Ogilvie syndrome also known as acute colonic pseudo-obstruction, is a rare acquired disorder that affects the large intestines (colon) characterized by abnormalities affecting the involuntary, rhythmic muscular contractions (peristalsis) within the colon 1. The colon is often significantly widened (dilated) without evidence of underlying mechanical or anatomic cause. Dilatation of the bowel is classically confined to the cecum and ascending colon with transition near the splenic flexure. Ogilvie syndrome (acute colonic pseudo-obstruction) is most commonly encountered in older adults with multiple underlying comorbidities but may also develop in otherwise healthy patients after traumatic injury or following surgical operation.
Although the signs and symptoms of Ogilvie syndrome mimic those of an intestinal blockage, there is no physical obstruction. Instead, the symptoms are due to nerve or muscle problems that affect peristalsis (the involuntary, rhythmic muscular contractions that move food, fluid, and air through the intestines). In people affected by Ogilvie syndrome, the symptoms come on suddenly and may include abdominal pain, nausea, vomiting, abdominal distention (swelling), constipation, and/or diarrhea. Symptoms and signs of Ogilvie syndrome usually manifest over the course of 3 to 5 days but may also develop more acutely, sometimes within 48 hours 1. If untreated, the condition can cause malnutrition, bacterial overgrowth in the intestines, and weight loss. Ogilvie syndrome is considered complicated when the patient develops any evidence of bowel ischemia, peritonitis, or perforation; the risk of complication increases directly with increasing cecal diameter and duration of illness.
The underlying cause of Ogilvie syndrome is poorly understood. Ogilvie syndrome most often occurs in individuals with a recent, serious medical condition or surgical procedure. Ogilvie syndrome usually occurs in adults when the colon becomes enlarged after surgery, illness or injury.
Advanced age, comorbidities associated with electrolyte imbalance or polypharmacy, and poor underlying functional status or immobility are all strongly associated with Ogilvie syndrome. Older adults who have been hospitalized, even for non-operative care, are at increased risk. In a frequently cited retrospective study of 400 patients with Ogilvie syndrome, non-operative trauma, severe infection, and admission for cardiovascular disease were each considered predisposing conditions in approximately 10% of cases. Although all surgical operations place a patient at some increased risk for ileus, major orthopedic and obstetric procedures are most associated with the development of Ogilvie syndrome.
There appears to be a relationship to cesarean operations for unclear reasons. It is important to remember that not all patients that develop Ogilvie syndrome are hospitalized. Elderly adults presenting to the emergency department or other acute care settings from long-term care facilities or nursing homes, those with an underlying progressive neurological disease, or with recent abdominal surgery are all at increased risk.
The incidence of Ogilvie syndrome is frequently cited as approximately 100 cases per 100,000 hospital admissions every year, although some underreporting is suspected. Prevalence appears to be slightly increased in males, but the reason for this is unknown. Average age at presentation is approximately 60 years. Almost all patients have multiple underlying co-morbidities, and those who are functionally dependent at baseline develop the disease with the highest frequency. Surgical patients are most likely to be diagnosed on postoperative days 3 to 5.
The diagnosis of Ogilvie syndrome (acute colonic pseudo-obstruction) is one of exclusion and that more common causes of functional or mechanical bowel dilatation must be investigated.
Treatment options for Ogilvie syndrome include supportive therapy with close observation that addresses associated symptoms, medications, decompression (a procedure that reduces pressure within the colon) with uncomplicated Ogilvie syndrome (acute colonic pseudo-obstruction) 2. Invasive procedures or surgery are indicated for disease refractory to conservative therapy or for those with a more severe initial presentation. Many patients recover with appropriate intervention although morbidity and mortality increase substantially in patients who develop complications at any time during management 3.
The intestines are part of the gastrointestinal (GI) tract and include the small intestine and the large intestine. The small intestine is the organ where most digestion occurs. The small intestine measures about 20 feet and includes the:
- duodenum, the first part of the small intestine
- jejunum, the middle section of the small intestine
- ileum, the lower end of the small intestine
The large intestine absorbs water from stool and changes it from a liquid to a solid form, which passes out of the body during a bowel movement. The large intestine measures about 5 feet and includes the:
- cecum, the first part of the large intestine, which is connected to the ileum
- colon, the part of the large intestine extending from the cecum to the rectum
- rectum, the lower end of the large intestine leading to the anus
Ogilvie syndrome causes
The exact cause and underlying mechanisms of Ogilvie syndrome are not fully understood and controversial; many clinical conditions that place a patient at increased risk have been identified.
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.
Ogilvie syndrome symptoms
The symptoms and severity of Ogilvie syndrome can vary greatly from one person to another 4. Ogilvie syndrome can potentially cause serious, life-threatening complications. Ogilvie syndrome most often occurs in hospitalized or institutionalized patients who have an underlying illness or have recently undergone surgery.
Ogilvie syndrome symptoms may include:
- abdominal swelling or bloating, also called distension
- abdominal pain
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.
Over time, Ogilvie syndrome can cause malnutrition, bacterial overgrowth in the intestines, and weight loss. Malnutrition is a condition that develops when the body does not get the right amount of the vitamins, minerals, and other nutrients it needs to maintain healthy tissues and organ function.
