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Pediatric Surgery pp 1107-1124 | Cite as

Short Bowel Syndrome

  • Michael E. HöllwarthEmail author
Reference work entry

Abstract

Short bowel syndrome occurs after extensive loss of small bowel leading to a status of intestinal failure characterized by significant problems in digesting and absorbing enteral nutrition. A very complex adaptation process of the intestine allows that 90% of the patients can be weaned after a long-term period from parenteral nutrition. A small number of patients will finally need surgical interventions either to reduce a very fast passage time or to lengthen the dilated intestinal remnants. Some patients will need intestinal or combined intestinal/liver transplantation either after nearly total loss of small bowel or after severe complication such as intestinal failure-associated liver insufficiency. The therapeutic decisions have to be tailored to each individual patient depending which parts of the intestine are lost or which dysfunctions of the intestinal remnants need a special treatment. Survival rates reach today nearly 90% including some children with long-term home parenteral support, additionally to the enteral nutrition.

Keywords

Short bowel syndrome Intestinal adaptation Intestinal failure Liver insufficiency Bacterial overgrowth Dysmotility Bacterial translocation Central venous line Sepsis 

References

  1. Abu-Elmagd KM, Costa G, Bond GJ, et al. Five hundred intestinal and multivisceral transplantations at a single center. Major advances with new challenges. Ann Surg. 2009;250:567–81.PubMedGoogle Scholar
  2. Bianchi A. Intestinal loop lengthening – a technique for increasing small intestinal length. J Pediatr Surg. 1980;15:145–51.PubMedPubMedCentralCrossRefGoogle Scholar
  3. Bianchi A. Longitudinal intestinal lengthening and tailoring: results in 20 children. J R Soc Med. 1997;90:429–32.PubMedPubMedCentralCrossRefGoogle Scholar
  4. Bloom SR, Polak JM. The hormonal pattern of intestinal adaptation. A major role for Enteroglukagon. Scand J Gastroenterol. 1982;74(Suppl):93–103.Google Scholar
  5. Briet F, Flourie B, Achour L, et al. Bacterial adaptation in patients with short bowel and colon in continuity. Gastroenterology. 1995;109:1446–53.PubMedCrossRefGoogle Scholar
  6. Brink MA, Mendez-Sanchez N, Carey MC. Bilirubin cycles enterohepatically after ileal resection in the rat. Gastroenterology. 1996;110:1945–57.PubMedCrossRefGoogle Scholar
  7. Byrne TA, Wilmore DW, Iyer K, et al. Growth hormone, glutamine, and an optimal diet reduces parenteral nutrition in patients with short bowel syndrome. Ann Surg. 2005;242:655–61.PubMedPubMedCentralCrossRefGoogle Scholar
  8. Chu HP, Brind J, Tomar R, et al. Significant reduction in central venous catheter-related bloodstream infections in children on HPN after starting treatment with Taurolidine line lock. JPGN. 2012;55:403–7.PubMedGoogle Scholar
  9. Cisler JJ, Buchman AL. Intestinal adaptation in short bowel syndrome. J Investig Med. 2005;53:402–13.PubMedCrossRefGoogle Scholar
  10. Cohran VC, Prozialeck JD, Cole CR. Redefining short bowel syndrome in the 21st century. Pediatr Res. 2017;  https://doi.org/10.1038/pr.2016.265. [Epub ahead of print]CrossRefPubMedGoogle Scholar
  11. Cole CR, Hansen NI, Higgins RD, et al. Very low birth weight preterm infants with surgical short bowel syndrome: incidence, morbidity and mortality, and growth outcome at 18 to 22 month. Pediantrics. 2008;122:e573–82.CrossRefGoogle Scholar
