Mirizzi syndrome


Mirizzi syndrome

Description, Causes and Risk Factors:

Mirizzi's syndrome is a rare complication in which a gallstone becomes impacted in the cystic duct or neck of the gallbladder causing compression of the CBD (common bile duct), resulting in CBD obstruction and jaundice. The obstructive jaundice can be caused by direct extrinsic compression by the stone or from fibrosis caused by chronic cholecystitis inflammation. A cholecystocholedochal fistula can occur.

Types:

Type I - No fistula present

    Type IA - Presence of the cystic duct.

  • Type IB - Obliteration of the cystic duct.

Types II-IV - Fistula present

    Type II - Defect smaller than 33% of the CBD diameter.

  • Type III - Defect 33-66% of the CBD diameter.

  • Type IV - Defect larger than 66% of the CBD diameter.

Mirizzi's syndrome is named after Doctor Pablo Mirizzi, who described a peculiar bile duct obstruction due to gallstones in 1948. However, this condition had been reported as early as in 1905. This syndrome refers to common hepatic duct obstruction caused by an extrinsic compression of an impacted stone in the Hartmann's pouch or cystic duct (type I). Predispositions are a low-lying cystic-choledochal duct juncture, contiguity of a large cystic duct with the common hepatic duct or a short cystic duct. Also inflammation contributes to the bile duct obstruction. If the obstruction persists the stone may erode into the common bile duct producing a cholecystocholedochal fistula (type II).

Mirizzi syndrome occurs in approximately 0.1% of patients with gallstones. It occurs in 0.7 to 2.5 percent of cholecystectomies. It affects males and females equally, but tends to affect older people more often. There is no evidence of race having any bearing on the epidemiology.

Symptoms:

Clinical presentation includes symptoms of obstructivejaundice: recurrent cholangitis, jaundice, fever, right upperquadrant pain and elevated liver test enzymes.

Diagnosis:

Diagnosis is made by physical examination, imagingstudy such as US (ultrasound), MRCT (multiplanar reformatted computerized tomography), and liver functional test.Differential diagnosis includes choledocholitiasis,Carroli's desease, hilar cholangiocarcinoma, pancreatic headcancer.

Treatment:

Surgery is the mainstay of therapy of Mirizzi syndrome, the dense inflammatory reaction in Calot's triangle (area bounded by the cystic artery, cystic duct, and common hepatic duct — important structures to identify in performing a laparoscopic cholecystectomy), as well as the frequent aberrant biliary anatomy, pose a difficult challenge to the unsuspecting surgeon when dealing with a Mirizzi syndrome. The two principal aims are (a) the safe completion of cholecystectomy without injuring the biliary system and (b) the appropriate management of the cholecystocholedochal fistula. Meticulous dissection and vigilance are essential in order to avoid inadvertent bile duct injury. If the diagnosis of Mirizzi syndrome is made preoperatively, an operative strategy that minimizes the risk of injury to the biliary tract can be carried out. However, a preoperative diagnosis of Mirizzi syndrome is seldom made because ERCP and direct cholangiography are not widely used. ERCP, direct cholangiography, or magnetic resonance cholangiography should be performed in patients with clinical jaundice and signs and symptoms suggestive of biliary obstruction.

A standardized surgical approach has been recommended based on the Csendes classification of the variants of Mirizzi syndrome:

    Type I - Cholecystectomy plus common bile duct exploration with T-tube placement. Exploration should be performed only if the CBD is easily exposed.

  • Type II - Suture of the fistula with absorbable material or choledochoplasty with the remnant gallbladder.

  • Type III - Choledochoplasty; suture of the fistula is not indicated.

  • Type IV - Bilio-enteric anastomosis is preferred since the entire wall of the common bile duct has been destroyed. The approach may vary with the type of fistula present; both the operative mortality and postoperative morbidity increase according to the severity of the lesion.

Laparoscopic Surgery: The Mirizzi syndrome presents a difficult challenge for laparoscopic surgery because the dense adhesions and edematous inflammatory tissue cause distortion of the normal anatomy and increase the risk for biliary injury. While it appears to be feasible, especially for type I anatomy, the routine use of laparoscopic surgery as the primary treatment of Mirizzi syndrome is controversial. It has been suggested, that a prudent approach for type 1 Mirizzi syndrome is to perform a trial laparoscopic dissection, but to have a low threshold to convert to an open procedure. This approach should be undertaken only by experienced laparoscopic surgeons.

Endoscopic Therapy: Endoscopic treatment with or without electrohydraulic lithotripsy (EHL) can be effective as a temporizing measure before surgery and can be definitive treatment for unsuitable surgical candidates. One report described the experience with 14 patients with Mirizzi syndrome treated with EHL. Twelve patients had a single stone and complete clearance was achieved with one treatment session; two had multiple stones and required an additional treatment session. Asymptomatic leakage of contrast medium from the cystic duct into the peritoneal cavity was observed in one patient after removal of a large impacted cystic duct stone. This patient recovered with conservative therapy and suffered no adverse events. In another series of 25 patients with cholangiographic evidence of Mirizzi syndrome, 12 were referred for surgery after preliminary endoscopic therapy and 13 were treated solely with endoscopy. Stones were completely removed in three and nine were treated with long-term stents; complications occurred in four patients. Endoscopic treatment of Mirizzi syndrome should be used as a temporizing measure before surgery. It can serve as a definitive treatment for those patients who are unsuitable surgical candidates when further endoscopic attempts can be made to disimpact and remove the stones. Long-term success appears to be most likely in patients with type II disease who do not have residual gallbladder stones.

NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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