What is paediatric short bowel syndrome?

Paediatric short bowel syndrome (SBS) results from the surgical removal (called resection) of extensive portions of the bowel.1 There are multiple causes of SBS in children2,3, including:

  • Necrotising enterocolitis (tissue of the intestine dying following birth)3,4
  • Intestinal atresia (a narrowing or absence of a portion of the intestine)3,5
  • Gastroschisis (a congenital defect where the bowel bulges out through the abdomen)3,6
  • Malrotation/volvulus (a rotation or twisting of the intestine)3
  • Long-segment Hirschsprung's disease (part or all of various sections of the intestine have no ganglion cells and therefore don't work correctly)3

SBS with intestinal failure (IF) is the term used when the function of the remaining bowel has been reduced below the minimum necessary for absorption of macronutrients and/or water and electrolytes required for maintenance of health. This means that the child would need nutritional supplementation given intravenously (known as parenteral support) to maintain their health and ongoing growth.1 The severity of SBS-IF can vary significantly and can be classified by the type and amount of support required, with more severe forms requiring greater levels of support.1

SBS-IF is when the function of the remaining bowel has been reduced below the minimum necessary for absorption of macronutrients and/or water and electrolytes1

Generally, the amount of bowel left is one of the most important factors determining the risk of SBS-IF and the likelihood of permanent dependence on parenteral support.3 Research has found that having remaining bowel length of <40cm was the single unfavourable predictive factor for being able to wean off PS.7

Symptoms

The symptoms of SBS in children can include diarrhoea and chronic dehydration.1 Children with SBS may also experience effects from poor nutritional status resulting in fatigue, weakness, problems concentrating, increased infection risk, impaired wound healing and slowing down of growth.1

How common is SBS-IF in children?

There are large variations in estimates of the prevalence* of SBS-IF in children in different locations around the world. This is thought to be because there has been no standardised definition of the condition and also because it is such a rare disorder.8 

*Prevalence is the proportion of a population who have (or had) a specific characteristic (e.g. an illness) in a given time period.9

References

  1. Georgeson KE, Breaux CW, Jr. Outcome and intestinal adaptation in neonatal short-bowel syndrome. J Pediatr Surg 1992;27:344–50
  2. Spencer AU, Neaga A, West B, et al. Pediatric short bowel syndrome: redefining predictors of success. Ann Surg 2005;242:403–409
  3. Schurink M, Hulscher JB, Nieuwenhuijs VB, et al. A surgical perspective of the outcome of a multidisciplinary intestinal rehabilitation program for children with short bowel syndrome in The Netherlands. Transplant Proc 2014;46:2102–2108
  4. 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 2001;139:27–33
  5. Van Manen M, Hendson L, Wiley M, et al. Early childhood outcomes of infants born with gastroschisis. J Pediatr Surg 2013;48(8):1682–1687
  6. Van Gossum A, Cabre E, Hébuterne X, et al. ESPEN Guidelines on Parenteral Nutrition: gastroenterology. Clin Nutr 2009;28:415–427
  7. Petit LM, Girard D, Ganousse-Mazeron S, et al. Weaning off prognosis factors of home parenteral nutrition for children with primary digestive disease. J Pediatr Gastroenterol Nutr 2016;62(3):462–468
  8. Wales PW, de Silva N, Kim J, Lecce L, To T, Moore A. Neonatal short bowel syndrome: population-based estimates of incidence and mortality rates. J Pediatr Surg 2004;39:690–695.
  9. National Institute of Health, National Institute of Mental Health. What is prevalence? http://www.nimh.nih.gov/health/statistics/prevalence

     

     

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