eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology

Short Bowel Syndrome

Author: Carmen Cuffari, MD, Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine
Contributor Information and Disclosures

Updated: Apr 15, 2009

Introduction

Background

Few conditions in pediatric gastroenterology pose as great a challenge as short bowel syndrome (SBS).1 Short bowel syndrome is the result of the alteration of intestinal digestion and absorption that occurs following extensive bowel resection. It represents a complex disorder that affects normal intestinal physiology with nutritional, metabolic, and infectious consequences.

Pathophysiology

The small intestine of the neonate is approximately 250 cm in length. By adulthood, the small intestine grows to approximately 750 cm. As a consequence, the infant and the young child have a favorable long-term prognosis compared to an adult in regards to potential intestinal growth after intestinal resection. Intestinal adaptation may take weeks to months to be achieved; in the interim, children who have had intestinal resection need nutritional support through various therapeutic measures, including parenteral nutrition. The duodenum and jejunum are responsible for the absorption of most dietary constituents except vitamin B-12 and bile acids.

The jejunum is characterized by long villi and a large absorptive area. The tight junctions are relatively large, rendering the epithelium more porous to larger molecules and the free and rapid flux of water and electrolytes. In comparison, the ileum has shorter villi and a less absorptive surface area than the jejunum. Furthermore, the tight junctions are tighter, permitting less flux of water and electrolytes from the vascular space into the intestinal lumen, and, consequently, the ileum is more efficient in the absorption of water. Although nutrients are less well absorbed in the ileum, it has site-specific receptors for the absorption of bile acids and vitamin B12. Moreover, many GI hormones that affect intestinal motility, including enteroglucagon and peptide YY, are produced in the ileum. The small intestinal sites of nutrient absorption are as follows: 

  • Duodenum - Iron
  • Jejunum - Carbohydrates, proteins, fat, vitamins
  • Ileum - Bile acids, vitamin B-12

In general, virtually all digestion and absorption is completed within the first 100-150 cm of jejunum in a healthy individual. In the absence of an intact colon, the minimum length of healthy bowel necessary to avoid parenteral nutrition is approximately 100 cm. Patients who have less than 100 cm of jejunum exhibit significant malabsorption. Although the ileum is limited in its capacity to form chylomicrons compared to the jejunum, studies have shown that the ileum has greater adaptive function as far as improving its absorptive function in the presence of short bowel syndrome. Similarly, studies in animals have shown that intestinal transit time is more likely to improve (ie, increase) in patients with proximal small-bowel resection as opposed to patients with distal small-bowel resection.

The jejunum cannot develop site-specific carriers for the transportation of vitamin B12 and bile salts, and, consequently, these are malabsorbed permanently in patients, following ileal resection. Furthermore, the loss of enteroglucagon and peptide YY cannot be underscored in the regulation of small-bowel motility.

Mortality/Morbidity

The leading causes of death in infants with short bowel syndrome who are being treated with parenteral nutrition are central line sepsis and liver failure with the prolonged use of parenteral nutrition.

Clinical

History

The history of a patient with short bowel syndrome (SBS) is typically of a child who was born with a congenital anomaly, such as an omphalocele, gastroschisis, or intestinal atresia, and who required a small-bowel resection. In addition, premature infants with necrotizing enterocolitis and require extensive bowel resection with or without the loss of the ileocecal junction also contribute to this patient population.2

Other patients present with a past medical history of intestinal ischemia from malrotation and volvulus that required intestinal resection. Congenital short bowel syndrome is a rare cause of short bowel syndrome. Over the last several years, the authors have noted numerous patients with short bowel syndrome associated with cloacal exstrophy. Although these children do not have short bowel syndrome in the classical sense, they behave clinically with all signs and symptoms associated with functional intestinal failure.

Children with short bowel syndrome may present with various medical issues, depending on the extent of their bowel resection and the level of medical complexity. The history should consider all the potential clinical ramifications of managing cases of short bowel syndrome, including the following:

  • Parenteral nutrition
    • The degree of home nutrition support necessary in the management of a child on total parenteral nutrition (TPN) is noteworthy.
    • Guidelines for the safe use of parenteral nutrition have been established by the American Society for Parenteral and Enteral Nutrition.3
    • Patients may present with issues entirely separate from the medical problems related to short bowel syndrome, including problems associated with intravenous access, infection, and signs and clinical symptoms associated with TPN-related liver disease.
  • Enteral nutrition
    • Quickly initiate enteral feeding for all children with short bowel syndrome.
    • Once again, patients may present with a history entirely separate from the medical problems related to short bowel syndrome, including gastrostomy or nasogastric tube issues. For example, gastrostomy tubes may accidentally fall out. In these patients, the immediate replacement of these tubes is important in maintaining the patency of the tube entry site.
    • Although complications are uncommon, be aware of potential gastric ulceration, gastrostomy tube migrations, and intestinal obstruction, which all may be associated with bilious vomiting and the risk for pancreatitis.
  • Nutrition
    • Closely monitor all children on long-term enteral and parenteral nutrition for specific nutritional issues.
    • Patients may present with various symptoms related to specific nutritional deficiency, including specific vitamin (or mineral) deficiencies and the related signs, symptoms, and electrolyte abnormalities and their potential complications.
  • Medical and surgical histories: Obtain a detailed account of the patient's past medical and surgical histories.
    • The pathology leading up to the surgical resection
    • The extent and location of bowel resection, the presence or absence of the ileocecal valve
    • Medical complications
    • TPN dependency
    • Enteral nutrition
    • Enteral access
    • Type of nutritional formula used
    • Nutritional supplements
    • Medications
    • Allergies
  • History of complications associated with short bowel syndrome

Physical

During the physical examination, pay close attention to these clinical signs.

  • Vitals
  • State of hydration
  • State of nutrition, as measured by a patient's weight for height and anthropometric measurements
  • Signs of sepsis
  • Form of nutritional therapy used in the patient (eg, central line access or enteral access)
  • Specific clinical signs of nutritional deficiency
  • Signs of liver disease

Causes

  • Necrotizing enterocolitis, intestinal atresias, and midgut volvulus are the most common causes of short bowel syndrome in the neonatal period.
  • Intussusception with ischemic small-intestinal injury is a common cause of short bowel syndrome in older infants and children.
  • Through innovations in the surgical management of patients with chronic inflammatory bowel disease, Crohn disease is a less frequently associated cause of short bowel syndrome.

More on Short Bowel Syndrome

Overview: Short Bowel Syndrome
Differential Diagnoses & Workup: Short Bowel Syndrome
Treatment & Medication: Short Bowel Syndrome
Follow-up: Short Bowel Syndrome
Multimedia: Short Bowel Syndrome
References
[ CLOSE WINDOW ]

References

  1. Goday PS. Short bowel syndrome: how short is too short?. Clin Perinatol. Mar 2009;36(1):101-10. [Medline].

  2. Cole CR, Hansen NI, Higgins RD, Ziegler TR, Stoll BJ. Very low birth weight preterm infants with surgical short bowel syndrome: incidence, morbidity and mortality, and growth outcomes at 18 to 22 months. Pediatrics. Sep 2008;122(3):e573-82. [Medline].

  3. Seres D, Sacks GS, Pedersen CA, et al. Parenteral nutrition safe practices: results of the 2003 American Society for Parenteral and Enteral Nutrition survey. JPEN J Parenter Enteral Nutr. May-Jun 2006;30(3):259-65. [Medline].

  4. Goulet OJ, Revillon Y, Jan D, et al. Neonatal short bowel syndrome. J Pediatr. Jul 1991;119(1 ( Pt 1)):18-23. [Medline].

  5. Cole CR, Ziegler TR. Small bowel bacterial overgrowth: a negative factor in gut adaptation in pediatric SBS. Curr Gastroenterol Rep. Dec 2007;9(6):456-62. [Medline].

  6. Jeppesen PB, Staun M, Tjellesen L, Mortensen PB. Effect of intravenous ranitidine and omeprazole on intestinal absorption of water, sodium, and macronutrients in patients with intestinal resection. Gut. Dec 1998;43(6):763-9. [Medline].

  7. Ascher DP, Shoupe BA, Maybee D, Fischer GW. Persistent catheter-related bacteremia: clearance with antibiotics and urokinase. J Pediatr Surg. Apr 1993;28(4):627-9. [Medline].

  8. Bernard DK, Shaw MJ. Principles of nutrition therapy for short-bowel syndrome. Nutr Clin Pract. Aug 1993;8(4):153-62. [Medline].

  9. Bloom SR. Gut hormones in adaptation. Gut. 1987;28 Suppl:31-5. [Medline].

  10. Buchman AL. The clinical management of short bowel syndrome: steps to avoid parenteral nutrition. Nutrition. Oct 1997;13(10):907-13. [Medline].

  11. 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].

  12. Capron JP, Gineston JL, Herve MA, Braillon A. Metronidazole in prevention of cholestasis associated with total parenteral nutrition. Lancet. Feb 26 1983;1(8322):446-7. [Medline].

  13. Cosnes L, Carbonnel F, Beaugerie L. Functional adaptation after extensive small bowel resection in humans. Eur J Gastroenterol Hepatol. 1994;6:197.