  12. Cole CR, Hansen NI, Higgins RD, et al. Bloodstream infections in very low birth weight infants with intestinal failure. J Pediatr. 2012;160:54–9.PubMedCrossRefGoogle Scholar
  13. D’Antiga L, Goulet O. Intestinal failure in children: the European view. JPGN. 2013;2:118–26.Google Scholar
  14. De Greef E, Mahler T, Janssen A, et al. The influence of neocate in pediatric short bowel syndrome on PN weaning. J Nutr Metab. 2010:Article ID 297575. 6 pagesGoogle Scholar
  15. Debongnie J, Philips S. Capacity of the human colon to absorb fluid. Gastroenterology. 1978;74:698–703.PubMedCrossRefGoogle Scholar
  16. Diamond IR, de Silva N, Pencharz PB, et al. Neonatal short bowel syndrome outcomes after establishment of the first Canadian multidisciplinary intestinal rehabilitation program: preliminary experience. J Pediatr Surg. 2007;42:806–11.PubMedCrossRefGoogle Scholar
  17. Dore M, Junco PT, Moreno AA, et al. Ultrashort bowel syndrome outcome in children treated in a multidisciplinary intestinal rehabilitation unit. Eur J Pediatr Surg. 2017;27(1):116–20.PubMedCrossRefGoogle Scholar
  18. Dorney SF, Ament ME, Berquist WE, et al. Improved survival in very short small bowel of infancy with use of long-term parenteral nutrition. J Pediatr. 1985;107:521–5.PubMedCrossRefGoogle Scholar
  19. Eizaguirre I, Aldazabal P, Urkia NG, et al. Escherichia coli translocation in experimental short bowel syndrome: probiotic supplementation and detection by polymerase chain reaction. Pediatr Surg Int. 2011;27:1301–5.PubMedCrossRefGoogle Scholar
  20. Fullerton BS, Sparks EA, Hall AM, et al. Enteral autonomy, cirrhosis, and long term transplant-free survival in pediatric intestinal failure patients. J Pediatr Surg. 2016;51(1):96–100.PubMedCrossRefGoogle Scholar
  21. Georgeson K, Halpin D, Figueroa R, et al. Sequential intestinal lengthening procedures for refractory short bowel syndrome. J Pediatr Surg. 1994;29:316–20.PubMedCrossRefGoogle Scholar
  22. Glick PL, de Lorimier AA, Ns A, et al. Colon interposition: an adjuvant operation for the short gut syndrome. J Pediatr Surg. 1984;19:719–25.PubMedCrossRefGoogle Scholar
  23. Goulet O, Baglin-Gobet S, Talbotec C. Outcome and long-term growth after extensive small bowel resection in the neonatal period: a survey of 87 children. Eur J Pediatr Surg. 2005;15:95–101.PubMedCrossRefGoogle Scholar
  24. Goulet O, Colomb-Jung V, Joly F. Role of the colon in short bowel syndrome and intestinal transplantation. JPGN. 2009;48(Suppl 2):S66–71.PubMedGoogle Scholar
  25. Goulet O, Dabbas-Tyan M, Talbotec C, et al. Effect of recombinant human growth hormone on intestinal absorption and body composition in children with short bowel syndrome. JPEN. 2010;34:513–20.CrossRefGoogle Scholar
  26. Goulet O, Olieman J, Ksiazyk J, et al. Neonatal short bowel syndrome as a model of intestinal failure: physiological background for enteral feeding. Clin Nutr. 2013;32:162–71.PubMedCrossRefGoogle Scholar
  27. 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. 2012;22:121–6.PubMedCrossRefGoogle Scholar
  28. Hadjittofi C, Coran AG, Mogilner JG, et al. Dietary supplementation with vitamin D stimulates intestinal epithelial cell turnover after massive small bowel resection in rats. Pediatr Surg Int. 2013;29:41–50.PubMedCrossRefGoogle Scholar
  29. Hess RA, Welch KB, Brown PI, et al. Survival outcomes of pediatric intestinal failure patients. Analysis of factors contributing to improved survival over the past two decades. J Surg Res. 2011;170:27–31.CrossRefGoogle Scholar
