Chronic Intestinal Pseudo-obstruction
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Chronic intestinal pseudo-obstruction (CIPO) is a very rare syndrome with chronic and recurrent symptoms that suggest
intestinal obstruction Bowel obstruction, also known as intestinal obstruction, is a mechanical or functional obstruction of the intestines which prevents the normal movement of the products of digestion. Either the small bowel or large bowel may be affected. Signs ...
in the absence of any mechanical blockage of the lumen. The most common symptoms of CIPO include
abdominal pain Abdominal pain, also known as a stomach ache, is a symptom associated with both non-serious and serious medical issues. Since the abdomen contains most of the body's vital organs, it can be an indicator of a wide variety of diseases. Given th ...
,
constipation Constipation is a bowel dysfunction that makes bowel movements infrequent or hard to pass. The Human feces, stool is often hard and dry. Other symptoms may include abdominal pain, bloating, and feeling as if one has not completely passed the ...
,
nausea Nausea is a diffuse sensation of unease and discomfort, sometimes perceived as an urge to vomit. It can be a debilitating symptom if prolonged and has been described as placing discomfort on the chest, abdomen, or back of the throat. Over 30 d ...
,
vomiting Vomiting (also known as emesis, puking and throwing up) is the forceful expulsion of the contents of one's stomach through the mouth and sometimes the nose. Vomiting can be the result of ailments like food poisoning, gastroenteritis, pre ...
,
dysphagia Dysphagia is difficulty in swallowing. Although classified under " symptoms and signs" in ICD-10, in some contexts it is classified as a condition in its own right. It may be a sensation that suggests difficulty in the passage of solids or l ...
, and abdominal distention. CIPO can lead to
malnutrition Malnutrition occurs when an organism gets too few or too many nutrients, resulting in health problems. Specifically, it is a deficiency, excess, or imbalance of energy, protein and other nutrients which adversely affects the body's tissues a ...
. Chronic intestinal pseudo-obstruction can be caused by a variety of other disorders or it can be idiopathic. Certain
genetic disorder A genetic disorder is a health problem caused by one or more abnormalities in the genome. It can be caused by a mutation in a single gene (monogenic) or multiple genes (polygenic) or by a chromosome abnormality. Although polygenic disorders ...
s can also cause CIPO. Mechanisms behind CIPO include abnormalities in the
smooth muscle Smooth muscle is one of the three major types of vertebrate muscle tissue, the others being skeletal and cardiac muscle. It can also be found in invertebrates and is controlled by the autonomic nervous system. It is non- striated, so-called bec ...
cells,
interstitial cells of Cajal Interstitial cells of Cajal (ICC) are interstitial cells found in the gastrointestinal tract. There are different types of ICC with different functions. ICC and another type of interstitial cell, known as platelet-derived growth factor recep ...
(ICCs), and intrinsic and extrinsic
neuron A neuron (American English), neurone (British English), or nerve cell, is an membrane potential#Cell excitability, excitable cell (biology), cell that fires electric signals called action potentials across a neural network (biology), neural net ...
s. The diagnosis of CIPO is made based on clinical symptoms and radiographic studies. Abdominal X-rays,
CT scan A computed tomography scan (CT scan), formerly called computed axial tomography scan (CAT scan), is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers or ...
s, endoscopies, laboratory tests, and biopsies may be used to make the diagnosis of CIPO. Treatment involves ensuring adequate nutrition and managing the symptoms of CIPO.
Enteral Enteral administration is food or drug administration via the human gastrointestinal tract. This contrasts with parenteral nutrition or drug administration (Greek ''para'', "besides" + ''enteros''), which occurs from routes outside the GI tract, ...
or
parenteral nutrition 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 ph ...
may be needed to maintain proper nutrition.
Analgesic An analgesic drug, also called simply an analgesic, antalgic, pain reliever, or painkiller, is any member of the group of drugs used for pain management. Analgesics are conceptually distinct from anesthetics, which temporarily reduce, and in s ...
s,
antiemetic An antiemetic is a drug that is effective against vomiting and nausea. Antiemetics are typically used to treat motion sickness and the side effects of opioid analgesics, general anaesthetics, and chemotherapy directed against cancer. They may ...
s, antisecretory,
antispasmodic An antispasmodic (synonym: spasmolytic) is a pharmaceutical drug or other agent that suppresses muscle spasms. Smooth muscle spasm One type of antispasmodics is used for smooth muscle relaxation, especially in tubular organs of the gastrointestina ...
s,
prokinetic agent A prokinetic agent (also prokineticin, gastroprokinetic agent, gastrokinetic agent or propulsive) is a type of drug which enhances gastrointestinal motility by increasing the frequency or strength of contractions, but without disrupting their rhyt ...
s,
laxative Laxatives, purgatives, or aperients are substances that loosen stools and increase bowel movements. They are used to treat and prevent constipation. Laxatives vary as to how they work and the side effects they may have. Certain stimulant, lubri ...
s, or antidiarrheal medications may be used to help manage the symptoms of CIPO. The long-term prognosis for CIPO is poor. Patients often require
parenteral nutrition 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 ph ...
due to their symptoms. The mortality rate for pediatric CIPO patients ranges from 10-25% before adulthood. " Intestinal pseudo-obstruction" is a broad term that refers to any
paralysis Paralysis (: paralyses; also known as plegia) is a loss of Motor skill, motor function in one or more Skeletal muscle, muscles. Paralysis can also be accompanied by a loss of feeling (sensory loss) in the affected area if there is sensory d ...
of the
intestines The gastrointestinal tract (GI tract, digestive tract, alimentary canal) is the tract or passageway of the digestive system that leads from the mouth to the anus. The tract is the largest of the body's systems, after the cardiovascular system. ...
that is not caused by a mechanical obstruction.
Ogilvie syndrome 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. Acute colonic pseudo-obstruction is characterized by massive dilatation of the cec ...
is an acute form of intestinal pseudo-obstruction.


