Mirizzi's syndrome

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Mirizzi’s syndrome is an uncommon clinical condition caused by the compression of common hepatic duct due to impaction of gallstone in the cystic duct or neck of the gallbladder. This results in acquired obstructive jaundice, which could be due to direct extrinsic compression of the bile duct or fibrosis caused by chronic cholecystitis (inflammation). The chronic inflammation and ulceration may also result in cholecystobiliary fistula or cholecystoenteric fistula. ERCP is the gold standard for diagnosis, however, ultrasonography, CT scan and MRCP are commonly done. Surgical management is the mainstay treatment for Mirizzi’s syndrome.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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• This clinical condition was first described in early 1900s by Kehr and Ruge. ^[1]
• However, the term “Mirizzi Syndrome” was adopted in 1948 after the work of Pablo Mirizzi. ^[1] Hence, it is named for Pablo Mirizzi.^[2][3]

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

• Mirizzi’s syndrome is caused by gallstone impaction in the cystic duct or neck of gallbladder resulting in chronic inflammation that leads to the compression, necrosis and fibrosis of common bile duct.
• This can result in fistula formation into the adjacent structures like common hepatic duct or common bile duct.
• The obstruction of bile duct either by direct compression from gallstone or scar formation results in obstructive jaundice.

• It can be divided into the following types: ^[1]

• Type I lesions involve external compression of the common bile duct without any fistula formation.

• Type II lesions involve cholecystobiliary fistula with erosion of less than one‐third of the circumference of the bile duct.

• Type III lesions are fistula that involve up to two‐thirds of the duct circumference.

• Type IV lesions are complete destruction of the bile duct. ^[2]

• Type V lesions described in 2008 by Beltran et al are any of the above 4 types plus the formation of cholecystoenteric fistula. ^{[3] [1]}

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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• Mirizzi’s syndrome is caused by gallstones that result in compression and inflammation of the common hepatic duct.

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Differential diagnosis of Mirizzi’s syndrome includes any other benign or malignant cause of obstructive jaundice such as: ^[1]

• gallbladder cancer
• cholangiocarcinoma
• pancreatic cancer
• sclerosing cholangitis
• metastatic disease

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Mirizzi’s syndrome is common in mean age varying from 53 to 70 years. However, it may occur at any age and in any patient with gallstones. ^[1]

70% of all cases are females. ^[1]

There is no evidence of race having any bearing on the epidemiology.

Occurs in approximately 0.1% of patients with gallstone disease^[2] and 0.7-1.4% of patients undergoing cholecystectomy^[3]

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• A cystic duct with low insertion into the common bile duct has been described as a risk factor for Mirizzi Syndrome. ^[1]
• A tortuous cystic duct is also thought to be a risk factor.

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Natural History

• Mirizzi syndrome frequently presents in an acute form. However, chronic form is equally common or an even more common form of presentation.
• Clinical presentation of Mirizzi’s syndrome could be non-specific.
• However, it presents most commonly with obstructive jaundice (60%-100%), accompanied by right upper quadrant abdominal pain (50%-100%) and fever. ^[1]
• Patient may be a known or suspected case of gallstone disease.
• Previous history of jaundice is sometimes present.
• Patients frequently present in the setting of acute cholecystitis, acute cholangitis, acute pancreatitis or gallstone ileus.

Complications

• Cholecystocholedochal fistula
• Cholecystoenteric fistula
• Cutaneous fistulas.
• Secondary biliary cirrhosis.
• Delayed onset biliary strictures.
• Operative and postoperative morbidity and mortality (according to the severity of lesion).

Prognosis

• Severe inflammation and delayed or missed diagnosis may result in high morbidity and mortality. Surgical management is the mainstay treatment. ^[2]