Intestinal pseudo-obstruction

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 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. 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.

Epidemiology

According to a 2013 study conducted in Japan, the estimated prevalence of CIPO was between 0.80 and 1.00 per 100,000 people, while its incidence rate was reported as 0.21 to 0.24 per 100,000. Furthermore, some studies have indicated that CIPO is more prevalent in females, a trend that may be attributable to the higher rates of secondary CIPO from conditions more common in this demographic. In the case of pediatric intestinal pseudo-obstruction (PIPO), the incidence was estimated at approximately 1 per 40,000 live births.

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.

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

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

• Hirschsprung's disease – the absence of colonic nerve cells
• Chagas' disease – a chronic parasitic infection of the colon leading to loss of nerve endings
• Kawasaki disease - a rare presentation for this particular autoimmune disorder of the vasculature
• Parkinson's disease - related to the neurodegeneration of gastrointestinal tract
• Autoimmune conditions — conditions including systemic lupus erythematosus and scleroderma lead to collagen vascular deposition and gastrointestinal motility disruption
• Mitochondrial disease - IPO is a known presentation for mitochondrial disease
• Endocrine disorders
• Certain medications.

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

Diagnosis

The symptoms of IPO are nonspecific. It is not unusual for patients to present repeatedly and to undergo numerous tests. 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. A diagnostic work-up may include:

• Gastric motility studies
• Imaging studies:
  • [[File:Ogilvie ct cross.jpg|thumb|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

Classification

Pseudo-obstruction syndromes are classified as acute or chronic based on their clinical appearance. Acute colonic pseudo-obstruction (ACPO; sometimes known as Ogilvie syndrome) causes the colon to become grossly dilated; if not decompressed, the individual risks perforation, peritonitis, and death. Chronic intestinal pseudo-obstruction is a chronic disorder.

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, pyridostigmine, metoclopramide, cisapride, erythromycin, and octreotide 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.

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.

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.

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. 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.

Potential treatments

Further research is necessary into other treatments which may alleviate symptoms. These include stem-cell transplantation and fecal microbiota transplantation. Cannabis 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

References

1. (May 2005). "Natural history of chronic idiopathic intestinal pseudo-obstruction in adults: a single center study". Clinical Gastroenterology and Hepatology.
2. (November 2004). "Advances in our understanding of the pathology of chronic intestinal pseudo-obstruction". Gut.
3. (2018). "Robbins basic pathology". Elsevier.
4. (March 2018). "Chronic Intestinal Pseudo-obstruction". Clinics in Colon and Rectal Surgery.
5. (2021-05-28). "Clinical Outcomes of Pediatric Chronic Intestinal Pseudo-Obstruction". Journal of Clinical Medicine.
6. Saunders MD. (October 2004). "Acute colonic pseudoobstruction". Current Gastroenterology Reports.
7. (February 2006). "Diagnosis and management of adult patients with chronic intestinal pseudoobstruction". Nutrition in Clinical Practice.
8. (2013). "Epidemiology and clinical experience of chronic intestinal pseudo-obstruction in Japan: a nationwide epidemiologic survey". Journal of Epidemiology.
9. (May 2020). "Update on chronic intestinal pseudo-obstruction". Current Opinion in Gastroenterology.
10. (July 2018). "Pathophysiology, Diagnosis, and Management of Chronic Intestinal Pseudo-Obstruction". Journal of Clinical Gastroenterology.
11. (May 2008). "Chronic intestinal pseudo-obstruction". World Journal of Gastroenterology.
12. (April 2009). "Nutritional challenge in pseudo-obstruction: the bridge between motility and nutrition". Journal of Pediatric Gastroenterology and Nutrition.
13. "Pediatric Intestinal Failure".
14. "Intestinal Failure".
15. Iida, Hiroshi. (2013). "Epidemiology and Clinical Experience of Chronic Intestinal Pseudo-Obstruction in Japan: A Nationwide Epidemiologic Survey". Journal of Epidemiology.
16. Gandhi, Jermie J.. (2025-02-01). "Pediatric Intestinal Pseudo-Obstruction: A Review". Pediatrics in Review.
17. (June 2013). "Chronic intestinal pseudo-obstruction". Nutrition in Clinical Practice.
18. (January 1999). "Family studies of infantile visceral myopathy: a congenital myopathic pseudo-obstruction syndrome". American Journal of Medical Genetics.
19. (April 1996). "The locus for a novel syndromic form of neuronal intestinal pseudoobstruction maps to Xq28". American Journal of Human Genetics.
20. (4 September 2017). "Hirschsprung disease".
21. (May 2004). "Intestinal pseudoobstruction in Kawasaki disease". Pediatrics.
22. (November 2018). "Intestinal Involvement in Kawasaki Disease". The Journal of Pediatrics.
23. (June 2004). "[Collagen diseases with gastrointestinal manifestations]". Nihon Rinsho Men'eki Gakkai Kaishi = Japanese Journal of Clinical Immunology.
24. (January 2017). "Gastrointestinal manifestations of mitochondrial disorders: a systematic review". Therapeutic Advances in Gastroenterology.
25. Cagir, Burt. (2018-07-23). "Intestinal Pseudo-Obstruction: Background, Anatomy, Pathophysiology".
26. (October 2001). "Effects of the enterokinetic prucalopride (R093877) on colonic motility in fasted dogs". Neurogastroenterology and Motility.
27. (February 2012). "Prucalopride for chronic intestinal pseudo-obstruction". Alimentary Pharmacology & Therapeutics.
28. (November 2006). "Gastric adenocarcinoma presenting with intestinal pseudoobstruction, successfully treated with octreotide". Indian Journal of Medical Sciences.
29. (April 2005). "Octreotide treatment for paraneoplastic intestinal pseudo-obstruction complicating SCLC". Lung Cancer.
30. (July 1999). "Chronic intestinal pseudo-obstruction: treatment and long term follow up of 44 patients". Archives of Disease in Childhood.
31. (October 2002). "Long-term outcome of congenital intestinal pseudoobstruction". Digestive Diseases and Sciences.
32. (November 2004). "Intestinal transplantation for total/near-total aganglionosis and intestinal pseudo-obstruction". Seminars in Pediatric Surgery.
33. [http://dsc.discovery.com/fansites/surgery/follow-up/six-organ/gretchen.html Discovery Channel – Multiorgan transplant]
34. (June 2017). "Pediatric enteric neuropathies: diagnosis and current management". Current Opinion in Pediatrics.
35. (October 2015). "Allogeneic haematopoietic stem cell transplantation for mitochondrial neurogastrointestinal encephalomyopathy". Brain: A Journal of Neurology.
36. (August 2013). "Role of endogenous cannabinoid system in the gut". Sheng Li Xue Bao.