Short bowel syndrome

Short bowel syndrome
Synonyms short gut syndrome, short gut, intestinal failure
A piece of diseased ileum following removal by surgery.
Classification and external resources
Specialty gastroenterology
ICD-10 K91.2
ICD-9-CM 579.3
DiseasesDB 12026
MedlinePlus 000237
eMedicine med/2746 ped/2088
MeSH D012778

Short bowel syndrome (SBS, or simply short gut) is a malabsorption disorder caused by a lack of functional small intestine.[1] The primary symptom is diarrhea, which can result in dehydration, malnutrition, and weight loss. Other symptoms may include bloating, heartburn, feeling tired, lactose intolerance, and foul smelling stool.[2] Complications can include anemia and kidney stones.[3]

Most cases are due to the surgical removal of a large portion of the small intestine.[2] This is most often required due to Crohn's disease in adults and necrotising enterocolitis in young children.[3] Other causes include damage to the small intestine from other means and being born with an abnormally short intestine.[2] It usually does not develop until less than 2 m (6.6 ft) of the normally 6.1 m (20 ft) small intestine remains.[1][2]

Treatment may include a specific diet, medications, or surgery. The diet may include slightly salty and slightly sweet liquids, vitamin and mineral supplements, small frequent meals, and the avoidance of high fat food. Occasionally nutrients need to be given through an intravenous line, known as parenteral nutrition. Medications used may include antibiotics, antacids, loperamide, teduglutide, and growth hormone. Different types of surgery, including an intestinal transplant, may help some people.[2]

Short bowel syndrome newly occurs in about three per million people each year.[2] There are estimated to be about 15,000 people with the condition in the United States.[3] It is classified as a rare disease by the European Medicines Agency.[4] Outcomes depend on the amount of bowel remaining and whether or not the small bowel remains connected with the large bowel.[3]

Definition

Intestinal failure is decreased intestinal function such that nutrients, water, and electrolytes are not sufficiently absorbed. Short bowel syndrome is when there is less than 2 m (6.6 ft) of working bowel and is the most common cause of intestinal failure.[1]

Signs and symptoms

The symptoms of short bowel syndrome can include:

Persons with short bowel syndrome may have complications caused by malabsorption of vitamins and minerals, such as deficiencies in vitamins A, D, E, K, B9 (folic acid), and B12, calcium, magnesium, iron, and zinc. These may appear as anemia, hyperkeratosis (scaling of the skin), easy bruising, muscle spasms, poor blood clotting, and bone pain.

Causes

Short bowel syndrome in adults and children is usually caused by surgery. This surgery may be done for:

Some children are also born with an abnormally short small intestine, known as congenital short bowel.[2]

Pathophysiology

The length of the small intestine can vary greatly, from as short as 2.75 m (9.0 ft) to as long as 10.49 m (34.4 ft).[5] On average it is about 6.1 m (20 ft).[2] Due to this variation it is recommended that following surgery the amount of bowel remaining be specified rather than the amount removed.[5]

Short bowel syndrome usually develops when there is less than 2 meters (6.6 feet) of the small intestine left to absorb sufficient nutrients.

In a process called intestinal adaptation, physiological changes to the remaining portion of the small intestine occur to increase its absorptive capacity. These changes include:

Treatments

Symptoms of short bowel syndrome are usually addressed with medication. These include:

In 2004, the USFDA approved a therapy that reduces the frequency and volume of total parenteral nutrition (TPN), comprising: NutreStore (oral solution of glutamine) and Zorbtive (growth hormone, of recombinant DNA origin, for injection) together with a specialized oral diet.[6] In 2012, an advisory panel to the USFDA voted unanimously to approve for treatment of SBS the agent teduglutide, a glucagon-like peptide-2 analog developed by NPS Pharmaceuticals, who intend to market the agent in the United States under the brandname Gattex.[7] Teduglutide had been previously approved for use in Europe and is marketed under the brand Revestive by Nycomed.[7]

