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Ogilvie syndrome

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Copyright 2012

NORD is very grateful to Prospere Remy, MD, Assistant Professor of Medicine, Albert Einstein College of Medicine, Bronx-Lebanon Hospital, GI Division, for assistance in the preparation of this report.

Synonyms of Ogilvie syndrome

Disorder Subdivisions

General Discussion

Summary
Ogilvie syndrome is a rare, acquired disorder characterized by abnormalities affecting the involuntary, rhythmic muscular contractions (peristalsis) within the colon. Peristalsis propels food and other material through the digestive system through the coordination of muscles, nerves and hormones. The colon is often significantly widened (dilated). Symptoms are similar to other forms of intestinal pseudo-obstruction and can include nausea, vomiting, abdominal bloating or swelling and constipation. The symptoms of Ogilvie syndrome mimic those of mechanical obstruction of the colon, but no such physical obstruction is present. Mechanical obstruction refers to something (e.g., tumor, scar tissue, etc.) physically blocking the passage of food and other material through the GI tract. Ogilvie syndrome is usually associated with an underlying disorder, trauma or surgery. Ogilvie syndrome can be managed with conservative treatment, but if unrecognized and untreated can lead to serious, potentially life-threatening complications.

Introduction
Ogilvie syndrome was first described in the medical literature in 1948 by a British surgeon named Sir William Ogilvie. The disorder is also known as acute colonic pseudo-obstruction (ACPO). It is not the same as chronic intestinal pseudo-obstruction, a similar, but distinct disorder.

Symptoms

The symptoms and severity of Ogilvie syndrome can vary greatly from one person to another. Ogilvie syndrome can potentially cause serious, life-threatening complications. The disorder most often occurs in hospitalized or institutionalized patients who have an underlying illness or have recently undergone surgery.

Common symptoms of Ogilvie syndrome include abdominal swelling (distention) and bloating, abdominal pain, nausea and vomiting. Some individuals have a history of chronic, sometimes severe constipation. Abdominal distention usually develops over several days, but can potentially develop rapidly within a 24-hour period. Colonic distention can be massive. Additional symptoms that can occur including fever, marked abdominal tenderness and an abnormal increase in the number of white blood cells (leukocytosis) often due to infection. Fever, marked abdominal tenderness, and leukocytosis are more common individuals with perforation or ischemia, but can occur in the absence of these conditions.

Distention of the colon in Ogilvie syndrome can potentially lead to serious, life-threatening complications including the formation of a hole in the wall of the colon (perforation) or lack of blood flow (ischemia) to the colon. Perforation may allow the contents of the colon to spill out into the abdominal cavity. A perforated bowel can cause intense abdominal pain, fever, and sepsis, a severe blood infection. The cecum, the large pouch that marks the beginning of the large intestines, is the area most often where the greatest dilation occurs and consequently is most at risk of perforation. Perforation in Ogilvie syndrome is rare developing in only 1-3 percent of affected individuals.

Ischemic bowel results in tissue damage or death in the affected portion of the bowel. Individuals with a perforated or ischemic bowel have a greater incidence of fever and may have signs of inflammation of the peritoneum (peritonitis). The peritoneum is the thin tissue that lines the inside of the abdominal wall and covers most of the abdominal organs.

Causes

The exact cause and underlying mechanisms of Ogilvie syndrome are not fully understood and controversial. The disorder most often occurs in individuals with a recent, serious medical condition or surgical procedure.

The list of conditions that have been associated with Ogilvie syndrome is extensive. The three most common conditions associated with Ogilvie syndrome are non-operative trauma, infection and heart disease, especially a heart attack (myocardial infarction) or congestive heart failure. The most common infections associated with Ogilvie syndrome are pneumonia and sepsis. Surgeries that have been associated with Ogilvie syndrome include abdominal, orthopedic (especially total hip replacement), neurologic, urologic and cardiac surgery. Severe pulmonary disease, malignancy, kidney (renal) disease, respiratory failure, metabolic disorders and severe electrolyte imbalances have also been associated with Ogilvie syndrome.

The use of certain medications has also been associated with the development of Ogilvie syndrome including neuroleptic medications, anticholinergics, amphetamines, steroids and narcotics.

Ogilvie syndrome most likely results from abnormalities affecting the autonomic nervous system's control of colonic motor function. The autonomic nervous system is the portion of the nervous system that controls or regulates certain involuntary body functions including heart rate, blood pressure, temperature regulation, breathing and more. The autonomic nervous system also controls or regulates the bowels and bladder.

Affected Populations

Ogilvie syndrome is believed to affect males and females in equal numbers, although one large study suggested that males may be affected more often than females. Ogilvie syndrome can potentially affect individuals of any age, but most often occurs in late middle age (mean age in the 60s). The exact incidence and prevalence of the disorder is unknown. Because cases can go unrecognized and resolve spontaneously, determining the true frequency of the disorder is the general population is difficult. Ogilvie syndrome generally develops in hospitalized or institutionalized individuals following an acute illness or surgery.

