Short bowel syndrome

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Short bowel syndrome
Other namesShort gut syndrome, short gut, intestinal failure
ResectedIleum.jpg
A piece of diseased ileum following removal by surgery.
Specialty Gastroenterology
Symptoms Diarrhea, dehydration, malnutrition, weight loss [1]
Complications Anemia, kidney stones [2]
CausesSurgical removal of a large portion of the small intestine [1]
Risk factors Crohn's disease, necrotising enterocolitis [2]
TreatmentSpecific diet, medications, surgery [1]
Medication Antibiotics, antacids, loperamide, teduglutide, growth hormone [1]
Prognosis Depends on amount of small bowel remaining [2]
Frequency3 per million per year, in which is a rare disorder [1]

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

Contents

Most cases are due to the surgical removal of a large portion of the small intestine. [1] This is most often required due to Crohn's disease in adults and necrotising enterocolitis in young children. [2] Other causes include damage to the small intestine from other means and being born with an abnormally short intestine. [1] 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] [3]

Treatment may include a specific diet, medications, or surgery. [1] The diet may include slightly salty and slightly sweet liquids, vitamin and mineral supplements, small frequent meals, and the avoidance of high fat food. [1] Occasionally, nutrients need to be given through an intravenous line, known as parenteral nutrition. [1] Medications used may include antibiotics, antacids, loperamide, teduglutide, and growth hormone. [1] Different types of surgery, including an intestinal transplant, may help some people. [1]

Short bowel syndrome newly occurs in about three per million people each year. [1] There are estimated to be about 15,000 people with the condition in the United States. [2] The prevalence in the United States is approximately 30 cases per million and in Europe it is approximately 1.4 cases per million (but the rate varies widely between countries). [4] The prevalence of short bowel syndrome has increased by more than 2 fold in the last 40 years. [4] It is classified as a rare disease by the European Medicines Agency. [5] Outcomes depend on the amount of bowel remaining and whether or not the small bowel remains connected with the large bowel. [2]

Signs and symptoms

The symptoms of short bowel syndrome can include:[ citation needed ]

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.[ citation needed ]

Causes

Short bowel syndrome in adults and children is most commonly caused by surgery (intestinal resection). [4] In those who undergo intestinal resection, approximately 15% eventually develop small bowel syndrome (75% of those due to 1 large resection and 25% due to multiple separate intestinal resections). [4] This surgery may be done for:

Some children are also born with an abnormally short small intestine, known as congenital short bowel. [1]

Surgical complications, requiring re-surgery, are a common cause of small bowel syndrome, contributing up to 50% of cases based on some estimates. [4] These surgical complications include internal hernias, volvuli, ischemia or profound hypotension. [4]

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). [6] On average it is about 6.1 m (20 ft). [1] Due to this variation it is recommended that following surgery the amount of bowel remaining be specified rather than the amount removed. [6]

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

The resection of specific areas of the small bowel can lead to distinct symptoms in short bowel syndrome. The resection of the ileum leads to a malabsorption of vitamin B12, bile acids and the fat soluble vitamins A, D, E and K. [4] Loss of the distal ileum also leads to loss of inhibitory hormones; leading to gastric hypersecretion, intestinal hypermotility (decreases in the intestinal transit time) leading to secretory diarrhea and macronutrient, micronutrient, vitamin and mineral deficiencies. [4] Loss of the ileocecal valve leads to small intestinal bacterial overgrowth(SIBO) as bacterial flora normally found in the large intestines migrate proximally and colonize the small intestines leading to further malabsorption. [4] SIBO leads to malabsorption as the bacteria colonizing the small intestine metabolize nutrients, directly competing with the intestinal absorption of nutrients. The bacteria colonizing the small intestines in SIBO may also cause bile acid deconjugation leading to malabsorption of lipids. [4]

In a process called intestinal adaptation, physiological changes to the remaining portion of the small intestine occur to increase its absorptive capacity. These changes usually take place over 1–2 years. [7] These changes include:

Osteoporosis is a very common comorbidity in people with short bowel syndrome who are on parenteral nutrition, with an estimated prevalence of 57-67%. [4] The contributing factors to the osteoporosis include malnutrition, vitamin D deficiency due to malabsorption and vitamin D deficiency due to scarce sunlight exposure due to chronic disability. [4]

Diagnosis

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. [3]

Treatments

Symptoms of short bowel syndrome are usually addressed with medication. These include:[ citation needed ]

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. [8] After 24 weeks of successful Phase III patient treatment trials, Teduglutide was shown to be relatively safe and effective with varying degrees of benefits and adverse effects per patient. [9] Adequate safety evaluations prove to be difficult due to a limited sample size available for study, however. [9] 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. [10] Teduglutide had been previously approved for use in Europe and is marketed under the brand Revestive by Nycomed. [10]

