Clinical History
87-year-old male patient presented with right upper quadrant pain, nausea, vomiting, weight loss, jaundice, and pale stool. Clinical examination and laboratory tests confirmed obstructive jaundice.
Imaging Findings
Ultrasonography (US) showed an impacted gallstone in the gallbladder neck, further gallstones in the body of the gallbladder, and intrahepatic biliary dilatation.
Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) of the liver and biliary tract including MR cholangiopancreatography (MRCP) demonstrated a gallstone impacted in the neck of the gallbladder causing extrinsic compression of the common hepatic duct, right and left central hepatic ducts with resultant intrahepatic biliary dilatation.
The intraoperative appearances were compatible with imaging findings. The patient underwent cholecystectomy, biliary reconstruction and hepaticojejunostomy with an uneventful recovery.
Discussion
Mirizzi syndrome is an uncommon condition in which the impacted gallstone in the gallbladder infundibulum or cystic duct is compressing the common bile duct with resultant obstruction.[1]
Although initially described by Kehr in 1905 and Ruge in 1908, the name “Mirizzi Syndrome” was used after Pablo Luis Mirizzi, an Argentinian surgeon, in 1948. [2, 3]
Pathophysiology involves bile duct compression with pressure effect from an embedded stone in the gallbladder neck or infundibulum. The consequent chronic inflammation and ulceration forms cholecystocholedochal and cholecystoenteric fistulae. [2]
Incidence is 4.7% - 5.7% per year in developing countries and below 1% in developed countries. Prevalence is in 4th to 7th decades with no sex predilection. [3]
Obstructive jaundice with right upper quadrant abdominal pain and fever in a patient with gallstone disease is the most frequent clinical manifestation. [4]
Laboratory results consist of elevated aminotransaminases, hyperbilirubinemia and leukocytosis. High cancer antigen (CA) 19-9 levels have been detected in some cases. [1]
Ultrasonography (US) with accuracy 29% and sensitivity 8.3% to 27% can show gallstones, atrophic gallbladder, cholecystitis, dilated common hepatic duct with a normal calibre distal CBD. [2]
Computed Tomography (CT) can display stones at the junction of the cystic duct and common hepatic duct, proximal biliary dilation and cholecystobiliary fistula. [5]
MRCP, the preferred noninvasive test with 50% accuracy, can reveal gallstone location, gallbladder inflammation, biliary dilation, common bile duct narrowing and fistulae. [2, 5]
Endoscopic retrograde cholangiopancreatography (ERCP) is a gold standard test despite of its invasiveness with accuracy 55% to 90%. It also has a therapeutic role in retrieval of stones and biliary stent placement. [2, 4]
More than 50% of diagnoses are only made during surgery.[4] The diagnosis can be established by intraoperative cholangiography or ultrasonography. [5]
Csendes et al. classification is useful for surgical planning.
• Type I A: extrinsic compression in the common hepatic duct (CHD) by impacted stone(s) in the infundibulum or cystic duct.
• Type I B: absence of cystic duct.
• Type II: cholecystocholedocal biliary fistula (CCBF) involving a third of the CHD wall circumference.
• Type III: CCBF with over two thirds of the CHD wall circumference.
• Type IV: CCBF involving the whole CHD wall circumference.
• Type V: any of above with a cholecystoenteric fistula.[5]
Surgical options are cholecystectomy for Csendes type I, partial cholecystectomy with choledochoplasty for type II and III, and biliary reconstruction with hepaticojejunostomy for type IV.[5]
High index of suspicion for preoperative diagnosis followed by tactful surgical planning and intraoperative recognition are crucial in treatment success. [6]
Differential Diagnosis List
Mirizzi syndrome
Cholangiocarcinoma
Sclerosing cholangitis
