Mirizzi syndrome

Mirizzi Syndrome Mirizzi syndrome is a rare condition characterized by an obstruction of the common hepatic duct due to an impacted gallstone in the cystic duct or neck of the gallbladder, leading to jaundice and other complications. This syndrome is named after Pablo Luis Mirizzi, an Argentinean surgeon who first described the condition in 1948.

Pathophysiology

Mirizzi syndrome occurs when a gallstone becomes lodged in the cystic duct or neck of the gallbladder, causing compression of the common hepatic duct. This can lead to obstruction of bile flow, resulting in jaundice. The inflammation and pressure can also cause erosion into the bile duct, leading to a cholecystocholedochal fistula in severe cases.

Classification

Mirizzi syndrome is classified into several types based on the extent of the obstruction and the presence of fistulas:

• Type I: External compression of the common hepatic duct by a stone in the cystic duct or neck of the gallbladder without a fistula.
• Type II: Presence of a cholecystocholedochal fistula with less than one-third of the bile duct circumference involved.
• Type III: Cholecystocholedochal fistula involving one-third to two-thirds of the bile duct circumference.
• Type IV: Cholecystocholedochal fistula involving more than two-thirds of the bile duct circumference.

Clinical Presentation

Patients with Mirizzi syndrome typically present with symptoms of obstructive jaundice, including:

• Jaundice
• Right upper quadrant abdominal pain
• Fever
• Nausea and vomiting

These symptoms can mimic other biliary tract diseases, making diagnosis challenging.

Diagnosis

The diagnosis of Mirizzi syndrome is often made using imaging studies, such as:

• Ultrasound: May show gallstones and dilation of the bile ducts.
• Magnetic Resonance Cholangiopancreatography (MRCP): Provides detailed images of the biliary tree and can help identify the site of obstruction.
• Endoscopic Retrograde Cholangiopancreatography (ERCP): Can be both diagnostic and therapeutic, allowing for visualization of the bile ducts and potential stone removal.

Treatment

The treatment of Mirizzi syndrome typically involves surgical intervention. The specific approach depends on the type of Mirizzi syndrome:

• Type I: Cholecystectomy with removal of the obstructing stone.
• Types II-IV: More complex surgical procedures may be required, such as bile duct reconstruction or repair of fistulas.

In some cases, endoscopic techniques may be used to manage the condition, especially if surgery is contraindicated.

Prognosis

The prognosis for patients with Mirizzi syndrome is generally good if the condition is diagnosed and treated promptly. However, delays in diagnosis or treatment can lead to complications such as biliary cirrhosis or cholangitis.

See Also

• Cholecystitis
• Choledocholithiasis
• Biliary Tract Diseases

External Links

• [Link to a reputable medical resource on Mirizzi syndrome]

NIH genetic and rare disease info

• NIH info on Mirizzi syndrome

Mirizzi syndrome is a rare disease.