Signs and symptoms

Symptoms of chronic intestinal pseudo-obstruction can vary depending on which segment of the
gastrointestinal tract The gastrointestinal tract (GI tract, digestive tract, alimentary canal) is the tract or passageway of the Digestion, digestive system that leads from the mouth to the anus. The tract is the largest of the body's systems, after the cardiovascula ...
is most involved and may evolve as the disease progresses. Patients may experience varying degrees of symptoms or be asymptomatic in between sub-occlusive episodes. Chronic intestinal pseudo-obstruction typically develops gradually over years, with gastrointestinal symptoms of increasing severity sometimes occurring years before the first sub-occlusive episode; less frequently, the onset occurs suddenly, with the patient presenting with a sub-occlusive episode. Patients with chronic intestinal pseudo-obstruction that involves the
small bowel The small intestine or small bowel is an organ in the gastrointestinal tract where most of the absorption of nutrients from food takes place. It lies between the stomach and large intestine, and receives bile and pancreatic juice through the p ...
commonly present with symptoms such as
constipation Constipation is a bowel dysfunction that makes bowel movements infrequent or hard to pass. The Human feces, stool is often hard and dry. Other symptoms may include abdominal pain, bloating, and feeling as if one has not completely passed the ...
,
abdominal pain Abdominal pain, also known as a stomach ache, is a symptom associated with both non-serious and serious medical issues. Since the abdomen contains most of the body's vital organs, it can be an indicator of a wide variety of diseases. Given th ...
,
nausea Nausea is a diffuse sensation of unease and discomfort, sometimes perceived as an urge to vomit. It can be a debilitating symptom if prolonged and has been described as placing discomfort on the chest, abdomen, or back of the throat. Over 30 d ...
, and
vomiting Vomiting (also known as emesis, puking and throwing up) is the forceful expulsion of the contents of one's stomach through the mouth and sometimes the nose. Vomiting can be the result of ailments like food poisoning, gastroenteritis, pre ...
.
Diarrhea Diarrhea (American English), also spelled diarrhoea or diarrhœa (British English), 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 d ...
may result from
bacterial overgrowth 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 (or large bowel), which is r ...
brought on by
small bowel The small intestine or small bowel is an organ in the gastrointestinal tract where most of the absorption of nutrients from food takes place. It lies between the stomach and large intestine, and receives bile and pancreatic juice through the p ...
dysmotility-induced stasis. In patients with primarily gastric involvement, postprandial bloating, early satiety, pain in the abdomen,
nausea Nausea is a diffuse sensation of unease and discomfort, sometimes perceived as an urge to vomit. It can be a debilitating symptom if prolonged and has been described as placing discomfort on the chest, abdomen, or back of the throat. Over 30 d ...
, and
vomiting Vomiting (also known as emesis, puking and throwing up) is the forceful expulsion of the contents of one's stomach through the mouth and sometimes the nose. Vomiting can be the result of ailments like food poisoning, gastroenteritis, pre ...
may be present along with significant
gastroparesis Gastroparesis (gastro- from Ancient Greek  – gaster, "stomach"; and -paresis, πάρεσις – "partial paralysis") is a medical disorder of ineffective neuromuscular contractions (peristalsis) of the stomach, resulting in food and l ...
.
Dysphagia Dysphagia is difficulty in swallowing. Although classified under " symptoms and signs" in ICD-10, in some contexts it is classified as a condition in its own right. It may be a sensation that suggests difficulty in the passage of solids or l ...
or symptoms of
gastroesophageal reflux disease Gastroesophageal reflux disease (GERD) or gastro-oesophageal reflux disease (GORD) is a chronic upper gastrointestinal disease in which stomach content persistently and regularly flows up into the esophagus, resulting in symptoms and/or ...
are seen in patients with esophageal involvement, while
abdominal distension Abdominal distension occurs when substances, such as air (gas) or fluid, accumulate in the abdomen causing its expansion. It is typically a symptom of an underlying disease or dysfunction in the body, rather than an illness in its own right. Peo ...
and
constipation Constipation is a bowel dysfunction that makes bowel movements infrequent or hard to pass. The Human feces, stool is often hard and dry. Other symptoms may include abdominal pain, bloating, and feeling as if one has not completely passed the ...
are signs of colonic involvement.


