Intestinal pseudo-obstruction

Intestinal pseudo-obstruction 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.[1] Clinical features can include abdominal pain, nausea, severe distension, vomiting, dysphagia, diarrhea and constipation, depending upon the part of the gastrointestinal tract involved.[2] The condition can begin at any age and it can be a primary condition (idiopathic or inherited) or caused by another disease (secondary).[3]

Intestinal pseudo-obstruction
SpecialtyGastroenterology

It can be chronic[4] or acute.[5]

Causes

In primary chronic intestinal pseudo-obstruction (the majority of chronic cases), the condition may be caused by an injury to the smooth muscle (myopathic) or the nervous system (neuropathic) of the gastrointestinal tract.[6]

In some cases there appears to be a genetic association.[7] One form has been associated with DXYS154.[8]

Secondary chronic intestinal pseudo-obstruction can occur as a consequence of a number of other conditions, including Kawasaki disease,[9] Parkinson's disease, Chagas' disease, Hirschsprung's disease, intestinal hypoganglionosis, collagen vascular diseases, mitochondrial disease, endocrine disorders and use of certain medications.[6] The term may be used synonymously with enteric neuropathy if a neurological cause is suspected.

Clinical manifestations

Clinical features of intestinal pseudo-obstruction can include abdominal pain, nausea, severe distension, vomiting, dysphagia, diarrhea and constipation, depending upon the part of the gastrointestinal tract involved.[2] In addition, in the moments in which abdominal colic occurs, an abdominal x-ray shows intestinal air fluid level. All of these features are also similar in true mechanical obstruction of the bowel.[3]

Diagnosis

Attempts must be made to determine whether there is a secondary cause amenable to treatment.[6]

Primary (idiopathic) intestinal pseudo-obstruction is diagnosed based on motility studies, x-rays and gastric emptying studies.

Treatment

Secondary chronic intestinal pseudo-obstruction is managed by treating the underlying condition.

There is no cure for primary chronic intestinal pseudo-obstruction. It is important that nutrition and hydration is maintained, and pain relief is given. Drugs that increase the propulsive force of the intestines have been tried, as have different types of surgery.

Medical treatment

Prucalopride,[10][11] pyridostigmine,[3] metoclopramide, cisapride, and erythromycin may be used, but they have not been shown to have great efficacy. In such cases, treatment is aimed at managing the complications. Linaclotide is a new drug that received approval from Food and Drug Administration in August 2012 and looks promising in the treatment of chronic intestinal pseudo-obstruction, gastroparesis and inertia coli.[12][13]

Intestinal stasis, which may lead to bacterial overgrowth and subsequently, diarrhea or malabsorption, is treated with antibiotics.

Nutritional deficiencies are treated by encouraging patients to avoid food high in fat and fibre, which are harder to digest and increase abdominal distention and discomfort, and have small, frequent meals (5–6 per day), focusing on liquids and soft food. Reducing intake of poorly absorbed sugar alcohols may be of benefit. Referral to an accredited dietitian is recommended. If dietary changes are unsuccessful in meeting nutritional requirements and stemming weight loss, enteral nutrition is used. Many patients eventually require parenteral nutrition.[6]

Total parenteral nutrition (TPN) is a form of long-term nutritional treatment needed for patients that have severe pseudoobstruction. After a period of no improvement of intestinal function or motility the decision to start TPN will be made, and the surgical procedure to add a long-term, more permanent IV to administer TPN will occur. Types of IV catheters to be placed will be a PICC line or central line which include mediports, Broviac, or Hickman lines depending on how long the physicians believe the patient will require TPN. Patients that are deemed TPN dependent will require constant checkups to monitor the catheter is working properly, check liver enzyme levels and look for signs of blood infections, as catheter blockage, liver damage, and infections of catheters are the main complications associated with long term TPN use and can result in sepsis and/or additional surgeries if not properly monitored. TPN nutritional feeds are given over a period of several hours to all day infusions, and are a mixture of all the vitamins, minerals, and calories similar to what one would get eating orally daily as well as any other specific nutritional needs the patient has at the moment. TPN format is typically changed depending on loss/gain of weight and bloodwork results, and is specially formulated to meet each individual patient's needs.[14]

