Intestinal pseudo-obstruction

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Intestinal pseudo-obstruction
Ogilvie ct coronal.jpg
Pronunciation
  • soo·doe/uhb·struhk·shn
Specialty Gastroenterology
Symptoms Abdominal pain, nausea, distention, vomiting, dysphagia, and constipation
Complications Intestinal 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 method Signs and symptoms consistent with a mechanical intestinal obstruction with no identifying lesion.
Differential diagnosis Intestinal obstruction, Crohn's disease, ovarian torsion, ovarian cyst, neoplasm, infection (parasitic)
TreatmentAimed at management of complications (e.g. nutrition, hydration, pain relief).
Prognosis 10–25% 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.

Contents

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. Ogilvie ct coronal.jpg
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]

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. [24]

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, [25] [26] pyridostigmine, [11] metoclopramide, cisapride, erythromycin, [9] and octreotide [9] [27] [28] 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. [29]

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. [29]

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. [30] [31] 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. [32]

Potential treatments

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

Related Research Articles

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

Crohn's disease is a type of inflammatory bowel disease (IBD) that may affect any segment of the gastrointestinal tract. Symptoms often include abdominal pain, diarrhea, fever, abdominal distension, and weight loss. Complications outside of the gastrointestinal tract may include anemia, skin rashes, arthritis, inflammation of the eye, and fatigue. The skin rashes may be due to infections as well as pyoderma gangrenosum or erythema nodosum. Bowel obstruction may occur as a complication of chronic inflammation, and those with the disease are at greater risk of colon cancer and small bowel cancer.

<span class="mw-page-title-main">Constipation</span> Bowel dysfunction

Constipation is a bowel dysfunction that makes bowel movements infrequent or hard to pass. The stool is often hard and dry. Other symptoms may include abdominal pain, bloating, and feeling as if one has not completely passed the bowel movement. Complications from constipation may include hemorrhoids, anal fissure or fecal impaction. The normal frequency of bowel movements in adults is between three per day and three per week. Babies often have three to four bowel movements per day while young children typically have two to three per day.

<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">Enteritis</span> Intestinal inflammatory disease

Enteritis is inflammation of the small intestine. It is most commonly caused by food or drink contaminated with pathogenic microbes, such as Serratia, but may have other causes such as NSAIDs, radiation therapy as well as autoimmune conditions like Crohn's disease and coeliac disease. Symptoms include abdominal pain, cramping, diarrhoea, dehydration, and fever. Related diseases of the gastrointestinal system involve inflammation of the stomach and large intestine.

<span class="mw-page-title-main">Inflammatory bowel disease</span> Medical condition

Inflammatory bowel disease (IBD) is a group of inflammatory conditions of the colon and small intestine, with Crohn's disease and ulcerative colitis (UC) being the principal types. Crohn's disease affects the small intestine and large intestine, as well as the mouth, esophagus, stomach and the anus, whereas UC primarily affects the colon and the rectum.

<span class="mw-page-title-main">Malabsorption</span> Medical condition

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.

Functional gastrointestinal disorders (FGID), also known as disorders of gut–brain interaction, include a number of separate idiopathic disorders which affect different parts of the gastrointestinal tract and involve visceral hypersensitivity and motility disturbances.

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

Gastrointestinal diseases refer to diseases involving the gastrointestinal tract, namely the esophagus, stomach, small intestine, large intestine and rectum, and the accessory organs of digestion, the liver, gallbladder, and pancreas.

<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, which is caused by a number of mechanisms.

<span class="mw-page-title-main">Short bowel syndrome</span> Medical condition

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

Intestinal malrotation is a congenital anomaly of rotation of the midgut. It occurs during the first trimester as the fetal gut undergoes a complex series of growth and development. Malrotation can lead to a dangerous complication called volvulus, in which cases emergency surgery is indicated. Malrotation can refer to a spectrum of abnormal intestinal positioning, often including:

<span class="mw-page-title-main">Lymphangiectasia</span> Medical condition

Lymphangiectasia, also known as "lymphangiectasis", is a pathologic dilation of lymph vessels. When it occurs in the intestines it is known as intestinal lymphangiectasia, colloquially recognized as Waldmann's disease in cases where there is no secondary cause. The primary defect lies in the inability of the lymphatic system to adequately drain lymph, resulting in its subsequent accumulation and leakage into the intestinal lumen. This condition, first described by Waldmann in 1961, is typically diagnosed in infancy or early childhood. However, it can also manifest in adults, exhibiting a broad spectrum of clinical symptoms.