  30. Höllwarth ME. Short bowel syndrome: pathophysiological and clinical aspects. Pathophysiology. 1999;6:1–19.CrossRefGoogle Scholar
  31. Höllwarth ME. Surgical strategies in short bowel syndrome. Pediatr Surg Int. 2017;33:413–419.PubMedCrossRefGoogle Scholar
  32. Höllwarth ME, Granger DN, Ulrich-Baker MG, et al. Pharmacologic enhancement of adaptive growth after extensive small bowel resection. Pediatr Surg Int. 1988;3:55–61.Google Scholar
  33. Hsieh J, Longuet C, Maida A, et al. Glucagon-like peptide-2 increases intestinal lipid absorption and chylomicron production via CD36. Gastroenterology. 2009;137:997–1005.PubMedCrossRefGoogle Scholar
  34. Iannoli P, Miller JH, Ryan CK, et al. Epidermal growth factor and human growth hormone accelerate adaptation after massive enterectomy in an additive, nutrient dependent, and site specific fashion. Surgery. 1997;122:721–9.PubMedCrossRefGoogle Scholar
  35. Inoue Y, Copeland EM, Souba WW. Growth hormone enhances amino acid uptake by the human small intestine. Ann Surg. 1994;219:715–24.PubMedPubMedCentralCrossRefGoogle Scholar
  36. Javid PJ, Malone FR, Dick AAS, et al. A contemporary analysis of parenteral nutrition- associated liver disease in surgical infants. J Pediatr Surg. 2011;46:1913–7.PubMedCrossRefGoogle Scholar
  37. Javid PJ, Sanchez SE, Horslen SP, et al. Intestinal lengthening and nutritional outcomes in children with short bowel syndrome. Am J Surg. 2013;205:576–80.PubMedCrossRefGoogle Scholar
  38. Jeppesen PB, Harmann B, Thulesen J, et al. Glucagon-like peptide 2 improves nutrient absorption and nutritional status in short bowel patients with no colon. Gastroenterology. 2001;120:806–15.PubMedCrossRefGoogle Scholar
  39. 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;143:1473–81.PubMedCrossRefGoogle Scholar
  40. Johnson JR. The trophic action of gastrointestinal hormones. Gastroenterology. 1976;70:278–88.PubMedCrossRefGoogle Scholar
  41. Jones BA, Hull MA, Potanos KM, et al. Report of 111 consecutive patients enrolled in the international serial transverse enteroplasty (STEP) data registry: a retrospective observational study. J Am Coll Surg. 2013;216:438–46.PubMedPubMedCentralCrossRefGoogle Scholar
  42. Kanamori Y, Hashizume K, Sugiyama M, et al. Combination therapy with Bifidobacterium breve, Lactobacillus casei, and galactooligosaccharides dramatically improved the intestinal function in a girl with short bowel syndrome: a novel symbiotic therapy for intestinal failure. Dig Dis Sci. 2001;46:2010–6.PubMedCrossRefGoogle Scholar
  43. Kang KHJ, Gutierrez IM, Zurakowski D, et al. Bowel re-dilatation following serial transverse enteroplasty (STEP). Pediatr Surg Int. 2012;28:1189–93.PubMedCrossRefGoogle Scholar
  44. Kim HB, Lee PW, Garza J, et al. Serial transverse enteroplasty for short bowel syndrome: a case report. J Pediatr Surg. 2003;38:881–5.PubMedCrossRefGoogle Scholar
  45. Kurkchubasche AG, Rowe MI, Smith SD. Adaptation in short bowel syndrome: reassessing old limits. J Pediatr Surg. 1993;28:1069–71.PubMedCrossRefGoogle Scholar
  46. Kurz R, Sauer H. Treatment and metabolic findings in extreme short-bowel syndrome with 11 cm jejunal remnant. J Pediatr Surg. 1983;18:257–63.PubMedCrossRefGoogle Scholar
  47. Madara JL, Trier JS. The functional morphology of the mucosa of the small intestine. In: Johnoson LR, editor. Physiology of the gastrointestinal tract. New York: Raven Press; 1994.Google Scholar
  48. Marino IR, Lauro A. Surgeon’s perspective on short bowel syndrome: Where are we? World J Transplant. 2018;8(6):198–202.PubMedPubMedCentralCrossRefGoogle Scholar
