Medicine:Intestinal pseudo-obstruction

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Intestinal pseudo-obstruction
Ogilvie ct coronal.jpg
Pronunciation
  • soo·doe/uhb·struhk·shn
SpecialtyGastroenterology
SymptomsAbdominal pain, nausea, distention, vomiting, dysphagia, and constipation
ComplicationsIntestinal failure, malabsorption, nutrient deficiencies, small intestinal bacterial overgrowth
DurationVaries according to etiology of disease. < 6 months is considered acute
CausesIdiopathic, Kawasaki disease, Parkinson's disease, Chagas disease, Hirschsprung's disease, intestinal hypogangliosis, collagen vascular disease, mitochondrial disease, endocrine disorders, medication side effects
Diagnostic methodSigns and symptoms consistent with a mechanical intestinal obstruction with no identifying lesion.
Differential diagnosisIntestinal obstruction, Crohn's disease, ovarian torsion, ovarian cyst, neoplasm, infection (parasitic)
TreatmentAimed at management of complications (e.g. nutrition, hydration, pain relief).
Prognosis10–25% mortality rate in chronic cases
FrequencyUnknown

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.[1] Clinical features mimic those seen with mechanical intestinal obstructions and can include abdominal pain, nausea, abdominal distension, vomiting, dysphagia and constipation[2][3] depending upon the part of the gastrointestinal tract involved.

It is a difficult condition to diagnose, requiring exclusion of any other mechanical cause of obstruction.[4] Many patients are diagnosed late in the course of disease after additional symptoms are seen. Mortality is also difficult to accurately determine. One retrospective study estimated mortality to be between 10 and 25% for chronic intestinal pseudo-obstruction (CIPO) and to vary greatly depending on the etiology of the condition.[5] When present for less than six months, it is diagnosed as acute IPO[6] or Ogilvie syndrome.[4] Longer than this is considered chronic.[7] Owing to the difficulty of diagnosis, few studies are available which have attempted to estimate its prevalence.[8]

The condition can begin at any age. Most studies describing CIPO are in pediatric populations.[9][10][4] It can be a primary condition (idiopathic or inherited) or caused by another disease (secondary).[11] It can be a result of myriad of etiologies including infectious, parasitic, autoimmune, genetic, congenital, neurologic, toxic, endocrinological, or anatomical pathology.

Treatment targets nutritional support, improving intestinal motility, and minimizing surgical intervention.[4] Bacterial overgrowth of the small intestine can occur in chronic cases – presenting as malabsorption, diarrhea, and nutrient deficiencies[12] – which may require the use of antibiotics.

Presentation

Clinical features of IPO can include abdominal pain, nausea, abdominal distension, vomiting, dysphagia, and constipation. Symptoms depend on the portion of the gastrointestinal tract involved[2] and the duration of symptoms. Symptoms may occur intermittently and over a prolonged period of time. It is not unusual for patients to present several times owing to the nonspecific nature of the symptoms.[4] Conditions and onset will vary if the disease is primary vs secondary and the underlying disease (if a secondary manifestation) and its management.

Symptoms indicative of advanced disease and possible intestinal failure include diarrhea, loss of appetite, sepsis, bloating, fatigue, signs of low volume status, and malabsorption including nutritional deficiencies and foul-smelling stools.[13][14]

Causes

In primary CIPO (the majority of chronic cases) the condition results from disruption of the intestine's ability to move food. These can be broadly classified as myopathic (affecting the smooth muscle), mesenchymopathic (affecting the interstitial cells of Cajal), or neuropathic (of the nervous system) of the gastrointestinal tract.[15]

In some cases there appears to be a genetic association.[16] One form has been associated with DXYS154, some associated with defective ACTG2 gene [17]

Secondary chronic intestinal pseudo-obstruction can occur as a consequence of a number of other conditions including:

The term may be used synonymously with enteric neuropathy if a neurological cause is suspected.

Diagnosis

CT-Scan showing a coronal section of the abdomen of an elderly lady with an IPO.

