Mirizzi's syndrome
Encyclopedia
Mirizzi's syndrome is a rare cause of acquired jaundice
. It is associated with large gallstone
s that compress the common hepatic duct
. 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.
s. 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.
s can reside chronically in the Hartmann's pouch
of the gallbladder
, causing inflammation
, necrosis
, scarring and ultimately fistula
formation into the adjacent common hepatic duct (CHD). As a result, the CHD becomes obstructed by either scar or stone, resulting in jaundice
. It can be divided into four types.
Type I - No fistula present
Types II-IV - Fistula present
and alkaline phosphatase
may or may not be present. Acute presentations of the syndrome include pancreatitis
or cholecystitis
.
Jaundice
Jaundice is a yellowish pigmentation of the skin, the conjunctival membranes over the sclerae , and other mucous membranes caused by hyperbilirubinemia . This hyperbilirubinemia subsequently causes increased levels of bilirubin in the extracellular fluid...
. It is associated with large gallstone
Gallstone
A gallstone is a crystalline concretion formed within the gallbladder by accretion of bile components. These calculi are formed in the gallbladder, but may pass distally into other parts of the biliary tract such as the cystic duct, common bile duct, pancreatic duct, or the ampulla of...
s that compress the common hepatic duct
Common hepatic duct
The common hepatic duct is the duct formed by the convergence of the right hepatic duct and the left hepatic duct . The common hepatic duct then joins the cystic duct coming from the gallbladder to form the common bile duct...
. The obstructive jaundice can be caused by direct extrinsic compression by the stone or from fibrosis caused by chronic cholecystitis
Cholecystitis
-Signs and symptoms:Cholecystitis usually presents as a pain in the right upper quadrant. This is known as biliary colic. This is initially intermittent, but later usually presents as a constant, severe pain. During the initial stages, the pain may be felt in an area totally separate from the site...
inflammation. A cholecystocholedochal fistula
Fistula
In medicine, a fistula is an abnormal connection or passageway between two epithelium-lined organs or vessels that normally do not connect. It is generally a disease condition, but a fistula may be surgically created for therapeutic reasons.-Locations:Fistulas can develop in various parts of the...
can occur.
Epidemiology
Mirizzi syndrome occurs in approximately 0.1% of patients with gallstoneGallstone
A gallstone is a crystalline concretion formed within the gallbladder by accretion of bile components. These calculi are formed in the gallbladder, but may pass distally into other parts of the biliary tract such as the cystic duct, common bile duct, pancreatic duct, or the ampulla of...
s. It occurs in 0.7 to 2.5 percent of cholecystectomies
Cholecystectomy
Cholecystectomy is the surgical removal of the gallbladder. It is the most common method for treating symptomatic gallstones. Surgical options include the standard procedure, called laparoscopic cholecystectomy, and an older more invasive procedure, called open cholecystectomy.-Open surgery:A...
.
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.
Pathophysiology
Multiple and large gallstoneGallstone
A gallstone is a crystalline concretion formed within the gallbladder by accretion of bile components. These calculi are formed in the gallbladder, but may pass distally into other parts of the biliary tract such as the cystic duct, common bile duct, pancreatic duct, or the ampulla of...
s can reside chronically in the Hartmann's pouch
Hartmann's pouch
Hartmann’s pouch is an out-pouching of the wall of the gallbladder at the junction of the neck of the gallbladder and the cystic duct. Its identification is useful in delineating biliary anatomy when performing a cholecystectomy....
of the gallbladder
Gallbladder
In vertebrates the gallbladder is a small organ that aids mainly in fat digestion and concentrates bile produced by the liver. In humans the loss of the gallbladder is usually easily tolerated....
, causing inflammation
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...
, necrosis
Necrosis
Necrosis is the premature death of cells in living tissue. Necrosis is caused by factors external to the cell or tissue, such as infection, toxins, or trauma. This is in contrast to apoptosis, which is a naturally occurring cause of cellular death...
, scarring and ultimately fistula
Fistula
In medicine, a fistula is an abnormal connection or passageway between two epithelium-lined organs or vessels that normally do not connect. It is generally a disease condition, but a fistula may be surgically created for therapeutic reasons.-Locations:Fistulas can develop in various parts of the...
formation into the adjacent common hepatic duct (CHD). As a result, the CHD becomes obstructed by either scar or stone, resulting in jaundice
Jaundice
Jaundice is a yellowish pigmentation of the skin, the conjunctival membranes over the sclerae , and other mucous membranes caused by hyperbilirubinemia . This hyperbilirubinemia subsequently causes increased levels of bilirubin in the extracellular fluid...
. It can be divided into four 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
Features
Mirizzi syndrome has no consistent or unique clinical features that distinguish it from other more common forms of obstructive jaundice. Symptoms of recurrent cholangitis, jaundice, right upper quadrant pain, and elevated bilirubinBilirubin
Bilirubin is the yellow breakdown product of normal heme catabolism. Heme is found in hemoglobin, a principal component of red blood cells. Bilirubin is excreted in bile and urine, and elevated levels may indicate certain diseases...
and alkaline phosphatase
Alkaline phosphatase
Alkaline phosphatase is a hydrolase enzyme responsible for removing phosphate groups from many types of molecules, including nucleotides, proteins, and alkaloids. The process of removing the phosphate group is called dephosphorylation...
may or may not be present. Acute presentations of the syndrome include pancreatitis
Pancreatitis
Pancreatitis is inflammation of the pancreas. It occurs when pancreatic enzymes that digest food are activated in the pancreas instead of the small intestine. It may be acute – beginning suddenly and lasting a few days, or chronic – occurring over many years...
or cholecystitis
Cholecystitis
-Signs and symptoms:Cholecystitis usually presents as a pain in the right upper quadrant. This is known as biliary colic. This is initially intermittent, but later usually presents as a constant, severe pain. During the initial stages, the pain may be felt in an area totally separate from the site...
.