Emphysematous cholecystitis is a fulminant variety of acute cholecystitis that differs in its pathology and epidemiology from cholecystitis induced by gallstones. The characteristic feature of this sinister variant of cholecystitis is the presence of gas in the lumen and wall of the gallbladder. The presence of gas may be detected elsewhere in the biliary tract or adjacent structures in addition to gas in the gallbladder wall. Emphysematous cholecystitis occurs in about 1% of all cases of acute cholecystitis but carries significantly higher morbidity and mortality. Individuals most susceptible to emphysematous cholecystitis are people with diabetes mellitus and those with a weak immune system.
The first reported case of emphysematous cholecystitis was by Stoltz in 1901. About 1% to 3% of patients with acute cholecystitis can develop this variant. This fulminant form of cholecystitis has been reported to be more common in older patients who also have diabetes mellitus. The prevalence of emphysematous cholecystitis is three times higher in males compared to females. There is also an association with peripheral vascular disease and immunosuppression. The mortality due to this life-threatening form of cholecystitis is reported to be around 15% to 25%.
Diabetes is noted to be present in 30% to 75% of patients with this disorder. The mean age of diagnosis is around 60.
Micro-organisms that have been isolated in patients with emphysematous cholecystitis include the following:
Ischemia of the gallbladder is considered the key etiological factor in the development of this uncommon, life-threatening condition. This situation develops predominantly in an age group greater than 50 years. There is an increased incidence, especially among male diabetics. In patients who develop emphysematous cholecystitis, gallstones are often detected; however, the proportion of acalculous cholecystitis is three-fold compared to the acute cholecystitis group.
Gallbladder ischemia as a result of vascular compromise of the cystic artery is the initiating factor in the development of this pathology. Poor perfusion results in ischemia and necrosis of the gallbladder wall. The presence of ischemic tissue results in a secondary infection, with gas-forming organisms, and leads to this condition. Gas produced by the gas-forming organisms is located in the lumen or wall of the gallbladder but can occasionally spread to other parts of the biliary tract as well as peritoneum and retroperitoneum.
A background of diabetes in many of these patients creates a microenvironment which promotes the growth of anaerobic bacteria. A pathological examination of removed specimens of emphysematous cholecystitis shows a higher degree of endarteritis obliterans when compared to acute cholecystitis. The causative organisms are E coli, Aerobactor aerogens, Klebsiella spp, and Salmonella spp. Gangrene and perforation and pericholecystic abscess may ensue. Emphysematous cholecystitis is also reported in patients undergoing hemodialysis, with changes during the procedure causing a compromise of visceral circulation including the cystic artery and leading to ischemia and devitalization of the gallbladder. This form of cholecystitis has a significant mortality, bacteria-produced endotoxin, and the higher incidence of gangrene and perforation of the gallbladder.
The symptoms of patients with emphysematous cholecystitis often are suggestive of acute cholecystitis which can be indistinguishable from that of any other acute upper abdominal pathology such as a liver abscess or a perforated duodenal ulcer. The usual combination of presenting symptoms are right upper quadrant pain and fever with nausea or vomiting. The lack of dramatic symptoms often does not alert the clinician to an underlying sinister pathology. The symptoms may be trivial in patients with associated diabetes and renal failure; however, the patient's condition may rapidly deteriorate despite the moderate pain. If not treated early, the patient can progress to a clinical picture of frank sepsis and shock. Signs of peritonitis is an indication of perforation. Whenever there is a delay, the patient can present with tachycardia, hypotension, and cardiovascular collapse.
Ultrasonography, the most common radiological imaging used for gallstone disease diagnosis, has a high specificity for detecting gas in the gallbladder wall. This indicates emphysematous cholecystitis, but the sensitivity is low. During an ultrasonogram, the air in the wall or lumen of the gallbladder can interfere with the clear visualization of the gallbladder The best imaging modality to confirm the presence of emphysematous cholecystitis is a contrast-enhanced abdominal CT scan.
The plain x-ray may reveal air and/or air-fluid levels in the gallbladder.
A CT scan will confirm the presence of gas in the gallbladder wall or lumen and provide accurate information about the presence of air or fluid in the pericholecystic tissue, free air in the peritoneum, or rarely, retroperitoneum. The presence of air in the peritoneum or retroperitoneum represents a more severe form of emphysematous cholecystitis. A suggested classification according to radiological findings as progressive stages starting with detection of air limited to the gallbladder lumen and progressing to findings of air in the gallbladder and the pericholecystic tissue as the most advanced stage. MRI can provide accurate information on the presence of intramural air and necrosis.
Blood work will usually reveal a leucocytosis, and liver function tests may be normal or abnormal. Serum glucose levels are usually elevated.
The definitive management of this condition is an emergency cholecystectomy upon diagnosis. It is possible to perform the cholecystectomy laparoscopically if gangrene or perforation of the gallbladder is not suspected. Clinical suspicion or confirmation of perforation and peritonitis indicates an open-cholecystectomy. Open surgery also is the preferred option if there is an associated pneumoperitoneum to rule out the presence of bowel perforation. The surgeon should have a low threshold for conversion to open due to the high chances of distorted anatomy or very fragile tissues. At surgery, a severely inflamed gallbladder is one with evidence of air in the pericholecystic tissue or foamy collection detected along the hepatoduodenal ligament or the right retroperitoneum. An intraoperative cholangiogram helps to avoid bile duct injury when the anatomy cannot be delineated clearly. In patients without evidence of peritonitis who cannot tolerate anesthesia because of a poor clinical condition, a reasonable intervention is to perform percutaneous radiological drainage as a temporizing option. The cholecystostomy can be followed by removal of the gallbladder at a later date once the patient's condition improves.
Stage 1: Gas in the gallbladder lumen with signs of cholecystitis. Air is only seen in some parts of the gallbladder
Stage 2: Gas in the entire gallbladder wall.
Stage 3: Gas in the pericholecystic fluid, within the wall and adjacent tissues, indicating gangrene and perforation.
Patients with emphysematous cholecystitis can develop the following complications:
Complications from surgery include:
Patients with emphysematous cholecystitis are critically ill and best managed in the intensive care unit. Aggressive hydration, broad-spectrum antibiotics, and cardiovascular support are necessary.
Once the diagnosis is made, the following should be consulted:
Early recognition of emphysematous cholecystitis is necessary to avoid the high mortality that this fulminant condition carries. In a patient with diabetes mellitus and renal failure who presents with right upper quadrant abdominal pain and hypotension, emphysematous cholecystitis should be high on the diagnostic possibilities
Emphysematous cholecystitis is a surgical emergency that carries a high morbidity and mortality. Thus, it is best managed by a multidisciplinary team that includes an endocrinologist, general surgeon, emergency department physician, ICU nurses, and an intensivist. As soon as the diagnosis is made the general surgeon should be consulted as the patient needs an emergency cholecystectomy.
In unstable patients, percutaneous drainage by the radiologist may be life-saving. The cholecystostomy can be followed by removal of the gallbladder at a later date once the patient's condition improves.
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