  49. Mayr JM, Schober PH, Weißensteiner U, et al. Morbidity and mortality of the short-bowel syndrome. Eur J Pediatr Surg. 1999;9:231–5.PubMedCrossRefGoogle Scholar
  50. Meier JJ, Nauck MA, Pott A, et al. Glucagon-like peptide 2 stimulates glucagon secretion, enhances lipid absorption, and inhibits gastric acid secretion in humans. Gastroenterology. 2006;130:44–54.PubMedCrossRefGoogle Scholar
  51. Miyasaka EA, Brown PI, Kadoura S, et al. The adolescent child with short bowel syndrome: new onset of failure to thrive and need for increased nutritional supplementation. J Pediatr Surg. 2010;45:1280–6.PubMedCrossRefGoogle Scholar
  52. Mogilner JG, Srugo I, Lurie M, et al. Effect of probiotics on intestinal regrowth and bacterial translocation after massive small bowel resection in rats. J Pediatr Surg. 2007;42:1365–71.PubMedCrossRefGoogle Scholar
  53. Mughal M, Irving M. Home parenteral nutrition in the United Kingdom and Ireland. Lancet. 1986;2:383–7.PubMedCrossRefGoogle Scholar
  54. Nightingale JM, Walker ER, Burnham W, et al. Octreotide ( a somatostatin analogue) improves the quality of life in some patients with a short intestine. Aliment Pharmacol Ther. 1989;3:367–73.PubMedCrossRefGoogle Scholar
  55. Nordgaard I, Hansen BST, Mortensen PB. Colon as digestive organ in patients with short bowel. Lancet. 1994;343:373–6.PubMedCrossRefGoogle Scholar
  56. O’Brien DP, Nelson LA, Huang FS, et al. Intestinal adaptation: structure, function, and regulation. Semin Pediatr Surg. 2001;10:56–64.PubMedCrossRefGoogle Scholar
  57. Olieman JF, Poley MJ, Gischler MJ, et al. Interdisciplinary management of infantile short bowel syndrome: resource consumption, growth, and nutrition. J Pediatr Surg. 2010;45:490–8.PubMedCrossRefGoogle Scholar
  58. Olieman JF, Penning C, Poley MJ, et al. Impact of infantile short bowel syndrome on long-term health-related quality of life: a cross sectional study. J Pediatr Surg. 2012;47:1309–16.PubMedCrossRefGoogle Scholar
  59. Oliveira SB, Cole CR. Insights into medical management of pediatric intestinal failure. Semin Pediatr Surg. 2018;27(4):256–60.PubMedCrossRefGoogle Scholar
  60. Ordonez F, Barbot-Trystram L, Lacaille F, et al. Intestinal absorption rate in children after small intestinal transplantation. Am J Clin Nutr. 2013;97:743–9.PubMedCrossRefGoogle Scholar
  61. Pakarinen MP, Kurvinen A, Koivusalo AI, et al. Long-term controlled outcomes after autologous intestinal reconstruction surgery in treatment of severe short bowel syndrome. J Pediatr Surg. 2013;48:339–44.PubMedCrossRefGoogle Scholar
  62. 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. 1997;225:401–7.PubMedPubMedCentralCrossRefGoogle Scholar
  63. Peretti N, Loras-Duclaux I, Kassai B, et al. Growth hormone to improve short bowel syndrome intestinal autonomy: a pediatric randomized open-label clinical trial. JPEN. 2011;35:723–31.CrossRefGoogle Scholar
  64. Peters JHC, Beishuizen A, Keur MB, et al. Assessment of small bowel function in critical illness: potential role of citrulline metabolism. J Intensive Care Med. 2011;26:105–10.PubMedCrossRefGoogle Scholar
  65. Peterson J, Kerner JA. New advances in the management of children with intestinal failure. JPEN. 2012;36(Suppl 1):36S–42S.CrossRefGoogle Scholar
  66. Pierro A, van Saene HKF, Jones MO, et al. Clinical impact of abnormal gut flora in infants receiving parenteral nutrition. Ann Surg. 1998;227:547–52.PubMedPubMedCentralCrossRefGoogle Scholar