The symptoms of IPO are nonspecific. It is not unusual for patients to present repeatedly and to undergo numerous tests.[4] Mechanical causes of intestinal obstruction must be excluded to reach a diagnosis of pseudo-obstruction. Attempts must also be made to determine whether the IPO is the result of a primary or secondary condition.[15] A diagnostic work-up may include:[14]

  • Gastric motility studies
  • Imaging studies:
    • CT-Scan showing a Cross-section of the abdomen of an elderly lady with an IPO.
      X-rays – may show intestinal air fluid levels (seen with true mechanical intestinal obstruction)
    • CT scans
    • Barium enema
  • Blood tests
  • Upper and lower endoscopies
  • Manometry – used to measure pressure of esophagus and stomach

Treatment

Treatment for IPO (acute or chronic) is aimed at removing the disease process and/or managing the complications present. Focus is placed on management of pain, gastrointestinal symptoms, nutritional deficiencies, fluid status, infection control, and improving quality of life. When CIPO is secondary to another disease, treatment is addressed towards the underlying condition. Surgery is sometimes required in severe cases of CIPO.

Medical treatment

Prucalopride,[24][25] pyridostigmine,[11] metoclopramide, cisapride, erythromycin[9] and octreotide[9][26][27] are medications that aim to enhance intestinal motility.

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 foods that increase distention and are difficult to digest (e.g. those high in fat and fibre), consuming 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 energy needs, enteral nutrition is used. Many patients eventually require parenteral nutrition.[15]

Total parenteral nutrition (TPN) is a form of long-term nutritional treatment reserved for patients that have severe pseudo-obstruction. TPN dependent patients require frequent checkups to monitor catheter function, check liver enzyme levels, and evaluate for signs of blood infections. 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.[28]

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. These may be used for feed (e.g. gastrostomy and jejunostomy) or to flush the intestines.

Colostomy or ileostomy can bypass affected parts if they are distal to (come after) the stoma. For instance, if only the colon is affected, an ileostomy may be helpful. Either of these ostomies are typically placed at or a few centimeters below the patient's navel per doctor recommendation based on the affected area of the intestines as well as concerns for patient comfort and future physical growth for children.[28]

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 localized 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 intestinal transplantation. This is only appropriate in the case of intestinal failure. These procedures are most frequently described in pediatric cases of CIPO.[29][30] One operation involving multi-organ transplant of the pancreas, stomach, duodenum, small intestine, and liver, and was performed by Doctor Kareem Abu-Elmagd on Gretchen Miller.[31]

Potential treatments

Further research is necessary into other treatments which may alleviate symptoms. These include stem-cell transplantation[9][32][33] and fecal microbiota transplantation.[9] Cannabis[34][35][unreliable source?] has not been studied with regards to CIPO. Any claims to its efficacy for use in CIPO are speculative.

Related disorders

  • Ogilvie syndrome: acute pseudoobstruction of the colon in severely ill debilitated patients.
  • Hirschsprung's disease: enlargement of the colon due to lack of development of autonomic ganglia.
  • Intestinal neuronal dysplasia: a disease of motor neurons leading to the bowels.
  • Bowel obstruction: mechanical or functional obstruction of the bowel, most commonly due to adhesions, hernias or neoplasms.
  • Enteric neuropathy: alternative name sometimes used for diagnosis in UK