Complications

Patients with chronic intestinal pseudo-obstruction often experience debilitating symptoms that result in nutrition compromise as well as the need for central
parenteral nutrition 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 ph ...
. According to estimates, between 30% and 50% of adult patients with chronic intestinal pseudo-obstruction will eventually need
parenteral nutrition 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 ph ...
, and up to two-thirds of patients experience some form of
nutritional deficiency Malnutrition occurs when an organism gets too few or too many nutrients, resulting in health problems. Specifically, it is a deficiency, excess, or imbalance of energy, protein and other nutrients which adversely affects the body's tissues a ...
.


Causes

Pseudo-obstruction in adults is a rare disorder that can have primary or secondary causes. Adult pseudo-obstruction's most frequent secondary causes include radiation enteritis,
amyloidosis Amyloidosis is a group of diseases in which abnormal proteins, known as amyloid fibrils, build up in tissue. There are several non-specific and vague signs and symptoms associated with amyloidosis. These include fatigue, peripheral edema, weigh ...
, paraneoplastic syndromes,
hypothyroidism Hypothyroidism is an endocrine disease in which the thyroid gland does not produce enough thyroid hormones. It can cause a number of symptoms, such as cold intolerance, poor ability to tolerate cold, fatigue, extreme fatigue, muscle aches, co ...
, usage of substances with
anticholinergic Anticholinergics (anticholinergic agents) are substances that block the action of the acetylcholine (ACh) neurotransmitter at synapses in the central nervous system, central and peripheral nervous system. These agents inhibit the parasympatheti ...
or
narcotic The term narcotic (, from ancient Greek ναρκῶ ''narkō'', "I make numb") originally referred medically to any psychoactive compound with numbing or paralyzing properties. In the United States, it has since become associated with opiates ...
effects,
diabetes Diabetes mellitus, commonly known as diabetes, is a group of common endocrine diseases characterized by sustained high blood sugar levels. Diabetes is due to either the pancreas not producing enough of the hormone insulin, or the cells of th ...
,
scleroderma Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs. The disease can be either localized to the skin or involve other organs, as well. Symptoms may include areas ...
, and other connective tissue disorders. Recently, viral infections such as Epstein-Barr virus,
Herpes Zoster Shingles, also known as herpes zoster or zona, is a viral disease characterized by a painful skin rash with blisters in a localized area. Typically the rash occurs in a single, wide mark either on the left or right side of the body or face. T ...
, and
Cytomegalovirus ''Cytomegalovirus'' (CMV) (from ''cyto-'' 'cell' via Greek - 'container' + 'big, megalo-' + -''virus'' via Latin 'poison') is a genus of viruses in the order '' Herpesvirales'', in the family '' Herpesviridae'', in the subfamily '' Betaherp ...
have drawn attention as potential causes of pseudo-obstruction. It's been demonstrated that pseudo-obstruction is caused by
mitochondrial disease Mitochondrial disease is a group of disorders caused by mitochondrial dysfunction. Mitochondria are the organelles that generate energy for the cell and are found in every cell of the human body except red blood cells. They convert the energy o ...
s. An occult
neoplasm A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...
may be the target of an immune response resulting in severe intestinal dysmotility. Of all the cancers linked to pseudo-obstruction, small-cell lung carcinoma is the most prevalent. However, in many cases, the symptoms of pseudoobstruction have no known cause; these patients are diagnosed with chronic idiopathic intestinal pseudo-obstruction.