Surgery

Prophylactic surgery

Antiperistaltic transverse coloplasty, proposed as a prophylactic measure against short bowel syndrome, retains transverse colon function after extensive colectomies, promoting improved stool consistency and quality of life. This technique involves repositioning the colon to simulate its original placement, potentially averting short bowel syndrome-related complications and benefiting patient outcomes. [11]

Therapeutic surgery

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. [12] The Bianchi and STEP procedures are usually performed by pediatric surgeons at quaternary hospitals who specialize in small bowel surgery.[ citation needed ]

Prognosis

After resection, having a remnant small bowel length of less than 75 cm (30 in) and a remaining large bowel length of less than 57% of the original length are both associated with subsequent dependence on parenteral nutrition. [4] 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%. [13] Some studies suggest that much of the mortality is due to a complication of the total parenteral nutrition (TPN), especially chronic liver disease. [14] As of 2006, much hope has been vested in Omegaven, a type of lipid TPN feed, for which recent case reports suggest the risk of liver disease is much lower. [15]

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

See also

Related Research Articles

<span class="mw-page-title-main">Diarrhea</span> Loose or liquid bowel movements

Diarrhea, also spelled diarrhoea or diarrhœa, is the condition of having at least three loose, liquid, or watery bowel movements in a day. It often lasts for a few days and can result in dehydration due to fluid loss. Signs of dehydration often begin with loss of the normal stretchiness of the skin and irritable behaviour. This can progress to decreased urination, loss of skin color, a fast heart rate, and a decrease in responsiveness as it becomes more severe. Loose but non-watery stools in babies who are exclusively breastfed, however, are normal.

<span class="mw-page-title-main">Crohn's disease</span> Type of inflammatory bowel disease

Crohn's disease is a chronic inflammatory bowel disease characterized by recurrent episodes of intestinal inflammation, primarily manifesting as diarrhea and abdominal pain. Unlike ulcerative colitis, inflammation can occur anywhere in the gastrointestinal tract, though it most frequently affects the ileum and colon, involving all layers of the intestinal wall. Symptoms may be non-specific and progress gradually, often delaying diagnosis. About one-third of patients have colonic disease, another third have ileocolic disease, and the remaining third have isolated ileal disease. Systemic symptoms such as chronic fatigue, weight loss, and low-grade fevers are common, along with extraintestinal manifestations affecting organs like the skin and joints. Complications can include bowel obstructions, fistulas, and an increased risk of intestinal cancers.

<span class="mw-page-title-main">Irritable bowel syndrome</span> Functional gastrointestinal disorder

Irritable bowel syndrome (IBS) is a functional gastrointestinal disorder characterized by a group of symptoms that commonly include abdominal pain, abdominal bloating and changes in the consistency of bowel movements. These symptoms may occur over a long time, sometimes for years. IBS can negatively affect quality of life and may result in missed school or work or reduced productivity at work. Disorders such as anxiety, major depression, and chronic fatigue syndrome are common among people with IBS.

<span class="mw-page-title-main">Parenteral nutrition</span> Intravenous feeding

Parenteral nutrition (PN), or intravenous feeding, is the feeding of nutritional products to a person intravenously, bypassing the usual process of eating and digestion. The products are made by pharmaceutical compounding entities or standard pharmaceutical companies. The person receives a nutritional mix according to a formula including glucose, salts, amino acids, lipids and vitamins and dietary minerals. It is called total parenteral nutrition (TPN) or total nutrient admixture (TNA) when no significant nutrition is obtained by other routes, and partial parenteral nutrition (PPN) when nutrition is also partially enteric. It is called peripheral parenteral nutrition (PPN) when administered through vein access in a limb rather than through a central vein as in central venous nutrition (CVN).

In medicine, the fecal fat test is a diagnostic test for fat malabsorption conditions, which lead to excess fat in the feces (steatorrhea).

<span class="mw-page-title-main">Diverticulitis</span> Digestive disease of the large intestine

Diverticulitis, also called colonic diverticulitis, is a gastrointestinal disease characterized by inflammation of abnormal pouches—diverticula—that can develop in the wall of the large intestine. Symptoms typically include lower abdominal pain of sudden onset, but the onset may also occur over a few days. There may also be nausea, diarrhea or constipation. Fever or blood in the stool suggests a complication. People may experience a single attack, repeated attacks, or ongoing "smouldering" diverticulitis.

<span class="mw-page-title-main">Malabsorption</span> Abnormality in absorption of food nutrients across the gastrointestinal tract

Malabsorption is a state arising from abnormality in absorption of food nutrients across the gastrointestinal (GI) tract. Impairment can be of single or multiple nutrients depending on the abnormality. This may lead to malnutrition and a variety of anaemias.

