Lund DP, Lillehei CW, et al. Nutritional and other postoperative management of neonates with short bowel syndrome correlates with clinical outcomes. J Pediatr. Jul 2001;139(1):27-33. [Medline].

  48. Baldwin-Price HK, Copp D, Singleton AO Jr. Reversed intestinal segments in the management of anenteric malabsorption syndrome. Ann Surg. Feb 1965;161:225-30. [Medline].

  49. Benedetti E, Baum C, Cicalese L, et al. Progressive functional adaptation of segmental bowel graft from living related donor. Transplantation. Feb 27 2001;71(4):569-71. [Medline].

  50. Chen MK, Badylak SF. Small bowel tissue engineering using small intestinal submucosa as a scaffold. J Surg Res. Aug 2001;99(2):352-8. [Medline].

  51. Cicalese L, Baum C, Brown M, et al. Segmental small bowel transplant from adult living-related donors. Transplant Proc. Feb-Mar 2001;33(1-2):1553. [Medline].

  52. Cicalese L, Rastellini C, Sileri P, et al. Segmental living related small bowel transplantation in adults. J Gastrointest Surg. Mar-Apr 2001;5(2):168-72; discussion 173. [Medline].

  53. Dudrick SJ, Wilmore DW, Vars HM, et al. Long-term total parenteral nutrition with growth, development, and positive nitrogen balance. Surgery. Jul 1968;64(1):134-42. [Medline].

  54. Ellegard L, Bosaeus I, Nordgren S, et al. Low-dose recombinant human growth hormone increases body weight and lean body mass in patients with short bowel syndrome. Ann Surg. Jan 1997;225(1):88-96. [Medline].

  55. Gerwig WH, Ghaphery A. Experimental attempt to delay alimentary transit after small bowel exclusion. Arch Surg. 1963;87:50-57.

  56. Greenberger NJ. State of the art: the management of the patient with short bowel syndrome. Am J Gastroenterol. Nov 1978;70(5):528-40. [Medline].

  57. Howard L, Heaphey L, Fleming CR, et al. Four years of North American registry home parenteral nutrition outcome data and their implications for patient management. JPEN J Parenter Enteral Nutr. Jul-Aug 1991;15(4):384-93. [Medline].

  58. Høverstad T. Studies of short-chain fatty acid absorption in man. Scand J Gastroenterol. Apr 1986;21(3):257-60. [Medline].

  59. Kocoshis S. Evolving concepts and improving prospects for neonates with short bowel syndrome. J Pediatr. Jul 2001;139(1):5-7. [Medline].

  60. Kono K, Sekikawa T, Iizuka H, et al. Interposed colon between remnants of the small intestine exhibits small bowel features in a patient with short bowel syndrome. Dig Surg. 2001;18(3):237-41. [Medline].

  61. Lennard-Jones JE. Review article: practical management of the short bowel. Aliment Pharmacol Ther. Dec 1994;8(6):563-77. [Medline].

  62. Lennard-Jones JE, Wood S. Coping with the short bowel syndrome. Hosp Update. 1991;27:797-807.

  63. Li-Ling, Irving M. The effectiveness of growth hormone, glutamine and a low-fat diet containing high-carbohydrate on the enhancement of the function of remnant intestine among patients with short bowel syndrome: a review of published trials. Clin Nutr. Jun 2001;20(3):199-204. [Medline].

  64. Lord LM, Schaffner R, DeCross AJ, et al. Management of the patient with short bowel syndrome. AACN Clin Issues. Nov 2000;11(4):604-18. [Medline].

  65. Meyer D, Thorwarth WM, Otto C, et al. Orthotopic liver/small bowel transplantation in rats: a microsurgical model inducing tolerance. Microsurgery. 2001;21(4):156-62. [Medline].

  66. 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. May 2001;96(5):1494-8. [Medline].

  67. Pansky B. Blood supply of the colon and appendix. In: Review of Gross Anatomy. 4th ed. New York, NY: Macmillan Publishing Co; 1979:340-341.

  68. Pansky B. Pancreas. In: Review of Gross Anatomy. 4th ed. New York, NY: Macmillan Publishing Co; 1979:332-333.

  69. Pansky B. The large intestine. In: Review of Gross Anatomy. 4th ed. New York, NY: Macmillan Publishing Co; 1979:338-339.

  70. Pansky B. Duodenum. In: Review of Gross Anatomy. 4th ed. New York, NY: Macmillan Publishing Co; 1979:330-331.

  71. Pearson PY, O'Connor DM, Schwartz MZ. Novel effect of leptin on small intestine adaptation. J Surg Res. May 15 2001;97(2):192-5. [Medline].

