Introduction
Background
Extensive calcium encrustation of the gallbladder wall has been variably termed calcified gallbladder, calcifying cholecystitis, or cholecystopathia chronica calcarea. The term "porcelain gallbladder" has been used to emphasize the blue discoloration and brittle consistency of the gallbladder wall at surgery. Some authorities eschew these terms and instead call all calcified gallbladders "porcelain gallbladders." The true incidence of porcelain gallbladder is unknown, but it is reported to be 0.6-0.8%, with a male-to-female ratio of 1:5. Most porcelain gallbladders (90%) are associated with gallstones.1
Patients with porcelain gallbladder are usually asymptomatic, and the condition is usually found incidentally on plain abdominal radiographs, sonograms, or computed tomography (CT) images. Surgical treatment of porcelain gallbladder is based on results from studies performed in 1931 and 1962, which revealed an association between porcelain gallbladder and gallbladder carcinoma. Porcelain gallbladder is an uncommon condition; recognizing the clinical and imaging characteristics of the disease is important because of the high frequency (22%) of adenocarcinoma in porcelain gallbladder.2 Nonetheless, the causal relationship between porcelain gallbladder and malignancy has not been established. Surgery should not be delayed even if the patient is asymptomatic, because the occurrence of carcinoma in porcelain gallbladder is remarkably high.3
Related Medscape topics:
Resource Center Gallbladder and Biliary Disease
CME Bariatric Emergencies
Related eMedicine topics:
Gallbladder, Carcinoma
Gallbladder Tumors
Gallbladder Cancer
Pathophysiology
Histologically, flakes of dystrophic calcium exist within the chronically inflamed gallbladder wall. The muscular wall of the gallbladder undergoes fibrotic changes. Microliths are diffusely scattered throughout the mucosa, submucosa, and glandular spaces, as well as in the Rokitansky-Aschoff sinuses. Calcification occurs in 2 forms: (1) a broad continuous band of calcification in the muscularis and (2) multiple punctate calcifications in the mucosa and glandular spaces of the mucosa. Gallstones are present in 90% of patients, and hydrops can obstruct the cystic duct. Most authorities consider gallbladder wall calcification to be secondary to a low-grade inflammation, but intramural hemorrhage and an imbalance in calcium metabolism are implicated as well.
Controversy still exists as to the causal relationship between porcelain gallbladder and malignancy. In an analysis of 10,741 cholecystectomies performed between 1955 and 1998, Towfigh et al identified 0.14% patients with porcelain gallbladder without any associated carcinoma. In addition, none of the patients with gallbladder carcinoma had a calcified gallbladder. The authors argue that this association is based on studies related to the surgical management of porcelain gallbladder performed in 1931 and 1962 that indicated a correlation between porcelain gallbladder and carcinoma. They suggest that with a better understanding of the natural history of porcelain gallbladder, the current management of these patients may change.4
Rooholamini et al retrospectively studied 59 cases of histologically proved gallbladder carcinoma and found an association with porcelain gallbladder in 4% of cases.5
In their study, Stephen and Berger also found a low risk of cancer in association with porcelain gallbladder. Gallbladder specimens from 25,900 patients from 1962-1999 were analyzed at Massachusetts GeneralHospital. The study recorded 2 types of calcified gallbladders: those with complete intramural calcification, and those with selective mucosal calcification. The authors found a significant association between mucosal calcification and gallbladder carcinoma; the incidence of cancer arising in a gallbladder with selective mucosal wall calcification was approximately 7%, with an odds ratio of 13.89 (P =.01). The study suggested a significant association between the pattern of calcification and gallbladder carcinoma; selective mucosal calcification poses a significant risk of cancer, whereas diffuse intramural calcification does not.6
Calcification in the right upper quadrant of the abdomen has several causes. Calcification can be categorized by the organ system in which it appears; for example, calcification can affect the liver, gallbladder, right kidney, digestive tract, peritoneal cavity, right adrenal gland, and retroperitoneum. Diseases associated with these organs include large gallbladder opaque calculi, milk-of-calcium bile (see Image 9), echinococcal cysts (see Images 10-11), schistosomiasis and other granulomatous diseases,7 old liver infarcts that have healed (see Image 12), calcified renal cysts, renal calculi, calcified nonparasitic liver cysts, primary and metastatic liver tumors, benign liver tumors, and calcification in old adrenal hemorrhage and adrenal masses.
