Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Short bowel syndrome


See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.


Your Question

My daughter was born with a jejunal atresia resulting in Short Bowel Syndrome.  I was curious as to long term prognosis and other relevant information.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is jejunal atresia?

Jejunal atresia is a genetic disorder in which people are born missing part of the fold of the stomach membrane that connects the small intestine to the back wall of the abdomen. As a result, one of the three portions of the small intestine (the jejunum) twists around one of the arteries of the colon called the marginal artery and causes a blockage (atresia). Jejunal atresia is the most common cause of bowel obstruction in the newborn.[1][2] The defect may occur alone (isolated) or with other birth defects.  The symptoms and treatment vary from patient to patient.
Last updated: 6/8/2011

What is short bowel syndrome?

Short bowel syndrome is a group of problems involving the small intestine that causes poor absorption of nutrients from food.[3] The main cause of short bowel syndrome is surgical removal of half or more of the small intestine to treat intestinal diseases, injuries, or defects present at birth. Signs and symptoms may include diarrhea, cramping, bloating, heartburn, dehydration, malnutrition, weight loss, food sensitivities, weakness, and fatigue. Examples of treatment include diet and nutritional support, and in some cases intestinal transplantation.[3]
Last updated: 4/29/2011

What is the typical long term prognosis of children with short bowel syndrome?

People with short bowel syndrome can lead a productive, lengthy, and happy life if their condition is managed appropriately.[4] Predictors of the overall long term prognosis of children with small bowel syndrome is influenced by the size and location of the resected intestine (i.e., whether it involves the ileocecal valve, duodenum, jejunum, or ileum),[5][6] and the development of liver disease (e.g., cholestasis).[6] The following free full-text articles provide further details regarding factors affecting long-term prognosis:

Spencer AU et al., Pediatric short bowel syndrome: Redefining predictors of success. Ann Surg. 2005 Sept;242(3):403-412. 
Brizee LS. Short Bowel Syndrome. University of Washington. 2007. Available at: http://depts.washington.edu/growing/Assess/SBS.htm

Initially, all people with short bowel syndrome require total parenteral nutrition (TPN). The goal of treatment is to gradually decrease the requirement for TPN and at best, to eliminate its need.

Some children with short bowel syndrome are able to eat by mouth and digest food in a matter of weeks to months[5] (optimal adaption of the intestine may evolve over the course of 1 to 2 years).[4] Your child's physician should counsel you regarding your child's nutritional status, treatment, and goals. Once children are able to eat, their doctor will likely recommend some modifications to their diet, and possibly vitamin and other nutrient supplementations. 

Children who are off of parenteral nutrition support still remain at risk for dehydration, bacterial overgrowth, and nutritional deficiencies. As a result they require long-term, regular monitoring. Symptoms of gastroenteritis should be reported to their doctors right away. Regular treatment with antibiotics to treat/prevent infections is often required.[5]

Despite careful treatment, some children with short bowel syndrome have very poor digestion and are unable to get adequate nutrients from diet alone. These children require long term TPN. In addition to the risks described above (i.e., dehydration, bacterial overgrowth, nutritional deficiencies), there are a number of additional challenges that can occur with long term TPN use (e.g., catheter infection, liver disease). The Oley Foundation exists to provide people on TPN and their families with information and support. The Foundation may be a helpful resource for learning more about long term prognosis and quality of life impacts of long term TPN use:

The Oley Foundation
214 Hun Memorial, MC-28
Albany Medical Center
Albany, NY 12208-3478
Toll-free: 1-800-776-OLEY 
Phone: 1-518-262-5079
Fax: 1-518-262-5528
Web site: http://www.oley.org/
Last updated: 6/8/2011

Where can I find further information resources on short bowel syndrome?

Please click on the link below to view a list of resources on our Short bowel syndrome Web page.
http://rarediseases.info.nih.gov/GARD/Condition/1502/Short_bowel_syndrome.aspx/Showall#Resources
Last updated: 6/8/2011

References
  • Bianchi DW, Crombleholme TM, D'Alton ME. Fetology. US: McGraw-Hill Companies; 2000;
  • Reddy KS. NORD Guide to Rare Disorders. Philadelphia: Lippincott Williams & Wilkins; 2003;
  • Short Bowel Syndrome. National Digestive Diseases Information Clearinghouse. http://digestive.niddk.nih.gov/ddiseases/pubs/shortbowel/index.htm. Accessed 4/29/2011.
  • Buchman AL, Scholapio J, Fryer J. AGA Technical Review on Short Bowel Syndrome and Intestinal Transplantation. Gastroenterology. 2003;
  • Brizee LS. Short bowel syndrome. University of Washington Children's Hospital and Regional Medical Center. 2007; http://depts.washington.edu/growing/Assess/SBS.htm. Accessed 4/29/2011.
  • Spencer AU et al. Pediatric short bowel syndrome: Redefining predictors of success. Ann Surg. 2005 Sept;