Related Disorders

Symptoms of the following disorders can be similar to those of Ogilvie syndrome. Comparisons may be useful for a differential diagnosis.

Chronic intestinal pseudo-obstruction (CIP) is a rare, potentially disabling gastrointestinal disorder characterized by abnormalities affecting the involuntary, rhythmic muscular contractions (a process called peristalsis) within the gastrointestinal (GI) tract. Peristalsis propels food and other material through the digestive system through the coordination of muscles, nerves and hormones. CIP usually results from abnormalities affecting the muscles or nerves that are involved in peristalsis. Consequently, peristalsis becomes altered and inefficient. The symptoms of CIP resemble those caused by mechanical obstruction of the GI tract. Mechanical obstruction refers to something (such as a tumor, scar tissue, etc.) physically blocking the passage of food and other material through the GI tract. In individuals with CIP no such physical obstruction is present, hence the term pseudo-obstruction. Common symptoms include nausea, abdominal pain, abdominal swelling (distention) and constipation. Ultimately, normal nutritional requirements cannot be met leading to unintended weight loss and malnourishment. CIP can potentially cause severe, even life-threatening complications. Some individuals with CIP may experience a sudden, rapid onset of the disorder that can be mistaken for Ogilvie syndrome. (For more information on this disorder, choose "chronic intestinal pseudo-obstruction" as your search term in the Rare Disease Database.)

The signs and symptoms of mechanical obstruction of the GI tract are almost indistinguishable from those associated with Ogilvie syndrome. Such symptoms include abdominal pain and bloating, nausea and vomiting and constipation. Mechanical obstruction can be caused by a variety of conditions including adhesions and scar tissue, foreign (ingested) material, gallstones, tumors, hernia, abnormal tissue growth, twisting of a loop of the intestines back around itself (volvulus), and intussusception, a condition in which one portion of the intestines slides into another much like a collapsing telescope. It is extremely important to distinguish Ogilvie syndrome from mechanical obstruction of the GI tract as the underlying process and potential treatments are different.

A variety of gastrointestinal disorders can have signs and symptoms that are similar to those seen in Ogilvie syndrome. Such disorders include irritable bowel syndrome (IBS), gastroparesis, functional dyspepsia, Crohn's disease, and cyclic vomiting syndrome. (For more information on these disorders, choose the specific disorder name as your search term in the Rare Disease Database.)

Standard Therapies

Diagnosis
A diagnosis of Ogilvie syndrome is made based upon identification of characteristic symptoms, a detailed patient history, a thorough clinical evaluation and a variety of specialized tests to rule out other conditions or identify underlying causes.

Clinical Testing and Work-Up
Ogilvie syndrome is virtually indistinguishable from mechanical obstruction based solely on signs and symptoms. X-ray examination of the colon will be performed to rule out mechanical obstruction. Plain abdominal films (radiographs) can reveal an abnormally expanded (dilated) colon. Plain abdominal radiographs can also reveal dilation and abnormal air-fluid levels in the small bowel, both of which are indicative of intestinal obstruction.

A water-soluble enema or computed tomography should be performed to rule out mechanical obstruction in cases where gas and distention does not occur throughout the entire colon. A water-soluble enema is a procedure that allows a physician to evaluate the large bowel. During the exam, a soft, thin tube is inserted into the anal passage. Dye is injected into the tube and x-rays will be taken. The dye will show the outline of the large bowel on the x-ray, revealing mechanical obstruction if present. During CT scanning, a computer and x-rays are used to create a film showing cross-sectional images of certain tissue structures such as the colon.

Treatment
There is no specific therapy for Ogilvie syndrome. Therapeutic options include support therapy, medications, decompression and surgery. Most therapeutic options have not undergone extensive controlled clinical study.

Specific therapeutic procedures and interventions may vary, depending upon numerous factors, such as disease progression; the presence or absence of certain symptoms; the status of the bowel; an individual's age and general health; and/or other elements. Decisions concerning the use of particular therapeutic options should be made by physicians and other members of the health care team in careful consultation with the patient based upon the specifics of his or her case; a thorough discussion of the potential benefits and risks, including possible side effects and long-term effects; patient preference; and other appropriate factors.

When planning treating, physicians must weigh conservative treatment and observation versus proceeding with a surgical decompression of the dilated colon. Most individuals respond to conservative management.

The initial management of Ogilvie syndrome requires an examination to detect signs of bowel perforation or ischemia. Signs of these complications require immediate medical intervention.

In cases where an identifiable cause for Ogilvie syndrome (e.g., respiratory failure, congestive heart failure, infection) has been established, treatment of the underlying condition is mandatory. If Ogilvie syndrome is linked to the use of certain medications, affected individuals must stop taking the medications.

Supportive therapy may include withholding oral food and fluid intake, administering intravenous fluids to correct fluid and electrolyte imbalances, employing nasogastric suction to limit the amount of air that is swallowed to avoid air from further expanding the colon, and inserting a long, thin tube into the rectum to allow the release of gas and stool, a procedure known as rectal tube to gravity drainage. Stopping the use of medications that can affect colon motility such as opiates and anticholinergics is also recommended. The success of supportive therapy varies among affected individuals.