Frequency
United States
The overall incidence of porcelain gallbladder in the United States appears to be identical to the international incidence (see International, below).
International
Because most cases of gallbladder calcification are not reported, determining the exact incidence is difficult; however, studies of cholecystectomy specimens reveal a 0.6-0.8% occurrence rate for extensive mural calcification.1
Mortality/Morbidity
The clinical importance of porcelain gallbladder lies in its significant association with gallbladder carcinoma. Because patients with gallbladder carcinoma usually have a poor prognosis, most authors agree that carcinoma occurs in association with porcelain gallbladder with sufficient frequency to warrant prophylactic cholecystectomy.
Race
No racial predilection is reported. In regions with a high incidence of gallstone disease, a high incidence of porcelain gallbladder might be expected; however, this relationship has not been demonstrated to date.
Calcified hydatid cysts in the liver are fairly common in endemic areas, such as the Middle East, Eastern and Mediterranean Europe, and North Africa. These cysts can mimic porcelain gallbladder on plain abdominal radiographs; however, the patient's country of origin or history of travel to endemic regions can suggest the diagnosis of calcified hydatid cysts. Ultrasonography will reveal the true gallbladder.
Sex
The male-to-female ratio is 1:5.1
Age
The mean age of patients is 54 years, with an age range of 38-70 years. Porcelain gallbladder is exceptionally rare in children; the only case described is of a 10-year-old girl who underwent a prophylactic cholecystectomy.8
Presentation
Characteristically, the condition is clinically covert, although a palpable mass may occasionally be found. The diagnosis of porcelain gallbladder is frequently made because of incidental findings on plain abdominal radiographs, sonograms, or CT images; sometimes, a mass may be palpable in the right upper quadrant of the abdomen.
The association between gallbladder malignancy and porcelain gallbladder was established on the basis of the results of studies performed in 1931 and 1962.4 Since then, sporadic case reports and collections of cases have appeared in the literature, and their findings have reinforced this association.
A study conducted by Towfigh et al contradicts this time-honored view. The authors reviewed the medical records of 10,741 patients who underwent cholecystectomies in the years 1955-1998. Pathology slides were evaluated for evidence of calcification and gallbladder carcinoma. The incidence of porcelain gallbladder was 0.14% (15 patients) in the series; 10 patients had symptoms suggestive of biliary colic or cholecystitis, and the diagnosis was incidental in 5.
All specimens examined showed histologic evidence of chronic cholecystitis and partial calcification of the gallbladder wall. Gallstones were found in 60% of the patients, but none had gallbladder carcinoma. During the same period, 0.82% of the patients had gallbladder carcinoma, none of whom had gallbladder wall calcification. The study did not reveal carcinoma in patients with porcelain gallbladder. In addition, none of the patients with gallbladder carcinoma had porcelain gallbladder. The authors concluded that with a better understanding of the natural history of porcelain gallbladder, patient treatment may change; however, until further studies confirm these findings, the importance of surgical treatment cannot be overemphasized, considering the frequency of carcinoma associated with porcelain gallbladder (as reported in previous studies).
Most authors agree that carcinoma occurs in association with porcelain gallbladder with sufficient frequency to warrant prophylactic cholecystectomy. Most carcinomas associated with porcelain gallbladder are diffusely infiltrating adenocarcinomas, although squamous cell carcinoma has been described as well. In rare cases, calcification that has precipitated in mucus within neoplastic glandular tissue may also be visible on plain radiographs; this calcification can mimic a carcinoma that is developing in a porcelain gallbladder.
