Mirizzi's syndrome

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Mirizzi's syndrome
Classification and external resources
ICD-9 576.2
DiseasesDB 33254
eMedicine radio/451

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 bile duct (CBD) or 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 (inflammation). A cholecystocholedochal fistula can occur.[1]

Epidemiology[edit]

Mirizzi's syndrome occurs in approximately 0.1% of patients with gallstones.[2] It is found in 0.7 to 2.5 percent of cholecystectomies.[1]

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[edit]

Multiple and large gallstones can become impacted in the Hartmann's pouch of the gallbladder, leading to inflammation, necrosis, 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. 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

Features[edit]

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 and alkaline phosphatase may or may not be present. Acute presentations of the syndrome include symptoms consistent with cholecystitis.

Diagnosis[edit]

Imaging by ultrasonography, MRCP, or CT scan usually make the diagnosis.[3] MRCP can be used to define the lesion anatomically prior to surgery.[citation needed] 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[edit]

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.[4]

Eponym[edit]

It is named for Pablo Luis Mirizzi, an Argentinian physician.[5][6]

References[edit]

  1. ^ a b 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
  2. ^ Hazzan, D; D Golijanin; P Reissman; SN Adler; E Shiloni (June 1999). "Combined endoscopic and surgical management of Mirizzi syndrome". Surgical Endoscopy 13 (6): 618–20. doi:10.1007/s004649901054. PMID 10347304. 
  3. ^ Ross, Jeffrey W; Gary S Sudakoff, Gregory B Snyder, Neela Lamki (editor), Bernard D Coombs (editor), Abraham H Dachman (editor), Robert M Krasny (editor), John Karani (editor) (2006-12-29). "Mirizzi syndrome". eMedicine. WebMD. Retrieved 2007-12-09. 
  4. ^ Aydin, U., P. Yazici, et al. (2008). "Surgical management of Mirizzi syndrome." Turk J Gastroenterol 19(4): 258–263. http://www.turkgastro.org/text.php?id=661
  5. ^ synd/3587 at Who Named It?
  6. ^ Mirizzi PL: Syndrome del conducto hepatico. J Int de Chir 1948; 8: 731–77

External links[edit]