Use of octreotide has been described.[15][16]

Cannabis has long been known to limit or prevent nausea and vomiting from a variety of causes. This has led to extensive investigations that have revealed an important role for cannabinoids and their receptors in the regulation of nausea and emesis. With the discovery of the endocannabinoid system, novel ways to regulate both nausea and vomiting have been discovered that involve the production of endogenous cannabinoids acting centrally.[17] The plant cannabis has been used in clinics for centuries, and has been known to be beneficial in a variety of gastrointestinal diseases, such as emesis, diarrhea, inflammatory bowel disease and intestinal pain. Moreover, modulation of the endogenous cannabinoid system in the gastrointestinal tract may provide a useful therapeutic target for gastrointestinal disorders.[18] While some GI disorders may be controlled by diet and pharmaceutical medications, others are poorly moderated by conventional treatments. Symptoms of GI disorders often include cramping, abdominal pain, inflammation of the lining of the large and/or small intestine, chronic diarrhea, rectal bleeding and weight loss. Patients with these disorders frequently report using cannabis therapeutically.[19]

In a 2012 animal study, cannabichromene was shown to normalize gastrointestinal hypermotility without reducing the transit time. The study notes that this result is of potential clinical interest, as the only drugs available for intestinal dysmotility are often associated with constipation.[20]

Procedures

Intestinal decompression by tube placement in a small stoma can also be used to reduce distension and pressure within the gut. The stoma may be a gastrostomy, jejunostomy, ileostomy or cecostomy, and may also be used to feed, in the case of gastrostomy and jejunostomy, or flush the intestines.

Colostomy or ileostomy can bypass affected parts if they are distal to (come after) the stoma. For instance, if only the large colon is affected, an ileostomy may be helpful. Either of these ostomies are typically placed at or a few centimeters below the patients belly button per doctor recommendation based on the affected area of the intestines as well as concerns for patient comfort and future physical growth for children.[14]

The total removal of the colon, called a colectomy or resection of affected parts of the colon may be needed if part of the gut dies (for instance toxic megacolon), or if there is a localised area of dysmotility.

Gastric and colonic pacemakers have been tried. These are strips placed along the colon or stomach which create an electric discharge intended to cause the muscle to contract in a controlled manner.

A potential solution, albeit radical, is a multi-organ transplant. The operation involved transplanting the pancreas, stomach, duodenum, small intestine, and liver, and was performed by Doctor Kareem Abu-Elmagd on Gretchen Miller.[21]