Genetics

There have also been reports of uncommon familial chronic intestinal pseudo-obstruction forms. There have been reports of X-linked, autosomal recessive, and syndromic autosomal dominant forms of chronic intestinal pseudo-obstruction. The Xq28 region has been mapped to an X-linked locus.


Mechanism

Abnormalities in the gastrointestinal control systems, including
smooth muscle Smooth muscle is one of the three major types of vertebrate muscle tissue, the others being skeletal and cardiac muscle. It can also be found in invertebrates and is controlled by the autonomic nervous system. It is non- striated, so-called bec ...
cells,
interstitial cells of Cajal Interstitial cells of Cajal (ICC) are interstitial cells found in the gastrointestinal tract. There are different types of ICC with different functions. ICC and another type of interstitial cell, known as platelet-derived growth factor recep ...
(ICCs), and intrinsic and extrinsic
neuron A neuron (American English), neurone (British English), or nerve cell, is an membrane potential#Cell excitability, excitable cell (biology), cell that fires electric signals called action potentials across a neural network (biology), neural net ...
s, can all contribute to the severe motor derangement seen in CIPO patients. Neuro-ICC-muscular anomalies in CIPO might be related to known diseases or idiopathic if no associated disorder is present. Furthermore, certain cases of CIPO exhibit syndromic manifestations and familial clustering, implying a genetic basis. Gut dysmotility is mostly caused by damage to the
enteric nervous system The enteric nervous system (ENS) is one of the three divisions of the autonomic nervous system (ANS), the others being the sympathetic nervous system (SNS) and parasympathetic nervous system (PSNS). It consists of a mesh-like system of neurons th ...
, both structurally and functionally. The
enteric nervous system The enteric nervous system (ENS) is one of the three divisions of the autonomic nervous system (ANS), the others being the sympathetic nervous system (SNS) and parasympathetic nervous system (PSNS). It consists of a mesh-like system of neurons th ...
 is highly sophisticated and can govern almost all gastrointestinal activities, including motility, independently of the central and peripheral neurological systems. Enteric neuropathies may share pathogenetic processes with
central nervous system The central nervous system (CNS) is the part of the nervous system consisting primarily of the brain, spinal cord and retina. The CNS is so named because the brain integrates the received information and coordinates and influences the activity o ...
neurodegenerative illnesses due to their close similarities. Neurodegenerative pathways involve altered
calcium Calcium is a chemical element; it has symbol Ca and atomic number 20. As an alkaline earth metal, calcium is a reactive metal that forms a dark oxide-nitride layer when exposed to air. Its physical and chemical properties are most similar to it ...
signaling, mitochondrial failure, and free radical generation. Patients with CIPO have reported alterations in the ICC network.
Electron microscopy An electron microscope is a microscope that uses a beam of electrons as a source of illumination. It uses electron optics that are analogous to the glass lenses of an optical light microscope to control the electron beam, for instance focusing i ...
and/or KIT immunolabelling, along with
confocal microscopy Confocal microscopy, most frequently confocal laser scanning microscopy (CLSM) or laser scanning confocal microscopy (LSCM), is an optical imaging technique for increasing optical resolution and contrast (vision), contrast of a micrograph by me ...
and image analysis, revealed a decrease in ICCs and structural abnormalities, including process loss and damage to the intracellular
cytoskeleton The cytoskeleton is a complex, dynamic network of interlinking protein filaments present in the cytoplasm of all cells, including those of bacteria and archaea. In eukaryotes, it extends from the cell nucleus to the cell membrane and is compos ...
and organelles. Significant changes in the ICC enteric network highlight the important involvement of nonneuronal cells in regulating gut motility.