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.
Ogilvie syndrome 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 (CT) 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.
Ogilvie syndrome treatment
The primary goal of treatment is urgent bowel decompression. Treatment options for Ogilvie syndrome include conservative therapy with observation, direct pharmacologic interventions, and endoscopic therapies. Surgical or open operative interventions are reserved for those failing endoscopic procedures, or for patients who develop ischemia or perforation 1.
Patients diagnosed with uncomplicated Ogilvie syndrome should be admitted to the hospital to a unit where they can be carefully monitored. Initial management includes nothing by mouth (NPO) status and placement of a nasal gastric tube to aid in decompression. Patients should be carefully resuscitated with fluid as indicated. Electrolyte abnormalities should be aggressively corrected as well as any other underlying disease exacerbations. Medications that impact colonic motility such as opiates and anticholinergics should be discontinued as soon as possible, and it is also recommended that laxative medications be stopped. Patients should attempt to ambulate as frequently as clinically tolerated and while in bed should be encouraged to lie prone or in a knee-to-chest position with frequent turns to promote passage of flatus. Frequent repeat abdominal examinations are required. Laboratory tests, especially complete blood count (CBC) and electrolytes, are recommended to be collected at least daily. A daily abdominal plain film should be obtained to monitor cecal dilatation. If there is no development of signs of complicated disease and cecal diameter remains less than 12 cm, this conservative approach should be continued for 72 hours and has a success rate of up to 90%.
Pharmacologic therapy should be initiated for patients who do not improve within 72 hours, have a duration of Ogilvie syndrome greater than 4 days, or develop a cecal diameter of greater than 12 cm. Neostigmine, a short-acting anticholinesterase, is the drug of choice for treatment of Ogilvie syndrome, and evidence and popularity for its use in uncomplicated disease is quickly increasing. Resolution of disease is defined clinically as the passage of flatus or stool and or decreasing cecal diameter. The medication is usually administered as a 2-mg, slow, IV push over 2 to 5 minutes. Contraindications and adverse effects should be reviewed before use. Common adverse effects include abdominal discomfort, salivation, and vomiting which are usually benign and transient. Patients should be closely monitored for more severe complications such as bradycardia and bronchorrhea. It is recommended that patients be on a continuous cardiac monitor during administration and for 30 additional minutes after administration. Atropine for symptomatic bradycardia should be available per ACLS guidelines. Glycopyrrolate can be used for concerning respiratory secretions. A clinical response is typically seen within 2 to 30 minutes of administration. Average efficacy after one dose of neostigmine in randomized controlled trials is approximately 90%, with most non-responders having a resolution of disease after a second 2-mg dose, which can be given 90 minutes to 3 hours after the first. A 24-hour neostigmine drip regimen has also been evaluated with a reported success rate of 85% and with fewer reported adverse effects. After an initial response to neostigmine, patients should continue to be monitored as approximately 30% are at risk of recurrence of cecal dilatation. There is evidence that oral administration of polyethylene glycol immediately after neostigmine response prevents this.
Patients who have contraindications to or fail pharmacologic therapy should be evaluated for endoscopic decompression. The procedure is considered technically difficult, and success rates are directly related to operator experience. Decompression is attempted through colonoscopy without gas insufflation or bowel preparation. After dilated bowel has been evaluated and suction has removed air, a colonic decompression tube is usually placed as close to the cecum as possible via the endoscope. The decompression tube is then left to drainage externally with intermittent flushes. There is an approximately 3% risk of iatrogenic perforation during this procedure, and reoccurrence is still possible although the placement of a decompression tube significantly reduces this event.
Traditional operative management is necessary when the interventions above are unsuccessful or when there is the development of bowel ischemia or perforation. If a perforation is not present, a cecostomy decompression procedure can be attempted. If there is severe ischemia found on endoscopy or perforation occurs, some degree of open resection is indicated with likely Hartmann procedure. Mortality rates increase substantially in Ogilvie syndrome when operative management is needed, regardless of whether perforation or non-viable bowel is present, and all attempts should be made to manage this disease more conservatively 5.
Ogilvie syndrome prognosis
The expected prognosis confounded by the association of multiple co-morbidities in the usual patient population as well as the underlying disease contributing to the development of Ogilvie syndrome. In general, expected mortality after diagnosis of uncomplicated Ogilvie syndrome is approximately 15%. Complicated Ogilvie syndrome occurs in 3% to 15% of patients which is associated with a much worse prognosis with average mortality rates between 30% and 40%. Factors associated with the development of complications include cecal diameter and duration of illness. In one study, no patients with a cecal diameter of less than 12 cm developed complicated disease, but 23% of patients with a cecal diameter of 14 cm did. Duration of dilation appears to have the most direct association with complicated disease and becomes concerning after 5 days, with a reported 5-fold increase in mortality rate after 7 days of unresolved disease. In patients who are actively treated, operative management has the highest mortality rate, which is likely due to operative therapy being reserved for those with complications or prolonged disease course; even without bowel ischemia, postoperative mortality rates are 26%. As can be seen, patient prognosis and survival depend on recognition, diagnosis, and timely colonic decompression.References
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