The mechanism by which a malignant transformation occurs in porcelain gallbladder is unknown, although degeneration and regeneration within the gallbladder epithelium is suggested to produce a carcinogenic stimulus. A chemical carcinogen formed or present within stagnant bile is also a possible cause of carcinoma. Using animal models, Petrov and Krotkina were able to induce gallbladder carcinoma by implanting hard foreign bodies in the gallbladder wall.9
Preferred Examination
Although most porcelain gallbladders are incidentally seen on plain abdominal radiographs, the definition and sensitivity provided by CT scanning appears to be far superior to the definition and sensitivity of radiography. CT is also superior to radiography for staging gallbladder carcinoma when it is a complication of porcelain gallbladder.
Sonograms do not depict porcelain gallbladder as well as CT scans do; sonographic findings can mimic those seen with a nonfunctioning gallbladder, large calculus, and emphysematous cholecystitis. (Patients with emphysematous cholecystitis usually have diabetes with no point tenderness [ie, diabetic neuropathy]. In one third of these patients, the white blood cell [WBC] count is within the normal range. High-level echoes that outline the gallbladder result from gas within the gallbladder wall. With emphysematous cholecystitis, the male-to-female ratio is 5:1.)
Occasionally, hepatobiliary surgeons may order angiograms when a malignant change has occurred and staging is required.
Limitations of Techniques
In porcelain gallbladder, plain radiographic findings are usually straightforward and are not often confused with findings related to other causes of calcification in the right upper quadrant. If doubt exists, cross-sectional imaging with a modality such as ultrasonography or CT can more accurately depict calcification in the appropriate organ.10
Porcelain gallbladder must be distinguished from large solitary calcified gallstones, which are seldom as large as porcelain gallbladders; however, exceptions can make a definite diagnosis difficult. Milky bile syndrome is characterized by radiopaque material that causes sufficient opacification of the gallbladder to cause it to be depicted on plain abdominal radiographs. Calculi in the cystic duct and/or Hartmann pouch usually obstruct the gallbladder, and the gallbladder wall will appear inflamed. The spontaneous expulsion of limy bile along with gallbladder calculi has been reported. The puttylike radiopaque material consists of calcium carbonate or, less commonly, calcium phosphate or calcium bilirubinate.
The appearance of limy bile syndrome may simulate that of the gallbladder after the oral or intravenous administration of contrast medium; differentiation between limy bile syndrome and the gallbladder requires knowing whether cholecystographic contrast medium has been administered to the patient.
Calcification of the gallbladder wall or milk-of-calcium bile may have identical appearances on sonograms; therefore, plain radiography is important in distinguishing these entities. Calcified hydatid cysts in the liver are fairly common in endemic areas, such as the Middle East, Eastern and Mediterranean Europe, and North Africa. These cysts can mimic porcelain gallbladder on plain abdominal radiographs; however, the patient's country of origin or history of travel to endemic regions suggest the diagnosis of calcified hydatid cysts. Ultrasonography will reveal the true gallbladder. Fataar et al described calcified gallbladder granulomas in schistosomal infestation that are dense enough to be seen on abdominal radiographs.7
Serpiginous calcification, as seen on plain radiographs of the abdomen in the region of the gallbladder neck, appears to indicate gallbladder schistosomiasis in patients from endemic areas.7 Calcifications in nonparasitic hepatic and renal cysts, in the adrenal gland, and in liver tumors usually are dissimilar to those in porcelain gallbladder. If confusion remains, sonograms or CT scans can be used to clarify the issue. Emphysematous cholecystitis can mimic porcelain gallbladder on sonograms; however, their clinical presentation is distinct from that of porcelain gallbladder. Ring-down shadows from gas within the gallbladder wall or lumen may be evident, and plain radiographs may show gas within the gallbladder fossa.
Differential Diagnoses
Other Problems to Be Considered
Right upper quadrant calcifications
Calculus disease of the gallbladder
More on Porcelain Gallbladder |
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References
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Further Reading
Keywords
calcified gallbladder, calcifying cholecystitis, cholecystopathia chronica calcarea, blue gallbladder wall, gallbladder discoloration, brittle gallbladder, calcified gallbladder, gallbladder wall calcification