  14. Cummings JH. Colonic absorption: the importance of short chain fatty acids in man. Scand J Gastroenterol Suppl. 1984;93:89-99. [Medline].

  15. Dudrick SJ, Latifi R, Fosnocht DE. Management of the short-bowel syndrome. Surg Clin North Am. Jun 1991;71(3):625-43. [Medline].

  16. Fine H, Levine GM, Shiau YF. Effects of cholecystokinin and secretin on intestinal structure and function. Am J Physiol. Sep 1983;245(3):G358-63. [Medline].

  17. Fordtran JS, Dietschy JM. Water and electrolyte movement in the intestine. Gastroenterology. Feb 1966;50(2):263-85. [Medline].

  18. Gazet JC, Kopp J. The surgical significance of the ileocecal junction. Surgery. 1964;56:565-73.

  19. Grant D. Current results of intestinal transplantation. The International Intestinal Transplant Registry. Lancet. Jun 29 1996;347(9018):1801-3. [Medline].

  20. Grosfeld JL, Rescorla FJ, West KW. Short bowel syndrome in infancy and childhood. Analysis of survival in 60 patients. Am J Surg. Jan 1986;151(1):41-6. [Medline].

  21. Hays TL, Saavedra JM, Mattis LE. The use of high-fat low-carbohydrate diets for advancement of enteral feedings in children with short bowel syndrome. Top Clin Nutr. 1995;10(4):35-41.

  22. Hofmann AF, Poley JR. Role of bile acid malabsorption in pathogenesis of diarrhea and steatorrhea in patients with ileal resection. I. Response to cholestyramine or replacement of dietary long chain triglyceride by medium chain triglyceride. Gastroenterology. May 1972;62(5):918-34. [Medline].

  23. Hwang TL, O'Dwyer ST, Smith RJ. Preservation of small bowel mucosa using glutamine enriched parenteral nutrition. Surg Forum. 1986;37:56-8.

  24. Hylander E, Ladefoged K, Jarnum S. Calcium absorption after intestinal resection. The importance of a preserved colon. Scand J Gastroenterol. Jul 1990;25(7):705-10. [Medline].

  25. Hyman PE, Everett SL, Harada T. Gastric acid hypersecretion in short bowel syndrome in infants: association with extent of resection and enteral feeding. J Pediatr Gastroenterol Nutr. Mar-Apr 1986;5(2):191-7. [Medline].

  26. Jeejeebhoy KN. Therapy of the short-gut syndrome. Lancet. Jun 25 1983;1(8339):1427-30. [Medline].

  27. Klish WJ, Putnam TC. The short gut. Am J Dis Child. Nov 1981;135(11):1056-61. [Medline].

  28. 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].

  29. Levine GM, Deren JJ, Yezdimir E. Small-bowel resection. Oral intake is the stimulus for hyperplasia. Am J Dig Dis. Jul 1976;21(7):542-6. [Medline].

  30. Lowry SF, Brennan MF. Abnormal liver function during parenteral nutrition: Relation to infusion excess. J Surg Res. 1979;26:300-7.

  31. Marotta RB, Floch MH. Dietary therapy of steatorrhea. Gastroenterol Clin North Am. Sep 1989;18(3):485-512. [Medline].

  32. Messing B, Pigot F, Rongier M, et al. Intestinal absorption of free oral hyperalimentation in the very short bowel syndrome. Gastroenterology. Jun 1991;100(6):1502-8. [Medline].

  33. Nightingale JM, Kamm MA, van der Sijp JR, et al. Disturbed gastric emptying in the short bowel syndrome. Evidence for a 'colonic brake'. Gut. Sep 1993;34(9):1171-6. [Medline].

  34. Nordgaard I, Hansen BS, Mortensen PB. Importance of colonic support for energy absorption as small-bowel failure proceeds. Am J Clin Nutr. Aug 1996;64(2):222-31. [Medline].

  35. Parker P, Stroop S, Greene H. A controlled comparison of continuous versus intermittent feeding in the treatment of infants with intestinal disease. J Pediatr. Sep 1981;99(3):360-4. [Medline].

  36. Purdum PP 3d, Kirby DF. Short-bowel syndrome: a review of the role of nutrition support. JPEN J Parenter Enteral Nutr. Jan-Feb 1991;15(1):93-101. [Medline].

  37. Reyes J, Todo S, Bueno J, et al. Intestinal transplantation in children: five-year experience. Transplant Proc. Oct 1996;28(5):2755-6. [Medline].

