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 common hepatic duct, resulting in obstruction and jaundice. The obstructive jaundice can be caused by direct extrinsic compression by the stone or from fibrosis caused by chronic

cholecystitis Cholecystitis is inflammation of the gallbladder. Symptoms include right upper abdominal pain, pain in the right shoulder, nausea, vomiting, and occasionally fever. Often gallbladder attacks (biliary colic) precede acute cholecystitis. The pain l ...

(inflammation). A cholecystocholedochal fistula can occur.Vitale M. Mirizzi Syndrome Type IV: An Atypical Presentation That Is Difficult to Diagnose Preoperatively. 2009. Society for Surgery of the Alimentary Tract.http://www.ssat.com/cgi-bin/abstracts/09ddw/P7.cgi

Presentation

Mirizzi's 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

bilirubin Bilirubin (BR) (Latin for "red bile") is a red-orange compound that occurs in the normal catabolic pathway that breaks down heme in vertebrates. This catabolism is a necessary process in the body's clearance of waste products that arise from the ...

and

alkaline phosphatase The enzyme alkaline phosphatase (EC 3.1.3.1, alkaline phosphomonoesterase; phosphomonoesterase; glycerophosphatase; alkaline phosphohydrolase; alkaline phenyl phosphatase; orthophosphoric-monoester phosphohydrolase (alkaline optimum), systematic ...

may or may not be present. Acute presentations of the syndrome include symptoms consistent with

. Surgery is extremely difficult as

Calot's triangle The cystohepatic triangle (or hepatobiliary triangle) is an anatomic space bordered by the cystic duct inferiorly, the common hepatic duct medially, and the inferior surface of the liver superiorly. The cystic artery lies within the hepatobiliary ...

is often obliterated and the risks of causing injury to the CBD are high.

Pathophysiology

Multiple and large gallstones can become impacted in the

Hartmann's pouch In vertebrates, the gallbladder, also known as the cholecyst, is a small hollow Organ (anatomy), organ where bile is stored and concentrated before it is released into the small intestine. In humans, the pear-shaped gallbladder lies beneath th ...

of the gallbladder, leading to chronic

inflammation Inflammation (from la, wikt:en:inflammatio#Latin, inflammatio) is part of the complex biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or Irritation, irritants, and is a protective response involving im ...

—which leads to compression of the common bile duct (CBD),

necrosis Necrosis () is a form of cell injury which results in the premature death of cells in living tissue by autolysis. Necrosis is caused by factors external to the cell or tissue, such as infection, or trauma which result in the unregulated dige ...

, fibrosis, and ultimately fistula formation into the adjacent common hepatic duct (CHD) or common bile duct (CBD). As a result, the CHD/CBD becomes obstructed by either scar or stone, resulting in obstructive

jaundice Jaundice, also known as icterus, is a yellowish or greenish pigmentation of the skin and sclera due to high bilirubin levels. Jaundice in adults is typically a sign indicating the presence of underlying diseases involving abnormal heme meta ...

. 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 CHD diameter * Type III – Defect 33–66% of the CHD diameter * Type IV – Defect larger than 66% of the CHD diameter

Diagnosis

Imaging by

ultrasonography Ultrasound is sound waves with frequencies higher than the upper audible limit of human hearing. Ultrasound is not different from "normal" (audible) sound in its physical properties, except that humans cannot hear it. This limit varies fr ...

, MRCP, or

CT scan A computed tomography scan (CT scan; formerly called computed axial tomography scan or CAT scan) is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers ...

usually make the diagnosis. MRCP can be used to define the lesion anatomically prior to surgery. Occasionally Mirizzi's syndrome is diagnosed or confirmed on

ERCP Endoscopic retrograde cholangiopancreatography (ERCP) is a technique that combines the use of endoscopy and fluoroscopy to diagnose and treat certain problems of the biliary or pancreatic ductal systems. It is primarily performed by highly skille ...

when requested to alleviate obstructive jaundice or cholangitis by means of an endoscopically placed stent, or when USS has been wrongly reported as choledocolithiasis.

Treatment

Simple cholecystectomy is suitable for type I patients. For types II–IV, subtotal cholecystectomy can be performed to avoid damage to the main bile ducts. Cholecystectomy and bilioenteric anastomosis may be required. Roux-en-Y hepaticojejunostomy has shown good outcome in some studies.

Epidemiology

Mirizzi's syndrome occurs in approximately 0.1% of patients with gallstones. It is found in 0.7 to 2.5 percent of

cholecystectomies Cholecystectomy is the surgical removal of the gallbladder. Cholecystectomy is a common treatment of symptomatic gallstones and other gallbladder conditions. In 2011, cholecystectomy was the eighth most common operating room procedure performed i ...

. 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.

Eponym

It is named for Pablo Luis Mirizzi (1893–1964), an Argentinian physician. Mirizzi was educated and trained in his hometown and later visited some of the best hospitals throughout the United States for further education and training. Mirizzi specialized in abdominal and thoracic surgery and would write prolifically on related surgical topics.Mirizzi PL: Syndrome del conducto hepatico. J Int de Chir 1948; 8: 731–77

References

External links

{{Gastroenterology Syndromes affecting the hepatobiliary system