References
  1. Stanghellini V, Cogliandro RF, De Giorgio R, et al. (May 2005). "Natural history of chronic idiopathic intestinal pseudo-obstruction in adults: a single center study". Clinical Gastroenterology and Hepatology. 3 (5): 449–58. doi:10.1016/S1542-3565(04)00675-5. PMID 15880314.
  2. De Giorgio R, Sarnelli G, Corinaldesi R, Stanghellini V (November 2004). "Advances in our understanding of the pathology of chronic intestinal pseudo-obstruction". Gut. 53 (11): 1549–52. doi:10.1136/gut.2004.043968. PMC 1774265. PMID 15479666.
  3. Antonucci A, Fronzoni L, Cogliandro L, et al. (May 2008). "Chronic intestinal pseudo-obstruction". World Journal of Gastroenterology. 14 (19): 2953–61. doi:10.3748/wjg.14.2953. PMC 2712158. PMID 18494042.
  4. Sutton DH, Harrell SP, Wo JM (February 2006). "Diagnosis and management of adult patients with chronic intestinal pseudoobstruction". Nutrition in Clinical Practice. 21 (1): 16–22. doi:10.1177/011542650602100116. PMID 16439766.
  5. Saunders MD (October 2004). "Acute colonic pseudoobstruction". Current Gastroenterology Reports. 6 (5): 410–6. doi:10.1007/s11894-004-0059-5. PMID 15341719.
  6. Gabbard SL, Lacy BE (June 2013). "Chronic intestinal pseudo-obstruction". Nutrition in Clinical Practice. 28 (3): 307–16. doi:10.1177/0884533613485904. PMID 23612903.
  7. Guzé CD, Hyman PE, Payne VJ (January 1999). "Family studies of infantile visceral myopathy: a congenital myopathic pseudo-obstruction syndrome". American Journal of Medical Genetics. 82 (2): 114–22. doi:10.1002/(SICI)1096-8628(19990115)82:2<114::AID-AJMG3>3.0.CO;2-H. PMID 9934973.
  8. Auricchio A, Brancolini V, Casari G, et al. (April 1996). "The locus for a novel syndromic form of neuronal intestinal pseudoobstruction maps to Xq28". American Journal of Human Genetics. 58 (4): 743–8. PMC 1914695. PMID 8644737.
  9. Akikusa JD, Laxer RM, Friedman JN (May 2004). "Intestinal pseudoobstruction in Kawasaki disease". Pediatrics. 113 (5): e504–6. doi:10.1542/peds.113.5.e504. PMID 15121996.
  10. Briejer MR, Prins NH, Schuurkes JA (October 2001). "Effects of the enterokinetic prucalopride (R093877) on colonic motility in fasted dogs". Neurogastroenterology and Motility. 13 (5): 465–72. doi:10.1046/j.1365-2982.2001.00280.x. PMID 11696108.
  11. Oustamanolakis P, Tack J (February 2012). "Prucalopride for chronic intestinal pseudo-obstruction". Alimentary Pharmacology & Therapeutics. 35 (3): 398–9. doi:10.1111/j.1365-2036.2011.04947.x. PMID 22221087.
  12. "Textbook of Gastroenterology" by Tadataka Yamada, Editor John Wiley & Sons, 2011 ISBN 144435941X, 9781444359411
  13. https://www.inspire.com/groups/agmd-gi-motility/discussion/some-good-news-and-a-new-drug-coming-soon/%5B%5D
  14. Heneyke S, Smith VV, Spitz L, Milla PJ (July 1999). "Chronic intestinal pseudo-obstruction: treatment and long term follow up of 44 patients". Archives of Disease in Childhood. 81 (1): 21–7. doi:10.1136/adc.81.1.21. PMC 1717974. PMID 10373127.
  15. Sharma S, Ghoshal UC, Bhat G, Choudhuri G (November 2006). "Gastric adenocarcinoma presenting with intestinal pseudoobstruction, successfully treated with octreotide". Indian Journal of Medical Sciences. 60 (11): 467–70. doi:10.4103/0019-5359.27974. PMID 17090868.
  16. Sørhaug S, Steinshamn SL, Waldum HL (April 2005). "Octreotide treatment for paraneoplastic intestinal pseudo-obstruction complicating SCLC". Lung Cancer. 48 (1): 137–40. doi:10.1016/j.lungcan.2004.09.008. PMID 15777981.
  17. Sharkey KA, Darmani NA, Parker LA (January 2014). "Regulation of nausea and vomiting by cannabinoids and the endocannabinoid system". European Journal of Pharmacology. 722: 134–46. doi:10.1016/j.ejphar.2013.09.068. PMC 3883513. PMID 24184696.
  18. Lin XH, Wang YQ, Wang HC, Ren XQ, Li YY (August 2013). "Role of endogenous cannabinoid system in the gut" (PDF). Sheng Li Xue Bao. 65 (4): 451–60. PMID 23963077.
  19. Gastrointestinal Disorders.
  20. Izzo AA, Capasso R, Aviello G, Borrelli F, Romano B, Piscitelli F, Gallo L, Capasso F, Orlando P, Di Marzo V (2012-05-17). "Inhibitory effect of cannabichromene, a major non-psychotropic cannabinoid extracted from Cannabis sativa, on inflammation-induced hypermotility in mice". British Journal of Pharmacology. 166 (4): 1444–60. doi:10.1111/j.1476-5381.2012.01879.x. PMC 3417459. PMID 22300105.
  21. Discovery Channel - Multiorgan transplant
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