  38. Scolapio JS, Camilleri M, Fleming CR, et al. Effect of growth hormone, glutamine, and diet on adaptation in short-bowel syndrome: a randomized, controlled study. Gastroenterology. Oct 1997;113(4):1074-81. [Medline].

  39. Sheldon GF, Peterson SR, Sanders R. Hepatic dysfunction during hyperalimentation. Arch Surg. Apr 1978;113(4):504-8. [Medline].

  40. Sial S, Koussayer T, Klein S. Nutritional management of a patient with short-bowel syndrome and large-volume jejunostomy output. Nutrition. Jan-Feb 1994;10(1):37-40; discussion 40-1. [Medline].

  41. Spagnuolo MI, Iorio R, Vegnente A, Guarino A. Ursodeoxycholic acid for treatment of cholestasis in children on long-term total parenteral nutrition: a pilot study. Gastroenterology. Sep 1996;111(3):716-9. [Medline].

  42. Thompson JS. Surgical management of short bowel syndrome. Surgery. Jan 1993;113(1):4-7. [Medline].

  43. Thompson JS, Rikkers LF. Surgical alternatives for the short bowel syndrome. Am J Gastroenterol. Feb 1987;82(2):97-106. [Medline].

  44. Todo S, Tzakis AG, Abu-Elmagd K, et al. Cadaveric small bowel and small bowel-liver transplantation in humans. Transplantation. Feb 1992;53(2):369-76. [Medline].

  45. Treem WR. Short bowel syndrome. In: Wyllie R, Hyams JS, eds. Pediatric Gastrointestinal Disease: Pathophysiology, Diagnosis, Management. Philadelphia, PA: WB Saunders; 1993:573-603.

  46. Vileisis RA, Inwood RJ, Hunt CE. Prospective controlled study of parenteral nutrition-associated cholestatic jaundice: effect of protein intake. J Pediatr. May 1980;96(5):893-7. [Medline].

  47. Woolf GM, Miller C, Kurian R, Jeejeebhoy KN. Diet for patients with a short bowel: high fat or high carbohydrate?. Gastroenterology. Apr 1983;84(4):823-8. [Medline].

  48. Zahavi I, Shaffer EA, Gall DG. Total parenteral nutrition-associated cholestasis: acute studies in infant and adult rabbits. J Pediatr Gastroenterol Nutr. Aug 1985;4(4):622-7. [Medline].

  49. Zurier RB, Campbell RG, Hashim SA, Van Itallie TB. Use of medium-chain triglyceride in management of patients with massive resection of the small intestine. N Engl J Med. Mar 3 1966;274(9):490-3. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

short bowel syndrome, SBS, short-bowel syndrome, total parenteral nutrition, TPN, malabsorption, omphalocele, gastroschisis, intestinal atresia, necrotizing enterocolitis, malrotation, volvulus, cloacal exstrophy, intestinal failure, pancreatitis, treatment, diagnosis, malabsorptive diarrhea, dehydration, gastroesophageal reflux, failure to thrive, midgut volvulus, Crohn disease, inflammatory bowel disease

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Carmen Cuffari, MD, Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine
Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Medical Editor

Jorge H Vargas, MD, Professor of Pediatrics and Clinical Professor of Pediatric Gastroenterology, David Geffen School of Medicine, University of California at Los Angeles; Consulting Physician, Department of Pediatrics, University of California at Los Angeles Health System
Jorge H Vargas, MD is a member of the following medical societies: American Liver Foundation, American Society for Gastrointestinal Endoscopy, American Society for Parenteral and Enteral Nutrition, Latin American Society of Pediatric Gastroenterology, Hepatology & Nutrition, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

David A Piccoli, MD, Chief, Division of Gastroenterology and Nutrition, Department of Pediatrics, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine
David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

CME Editor

Steven M Schwarz, MD, FAAP, FACN, AGAF, Professor of Pediatrics, Children's Hospital at Downstate, SUNY-Downstate Medical Center
Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research
Disclosure: TAP Pharmaceuticals Honoraria Speaking and teaching; Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor

Chief Editor

Jatinder Bhatia, MBBS, Professor of Pediatrics, Chief, Section of Neonatology, Department of Pediatrics, Medical College of Georgia
Jatinder Bhatia, MBBS is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Dietetic Association, American Federation for Clinical Research, American Pediatric Society, American Society for Clinical Nutrition, American Society for Parenteral and Enteral Nutrition, New York Academy of Sciences, Society for Pediatric Research, and Southern Society for Pediatric Research
Disclosure: Mead Johnson Consulting fee Consulting; Mead Johnson Honoraria Speaking and teaching; Dey LP Consulting fee Consulting; Dey LP Honoraria Speaking and teaching; Ovation Honoraria Speaking and teaching

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.