Mirizzi's syndrome

Overview

Mirizzi's syndrome is a rare complication of gallstone disease characterized by obstruction or compression of the common hepatic duct (CHD) due to an impacted gallstone in the cystic duct or Hartmann’s pouch of the gallbladder. This obstruction can lead to cholestasis, inflammation, and in severe cases, the formation of a cholecystocholedochal fistula (a direct communication between the gallbladder and the common bile duct).

First described by Pablo Luis Mirizzi in 1948, the syndrome remains uncommon but clinically significant because of its potential to mimic other hepatobiliary conditions such as cholangiocarcinoma. Prompt recognition and appropriate management are essential to prevent complications like biliary cirrhosis, sepsis, or bile duct injury during surgery.

Causes

The primary cause of Mirizzi’s syndrome is chronic gallstone disease. Specifically, the syndrome develops when a gallstone becomes impacted in the cystic duct or Hartmann’s pouch (an outpouching of the gallbladder wall), causing:

• Mechanical compression of the adjacent common hepatic duct

• Chronic inflammation and fibrosis of surrounding tissues

• Formation of a fistula between the gallbladder and the common bile duct in advanced cases

Risk factors include:

• Long-standing cholelithiasis (gallstones)

• Recurrent cholecystitis

• Thickened gallbladder wall or fibrotic adhesions from prior infections

In rare cases, repeated inflammation may erode the bile duct wall, resulting in fistula formation, a hallmark of advanced Mirizzi’s syndrome.

Symptoms

Mirizzi’s syndrome presents with symptoms typical of obstructive jaundice and cholecystitis. These may include:

Common Symptoms

• Right upper quadrant abdominal pain (biliary colic)

• Jaundice (yellowing of the skin and eyes)

• Fever (may suggest superimposed cholangitis)

• Nausea and vomiting

• Dark urine and pale stools (due to bile obstruction)

• Pruritus (itching from bile salt accumulation)

Symptoms can be intermittent or progressive. In cases where a cholecystocholedochal fistula develops, patients may also present with signs of chronic infection or recurrent biliary sepsis.

Diagnosis

Diagnosing Mirizzi’s syndrome can be challenging because it mimics other causes of biliary obstruction. A combination of imaging and endoscopic studies is often needed for accurate diagnosis. The diagnostic steps include:

Laboratory Tests

• Elevated bilirubin (mainly conjugated)

• Elevated alkaline phosphatase (ALP) and gamma-glutamyl transferase (GGT)

• Mild to moderate elevation of transaminases (AST, ALT)

• Leukocytosis if infection is present

Imaging Studies

• Ultrasound: May show gallstones, gallbladder wall thickening, and bile duct dilatation

• CT scan: Provides better anatomical detail, useful in ruling out malignancy

• Magnetic Resonance Cholangiopancreatography (MRCP): Non-invasive tool to visualize biliary anatomy and detect compression or fistulas

• Endoscopic Retrograde Cholangiopancreatography (ERCP): Gold standard for diagnosis, allows for visualization, stone extraction, and stent placement

During surgery, Mirizzi’s syndrome is often discovered intraoperatively when the anatomy of the biliary tract appears distorted due to inflammation or fistula formation. Classification systems (such as Csendes classification) are used to define the extent of disease and guide treatment.

Treatment

Treatment of Mirizzi’s syndrome depends on the severity of the condition and the presence of complications such as fistula formation or infection. Management typically involves surgical intervention, with or without preoperative endoscopic procedures.

Initial Management

• Antibiotics: For cases with cholangitis or infection

• Endoscopic stenting: ERCP with stent placement to relieve obstruction preoperatively, especially in high-risk surgical patients

Surgical Management

• Cholecystectomy: Surgical removal of the gallbladder; may be open or laparoscopic depending on severity and surgeon experience

• Bile duct repair or reconstruction: Required in advanced cases with cholecystocholedochal fistulas (e.g., Roux-en-Y hepaticojejunostomy)

• Intraoperative cholangiography: To define biliary anatomy and avoid bile duct injury

Patients with extensive inflammation or distorted anatomy may require conversion from laparoscopic to open surgery to safely complete the procedure. In severe or complex cases, management at a tertiary care center with hepatobiliary expertise is recommended.

Prognosis

The prognosis for Mirizzi’s syndrome is generally good when diagnosed early and treated appropriately. However, delayed diagnosis or mismanagement can lead to serious complications such as:

• Bile duct injury during surgery

• Biliary fistula formation

• Recurrent cholangitis

• Secondary biliary cirrhosis from prolonged obstruction

With timely surgical intervention and appropriate supportive care, most patients recover well, though recovery may be prolonged in those with severe inflammation or multiple comorbidities. Long-term follow-up is advised in patients with complex bile duct reconstructions to monitor for stricture or recurrent obstruction.