Short bowel syndrome

SBS has a highly variable prognosis depending on the health of the bowel, and the type and extent of surgical resection. The bowel generally gains absorptive capacity with time, a process called intestinal adaptation. Approximately 50% of patients are able to be weaned from parenteral nutrition (PN) within 2 years.[3]Capriati T, Mosca A, Alterio T, et al. To wean or not to wean: the role of autologous reconstructive surgery in the natural history of pediatric short bowel syndrome on behalf of Italian Society for Gastroenterology, Hepatology and Nutrition (SIGENP). Nutrients. 2020 Jul 18;12(7):2136. https://www.doi.org/10.3390/nu12072136 http://www.ncbi.nlm.nih.gov/pubmed/32708377?tool=bestpractice.com [4]Massironi S, Cavalcoli F, Rausa E, et al. Understanding short bowel syndrome: current status and future perspectives. Dig Liver Dis. 2020 Mar;52(3):253-61. https://www.doi.org/10.1016/j.dld.2019.11.013 http://www.ncbi.nlm.nih.gov/pubmed/31892505?tool=bestpractice.com Prognosis is directly related to length of bowel and dependency on PN. The highest-risk patients are those with <50 cm of remaining bowel, those with proximal jejunostomies, and those with mesenteric thrombosis or radiation enteritis as an underlying etiology.[17]Messing B, Crenn P, Beau P, et al. Long-term survival and parenteral nutrition dependence in adult patients with the short bowel syndrome. Gastroenterology. 1999 Nov;117(5):1043-50. http://www.ncbi.nlm.nih.gov/pubmed/10535866?tool=bestpractice.com Some patients have lived up to 50 years or more; 5-year survival rate in patients with no malignancy is approximately 75%.[17]Messing B, Crenn P, Beau P, et al. Long-term survival and parenteral nutrition dependence in adult patients with the short bowel syndrome. Gastroenterology. 1999 Nov;117(5):1043-50. http://www.ncbi.nlm.nih.gov/pubmed/10535866?tool=bestpractice.com

Adults

Limited small bowel resection

• Patients with >100 cm of residual bowel and an intact small bowel-colonic anastomosis with the entire colon remaining have an excellent prognosis. Intestinal adaptation and dietary instruction allows these patients to eventually live normal lives with relatively little morbidity or mortality.

Terminal ileum resection

• Intestinal adaptation cannot replace the terminal ileum's role in bile salt and vitamin B12 absorption. While prognosis is still excellent, lifelong management of bile salt and vitamin malabsorption is required. Fat-soluble vitamin supplementation may be required.

Extensive bowel resection

• Weaning from PN is more difficult in patients with ≤100 cm of residual small bowel, particularly in those who lack a colon or colonic anastomosis.

Intestinal transplantation

• Mortality and morbidity rates are significant, but are improving due to an increase in surgical experience and improved immunosuppression regimens. One-year survival after intestinal transplant for short bowel syndrome is 88%, and 5-year survival is 62%.[47]US Department of Health & Human Services. Organ Procurement and Transplantation Network. Intestine Kaplan-Meier patient survival rates for transplants performed: 2008 - 2015. May 2022 [internet publication]. https://optn.transplant.hrsa.gov/data/view-data-reports/national-data/ The prognosis is worse if patients remain PN dependent.

Children

Children usually acquire SBS because of a congenital abnormality or a catastrophic event such as midgut volvulus. It remains one of the most morbid conditions of infancy and childhood, with survival rates from 73% to 89%.[48]Duro D, Kamin D, Duggan C. Overview of pediatric short bowel syndrome. J Pediatr Gastroenterol Nutr. 2008 Aug;47(supp 1):S33-S36. http://www.ncbi.nlm.nih.gov/pubmed/18667916?tool=bestpractice.com Younger children may have a more adaptable intestine than adults; there are anecdotal reports of children living independent of PN with as little as 10 cm of residual small intestine. If children and infants (particularly preterm infants) become dependent on PN, the risk of liver failure is much greater than in adults.