Mirizzi’s Syndrome

Mirizzi’s Syndrome Definition

Mirizzi’s Syndrome is a health condition that involves an obstruction of the common hepatic duct or the common bile duct, as an end result of an extrinsic compression by an impacted stone which is located in the cystic duct, or in the Hartmann’s pouch found in the gallbladder.

The obstruction may lead to jaundice of the affected person   due to the mechanical compression by the stone, or by an inflammation from a fibrosis that develops from chronic cholecystitis. It has an estimate to occur about 0.1% among patients who have gallstones and between 0.7% to 2.5% of the cases who have cholecystectomies, with ages between 53-70 years old and affecting the female group.

Symptoms of Mirizzi Syndrome

The clinical manifestations of Mirizzi’s syndrome has no definite unique characteristics to differentiate it more specifically with other existing common forms of obstructive jaundice formation.

Its symptoms may be present or may not be present and these are:

• Obstructive jaundice
• Cholangitis
• Pain in the right upper quadrant of the abdomen
• Fever together a known or suspected gallstone problem
• Elevated levels of alkaline phosphatase and bilirubin and leukocytosis

Patients with Mirrizi’s syndrome can present itself according to any similar setting such as in the cases of an existing acute cholangitis, acute pancreatitis and acutecholecystits. According to a recent study, it has been associated with a gallstone ileus setting that all surgeons have to take into consideration as another form of clinical presentation.

Pathophysiology

The formation of large and multiple gallstones can become impacted in the gallbladder, specifically in the Hartmann’s pouch.  This results to a chronic inflammation associated with some episodes of cholangitis; that can then in turn lead to the compression of the common bile duct, fibrosis can be noted, necrosis can occur, and ultimately affecting the nearby common bile duct or hepatic duct through a fistula formation. The obstructions of both ducts by any type of scar or stone being formed ends up with an obstructive jaundice that is noticeable.

Types

Mirizzi’s Syndrome can be classified into four types and these are:

• Type I where no fistula is present in a patient

Type IA, there is an existing cystic duct

Type IB, there is an eradication of the cystic duct

• Type II-IV, where patients have the fistula formation

Type II, the defect is 33% smaller than that of a common bile duct diameter

Type III, the defect is between 33% to 36% than that of a common bile duct diameter

Type IV, the defect is 66% larger than that of a common bile duct diameter

Diagnosis

The making of a preoperative diagnosis of Mirizzi syndrome is considered essential together with the surgical planning to resolve the problem.

The following procedures can be done:

• Ultrasonography which might present the existence of a large gallstone or the formation of smaller multiple gallstones which are impacted in the common hepatic duct
• Computed tomography can identify the gallbladder thickness and dilatation of the bile duct with stones
• Magnetic resonance imaging which can view an extrinsic compression affecting the bile duct and it can help to determine the presence or absence of a fistula
• Endoscopic retrograde cholangiopancreatography is used to confirm the diagnosis of the problem

Intraoperative diagnosis is also done during the surgical procedure.

Treatment

The main treatment for Mirizzi syndrome involves a surgical procedure.

1. Classic cholecystectomy for Type I to open the gallbladder, emptying the gallstone/gallstones and removing the gallbladder with a dissection being started at the fundus.
2. Subtotal cholecystectomy for Type II to remove the gallbladder, leaving a 5mm remnant of gallbladder wall around the cholecystobiliary fistula, with the purpose of aiding the closure of the impaired bile duct
3. Subtotal cholecystectomy for Type III, leaving a 1 cm gallbladder wall flap to support the bile duct repair
4. Bilioenteric anastomosis through a hepaticojejunostomy en-Y-de-Roux for Type IV patients
5. Operative cholangiography through the Kehr tube is done for all types of Mirizzi syndrome to provide protection of the bile duct suture with a Kehr tube

References:

1. mirizzi syndrome PATHOGENESIS, CLASSIFICATION, DIAGNOSIS at http://www.uptodate.com/contents/mirizzi-syndrome#H1
2. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3442202/
3. http://www.scielo.br/scielo.php?pid=S0004-28032005000100005&script=sci_arttext#tab01
4. Erben Y, Benavente-Chenhalls LA, Donohue JM, et al (2011). Diagnosis and treatment of Mirizzi syndrome: 23-year Mayo Clinic experience. J Am Coll Surg 213:114.
5. Antoniou SA, Antoniou GA, Makridis C (2010). Laparoscopic treatment of Mirizzi syndrome: a systematic review. Surg Endosc 24:33.
6. Prasad TL, Kumar A, Sikora SS, et al (2006). Mirizzi syndrome and gallbladder cancer. J Hepatobiliary Pancreat Surg 13:323.