Diagnosis

The diagnosis of CIPO is mostly clinical, backed by radiographic documentation of dilated bowel with air-fluid level after exclusion of organic lesions obstructing the gut lumen, as determined by radiologic and/or
endoscopic An endoscopy is a procedure used in medicine to look inside the body. The endoscopy procedure uses an endoscope to examine the interior of a hollow organ or cavity of the body. Unlike many other medical imaging techniques, endoscopes are insert ...
examinations. Thus, diagnostic investigations in patients with suspected CIPO are required to rule out mechanical blockage, identify possible sources of secondary forms, investigate underlying pathophysiological mechanisms, and reveal potential consequences. In order to look into possible intestinal obstruction, radiologic examinations are essential. Simple and cheap to perform, plain film
x-ray An X-ray (also known in many languages as Röntgen radiation) is a form of high-energy electromagnetic radiation with a wavelength shorter than those of ultraviolet rays and longer than those of gamma rays. Roughly, X-rays have a wavelength ran ...
s can show the traditional indicator of air-fluid levels and dilated bowel loops, which is best seen in the erect film.
Abdominal x-ray An abdominal x-ray is an x-ray of the abdomen. It is sometimes abbreviated to AXR, or KUB (for kidneys, ureters, and urinary bladder). Indications In adults, abdominal X-rays have a very low specificity and cannot rule out suspected obstructi ...
s, however, are unable to definitively differentiate between genuine mechanical
intestinal obstruction Bowel obstruction, also known as intestinal obstruction, is a mechanical or functional obstruction of the intestines which prevents the normal movement of the products of digestion. Either the small bowel or large bowel may be affected. Signs ...
and pseudo-obstruction. Afterwards, additional information is obtained by abdominal computed tomography (CT) imaging in order to rule out an extraluminal, gut wall, or intraluminal mechanically obstructive lesion. Endoscopy's primary function in CIPO is to rule out mechanical obstructions in the foregut while also making it easier to get biopsies in order to rule out
celiac disease Coeliac disease (British English) or celiac disease (American English) is a long-term autoimmune disorder, primarily affecting the small intestine. Patients develop intolerance to gluten, which is present in foods such as wheat, rye, spel ...
.
Colonoscopy Colonoscopy () or coloscopy () is a medical procedure involving the Endoscopy, endoscopic examination of the large bowel (colon) and the distal portion of the small bowel. This examination is performed using either a Charge-coupled device, CCD ...
is not usually necessary for the diagnosis of CIPO; however, in certain cases, it may be done for therapeutic decompression. Identification of secondary forms of CIPO associated with potentially treatable disorders can be facilitated by laboratory testing. As a result, tests should be performed for serum
glucose Glucose is a sugar with the Chemical formula#Molecular formula, molecular formula , which is often abbreviated as Glc. It is overall the most abundant monosaccharide, a subcategory of carbohydrates. It is mainly made by plants and most algae d ...
, thyroid-stimulating hormone,
albumin Albumin is a family of globular proteins, the most common of which are the serum albumins. All of the proteins of the albumin family are water- soluble, moderately soluble in concentrated salt solutions, and experience heat denaturation. Alb ...
, liver enzymes,
vitamin B12 Vitamin B12, also known as cobalamin, is a water-soluble vitamin involved in metabolism. One of eight B vitamins, it serves as a vital cofactor (biochemistry), cofactor in DNA synthesis and both fatty acid metabolism, fatty acid and amino a ...
, total blood count, and inflammatory markers (such as
C-reactive protein C-reactive protein (CRP) is an annular (ring-shaped) pentameric protein found in blood plasma, whose circulating concentrations rise in response to inflammation. It is an acute-phase protein of hepatic origin that increases following interleukin ...
and
erythrocyte sedimentation rate The erythrocyte sedimentation rate (ESR or sed rate) is the rate at which red blood cells in anticoagulated whole blood descend in a standardized tube over a period of one hour. It is a common hematology test, and is a non-specific measure of in ...
). Patients with paraneoplastic syndrome should have their circulating antineuronal antibodies, such as ANNA-1/anti-Hu, identified. Antroduodenal manometry is a useful tool for figuring out the pathogenesis of CIPO symptoms. The amplitude of contractions as well as the temporal and geographical structure of phasic contractions are evaluated by antroduodenal manometry. Enteric neuromuscular function is intact when normal patterns are present, such as the migrating motor complex during fasting and a shift to the postprandial motility pattern after a test meal. Manometry is important to distinguish between two conditions:
neuropathy Peripheral neuropathy, often shortened to neuropathy, refers to damage or disease affecting the nerves. Damage to nerves may impair sensation, movement, gland function, and/or organ function depending on which nerve fibers are affected. Neuropa ...
, in which contractions maintain normal amplitude but are uncoordinated and lack normal physiologic patterns, and
myopathy In medicine, myopathy is a disease of the muscle in which the muscle fibers do not function properly. ''Myopathy'' means muscle disease ( Greek : myo- ''muscle'' + patheia '' -pathy'' : ''suffering''). This meaning implies that the primary defec ...
, in which contraction amplitude is diminished but spatial and temporal organization is retained.