<span class="mw-page-title-main">Small intestinal bacterial overgrowth</span> Medical condition

Small intestinal bacterial overgrowth (SIBO), also termed bacterial overgrowth, or small bowel bacterial overgrowth syndrome (SBBOS), is a disorder of excessive bacterial growth in the small intestine. Unlike the colon, which is rich with bacteria, the small bowel usually has fewer than 100,000 organisms per millilitre. Patients with bacterial overgrowth typically develop symptoms which may include nausea, bloating, vomiting, diarrhea, malnutrition, weight loss, and malabsorption by various mechanisms.

<span class="mw-page-title-main">Colestyramine</span> Pharmaceutical drug

Colestyramine (INN) or cholestyramine (USAN) is a bile acid sequestrant, which binds bile in the gastrointestinal tract to prevent its reabsorption. It is a strong ion exchange resin, which means it can exchange its chloride anions with anionic bile acids in the gastrointestinal tract and bind them strongly in the resin matrix. The functional group of the anion exchange resin is a quaternary ammonium group attached to an inert styrene-divinylbenzene copolymer.

<span class="mw-page-title-main">Blind loop syndrome</span> Medical condition

Blind loop syndrome, also known as stagnant loop syndrome, is a state that occurs when the normal bacterial flora of the small intestine proliferates to numbers that cause significant derangement to the normal physiological processes of digestion and absorption. In some cases of blind loop syndrome, overgrowth of pathogenic non-commensal bacteria has also been noted. It has long been understood that from birth, and throughout life, large amounts of bacteria reside symbiotically within animal gastrointestinal tracts such as the human gastrointestinal tract. The understanding of this gut flora has even led to novel treatments for bowel irregularity that utilize so called "probiotics" or good bacteria that aid in normal digestion. The problem of blind loop syndrome arises when the bacterial colonies residing in the upper gastrointestinal tract begin to grow out of control or are altered in their makeup thereby creating a burden on the normal physiological processes occurring in the small intestine. This results in problems, among others, of: vitamin B12 deficiency, fat malabsorption and steatorrhea, fat-soluble vitamin deficiencies and intestinal wall injury.

<span class="mw-page-title-main">Intestinal pseudo-obstruction</span> Medical condition

Intestinal pseudo-obstruction (IPO) is a clinical syndrome caused by severe impairment in the ability of the intestines to push food through. It is characterized by the signs and symptoms of intestinal obstruction without any lesion in the intestinal lumen. Clinical features mimic those seen with mechanical intestinal obstructions and can include abdominal pain, nausea, abdominal distension, vomiting, dysphagia and constipation depending upon the part of the gastrointestinal tract involved.

<span class="mw-page-title-main">Bowel resection</span> Surgical procedure in which a part of an intestine is removed

A bowel resection or enterectomy is a surgical procedure in which a part of an intestine (bowel) is removed, from either the small intestine or large intestine. Often the word enterectomy is reserved for the sense of small bowel resection, in distinction from colectomy, which covers the sense of large bowel resection. Bowel resection may be performed to treat gastrointestinal cancer, bowel ischemia, necrosis, or obstruction due to scar tissue, volvulus, and hernias. Some patients require ileostomy or colostomy after this procedure as alternative means of excretion. Complications of the procedure may include anastomotic leak or dehiscence, hernias, or adhesions causing partial or complete bowel obstruction. Depending on which part and how much of the intestines are removed, there may be digestive and metabolic challenges afterward, such as short bowel syndrome.

<span class="mw-page-title-main">Microvillous inclusion disease</span> Medical condition

Microvillus inclusion disease, previously known as Davidson's disease, congenital microvillus atrophy and, less specifically, microvillus atrophy, is a rare genetic disorder of the small intestine that is inherited in an autosomal recessive pattern.

Jejunoileal bypass (JIB) was a surgical weight-loss procedure performed for the relief of morbid obesity from the 1950s through the 1970s in which all but 30 cm (12 in) to 45 cm (18 in) of the small bowel were detached and set to the side.

<span class="mw-page-title-main">Jejunostomy</span>

Jejunostomy is the surgical creation of an opening (stoma) through the skin at the front of the abdomen and the wall of the jejunum. It can be performed either endoscopically, or with open surgery.

Bile acid malabsorption (BAM), known also as bile acid diarrhea, is a cause of several gut-related problems, the main one being chronic diarrhea. It has also been called bile acid-induced diarrhea, cholerheic or choleretic enteropathy, bile salt diarrhea or bile salt malabsorption. It can result from malabsorption secondary to gastrointestinal disease, or be a primary disorder, associated with excessive bile acid production. Treatment with bile acid sequestrants is often effective. Depending on the severity of symptoms, it may be recognised as a disability in the United Kingdom under the Equality Act 2010.

