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Pediatric Short Bowel Syndrome Clinical Presentation

  • Author: Carmen Cuffari, MD; Chief Editor: Jatinder Bhatia, MBBS, FAAP  more...
 
Updated: Jul 08, 2016
 

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.[1]

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.[4]
    • 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
    • Malabsorptive diarrhea
    • Dehydration
    • Vomiting
    • Bloating
    • Gastroesophageal reflux
    • Failure to thrive
    • Drug toxicities
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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
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Causes

See the list below:

  • 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.
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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, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David A Piccoli, MD Chief of Pediatric Gastroenterology, Hepatology and Nutrition, 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, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Chief Editor

Jatinder Bhatia, MBBS, FAAP Professor of Pediatrics, Medical College of Georgia, Georgia Regents University; Chief, Division of Neonatology, Director, Fellowship Program in Neonatal-Perinatal Medicine, Director, Transport/ECMO/Nutrition, Vice Chair, Clinical Research, Department of Pediatrics, Children's Hospital of Georgia

Jatinder Bhatia, MBBS, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Pediatric Society, American Society for Nutrition, American Society for Parenteral and Enteral Nutrition, Academy of Nutrition and Dietetics, Society for Pediatric Research, Southern Society for Pediatric Research

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Gerber.

Additional Contributors

Jorge H Vargas, MD Professor of Pediatrics and Clinical Professor of Pediatric Gastroenterology, University of California, Los Angeles, David Geffen School of Medicine; 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, Latin American Society of Pediatric Gastroenterology, Hepatology & Nutrition, American Society for Gastrointestinal Endoscopy, American Society for Parenteral and Enteral Nutrition, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

References
  1. 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. 2008 Sep. 122(3):e573-82. [Medline]. [Full Text].

  2. Weih S, Kessler M, Fonouni H, Golriz M, Hafezi M, Mehrabi A, et al. Current practice and future perspectives in the treatment of short bowel syndrome in children-a systematic review. Langenbecks Arch Surg. 2011 Nov 22. [Medline].

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

  4. 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. 2006 May-Jun. 30(3):259-65. [Medline].

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

  6. Novak B. Long-term teduglutide frees some short bowel patients from parenteral support. Medscape Medical News. January 27, 2014. [Full Text].

  7. Jeppesen PB. Teduglutide, a novel glucagon-like peptide 2 analog, in the treatment of patients with short bowel syndrome. Therap Adv Gastroenterol. 2012 May. 5(3):159-71. [Medline].

  8. 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. 1998 Dec. 43(6):763-9. [Medline].

  9. Guo M, Li Y, Li J. Role of Growth Hormone, Glutamine and Enteral Nutrition in Pediatric Short Bowel Syndrome: A Pilot Follow-Up Study. Eur J Pediatr Surg. 2011 Dec 7. [Medline].

  10. Greenberg RG, Moran C, Ulshen M, Smith PB, Benjamin DK Jr, Cohen-Wolkowiez M. Outcomes of catheter-associated infections in pediatric patients with short bowel syndrome. J Pediatr Gastroenterol Nutr. 2010 Apr. 50(4):460-2. [Medline]. [Full Text].

  11. Chung PH, Wong KK, Wong RM, Tsoi NS, Chan KL, Tam PK. Clinical experience in managing pediatric patients with ultra-short bowel syndrome using omega-3 fatty acid. Eur J Pediatr Surg. 2010 Mar. 20(2):139-42. [Medline].

  12. Park KT, Nespor C, Kerner J Jr. The use of Omegaven in treating parenteral nutrition-associated liver disease. J Perinatol. 2011 Apr. 31 Suppl 1:S57-60. [Medline].

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

  28. 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.

  29. 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. 1972 May. 62(5):918-34. [Medline].

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

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

  32. 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. 1986 Mar-Apr. 5(2):191-7. [Medline].

  33. Infantino BJ, Mercer DF, Hobson BD, Fischer RT, Gerhardt BK, Grant WJ, et al. Successful Rehabilitation in Pediatric Ultrashort Small Bowel Syndrome. J Pediatr. 2013 Jul 15. [Medline].

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

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

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

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

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

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

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

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

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

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

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

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

  46. 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. 1997 Oct. 113(4):1074-81. [Medline].

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

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

  49. 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. 1996 Sep. 111(3):716-9. [Medline].

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

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

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

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

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

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

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

  57. 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. 1966 Mar 3. 274(9):490-3. [Medline].

 
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Small-bowel obstruction caused by intussusception in a 5-month-old patient is visible on plain radiograph.
 
 
 
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