Treatment

Treatment of CIPO is difficult. Following diagnosis, treatment goals should include preventing needless surgery, reestablishing electrolyte and fluid balance, enhancing nutritional status, managing infections, and reducing pain,
nausea Nausea is a diffuse sensation of unease and discomfort, sometimes perceived as an urge to vomit. It can be a debilitating symptom if prolonged and has been described as placing discomfort on the chest, abdomen, or back of the throat. Over 30 d ...
,
vomiting Vomiting (also known as emesis, puking and throwing up) is the forceful expulsion of the contents of one's stomach through the mouth and sometimes the nose. Vomiting can be the result of ailments like food poisoning, gastroenteritis, pre ...
, and
bloating Abdominal bloating (or simply bloating) is a short-term disease that affects the gastrointestinal tract. Bloating is generally characterized by an excess buildup of gas, air or fluids in the stomach. A person may have feelings of tightness, pressu ...
symptoms. If an underlying illness is the cause of CIPO, it should also be promptly addressed. Maximizing oral intake is often recommended. Making up for any nutritional deficiencies should be the first step in any dietary treatment plan. Patients should be urged to eat small, frequent meals (five to six times a day), emphasizing protein and liquid calories, and to stay away from high-fiber and fat items.
Enteral feeding A feeding tube is a medical device used to provide nutrition to people who cannot obtain nutrition by mouth, are unable to swallow safely, or need nutritional supplementation. The state of being fed by a feeding tube is called gavage, enteral fee ...
needs to be the next option if food intake is insufficient to meet nutritional needs. An enteral formula at goal rate can be used to finish a nasogastric or nasojejunal feeding trial prior to the implantation of a permanent
feeding tube A feeding tube is a medical device used to provide nutrition to people who cannot obtain nutrition by mouth, are unable to swallow safely, or need nutritional supplementation. The state of being fed by a feeding tube is called gavage, enteral f ...
. Permanent enteral access may be inserted if it is tolerated without causing severe discomfort. It is usually recommended to bypass the stomach and feed directly into the small intestine if delayed gastric emptying is evident. In patients with CIPO,
parenteral nutrition 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 ph ...
is generally avoided unless enteral intake is insufficient to meet nutritional needs. This is because
parenteral nutrition 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 ph ...
has been linked to
pancreatitis Pancreatitis is a condition characterized by inflammation of the pancreas. The pancreas is a large organ behind the stomach that produces digestive enzymes and a number of hormone A hormone (from the Ancient Greek, Greek participle , "se ...
,
thrombus A thrombus ( thrombi) is a solid or semisolid aggregate from constituents of the blood (platelets, fibrin, red blood cells, white blood cells) within the circulatory system during life. A blood clot is the final product of the blood coagulatio ...
formation,
cellulitis Cellulitis is usually a bacterial infection involving the inner layers of the skin. It specifically affects the dermis and subcutaneous fat. Signs and symptoms include an area of redness which increases in size over a few days. The borders of ...
,
sepsis Sepsis is a potentially life-threatening condition that arises when the body's response to infection causes injury to its own tissues and organs. This initial stage of sepsis is followed by suppression of the immune system. Common signs and s ...
, and nonalcoholic fatty liver disease, which can progress to
fibrosis Fibrosis, also known as fibrotic scarring, is the development of fibrous connective tissue in response to an injury. Fibrosis can be a normal connective tissue deposition or excessive tissue deposition caused by a disease. Repeated injuries, ch ...
and
cirrhosis Cirrhosis, also known as liver cirrhosis or hepatic cirrhosis, chronic liver failure or chronic hepatic failure and end-stage liver disease, is a chronic condition of the liver in which the normal functioning tissue, or parenchyma, is replaced ...
. Unfortunately, because to the degenerative nature of the condition and the absence of viable treatments, many CIPO patients may eventually need
parenteral nutrition 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 ph ...
. When receiving
parenteral nutrition 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 ph ...
, patients should be encouraged and advised to consume as much oral food as they can tolerate. The pharmaceutical treatment of CIPO aims to regulate symptoms and prevent complications. It is frequently necessary to co-prescribe
analgesic An analgesic drug, also called simply an analgesic, antalgic, pain reliever, or painkiller, is any member of the group of drugs used for pain management. Analgesics are conceptually distinct from anesthetics, which temporarily reduce, and in s ...
s,
antiemetic An antiemetic is a drug that is effective against vomiting and nausea. Antiemetics are typically used to treat motion sickness and the side effects of opioid analgesics, general anaesthetics, and chemotherapy directed against cancer. They may ...
s, antisecretory,
antispasmodic An antispasmodic (synonym: spasmolytic) is a pharmaceutical drug or other agent that suppresses muscle spasms. Smooth muscle spasm One type of antispasmodics is used for smooth muscle relaxation, especially in tubular organs of the gastrointestina ...
s,
laxative Laxatives, purgatives, or aperients are substances that loosen stools and increase bowel movements. They are used to treat and prevent constipation. Laxatives vary as to how they work and the side effects they may have. Certain stimulant, lubri ...
s, or antidiarrheal medications.
Prokinetics A prokinetic agent (also prokineticin, gastroprokinetic agent, gastrokinetic agent or propulsive) is a type of drug which enhances gastrointestinal motility by increasing the frequency or strength of contractions, but without disrupting their rhyt ...
are frequently used in an effort to regulate visceral sensitivity and enhance gastrointestinal motility.