References

  1. ↑ "Natural history of chronic idiopathic intestinal pseudo-obstruction in adults: a single center study". Clinical Gastroenterology and Hepatology 3 (5): 449–58. May 2005. doi:10.1016/S1542-3565(04)00675-5. PMID 15880314. 
  2. ↑ 2.0 2.1 "Advances in our understanding of the pathology of chronic intestinal pseudo-obstruction". Gut 53 (11): 1549–52. November 2004. doi:10.1136/gut.2004.043968. PMID 15479666. 
  3. ↑ Robbins basic pathology. Vinay Kumar, Abul K. Abbas, Jon C. Aster, James A. Perkins (10th ed.). Philadelphia, Pa.: Elsevier. 2018. pp. Chapter 5: intestinal obstruction. ISBN 978-0-323-39413-0. OCLC 972900144. https://www.worldcat.org/oclc/972900144. 
  4. ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 El-Chammas, Khalil; Sood, Manu R. (March 2018). "Chronic Intestinal Pseudo-obstruction". Clinics in Colon and Rectal Surgery 31 (2): 99–107. doi:10.1055/s-0037-1609024. ISSN 1531-0043. PMID 29487492. 
  5. ↑ Ko, Dayoung; Yang, Hee-Beom; Youn, Joong; Kim, Hyun-Young (2021-05-28). "Clinical Outcomes of Pediatric Chronic Intestinal Pseudo-Obstruction". Journal of Clinical Medicine 10 (11): 2376. doi:10.3390/jcm10112376. ISSN 2077-0383. PMID 34071279. 
  6. ↑ Saunders MD (October 2004). "Acute colonic pseudoobstruction". Current Gastroenterology Reports 6 (5): 410–6. doi:10.1007/s11894-004-0059-5. PMID 15341719. 
  7. ↑ "Diagnosis and management of adult patients with chronic intestinal pseudoobstruction". Nutrition in Clinical Practice 21 (1): 16–22. February 2006. doi:10.1177/011542650602100116. PMID 16439766. http://ncp.sagepub.com/cgi/pmidlookup?view=long&pmid=16439766. 
  8. ↑ Iida, Hiroshi; Ohkubo, Hidenori; Inamori, Masahiko; Nakajima, Atsushi; Sato, Hajime (2013). "Epidemiology and clinical experience of chronic intestinal pseudo-obstruction in Japan: a nationwide epidemiologic survey". Journal of Epidemiology 23 (4): 288–294. doi:10.2188/jea.je20120173. ISSN 1349-9092. PMID 23831693. 
  9. ↑ 9.0 9.1 9.2 9.3 9.4 Zenzeri, Letizia; Tambucci, Renato; Quitadamo, Paolo; Giorgio, Valentina; De Giorgio, Roberto; Di Nardo, Giovanni (May 2020). "Update on chronic intestinal pseudo-obstruction". Current Opinion in Gastroenterology 36 (3): 230–237. doi:10.1097/MOG.0000000000000630. ISSN 1531-7056. PMID 32073506. https://pubmed.ncbi.nlm.nih.gov/32073506. 
  10. ↑ Downes, Thomas J.; Cheruvu, Manikandar S.; Karunaratne, Tennekoon B.; De Giorgio, Roberto; Farmer, Adam D. (July 2018). "Pathophysiology, Diagnosis, and Management of Chronic Intestinal Pseudo-Obstruction". Journal of Clinical Gastroenterology 52 (6): 477–489. doi:10.1097/MCG.0000000000001047. ISSN 1539-2031. PMID 29877952. https://pubmed.ncbi.nlm.nih.gov/29877952. 
  11. ↑ 11.0 11.1 "Chronic intestinal pseudo-obstruction". World Journal of Gastroenterology 14 (19): 2953–61. May 2008. doi:10.3748/wjg.14.2953. PMID 18494042. 
  12. ↑ Cucchiara, Salvatore; Borrelli, Osvaldo (April 2009). "Nutritional challenge in pseudo-obstruction: the bridge between motility and nutrition". Journal of Pediatric Gastroenterology and Nutrition 48 (Suppl 2): S83–85. doi:10.1097/MPG.0b013e3181a15bfe. ISSN 1536-4801. PMID 19300134. https://pubmed.ncbi.nlm.nih.gov/19300134. 
  13. ↑ "Pediatric Intestinal Failure". https://childrensnational.org/visit/conditions-and-treatments/stomach-digestion-gi/intestinal-failure. Retrieved 8 November 2021. 
  14. ↑ 14.0 14.1 "Intestinal Failure". https://www.topdoctors.co.uk/medical-dictionary/intestinal-failure. 
  15. ↑ 15.0 15.1 15.2 15.3 "Chronic intestinal pseudo-obstruction". Nutrition in Clinical Practice 28 (3): 307–16. June 2013. doi:10.1177/0884533613485904. PMID 23612903. 
  16. ↑ "Family studies of infantile visceral myopathy: a congenital myopathic pseudo-obstruction syndrome". American Journal of Medical Genetics 82 (2): 114–22. January 1999. doi:10.1002/(SICI)1096-8628(19990115)82:2<114::AID-AJMG3>3.0.CO;2-H. PMID 9934973. 
  17. ↑ "The locus for a novel syndromic form of neuronal intestinal pseudoobstruction maps to Xq28". American Journal of Human Genetics 58 (4): 743–8. April 1996. PMID 8644737. 
