Mirizzi's syndrome

Named after the Argentinean surgeon Pablo Luis Mirizzi, Mirizzi syndrome is the compression of the hepatic duct secondary to stone impaction and chronic inflammation in the adjacent gallbladder Hartman pouch or in the gallbladder infundibulum and cystic duct (1,2).

• this inflammatory process may lead to development of cholecystobiliary and cholecystoenteric fistulas as different stages of the same disease
• Csendes classification divided this condition into 5 types - from type I or simple external compression of the common hepatic duct to type V, presence of a cholecystoenteric fistula together with any other type of Mirizzi (1)

The condition is rare in Western countries (less than 1% a year) but is more common in underdeveloped countries e.g. - in Latin-America, the reported incidence range from 4.7% to 5.7% (2).

The condition may occur at any age and in any patient with gallstones

• it has a mean age varying from 53 to 70 years
• it is common in females with a frequency of around 70% of all cases

Clinical presentation of Mirizzi syndrome is unspecific. Common presentations of Mirizzi syndrome in patients with known or suspected gallstone disease include:

• obstructive jaundice (60%-100%)
• accompanied by abdominal pain over the right upper abdominal quadrant (50%-100%)
• fever

Diagnosis of Mirizzi syndrome is based on clinical features together with a high degree of suspicion or surgical intuition and preoperative radiological images and endoscopic procedures (2).

• failure of diagnosis of the condition is associated with high preoperative morbidity and mortality
• preoperative diagnosis of Mirizzi syndrome is made in only 8% to 62.5% of patients
• preoperative investigations include
  • ultrasonography – may show
    • one large gallstone or multiple smaller stones in the neck of a contracted gallbladder
    • dilated extra and intra hepatic duct above the level of the obstruction
    • normal size common bile duct under the level of the obstruction
  • CT – radiological signs are nonspecific, but is useful in excluding malignancy in the porta hepatis area or in the liver
  • ERCP - useful to confirm the presence of Mirizzi syndrome with or without fistulas (2)
• intraoperative diagnosis of Mirizzi syndrome - around 50% are diagnosed during surgery

The standard treatment of Mirizzi syndrome is open cholecystectomy.

• if no fistulas - subtotal cholecystectomy
• small fistula (type II) - subtotal cholecystectomy with a T tube inserted into the fistula
• larger fistulae (types III and IV) – hepaticojejunostomy

Frozen section histology is indicated intraoperatively in all cases of Mirizzi syndrome since 6-27% of patients with a diagnosis Mirizzi syndrome had carcinoma of the gallbladder (1)

Reference:

• (1) Elwood DR. Cholecystitis. Surg Clin North Am. 2008;88(6):1241-52
• (2) Beltrán MA. Mirizzi syndrome: history, current knowledge and proposal of a simplified classification. World J Gastroenterol. 2012;18(34):4639-50