  67. Pironi L, Paganelli GM, Miglioli M, et al. Morphologic and cytoproliferative patterns of duodenal mucosa in two patients after long-term parenteral nutrition: changes with oral refeeding and relation to intestinal resection. JPEN. 1994;18:351–4.CrossRefGoogle Scholar
  68. Porus RL. Epithelial hyperplasia following massive bowel resection in man. Gastroenterology. 1965;48:753–7.PubMedCrossRefGoogle Scholar
  69. Puder M, Valim C, Meisl JA, et al. Parenteral fish oil improves outcomes in patients with parenteral nutrition-associated liver injury. Ann Surg. 2009;250:395–402.PubMedPubMedCentralGoogle Scholar
  70. Pul M, Yilmaz N, Gürses N, et al. Enhancement by testosterone of adaptive growth after small bowel resection. Isr J Med Sci. 1991;27:339–42.PubMedGoogle Scholar
  71. Ramos-Gonzalez G, Kim HB. Autologous intestinal reconstruction surgery. Semin Pediatr Surg. 2018;27(4):261–6.PubMedCrossRefGoogle Scholar
  72. Raphael BP, Duggan CH. Prevention and treatment of intestinal failure-associated liver disease in children. Semin Liver Dis. 2012;32:341–7.PubMedCrossRefGoogle Scholar
  73. Reddy VS, Patole SK, Rao S. Role of probiotics in short bowel syndrome in infants and children – a systematic review. Nutrients. 2013;5:679–99.PubMedPubMedCentralCrossRefGoogle Scholar
  74. Rhoads JM, Plunkett E, Galanko J, et al. Serum citrulline levels correlate with enteral tolerance and bowel length in infants with short bowel syndrome. J Pediatr. 2005;146:542–7.PubMedCrossRefGoogle Scholar
  75. Rickham PP. Massive intestinal resection in newborn infants. Ann R Coll Surg. 1967;41:480–5.Google Scholar
  76. Ricotta J, Zuidema GD, Gadacz TR, et al. Construction of an ileocecal valve and its role in massive resection of the small intestine. Surg Gynecol Obstet. 1981;152:310–4.PubMedGoogle Scholar
  77. Salloum RM, Stevens BR, Schultz GS, et al. Regulation of small intestine glutamine transport by epidermal growth factor. Surgery. 1993;113:552–9.PubMedGoogle Scholar
  78. Schalamon J, Mayr JM, Höllwarth ME. Mortality and economics in short bowel syndrome. Best Pract Res Clin Gastroenterol. 2003;17:931–42.PubMedCrossRefGoogle Scholar
  79. Schimpl G, GFeierl G, Linni K, et al. Bacterial translocation in short-bowel syndrome rats. Eur J Pediatr Surg. 1999;9:224–7.PubMedCrossRefGoogle Scholar
  80. 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.PubMedCrossRefGoogle Scholar
  81. Sigalet DL. Short bowel syndrome in infants and children: an overview. Semin Pediatr Surg. 2001;10:49–55.PubMedCrossRefGoogle Scholar
  82. Sommovilla J, Warner BW. Surgical options to enhance intestinal function in patients with short bowel syndrome. Curr Opin Pediatr. 2014;26(3):350–5.PubMedPubMedCentralCrossRefGoogle Scholar
  83. Sondheimer JM, Cadnapaphornchai M, Sontag M, et al. Predicting the duration of dependence on parenteral nutrition after neonatal intestinal resection. J Pediatr. 1998;132:80–4.PubMedCrossRefGoogle Scholar
  84. Spencer AU, Neaga A, West B, et al. Pediatric short bowel syndrome. Redefining predictors of success. Ann Surg. 2005;242:403–12.PubMedPubMedCentralGoogle Scholar
  85. Stanger JD, Oliveira C, Blackmore C, et al. The impact of multi-disciplinary intestinal rehabilitation programs on the outcome of pediatric patients with intestinal failure: a systematic review and meta-analysis. J Pediatr Surg. 2013;48:983–92.PubMedCrossRefGoogle Scholar
  86. Stringer MD, Putins JW. Short bowel syndrome. Arch Dis Child. 1995;73:170–3.PubMedPubMedCentralCrossRefGoogle Scholar