  72. Pertsemlidis D, Kark AE. Antiperistaltic segments for the treatment of short bowel syndrome. Am J Gastroenterol. Dec 1974;62(6):526-30. [Medline].

  73. Ploeg RJ, D'Alessandro AM. Intestinal transplantation: a clinical update. Scand J Gastroenterol Suppl. 1995;212:79-89. [Medline].

  74. Scolapio JS, McGreevy K, Tennyson GS, et al. Effect of glutamine in short-bowel syndrome. Clin Nutr. Aug 2001;20(4):319-23. [Medline].

  75. Sedgwick CE, Viglotti J. Hyperalimentation. Surg Clin North Am. Jun 1971;51(3):681-6. [Medline].

  76. Stern LE, Erwin CR, O'Brien DP, et al. Epidermal growth factor is critical for intestinal adaptation following small bowel resection. Microsc Res Tech. Oct 15 2000;51(2):138-48. [Medline].

  77. Tatsumi Y, Matis LA, Iramajiri K, et al. The molecular mechanism of the functional development of the TcR-gamma delta cells and the effects of cytokines. 9th Congr Immunology. 1995;1499.

  78. Terra RM, Plopper C, Waitzberg DL, et al. Remaining small bowel length: association with catheter sepsis in patients receiving home total parenteral nutrition: evidence of bacterial translocation. World J Surg. Dec 2000;24(12):1537-41. [Medline].

  79. Thompson JS, Pinch LW, Young R, et al. Long-term outcome of intestinal lengthening. Transplant Proc. Sep 2000;32(6):1242-3. [Medline].

  80. Vanderhoof JA. Short bowel syndrome in children and small intestinal transplantation. Pediatr Clin North Am. Apr 1996;43(2):533-50. [Medline].

  81. Vernon AH, Georgeson KE. Surgical options for short bowel syndrome. Semin Pediatr Surg. May 2001;10(2):91-8. [Medline].

  82. Whang EE, Shen KR. Intestinal adaptation: a new era. JPEN J Parenter Enteral Nutr. Mar-Apr 2001;25(2):100. [Medline].

  83. Wilmore DW, Robinson MK. Short bowel syndrome. World J Surg. Dec 2000;24(12):1486-92. [Medline].

  84. Zhou X, Li YX, Li N, et al. Glutamine enhances the gut-trophic effect of growth hormone in rat after massive small bowel resection. J Surg Res. Jul 2001;99(1):47-52. [Medline].

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Keywords

short-bowel syndrome, short bowel syndrome, Crohn's disease, total parenteral nutrition, bowel transplant, intestinal atresia, atresia intestinal, jejunal atresia, intestinal transplant, short gut syndrome, anenteric malabsorption syndrome

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Contributor Information and Disclosures

Author

Burt Cagir, MD, FACS, Assistant Professor of Surgery, State University of New York, Upstate Medical Center; Consulting Staff, Director of Surgical Research, Robert Packer Hospital; Associate Program Director, Department of Surgery, Guthrie Clinic
Burt Cagir, MD, FACS is a member of the following medical societies: American College of Surgeons, American Medical Association, and Society for Surgery of the Alimentary Tract
Disclosure: Nothing to disclose.

Coauthor(s)

Michael AJ Sawyer, MD, Consulting Staff, Department of Surgery, Southwestern Medical Center; Consulting Staff, Department of Surgery, Comanche County Memorial Hospital; Consulting Staff, Great Plains Surgical Clinic, Inc
Michael AJ Sawyer, MD is a member of the following medical societies: American College of Surgeons, Society for Surgery of the Alimentary Tract, Society of American Gastrointestinal and Endoscopic Surgeons, and Society of Laparoendoscopic Surgeons
Disclosure: Nothing to disclose.

Medical Editor

Juan B Ochoa, MD, Assistant Professor, Department of Surgery, University of Pittsburgh
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

David L Morris, MD, PhD, Professor, Department of Surgery, St George Hospital, University of New South Wales, Australia
Disclosure: RFA Medical None Director; MRC Biotec None Director

CME Editor

Paolo Zamboni, MD, Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy
Paolo Zamboni, MD is a member of the following medical societies: American Venous Forum and New York Academy of Sciences
Disclosure: Nothing to disclose.

Chief Editor

John Geibel, MD, DSc, MA, Vice Chairman, Professor, Department of Surgery, Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology, Yale University School of Medicine; Director of Surgical Research, Department of Surgery, Yale-New Haven Hospital
John Geibel, MD, DSc, MA is a member of the following medical societies: American Gastroenterological Association, American Physiological Society, American Society of Nephrology, Association for Academic Surgery, International Society of Nephrology, New York Academy of Sciences, and Society for Surgery of the Alimentary Tract
Disclosure: AMGEN Royalty Other

 
 
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