Mirizzi Syndrome: A Rare Gallstone Complication – Symptoms & Treatment

What is Mirizzi syndrome?

Mirizzi syndrome is a rare complication of chronic gallstone disease where a gallstone impacted in the cystic duct or the neck of the gallbladder compresses the common hepatic duct (CHD) or common bile duct (CBD) from the outside, causing obstructive jaundice. It can also lead to a cholecystocholedochal fistula (abnormal connection between the gallbladder and the bile duct). The condition is named after the Argentine surgeon Pablo Mirizzi, who described it in 1948. Mirizzi syndrome is found in 0.5‑2% of patients undergoing cholecystectomy and is often missed preoperatively, leading to a high risk of bile duct injury during surgery.

McSherry / Csendes classification

Mirizzi syndrome is classified into five types (Csendes classification, modified), which guide surgical management:

• Type I: External compression of the common hepatic duct by a stone in the cystic duct or gallbladder neck. No fistula.
• Type II: Cholecystocholedochal fistula involving less than one‑third of the circumference of the bile duct.
• Type III: Fistula involving one‑third to two‑thirds of the bile duct circumference.
• Type IV: Fistula involving more than two‑thirds of the bile duct circumference.
• Type V: Fistula with cholecystoenteric fistula (gallstone ileus variant).

Higher types (III‑V) are associated with greater surgical difficulty and risk of bile duct injury.

Symptoms – jaundice, pain, recurrent cholangitis

Symptoms of Mirizzi syndrome are often non‑specific and can mimic choledocholithiasis or malignant obstruction:

• Obstructive jaundice (yellow skin/eyes, dark urine, pale stools) – present in 70‑90% of patients.
• Right upper quadrant or epigastric pain – often chronic, intermittent.
• Recurrent episodes of cholangitis (fever, jaundice, RUQ pain).
• Weight loss and anorexia (may suggest malignancy).
• Elevated alkaline phosphatase, GGT, and bilirubin.

Diagnosis – ultrasound, CT, MRCP, ERCP

Pre‑operative diagnosis is crucial to avoid bile duct injury. Imaging findings:

• Ultrasound: Shows gallstones, a dilated intrahepatic bile duct, and a stone impacted at the gallbladder neck/cystic duct. May show a “triangular sign” (convergence of the gallbladder wall and bile duct).
• CT scan: Visualises the stone, bile duct dilation, and may show the fistula.
• MRCP (magnetic resonance cholangiopancreatography): Excellent for demonstrating the level of bile duct obstruction, the impacted stone, and the presence of a fistula. Sensitivity >90%.
• ERCP (endoscopic retrograde cholangiopancreatography): Invasive but can confirm the diagnosis, show the fistula, and allow placement of a biliary stent. However, ERCP carries a risk of cholangitis if the fistula is present.
• Endoscopic ultrasound (EUS): Useful for visualising the stone and assessing the fistula.

Treatment – surgical (subtotal cholecystectomy, fistula repair)

Surgery is the mainstay of treatment, but it is challenging and should be performed by an experienced hepatobiliary surgeon.

• Type I (no fistula): Laparoscopic or open cholecystectomy with careful dissection to avoid bile duct injury. If the stone is impacted, a subtotal cholecystectomy (leaving the posterior wall attached to the liver) may be safer.
• Types II‑IV (with fistula): Open surgery is preferred. The gallbladder is opened, the stone removed, and the fistula is inspected. The remaining gallbladder wall is excised (subtotal cholecystectomy). The bile duct is repaired over a T‑tube or by primary closure with a choledochoplasty using the remnant gallbladder wall.
• Type V (with cholecystoenteric fistula): Enterolithotomy + fistula repair + cholecystectomy (may be staged).
• Pre‑operative ERCP: May be used to place a biliary stent to decompress the bile duct before surgery, especially in patients with cholangitis.

Complications – bile duct injury, stricture, recurrence

Despite careful surgery, complications are common:

• Bile duct injury (10‑20%): Can occur during dissection because the anatomy is distorted.
• Post‑operative bile leak (5‑10%).
• Bile duct stricture (5‑15%) – may require endoscopic dilation or reoperation.
• Recurrent cholangitis.
• Recurrent stones (rare).

Prognosis and outcomes

With appropriate surgical management, the prognosis is good. However, patients with type III‑IV fistulas have higher morbidity (20‑30%) and a longer hospital stay. Bile duct injury remains the most feared complication. Long‑term follow‑up with LFTs and imaging is recommended to detect strictures.

Interactive FAQ – Mirizzi syndrome

Disclaimer: This information is for educational purposes. Mirizzi syndrome requires expert surgical management. If you have gallstones and unexplained jaundice, consult a gastroenterologist or surgeon at Vivekananda Hospital.