  87. Tappenden KA, Thomson ABR, Wild GE, et al. Short-chain fatty acid- supplemented total parenteral nutrition enhances functional adaptation to intestinal resection in rats. Gastroenterology. 1997;112:792–802.PubMedCrossRefGoogle Scholar
  88. Thompson JS. Epidermal growth factor and the short bowel syndrome. JPEN. 1999;23(Suppl):S113–6.CrossRefGoogle Scholar
  89. Thompson JS, Langnas AN. Surgical approaches to improving intestinal function in the short- bowel syndrome. Arch Surg. 1999;134:706–9.PubMedCrossRefGoogle Scholar
  90. Thompson JS, Pinch LW, Young R, et al. Long-term outcome of intestinal lengthening. Transplant Proc. 2000;32:1242–3.PubMedCrossRefGoogle Scholar
  91. Touloukian RJ, Walker Smith GJ. Normal intestinal length in preterm infants. J Pediatr Surg. 1983;6:720–3.CrossRefGoogle Scholar
  92. Ulrich-Baker MG, Höllwaerth ME, Kvietys PR, et al. Blood flow response to small bowel resection. Am J Phys. 1986;251:G815–22.CrossRefGoogle Scholar
  93. Vanderhoof JA, Young RJ. Hydrolyzed versus nonhydrolyzed protein diet in short bowel syndrome in children. JPGN. 2004;38:107–8.PubMedGoogle Scholar
  94. Vanderhoof JA, Park JHY, Herrington MK, et al. Effects of dietary menhaden oil on mucosal adaptation after small bowel resection in rats. Gastroenterology. 1994;106:94–9.PubMedCrossRefGoogle Scholar
  95. Vanderhoof JA, Young RJ, Murray N, et al. Treatment strategies for small bowel bacterial overgrowth in short bowel syndrome. JPGN. 1998;27:155–60.PubMedGoogle Scholar
  96. Vanderhoof JA, Young RJ, Thompson JS. New and emerging therapies for short bowel syndrome in children. Pediatr Drugs. 2003;5:525–31.CrossRefGoogle Scholar
  97. Vernon AH, Georgeson KE. Surgical options for short bowel syndrome. Semin Pediatr Surg. 2001;10:91–8.PubMedCrossRefGoogle Scholar
  98. Vitek L, Carey MC. Enterohepatic cycling of bilirubin as a cause of “black” pigment gallstones in adult life. Eur J Clin Investig. 2003;33:799–810.CrossRefGoogle Scholar
  99. Wales PW, de Silva N, Kim J, et al. Neonatal short bowel syndrome: population-based estimates of incidence and mortality rates. J Pediatr Surg. 2004;39:690–5.PubMedCrossRefGoogle Scholar
  100. Wales PW, Nasr A, de Silva N, et al. Human growth hormone and glutamine for patients with short bowel syndrome (review). Cochrane Database Syst Rev. 2010;6:1–29.Google Scholar
  101. Wales PW, Kosar C, Carricato M, et al. Ethanol lock therapy to reduce the incidence of catheter-related bloodstream infections in home parenteral nutrition patients with intestinal failure: preliminary experience. J Pediatr Surg. 2011;46:951–6.PubMedCrossRefGoogle Scholar
  102. Wallander J, Ewald U, Läckgren G, et al. Extreme short bowel syndrome in neonates: an indication for small bowel transplantation? Transplant Proc. 1992;24:1230–5.PubMedGoogle Scholar
  103. Wilmore DW. Factors correlating with a successful outcome following extensive intestinal resection in newborn infants. J Pediatr. 1972;80:88–95.PubMedCrossRefGoogle Scholar
  104. Winsett DE, Ulshen DM, Hoyt EC, et al. Regulation and localisation of the insulin-like growth factor system in small bowel during altered nutritional status. Am J Phys. 1995;268:G631–40.Google Scholar
  105. Ziegler TR, Mantell MP, Chow JC, et al. Gut adaptation and the insulin-like growth factor system: regulation by glutamine and IGF-I administration. Am J Physiol. 1996;271:G866–75.PubMedGoogle Scholar

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Authors and Affiliations

  1. 1.Department of Paediatric and Adolescent SurgeryMedical University of GrazGrazAustria

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