  18. ↑ "Hirschsprung disease". 4 September 2017. https://rarediseases.info.nih.gov/diseases/6660/hirschsprungs-disease. 
  19. ↑ "Intestinal pseudoobstruction in Kawasaki disease". Pediatrics 113 (5): e504–6. May 2004. doi:10.1542/peds.113.5.e504. PMID 15121996. 
  20. ↑ Colomba, Claudia; La Placa, Simona; Saporito, Laura; Corsello, Giovanni; Ciccia, Francesco; Medaglia, Alice; Romanin, Benedetta; Serra, Nicola et al. (November 2018). "Intestinal Involvement in Kawasaki Disease". The Journal of Pediatrics 202: 186–193. doi:10.1016/j.jpeds.2018.06.034. ISSN 1097-6833. PMID 30029859. 
  21. ↑ Salari, Mehri; Fayyazi, Emad; Mirmosayyeb, Omid (2016). "Gastrointestinal dysfunction in idiopathic Parkinsonism: A narrative review". Journal of Research in Medical Sciences 21: 126. doi:10.4103/1735-1995.196608. ISSN 1735-1995. PMID 28331512. 
  22. ↑ Takahashi, Hiroki; Ohara, Mikiko; Imai, Kohzoh (June 2004). "[Collagen diseases with gastrointestinal manifestations]". Nihon Rinsho Men'eki Gakkai Kaishi = Japanese Journal of Clinical Immunology 27 (3): 145–155. doi:10.2177/jsci.27.145. ISSN 0911-4300. PMID 15291251. 
  23. ↑ Finsterer, Josef; Frank, Marlies (January 2017). "Gastrointestinal manifestations of mitochondrial disorders: a systematic review". Therapeutic Advances in Gastroenterology 10 (1): 142–154. doi:10.1177/1756283X16666806. ISSN 1756-283X. PMID 28286566. 
  24. ↑ "Effects of the enterokinetic prucalopride (R093877) on colonic motility in fasted dogs". Neurogastroenterology and Motility 13 (5): 465–72. October 2001. doi:10.1046/j.1365-2982.2001.00280.x. PMID 11696108. 
  25. ↑ "Prucalopride for chronic intestinal pseudo-obstruction". Alimentary Pharmacology & Therapeutics 35 (3): 398–9. February 2012. doi:10.1111/j.1365-2036.2011.04947.x. PMID 22221087. 
  26. ↑ "Gastric adenocarcinoma presenting with intestinal pseudoobstruction, successfully treated with octreotide". Indian Journal of Medical Sciences 60 (11): 467–70. November 2006. doi:10.4103/0019-5359.27974. PMID 17090868. 
  27. ↑ "Octreotide treatment for paraneoplastic intestinal pseudo-obstruction complicating SCLC". Lung Cancer 48 (1): 137–40. April 2005. doi:10.1016/j.lungcan.2004.09.008. PMID 15777981. 
  28. ↑ 28.0 28.1 "Chronic intestinal pseudo-obstruction: treatment and long term follow up of 44 patients". Archives of Disease in Childhood 81 (1): 21–7. July 1999. doi:10.1136/adc.81.1.21. PMID 10373127. 
  29. ↑ Mousa, Hayat; Hyman, Paul E.; Cocjin, Jose; Flores, Alejandro F.; Di Lorenzo, Carlo (October 2002). "Long-term outcome of congenital intestinal pseudoobstruction". Digestive Diseases and Sciences 47 (10): 2298–2305. doi:10.1023/a:1020199614102. ISSN 0163-2116. PMID 12395903. https://pubmed.ncbi.nlm.nih.gov/12395903. 
  30. ↑ Bond, Geoffrey J.; Reyes, Jorge D. (November 2004). "Intestinal transplantation for total/near-total aganglionosis and intestinal pseudo-obstruction". Seminars in Pediatric Surgery 13 (4): 286–292. doi:10.1053/j.sempedsurg.2004.10.016. ISSN 1055-8586. PMID 15660322. https://pubmed.ncbi.nlm.nih.gov/15660322. 
  31. ↑ Discovery Channel – Multiorgan transplant
  32. ↑ Westfal, Maggie L.; Goldstein, Allan M. (June 2017). "Pediatric enteric neuropathies: diagnosis and current management". Current Opinion in Pediatrics 29 (3): 347–353. doi:10.1097/MOP.0000000000000486. ISSN 1531-698X. PMID 28319561. 
  33. ↑ Halter, Joerg P.; Michael, W.; Schüpbach, M.; Mandel, Hanna; Casali, Carlo; Orchard, Kim; Collin, Matthew; Valcarcel, David et al. (October 2015). "Allogeneic haematopoietic stem cell transplantation for mitochondrial neurogastrointestinal encephalomyopathy". Brain: A Journal of Neurology 138 (Pt 10): 2847–2858. doi:10.1093/brain/awv226. ISSN 1460-2156. PMID 26264513. 
  34. ↑ "Role of endogenous cannabinoid system in the gut". Sheng Li Xue Bao 65 (4): 451–60. August 2013. PMID 23963077. http://www.actaps.com.cn/qikan/manage/wenzhang/2013-4-12.pdf. 
  35. ↑ Gastrointestinal Disorders.

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