Outlook

CIPO typically progresses over time. Patients may experience symptom-free intervals followed by periods of severe symptoms that require emergency room visits and hospitalizations. According to one study, only 11% of people with CIPO experience asymptomatic periods between subacute obstructive episodes and do not need ongoing medical treatment. CIPO sufferers often require complete
parenteral nutrition 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 ph ...
due to their debilitating symptoms. Nutrition deficiency affects up to two-thirds of CIPO patients, with 30%-50% of adults requiring
parenteral nutrition 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 ph ...
(PN) at some point. The long-term prognosis for patients with CIPO is dismal. Chronic abdominal discomfort can lead to opiate dependence for many patients. In a long-term study of 59 patients with CIPO, 4 died from disease-related complications and 4 had small bowel transplant. Pediatric CIPO patients have a mortality rate ranging from 10% to 25% before reaching adulthood.


Epidemiology

CIPO is a rare condition, hence specific information about its incidence and prevalence is unclear. According to a pediatric tertiary care institution, roughly 100 infants are born with CIPO each year in the US. This statistic does not accurately reflect the prevalence of CIPO because it excludes patients who develop it later in life. A review of 378 institutions by the Japanese Society of Gastroenterology found only 160 cases with suspected CIPO, with 138 (86.3%) meeting the rigorous criteria. Research indicates that females are more likely than males to experience CIPO, as seen by published adult patient studies.


See also

*
Gastroparesis Gastroparesis (gastro- from Ancient Greek  – gaster, "stomach"; and -paresis, πάρεσις – "partial paralysis") is a medical disorder of ineffective neuromuscular contractions (peristalsis) of the stomach, resulting in food and l ...
*
Functional gastrointestinal disorder 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 human gastrointestinal tract, gastrointestinal tract and involv ...


References


Further reading

* * * *


External links


Children's Hospital of Philadelphia

Boston Children's Hospital
{{Medical resources , ICD11 = {{ICD11, DA90.2 {{ICD11, DA93.Y , ICD10 = {{ICD10, K59.8 , ICD10CM = , ICD9 = , ICDO = , OMIM = 243180 , OMIM_mult = {{OMIM, 300048, , none {{OMIM, 601223, , none {{OMIM, 609629, , none , MeshID = , DiseasesDB = , SNOMED CT = 235828008 , Curlie = , MedlinePlus = , eMedicineSubj = , eMedicineTopic = , PatientUK = , NCI = , GeneReviewsNBK = , GeneReviewsName = , NORD = chronic-intestinal-pseudo-obstruction , GARDNum = 12744 , GARDName = Chronic intestinal pseudoobstruction , RP = 180450 , AO = , WO = , OrthoInfo = , Orphanet = 2978 , Scholia = , OB = Diseases of intestines Gastrointestinal motility disorders