1. Mirizzi Syndrome An Uncommon Gallstone Complication
Dr. Ma Ka Wing
Queen Elizabeth Hospital

2. Common gallstone complications
I think everyone of us are quite familiar with those common gallstone complications. As shown in this slide, depending on different location, gallstone could result in different problems like cholecystitis, cholangitis and pancreatitis but if …

3. 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
But if, the gallstone impact at the cystic duct/ gallbladder neck region, abutting or eroding the lateral wall of the common duct, leading to biliary obstruction., we call it Mirizzi syndome.

4. How uncommon is it?
Variable, from 0.3% to 3% of patients having cholecystectomy
More common in South America
How common is it?
Different series has different figure, ranging from 0.3% and 3%
In our hospital, its around 1 case per year, it’s uncommon, definitely not rare

5. 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 ( to )
Before we go into details, lets have some information about the man behind the syndrome
apart from giving his name to the syndrome, he is also well-known by performing the first OC, and that’s why also called mirizzigraphia
Leopardi LN, Maddern GJ. Pablo Luis Mirizzi: the man behind the syndrome. ANZ J Surg Dec;77(12):

6. 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 inflammation
Morelli A, Narducci F, Ciccone R. Can Mirizzi syndrome be
classified into acute and chronic form? An endoscopic retrograde
cholangiography (ERC) study. Endoscopy 1978; 10:
109–12.
In the past, different classifications suggested in the literatures, for example, …..
But these classification is no longer used nowadays…
Starling JR, Matallana RH. Benign mechanical obstruction of
the 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 and
therapeutic viewpoints. Hepatogastroenterology 1997; 44:
63–7.

7. How is it classified?
McSherry and Csendes classifications are most commonly used
Mcsherry and Csendes classification are most commonly used in the literature, and used for communication among surgeons

8. 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.

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

10. 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

11. 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

12. 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
These imagings have low sensitivity and specificity

13. ERCP Remains the most important investigation
serves both diagnostic and therapeutic purposes..
And eventually ERCP has to be performed

14. Diagnostic purposes Radiological assessment Microbiological 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

15. Therapeutic purpose
Insertion of biliary stent to relieve biliary obstruction
Bring down bilirubin before operation
Remove the stone with special instruments

16. Despite of these… Pre-operative diagnostic rate remains low
The quoted rate in the literatures were %
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
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
Lai EC, Lau WY. Mirizzi syndrome: history, present and future development. ANZ J Surg Apr;76(4):251-7.

17. Management options Surgical Non-surgical Open surgery
Laparoscopic surgery
Non-surgical
Endoscopic
Interventional radiology
Percutaneous transhepatic stone removal
Extracorporeal shock-wave lithotripsy
Oral dissolution therapy

18. 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 Apr;76(4):251-7.

19. 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

20. Methods of reconstruction

21. 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

22. 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 Jan; 24 (1):33-9. Epub 2009 May 23.

23. Endoscopic treatment Method Drawbacks
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 syndrome
by peroral cholangioscopy. Gastrointest. Endosc. 2000; 52:
639–44.

24. 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

25. 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

26. Thank you

28. 0.3% to 3%, why so variable?
depends on accessibility of medical services, i.e. USG, lap chole…
Lifestyle
BMI….

29. 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 Dec;29 (12):
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:
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:

30. 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

31. Type V Mirizzi…

33. How does it present? Common Less common As malignancy 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
Less commonly, it may present as gallstone ileus if the stone pass through the fistula and get impacted at the most narrow part of the bowel. AXR showing SB IO with aerobilia is the pathonomonic sign
And even more less commonly, the gallstone pas through the fistula and migrate proximally and impacted and the first part of duodenum, leading to gastric outlet obstruction, it gives rise to the Bouveret’s syndtome

34. Gallstone ileus(the Rigler’s triad)

35. Bouveret’s syndrome

36. Bouveret’s syndrome