Mirizzi Syndrome: Causes, Symptoms, Diagnosis, Treatment and Association with Gallbladder Cancer

It is characterized by extrinsic compression of the common hepatic duct by stones impacted in the cystic duct or in the neck of the gallbladder.

The syndrome comprises a spectrum of anatomical alterations, where the most significant anomaly is the formation of a biliary-biliary fistula.

The gallbladder is made up of the fundus, body, infundibulum, and neck. The body extends from the bottom towards the conical portion or neck.

The neck generally forms a smooth curve, the convexity of which forms the infundibulum or Hartmann’s pouch.

The gallbladder is connected in your neck to the cystic duct that empties into the common bile duct.

Large gallstones can impact the cystic duct or Hartmann’s pouch .

These stones can cause obstruction of the common hepatic duct by mechanical obstruction of the hepatic duct due to the proximity of the cystic duct and the common hepatic duct, and secondary inflammation with frequent episodes of cholangitis .

In rare cases, chronic inflammation can lead to necrosis of the bile duct wall and erosion of the anterior or lateral wall of the common bile duct by impacted stones leading to cholecystohepatic fistula or cholecystochedoloedol.

Mirizzi Syndrome is a rare and difficult to manage clinical entity. However, recent advances in technology have provided surgeons with new options for more effective diagnosis and treatment of this condition.

Kehr and Ruge were the first to describe this condition in the early 1900s, although the term ‘Mirizzi Syndrome’ was not adopted until after Mirizzi’s work in 1948.

This syndrome is a rare complication of chronic gallstone disease.

Physiopathologically, this condition involves extrinsic compression of the bile duct by pressure applied indirectly by an impacted stone in the infundibulum or neck of the gallbladder.

In turn, the resulting chronic inflammation and ulceration form varying degrees of cholecystobiliary fistula, in addition, cholecystoiderial fistula can also occur.

Causes of Mirizzi syndrome

This syndrome is caused by extrinsic compression of an impacted stone in the cystic duct or Hartmann’s bursa of the gallbladder.

Symptoms of Mirizzi syndrome

The clinical presentation of Mirizzi syndrome varies from asymptomatic to non-specific, with obstructive jaundice and elevated liver enzymes being the most common, abdominal pain in the right upper quadrant and constitutional symptoms such as fever, nausea, vomiting, diarrhea and constipation .

Although rare, Mirizzi syndrome can also present with gallstone ileus .

The nature of these presentations often causes this condition to be mistaken for a biliary tract neoplasm, and therefore misdiagnosis is common.

Diagnosis of Mirizzi syndrome

Preoperative diagnosis of Mirizzi syndrome followed by careful surgical planning is of utmost importance

The most widely used diagnostic modalities are ultrasound, computed tomography, magnetic resonance cholangiopancreaticography, and endoscopic retrograde cholangiopancreaticography.

Endoscopic retrograde cholangiopancreatography is an invasive procedure that is not only useful to confirm the presence of Mirizzi syndrome with or without cholecystobiliary or cholecystoester fistulas, but also for therapeutic means that allow stone removal, stenting, and other procedures

Safe and effective surgical therapy is facilitated by an accurate preoperative diagnosis. However, such a diagnosis is often overlooked before the operation, although more advanced cases of the disease are easier to detect before surgery.

Mirizzi syndrome treatment

The treatment of Mirizzi syndrome is surgical. Mirizzi syndrome is important to surgeons because preoperative diagnosis is not always possible and because surgical treatment of this condition is associated with a significantly increased risk of bile duct injury.

Open cholecystectomy has been the standard treatment; however, laparoscopy has challenged this approach.

The importance and the ultimate goal of all classification systems for Mirizzi syndrome is to allow the surgeon to tailor the surgical approach to the individual case.

Type I.

External compression of the bile duct. Open cholecystectomy, open subtotal cholecystectomy, laparoscopic cholecystectomy, and laparoscopic subtotal cholecystectomy are recommended as treatment.

Type IIa

Cholecystobiliary fistula with a bile duct diameter less than 50%. Open cholecystectomy and open subtotal cholecystectomy are recommended as treatment.

Tipo IIb

Cholecystobiliary fistula with a bile duct diameter greater than 50%. Open subtotal cholecystectomy and biliary-enteric diversion from side to side to the duodenum en-Y-de-Roux to the jejunum are recommended as treatment.

Type IIIa

Cholecystobiliary fistula and cholecysto-enteric fistula without gallstone ileus. Simple closure of the fistula and treatment of the gallbladder are recommended as treatment depending on the presence of type I, type IIa or type IIb Mirizzi.

Type IIIb

Cholecystobiliary fistula and cholecysto-enteric fistula with gallstone ileus. It is recommended as a treatment for gallstone ileus and deferred treatment for the gallbladder depending on the presence of type I, type IIa or type IIb Mirizzi.

Association with gallbladder cancer

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.