Serial transverse enteroplasty (STEP) is a surgical procedure used primarily in the treatment of short bowel syndrome (SBS). In STEP, by making cuts in the intestine and creating a zigzag pattern, surgeons lengthen the amount of bowel available to absorb nutrients. The procedure was first performed in 2003 and more than 100 patients had undergone the surgery by 2013.

Serum-derived bovine immunoglobulin/protein isolate (SBI) is a medical food product derived from bovine serum obtained from adult cows in the United States. It is sold under the name EnteraGam.

<span class="mw-page-title-main">Intestine transplantation</span> Surgical replacement of the small intestine

Intestine transplantation is the surgical replacement of the small intestine for chronic and acute cases of intestinal failure. While intestinal failure can oftentimes be treated with alternative therapies such as parenteral nutrition (PN), complications such as PN-associated liver disease and short bowel syndrome may make transplantation the only viable option. One of the rarest type of organ transplantation performed, intestine transplantation is becoming increasingly prevalent as a therapeutic option due to improvements in immunosuppressive regimens, surgical technique, PN, and the clinical management of pre and post-transplant patients.

Intestinal bypass is a bariatric surgery performed on patients with morbid obesity to create an irreversible weight loss, when implementing harsh restrictions on the diets have failed. Jejunocolic anastomosis was firstly employed. Nonetheless, it led to some unexpected complications such as severe electrolyte imbalance and liver failure. It was then modified to jejunoileal techniques. Viewed as a novel form of treatment for obesity, many intestinal bypass operations were carried out in the 1960s and 1980s. Significant weight loss was observed in patients, but this surgery also resulted in several complications, for instance, nutritional deficiencies and metabolic problems. Due to the presence of surgical alternatives and anti-obesity medications, intestinal bypass is now rarely used.

References

  1. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 "Short Bowel Syndrome". NIDDK. July 2015. Archived from the original on 20 October 2016. Retrieved 20 October 2016.
  2. 1 2 3 4 5 6 7 Ferri FF (2014). Ferri's Clinical Advisor 2015: 5 Books in 1. Elsevier Health Sciences. p. 1074. ISBN   9780323084307. Archived from the original on 2016-10-21.
  3. 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. doi:10.1016/j.bpg.2016.02.011. PMID   27086884.
  4. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 Massironi S, Cavalcoli F, Rausa E, Invernizzi P, Braga M, Vecchi M (March 2020). "Understanding short bowel syndrome: Current status and future perspectives". Digestive and Liver Disease. 52 (3): 253–261. doi:10.1016/j.dld.2019.11.013. hdl: 2434/756015 . PMID   31892505. S2CID   209524482.
  5. ↑ "Short bowel syndrome", orphanet , February 2012, archived from the original on March 4, 2016, retrieved November 16, 2012
  6. 1 2 DiBaise JK, Parrish CR, Thompson JS (2016). Short Bowel Syndrome: Practical Approach to Management. CRC Press. p. 31. ISBN   9781498720809. Archived from the original on 2016-10-20.
  7. 1 2 Buchman AL, Scolapio J, Fryer J (April 2003). "AGA technical review on short bowel syndrome and intestinal transplantation". Gastroenterology. 124 (4): 1111–1134. doi:10.1016/S0016-5085(03)70064-X. PMID   12671904.
  8. ↑ Byrne TA, Wilmore DW, 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, PMC   1409868 , PMID   16244538
  9. 1 2 Jeppesen P (2012). "Therapeutic Advances in Gastroenterology". 5 (3): 159–171. doi:10.1177/1756283X11436318. PMC   3342570 . PMID   22570676.{{cite journal}}: Cite journal requires |journal= (help)
  10. 1 2 Debra Sherman (October 16, 2012). Leslie Adler, Matthew Lewis (eds.). "FDA advisers back NPS's drug for short bowel syndrome". Reuters. Archived from the original on November 11, 2012. Retrieved November 16, 2012.
  11. ↑ Segura-Sampedro JJ, Morales-Soriano R, Rodríguez-Pino JC, Pineño Flores C, Craus-Miguel A (2023-08-26). "Antiperistaltic Transverse Coloplasty: A Salvage Procedure in Extensive Bowel and Colorectal Resections to Avoid Intestinal Failure". Annals of Surgical Oncology. 30 (12): 7236–7239. doi: 10.1245/s10434-023-14165-0 . hdl: 20.500.13003/19357 . ISSN   1534-4681. PMC   10562490 . PMID   37626252.
  12. ↑ Innovation at Work: The STEP Procedure, Boston Children's Hospital, Center for Advanced Intestinal Rehabilitation, archived from the original on May 19, 2011, retrieved June 17, 2010
  13. ↑ 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)
  14. ↑ 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.
  15. ↑ 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. S2CID   46567040.
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