Short bowel syndrome

Short bowel syndrome
Resected diseased ileum.
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, also short gut syndrome or simply short gut) is a malabsorption disorder caused by the surgical removal of the small intestine, or rarely due to the complete dysfunction of a large segment of bowel. Most cases are acquired, although some children are born with a congenital short bowel. It usually does not develop unless more than two thirds of the small intestine have been removed. SBS has been designated a rare disease by the EMA,^[1] and is eligible for orphan drug designation according to the U.S. Food and Drug Administration (FDA).^[2] There are roughly 3,500 adults and 9,000 children with SBS in the USA alone.

Signs and symptoms

The symptoms of short bowel syndrome can include:

Abdominal pain
Diarrhea and steatorrhea (oily or sticky stool, which can be malodorous)
Fluid depletion
Weight loss and malnutrition
Fatigue

Patients 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 for:

Crohn's disease, an inflammatory disorder of the digestive tract
Volvulus, a spontaneous twisting of the small intestine that cuts off the blood supply and leads to tissue death
Tumors of the small intestine
Injury or trauma to the small intestine
Necrotizing enterocolitis (premature newborn)
Bypass surgery to treat obesity
Surgery to remove diseases or damaged portion of the small intestine

Pathophysiology

In healthy adults, the small intestine has an average length of approximately 6 meters (19.7 feet). Short bowel syndrome usually develops when there is less than 2 meters (6.6 feet) of the small intestine left to absorb sufficient nutrients.

Short bowel syndrome caused by the surgical removal of a portion of the bowel may be a temporary condition, due to the adaptive property of the small intestine.

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:

Enlargement and lengthening of the villi found in the lining
Increase in the diameter of the small intestine
Slow down in peristalsis or movement of food through the small intestine

Treatments

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

Anti-diarrheal medicine (e.g. loperamide, codeine)
Vitamin, mineral supplements and L-glutamine powder mixed with water
H2 blocker and proton pump inhibitors to reduce stomach acid
Lactase supplement (to improve the bloating and diarrhoea associated with lactose intolerance)

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.^[3] 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.^[4] Teduglutide had been previously approved for use in Europe and is marketed under the brand Revestive by Nycomed.^[4]

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.^[5] 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%.^[6] Some studies suggest that much of the mortality is due to a complication of the total parenteral nutrition (TPN), especially chronic liver disease.^[7] 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.^[8]

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

↑ "Short bowel syndrome", orphanet, February 2012, retrieved November 16, 2012
↑ "teduglutide", Orphan Drug Product Designations (database record) (U.S. Food and Drug Administration), June 2000, retrieved November 16, 2012
↑ 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
1 2 Debra Sherman (October 16, 2012). Leslie Adler and Matthew Lewis, ed. "FDA advisers back NPS's drug for short bowel syndrome". Reuters. Retrieved November 16, 2012.
↑ Innovation at Work: The STEP Procedure, Boston Children's Hospital, Center for Advanced Intestinal Rehabilitation, retrieved June 17, 2010
↑ 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 1357748. PMID 16135926. (mean follow-up time was 5.1 years)
↑ 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.
↑ 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.

External links

Diseases of the digestive system (primarily K20–K93, 530–579)

Upper GI tract

Esophagus	Esophagitis Candidal Eosinophilic Herpetiform Rupture Boerhaave syndrome Mallory-Weiss syndrome UES Zenker's diverticulum LES Barrett's esophagus Esophageal motility disorder Nutcracker esophagus Achalasia Diffuse esophageal spasm Gastroesophageal reflux disease (GERD) Laryngopharyngeal reflux (LPR) Esophageal stricture Megaesophagus

Stomach	Gastritis Atrophic Ménétrier's disease Gastroenteritis Peptic (gastric) ulcer Cushing ulcer Dieulafoy's lesion Dyspepsia Pyloric stenosis Achlorhydria Gastroparesis Gastroptosis Portal hypertensive gastropathy Gastric antral vascular ectasia Gastric dumping syndrome Gastric volvulus

Lower GI tract:
Intestinal/
Enteropathy

Small intestine (Duodenum/Jejunum/Ileum)	Enteritis Duodenitis Jejunitis Ileitis Peptic (duodenal) ulcer Curling's ulcer Malabsorption: Coeliac Tropical sprue Blind loop syndrome Small bowel bacterial overgrowth syndrome Whipple's Short bowel syndrome Steatorrhea Milroy disease bile acid malabsorption

Large intestine (Appendix/Colon)	Appendicitis Colitis Pseudomembranous Ulcerative Ischemic Microscopic Collagenous Lymphocytic Functional colonic disease IBS Intestinal pseudoobstruction / Ogilvie syndrome Megacolon / Toxic megacolon Diverticulitis/Diverticulosis

Large and/or small	Enterocolitis Necrotizing Gastroenterocolitis IBD Crohn's disease Vascular: Abdominal angina Mesenteric ischemia Angiodysplasia Bowel obstruction: Ileus Intussusception Volvulus Fecal impaction Constipation Diarrhea Infectious Intestinal adhesions

Rectum	Proctitis Radiation proctitis Proctalgia fugax Rectal prolapse Anismus

Anal canal	Anal fissure/Anal fistula Anal abscess Anal dysplasia Pruritus ani

GI bleeding/BIS

Accessory

Liver	Hepatitis Viral hepatitis Autoimmune hepatitis Alcoholic hepatitis Cirrhosis PBC Fatty liver NASH Vascular Budd-Chiari syndrome Hepatic veno-occlusive disease Portal hypertension Nutmeg liver Alcoholic liver disease Liver failure Hepatic encephalopathy Acute liver failure Liver abscess Pyogenic Amoebic Hepatorenal syndrome Peliosis hepatis Metabolic disorders Wilson's Disease Hemochromatosis

Gallbladder	Cholecystitis Gallstones/Cholecystolithiasis Cholesterolosis Rokitansky-Aschoff sinuses Postcholecystectomy syndrome Porcelain gallbladder

Bile duct/ Other biliary tree	Cholangitis Primary sclerosing cholangitis Secondary sclerosing cholangitis Ascending Cholestasis/Mirizzi's syndrome Biliary fistula Haemobilia Gallstones/Cholelithiasis Common bile duct Choledocholithiasis Biliary dyskinesia Sphincter of Oddi dysfunction

Pancreatic	Pancreatitis Acute Chronic Hereditary Pancreatic abscess Pancreatic pseudocyst Exocrine pancreatic insufficiency Pancreatic fistula

Abdominopelvic

Hernia	Diaphragmatic Congenital Hiatus Inguinal Indirect Direct Umbilical Femoral Obturator Spigelian Lumbar Petit's Grynfeltt-Lesshaft Undefined location Incisional Internal hernia

Peritoneal	Peritonitis Spontaneous bacterial peritonitis Hemoperitoneum Pneumoperitoneum

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