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

Ogilvie syndrome, or acute colonic pseudo-obstruction is the acute dilatation of the colon in the absence of any mechanical obstruction in severely ill patients.

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

Chronic intestinal pseudo-obstruction (CIPO) is an uncommon syndrome with chronic and recurrent symptoms that suggest intestinal obstruction in the absence of any mechanical blockage of the lumen. The most common symptoms of CIPO include abdominal pain, constipation, nausea, vomiting, dysphagia, and abdominal distention. CIPO can lead to malnutrition.

<span class="mw-page-title-main">Mitochondrial neurogastrointestinal encephalopathy syndrome</span> Medical condition

Mitochondrial neurogastrointestinal encephalopathy syndrome (MNGIE) is a rare autosomal recessive mitochondrial disease. It has been previously referred to as polyneuropathy, ophthalmoplegia, leukoencephalopathy, and intestinal pseudoobstruction. The disease presents in childhood, but often goes unnoticed for decades. Unlike typical mitochondrial diseases caused by mitochondrial DNA (mtDNA) mutations, MNGIE is caused by mutations in the TYMP gene, which encodes the enzyme thymidine phosphorylase. Mutations in this gene result in impaired mitochondrial function, leading to intestinal symptoms as well as neuro-ophthalmologic abnormalities. A secondary form of MNGIE, called MNGIE without leukoencephalopathy, can be caused by mutations in the POLG gene.

<span class="mw-page-title-main">Superior mesenteric artery syndrome</span> Medical condition

Superior mesenteric artery (SMA) syndrome is a gastro-vascular disorder in which the third and final portion of the duodenum is compressed between the abdominal aorta (AA) and the overlying superior mesenteric artery. This rare, potentially life-threatening syndrome is typically caused by an angle of 6–25° between the AA and the SMA, in comparison to the normal range of 38–56°, due to a lack of retroperitoneal and visceral fat. In addition, the aortomesenteric distance is 2–8 millimeters, as opposed to the typical 10–20. However, a narrow SMA angle alone is not enough to make a diagnosis, because patients with a low BMI, most notably children, have been known to have a narrow SMA angle with no symptoms of SMA syndrome.

<span class="mw-page-title-main">Prucalopride</span> Drug used to treat chronic constipation

Prucalopride, sold under brand names Resolor and Motegrity among others, is a medication acting as a selective, high affinity 5-HT4 receptor agonist which targets the impaired motility associated with chronic constipation, thus normalizing bowel movements. Prucalopride was approved for medical use in the European Union in 2009, in Canada in 2011, in Israel in 2014, and in the United States in December 2018. The drug has also been tested for the treatment of chronic intestinal pseudo-obstruction.

A prokinetic agent is a type of small peptide drug which enhances gastrointestinal motility by increasing the frequency or strength of contractions, but without disrupting their rhythm. They are used to treat certain gastrointestinal symptoms, including abdominal discomfort, bloating, constipation, heart burn, nausea, and vomiting; and certain gastrointestinal disorders, including irritable bowel syndrome, gastritis, gastroparesis, and functional dyspepsia.

<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.

A low-FODMAP diet is a person's global restriction of consumption of all fermentable carbohydrates (FODMAPs), recommended only for a short time. A low-FODMAP diet is recommended for managing patients with irritable bowel syndrome (IBS) and can reduce digestive symptoms of IBS including bloating and flatulence.

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. S2CID   32605317.
  2. 1 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. ↑ 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.{{cite book}}: CS1 maint: others (link)
  4. 1 2 3 4 5 6 7 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. PMC   5825855 . PMID   29487492.
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  9. 1 2 3 4 5 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. S2CID   211193582.
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  20. ↑ Colomba, Claudia; La Placa, Simona; Saporito, Laura; Corsello, Giovanni; Ciccia, Francesco; Medaglia, Alice; Romanin, Benedetta; Serra, Nicola; Di Carlo, Paola; Cascio, Antonio (November 2018). "Intestinal Involvement in Kawasaki Disease". The Journal of Pediatrics. 202: 186–193. doi: 10.1016/j.jpeds.2018.06.034 . hdl: 10447/350574 . ISSN   1097-6833. PMID   30029859. S2CID   51704336.
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  32. ↑ Discovery Channel – Multiorgan transplant
  33. ↑ 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. PMC   5475271 . PMID   28319561.
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