One of the few treatment options for Ogilvie syndrome that has undergone clinical study is a medication known as neostigmine. Studies have shown that intravenous administration of neostigmine has led to rapid decompression of the colon in individuals with Ogilvie syndrome who did not respond to conservative management. Neostigmine is believed to interfere with the breakdown of the neurotransmitter acetylcholine, increasing the duration and activity of acetylcholine. Acetylcholine helps the communication between nerve and muscle cells, such as those in the GI tract. Neostigmine improves GI motility and increases the transit of food and other material through the GI tract. Although infrequent, colonic distention can recur following successful treatment with neostigmine.

Some individuals with Ogilvie syndrome may be treated with colonic decompression, a procedure that reduces pressure within the colon. Usually, this treatment is reserved for individuals with persistent, marked colonic distention who have not responded to other treatment options. A specific procedure known as colonoscopic decompression, in which a thin, flexible tube is inserted into the anal passage and threaded up to the colon, may be used. Although colonoscopic decompression has not undergone clinical study, numerous reports in the medical literature cite it as an effective method for removing air from the colon and, potentially, reducing the risk of perforation. In addition, some individuals may require the insertion of a decompression tube within the colon in to order to achieve decompression or reduce the risk of recurrence. Decompression techniques carry risks including perforation. There is also a risk of disease recurrence.

In rare cases, individuals with Ogilvie syndrome may require surgical intervention. Surgery is used when affected individuals have signs of perforation or ischemia or have failed to respond to other treatment options. Surgery can be associated with significant morbidity and mortality due in part to the severity of the underlying condition. The specific type of surgery used may vary depending upon the status of the bowel. If perforation or ischemia is not present, a cecostomy is usually performed. A cecostomy is a procedure in which an artificial opening is created in the cecum, allowing physicians to "vent" excess air or contents from the colon. This procedure has a high success rate in individuals with Ogilvie syndrome.

If perforation or ischemia is present, surgery to remove a portion of the colon (subtotal colectomy) may be necessary. The specific surgical procedure used can vary based upon several factors such as the status of the cecum.

Investigational Therapies

There are anecdotal case reports in the medical literature of successful treatment of Ogilvie syndrome with medications including erythromycin, cisapride and metoclopramide. Cisapride, however, is no longer widely available after being voluntarily pulled from the market in 2000 because of an association with serious cardiac arrhythmias.

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, in the main, contact:
www.centerwatch.com

Organizations related to Ogilvie syndrome

References

TEXTBOOKS
Saunders MD. Ogilvie's Syndrome. In: Evidence-based Gastroenterology and Hepatology, McDonald JWD, Burroughs AK, Feagan BG, Fennerty MB, editors. 2010 Wiley-Blackwell, Hoboken, NJ. Pp. 377-384.

Gladstone L, Bernstein M, Teixeira M, Harris A. Pseudo-Obstruction (Ogilvie's), Cathartic Colon-Laxative Abuse, and Melanosis. In: Diseases of the Colon, Wexner SD, Stollman N, editors. 2007 Informa Healthcare USA, New York, NY. Pp. 449-462.

JOURNAL ARTICLES
De Giorgio R, Knowles CH. Acute colonic pseudo-obstruction. Br J Surg. 2009;96:229-239. http://www.ncbi.nlm.nih.gov/pubmed/19224517

McNamara R, Mihalakis MJ. Acute colonic pseudo-obstruction: rapid correction with neostigmine in the emergency department. J Emerg Med. 2008;35:167-170. http://www.ncbi.nlm.nih.gov/pubmed/18242923

Saunders MD. Acute colonic pseudo-obstruction. Best Pract Res Clin Gastroenterol. 2007;21:671-687. http://www.ncbi.nlm.nih.gov/pubmed/17643908

Da-Peng J, Zhao-Zhu Li, Sheng-Yang G, Yu-Bo Z. Treatment of pediatric Ogilvie's syndrome with low-dose erythromycin: a case report. World J Gastroenterol. 2007;13:2002-2003. http://www.wjgnet.com/1007-9327/13/2002.pdf.

Saunders MD, Kimmey MB. Systemic review: acute colonic pseudo-obstruction. Aliment Pharmacol Ther. 2005;22:917-925. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2036.2005.02668.x/pdf

Maloney N, Vargas HD. Acute intestinal pseudo-obstruction (Ogilvie's syndrome). Clin Colon Rectal Surg. 2005;18:96-101. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2780141/?tool=pubmed

Ponec RJ, Saunders MD, Kimmey MB. Neostigmine for the treatment of acute colonic pseudo-obstruction. N Engl J Med. 1999;341:137-141. http://www.nejm.org/doi/pdf/10.1056/NEJM199907153410301

INTERNET
Remy P, Kumbum K. Ogilvie Syndrome. Emedicine Journal, Dec 9, 2009. Available at: http://emedicine.medscape.com/article/184579-overview Accessed on: December 22, 2012.

Report last updated: 2012/03/13 00:00:00 GMT+0