Definition

What is Mirizzi syndrome?

Mirizzi syndrome is defined as common hepatic duct obstruction caused by an extrinsic compression from an impacted stone in the cystic duct or Hartmann’s pouch of the gallbladder.

Mirizzi syndrome has been classified based on the presence and extent of a cholecystobiliary fistula:

  • Type I (11 percent of Mirizzi syndrome) – External compression of the common hepatic duct due to a stone impacted at the neck/infundibulum of the gallbladder or at the cystic duct.
  • Type II (41 percent of Mirizzi syndrome) – The fistula involves less than one-third of the circumference of the common bile duct.
  • Type III (44 percent of Mirizzi syndrome) – Involvement of between one-third and two-thirds of the circumference of the common bile duct.
  • Type IV (4 percent of Mirizzi syndrome) – Destruction of the entire wall of the common bile duct.

How common is Mirizzi syndrome?

Mirizzi syndrome is estimated to occur in 0.05 to 4 percent of patients undergoing surgery for cholelithiasis. Approximately 50 to 77 percent of patients with Mirizzi syndrome are women, which may in part be due to a higher incidence of gallstones in women. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of Mirizzi syndrome?

The common symptoms of Mirizzi syndrome are:

  • Jaundice
  • Fever
  • Right upper quadrant pain

However, all three symptoms are only present in 44 to 71 percent of patients. Pain is the most common presenting feature (54 to 100 percent), followed by jaundice (24 to 100 percent) and cholangitis (6 to 35 percent). Up to one-third of patients have acute cholecystitis on presentation, and in rare cases, acute pancreatitis.

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you or your loved one has any signs or symptoms listed above or you have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes Mirizzi syndrome?

The gallbladder consists of the fundus, body, infundibulum, and neck. The body extends from the fundus into the tapered portion, or neck. The neck usually forms a gentle curve, the convexity of which forms the infundibulum, or Hartmann’s pouch. The gallbladder is connected at its neck to the cystic duct which empties into the common bile duct. Large gallstones can become impacted in the cystic duct or in Hartmann’s pouch. These stones can produce common hepatic duct obstruction by mechanical obstruction of the hepatic duct because of the proximity of the cystic duct and the common hepatic duct, and secondary inflammation with frequent episodes of cholangitis. In rare cases, chronic inflammation may result in bile duct wall necrosis and erosion of the anterior or lateral wall of the common bile duct by impacted stones leading to cholecystobiliary (cholecystohepatic or cholecystocholedochal) fistula.

Risk factors

What increases my risk for Mirizzi syndrome?

Mirizzi syndrome has been associated with gallbladder cancer. It has been hypothesized that recurrent inflammation and biliary stasis may predispose to both conditions. The reported prevalence of gallbladder cancer in patients with Mirizzi syndrome undergoing cholecystectomy ranges from 5 to 28 percent. In a retrospective study of 4800 patients who underwent cholecystectomy, Mirizzi syndrome was present in 133 patients, of whom seven (5 percent) had gallbladder cancer. Please consult with your doctor for further information.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is Mirizzi syndrome diagnosed?

The diagnosis of Mirizzi syndrome requires the presence of the following on abdominal imaging (eg, transabdominal ultrasonography, contrast-enhanced computed tomography [CT], magnetic resonance cholangiopancreatogram [MRCP]):

  • Dilatation of the biliary system above the level of the gallbladder neck
  • The presence of a stone impacted in the gallbladder neck
  • An abrupt change to a normal diameter of the common duct below the level of the stone

How is Mirizzi syndrome treated?

Surgery

Surgery is the mainstay of therapy for Mirizzi syndrome, permitting removal of the causal factors: the inflamed gallbladder and the impacted stone. The surgical approach to Mirizzi syndrome is based on the presence and type of cholecystobiliary fistula:

  • Type I – Partial or total cholecystectomy, either laparoscopic or open. Common bile duct exploration is typically not required.
  • Type II – Cholecystectomy plus closure of the fistula, either by suture repair with absorbable material, T tube placement, or choledochoplasty with the remnant gallbladder.
  • Type III – Choledochoplasty or bilioenteric anastomosis (choledochoduodenostomy, cholecystoduodenostomy, or choledochojejunostomy) depending on the size of the fistula. Suture of the fistula is not indicated.
  • Type IV – Bilioenteric anastomosis, typically choledochojejunostomy, is preferred because the entire wall of the common bile duct has been destroyed.

If the diagnosis of Mirizzi syndrome is made preoperatively, endoscopic retrograde cholangiopancreatography can be both diagnostic and therapeutic as a temporizing measure before surgery, as stenting across the obstruction allows decompression of the common bile duct in patients with obstructive jaundice or cholangitis. Endoscopic removal of common bile duct stones may eliminate the need for common bile duct exploration at the time of surgery.

If Mirizzi syndrome is diagnosed incidentally at the time of cholecystectomy, intraoperative cholangiogram should be performed prior to cholecystectomy to confirm the diagnosis and characterize the biliary anatomy.

Endoscopic therapy

For patients who are unsuitable surgical candidates, endoscopic retrograde cholangiopancreatography with stenting can be definitive treatment for Mirizzi syndrome.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage Mirizzi syndrome?

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Sources

Review Date: March 27, 2018 | Last Modified: March 27, 2018

Want to live your best life?
Get the Hello Doktor Daily newsletter for health tips, wellness updates and more.