Surgical procedures to lengthen dilated bowel include the Bianchi procedure, where the bowel is cut in half and one end is sewn to the other, and a newer procedure called serial transverse enteroplasty (STEP), where the bowel is cut and stapled in a zigzag pattern. Heung Bae Kim, MD, and Tom Jaksic, MD, both of Children's Hospital Boston, devised the STEP procedure in the early 2000s. The procedure lengthens the bowel of children with SBS and may allow children to avoid the need for intestinal transplantation. As of June 2009, Kim and Jaksic have performed 18 STEP procedures.[8] The Bianchi and STEP procedures are usually performed by pediatric surgeons at quaternary hospitals who specialize in small bowel surgery.

Prognosis

There is no cure for short bowel syndrome except transplant. In newborn infants, the 4-year survival rate on parenteral nutrition is approximately 70%. In newborn infants with less than 10% of expected intestinal length, 5 year survival is approximately 20%.[9] Some studies suggest that much of the mortality is due to a complication of the total parenteral nutrition (TPN), especially chronic liver disease.[10] Much hope is vested in Omegaven, a type of lipid TPN feed, in which recent case reports suggest the risk of liver disease is much lower.[11]

Although promising, small intestine transplant has a mixed success rate, with postoperative mortality rate of up to 30%. One-year and 4-year survival rate are 90% and 60%, respectively.

References

  1. 1 2 3 Pironi, L (April 2016). "Definitions of intestinal failure and the short bowel syndrome.". Best practice & research. Clinical gastroenterology. 30 (2): 173–85. PMID 27086884.
  2. 1 2 3 4 5 6 7 8 "Short Bowel Syndrome". NIDDK. July 2015. Retrieved 20 October 2016.
  3. 1 2 3 4 Ferri, Fred F. (2014). Ferri's Clinical Advisor 2015: 5 Books in 1. Elsevier Health Sciences. p. 1074. ISBN 9780323084307.
  4. ↑ "Short bowel syndrome", orphanet, February 2012, retrieved November 16, 2012
  5. 1 2 DiBaise, John K.; Parrish, Carol Rees; Thompson, Jon S. (2016). Short Bowel Syndrome: Practical Approach to Management. CRC Press. p. 31. ISBN 9781498720809.
  6. ↑ Byrne, Theresa A.; Wilmore, Douglas W.; et al. (November 2005), "Growth Hormone, Glutamine, and an Optimal Diet Reduces Parenteral Nutrition in Patients With Short Bowel Syndrome: A Prospective, Randomized, Placebo-Controlled, Double-Blind Clinical Trial", Ann. Surg., 242 (5): 655–661, doi:10.1097/01.sla.0000186479.53295.14
  7. 1 2 Debra Sherman (October 16, 2012). Leslie Adler; Matthew Lewis, eds. "FDA advisers back NPS's drug for short bowel syndrome". Reuters. Retrieved November 16, 2012.
  8. ↑ Innovation at Work: The STEP Procedure, Boston Children's Hospital, Center for Advanced Intestinal Rehabilitation, retrieved June 17, 2010
  9. ↑ Spencer AU, Neaga A, West B, et al. (September 2005). "Pediatric short bowel syndrome: redefining predictors of success". Ann. Surg. 242 (3): 403–9; discussion 409–12. doi:10.1097/01.sla.0000179647.24046.03. PMC 1357748Freely accessible. PMID 16135926. (mean follow-up time was 5.1 years)
  10. ↑ Vanderhoof JA, Langnas AN (1997). "Short-bowel syndrome in children and adults". Gastroenterology. 113 (5): 1767–78. doi:10.1053/gast.1997.v113.pm9352883. PMID 9352883.
  11. ↑ Gura KM, Duggan CP, Collier SB, et al. (2006). "Reversal of parenteral nutrition-associated liver disease in two infants with short bowel syndrome using parenteral fish oil: implications for future management". Pediatrics. 118 (1): e197–201. doi:10.1542/peds.2005-2662. PMID 16818533.
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