Mirizzi's syndrome is a rare complication in which a gallstone becomes impacted in the

cystic duct The cystic duct is the duct that (typically) joins the gallbladder and the common hepatic duct; the union of the cystic duct and common hepatic duct forms the bile duct (formerly known as the common bile duct). Its length varies. Anatomy ...

or neck of the

gallbladder 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 t ...

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 quadrant (abdomen), right upper abdominal pain, pain in the right shoulder, nausea, vomiting, and occasionally fever. Often gallbladder attacks (biliary colic) precede ...

(inflammation). A cholecystocholedochal

fistula In anatomy, a fistula (: fistulas or fistulae ; from Latin ''fistula'', "tube, pipe") is an abnormal connection (i.e. tube) joining two hollow spaces (technically, two epithelialized surfaces), such as blood vessels, intestines, or other h ...

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) (adopted from German, originally bili—bile—plus ruber—red—from Latin) is a red-orange compound that occurs in the normcomponent of the straw-yellow color in urine. Another breakdown product, stercobilin, causes the brown ...

and

alkaline phosphatase The enzyme alkaline phosphatase (ALP, alkaline phenyl phosphatase, also abbreviated PhoA) is a phosphatase with the physiological role of dephosphorylating compounds. The enzyme is found across a multitude of organisms, prokaryotes and eukaryo ...

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

. Surgery is extremely difficult as Calot's triangle is often obliterated and the risks of causing injury to the CBD are high.

Pathophysiology

Multiple and large

gallstone A gallstone is a stone formed within the gallbladder from precipitated bile components. The term cholelithiasis may refer to the presence of gallstones or to any disease caused by gallstones, and choledocholithiasis refers to the presence of ...

s can become impacted in the Hartmann's pouch of the

, leading to chronic

inflammation Inflammation (from ) is part of the biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. The five cardinal signs are heat, pain, redness, swelling, and loss of function (Latin ''calor'', '' ...

—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. The term "necrosis" came about in the mid-19th century and is commonly attributed to German pathologist Rudolf Virchow, who i ...

, fibrosis, and ultimately

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, less frequently, 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 ...

. 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 Medical ultrasound includes diagnostic techniques (mainly imaging) using ultrasound, as well as therapeutic applications of ultrasound. In diagnosis, it is used to create an image of internal body structures such as tendons, muscles, joints, ...

, MRCP, or

CT scan A computed tomography scan (CT scan), formerly called computed axial tomography scan (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 or ...

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 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

s. It is found 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.

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