Mirizzi Syndrome An Uncommon Gallstone Complication

Dr. Ma Ka Wing

Queen Elizabeth Hospital

Common gallstone complications

What is Mirizzi syndrome ?

the gallstone impacted at the gallbladder neck/Hartmann’s pouch

Causing chronic inflammation and fibrosis

Result in obstruction or erosion of the common duct

How uncommon is it?

Variable, from 0.3% to 3% of patients having cholecystectomy

More common in South America

Who is Mirizzi?

Mirizzi is an Argentine Surgeon

He carried out the first operative cholangiogram in 1931, also known as the “mirizzigraphia”

Pablo Luis Mirizzi (25- 01-1893 to 28-08-1964)

Leopardi LN, Maddern GJ. Pablo Luis Mirizzi: the man behind the syndrome. ANZ J Surg. 2007 Dec;77(12):1062-4.

Is there a classification for the disease?

Yes, many Acute vs chronic Cystic duct variant vs no variant Obstruction due to stone vs obstruction

due to inflammationMorelli A, Narducci F, Ciccone R. Can Mirizzi syndrome beclassified into acute and chronic form? An endoscopic retrogradecholangiography (ERC) study. Endoscopy 1978; 10:109–12.Starling JR, Matallana RH. Benign mechanical obstruction ofthe common hepatic duct (Mirizzi syndrome). Surgery 1980; 88:737–40.

Nagakawa T, Ohta T, Kayahara M, Ueno K, Konishi I, Sanada H.A new classification of Mirizzi syndrome from diagnostic andtherapeutic viewpoints. Hepatogastroenterology 1997; 44:63–7.

How is it classified? McSherry and Csendes classifications

are most commonly used

McSherry Classification

Mirizzi syndrome classified into two types based on the ERCP features Type I: CHD compression without fistula Type II: presence of

cholecystocholedochal fistula

McSherry CK, Ferstenberg H, Virship M. The Mirizzi syndrome:suggested classification and surgical therapy. Surg. Gastroenterol.1982; 1: 219–25.

Csendes Classification Mirizzi syndrome classified into four types

type I: extrinsic compression of common duct due to an impacted stone at gallbladder neck or cystic duct

Type II: cholecystobiliary (either cholecystohepatic or cholecystocholedochal) fistula with the defect less than 1/3 of the duct circumference

Type III: fistula formation, wall defect up to 2/3 Type IV: fistula formation, complete destruction

of the duct wall

Csendes A, Diaz JC, Burdiles P, Maluenda F, Nava O. Mirizzisyndrome and cholecystobiliary fistula: a unifying classification.Br. J. Surg. 1989; 76: 1139–43.

How to diagnose

Diagnosis of Mirizzi syndrome is difficult

biochemical profile not specific elevated bilirubin Elevated white cell count Deranged liver function

Further investigations are needed

Imaging

USG As an baseline for jaundice patient Should see

A large gallstone contracted or indiscernible gallbladder Dilated upper CBD +/- IHDs

These findings are not specific for Mirizzi syndrome

Imagings (2)… CT scan

Should be performed to rule out malignant causes of biliary obstruction

Distinguish features include A large gallstone Contracted GB Dilated CHD and IHD Soft tissue mass at upper

CBD, reported as Ca GB/cholangioCa usually

ERCP

Remains the most important investigation

serves both diagnostic and therapeutic purposes..

Diagnostic purposes Radiological assessment

Typical features: Curvilinear extrinsic

compression of CHD from lateral

Dilated CHD and IHD “relatively” normal

CBD Return of pus after

CBD cannulation Microbiological

assessment Bile x c/st

Cytological assessment Brush cytology

Therapeutic purpose

Insertion of biliary stent to relieve biliary obstruction

Bring down bilirubin before operation Remove the stone with special

instruments

Despite of these… Pre-operative diagnostic rate remains low The quoted rate in the literatures were 8-

62.5% actually not very important not recognizing

it before OT but it is disastrous if not recognized intra-op

Fail to recognize this condition may lead to significant morbidity and mortality

Lai EC, Lau WY. Mirizzi syndrome: history, present and future development. ANZ J Surg. 2006 Apr;76(4):251-7.

Baer HU, Matthews JB, Schweizer WP, Gertsch P, Blumgart LH. Management of the mirizzi syndrome and the surgical implication of the cholecystocholedochal fistula. Br. J. Surg. 1990;77:743-5

Management options Surgical

Open surgery Laparoscopic surgery

Non-surgical Endoscopic Interventional radiology

Percutaneous transhepatic stone removal Extracorporeal shock-wave lithotripsy Oral dissolution therapy

Open surgery Remains the gold standard of treatment

with good short term and long term result.

Lai EC, Lau WY. Mirizzi syndrome: history, present and future development. ANZ J Surg. 2006 Apr;76(4):251-7.

How do we do it? Kocher’s incision Frozen section should be sent if malignancy is suspected Mobilize the gallbladder using the fundus first approach Transect gallbladder at around Hartmann’s pouch region

(partial cholecystectomy) Remove the stone, if there is a gush of bile, this suggest

presence of cholecystobiliary fistula Then you have to decide whether to..

Repair or reconstruct according to extent of destruction

ECBD or not depends on suspicion of residual stone in common duct

T-tube or not depends on likelihood of biliary stricture and bile leaks from repair site

Methods of reconstruction

Controversies in management

Treatment approach Which repair method is the best? Direct

repair or HJ for all the case

Placement of t-tube When and where to insert t-tube? Proximal, distal or right into the fistula?

No randomized control trial to answer these questions

Laparoscopic surgery

Technically feasible but more risky Most series involved small case

number and the successful cases were limited to mild disease (type I or II)

Higher complication rate, re-operation rate and conversion rate (near 100% conversion for type II disease)

Antoniou SA, Antoniou GA, Makridis C. Laparoscopic treatment of Mirizzi syndrome: a systematic review. Surg Endosc. 2010 Jan; 24 (1):33-9. Epub 2009 May 23.

Endoscopic treatment Method

Use of mother-and-baby scope

Fragment the stone with EHL

Extract the stone with basket

Drawbacks Stone not easily

accessible, especially for type I

May need multiple sessions and time consuming

Reserve for poor surgical candidate

Tsuyuguchi T, Saisho H, Ishihara T, Yamaguchi T, Onuma EK.Long-term follow-up after treatment of Mirizzi syndromeby peroral cholangioscopy. Gastrointest. Endosc. 2000; 52:639–44.

Other treatment options

Percutaneous transhepatic stone removal Reserved for patient with high operative

risk

Oral dissolution therapy May not work for large stone and

obstructed cystic duct Cholesterol stones are not as common as

compared to the western patients

To conclude

Mirizzi syndrome is uncommon but important

ERCP and CT are the two important investigations

treatment should be individualized open surgery with adequate

treatment often provide satisfactory outcome

Thank you

0.3% to 3%, why so variable?

depends on accessibility of medical services, i.e. USG, lap chole…

Lifestyle BMI….

Male or female, which is more common? Series said male..

Tan KY, Ching HC, Chen CYY, Tan SM, Poh BK, Hoe MNY. Mirizzi syndrome: noteworthy aspects of a retrospective study in one centre. ANZ J Surg. 2004; 74:833-7.

Al-Akeely MH, Alam MK, Bismar HA, Khalid K, Al-Teimi I, Al-Dossary NF. Mirizzi syndrome: ten years experience from a teaching hospital in Riyadh. World J Surg. 2005 Dec;29 (12):1687-92.

Series said female.. Csendes A, Diaz JC, Burdiles P, Maluenda F, Nava O. Mirizzi

syndrome and cholecystobiliary fistula: a unifying classification. Br. J. Surg. 1989;76:1139-43.

Chan CY, Liau KH, Ho CK, et al. Mirizzi syndrome: a diagnostic and operative challenge. Surg. J. R. Coll. Surg. Edinb. Irel. 2003; 1: 273-8

McSherry CK, Ferstenberg H, Virshup M. The Mirizzi syndrome: suggested classification and surgical therapy. Surg Gastroenterol 1982; 1: 219-225

Type V Mirizzi?

Csendes group introduce type V Mirizzi syndrome in a recent publication in World J surgery

All Mirizzi syndrome with coexciting cystoenteric fistula will classified type V

Type V Mirizzi…

How does it present?

Common Cholecystitis Cholangitis

Less common In ileum: gallstone ileus In duodenum: Bouveret’s syndrome (gastric

outlet obstruction due to gallstone) As malignancy

Carcinoma of gallbladder cholangiocarcinoma

Gallstone ileus(the Rigler’s triad)

Bouveret’s syndrome

Bouveret’s syndrome