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REVIEW ARTICLE
MIRIZZI SYNDROME: HISTORY, PRESENT AND
FUTURE DEVELOPMENT
ERIC C. H. LAI AND WAN YEE LAU
Department of Surgery, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin,
New Territories, Hong Kong
Background: Mirizzi syndrome was reported in 0.3–3% of patients undergoing cholecystectomy. The distortion of anatomy and
the presence of cholecystocholedochal fistula increase the risk of bile duct injury during cholecystectomy.
Methods: A Medline search was undertaken to identify articles that were published from 1974 to 2004. Additional papers were
identified by a manual search of the references from the key articles.
Results: A preoperative diagnosis was made in 8–62.5% of cases. Open surgical treatment gave good short-term and long-term
results. There was a lack of good data in laparoscopic treatment. Conversion to open surgery rates was high, and bile duct injury rate
varied from 0 to 22.2%.
Conclusion: A high index of clinical suspicion is required to make a preoperative or intraoperative diagnosis, which leads to good
surgical planning to treat the condition. Open surgery is the gold standard. Mirizzi syndrome should still be considered as a contra-
indication for laparoscopic surgery.
Key words: bile duct injury, cholecystectomy, gall bladder neoplasm, laparoscopy, Mirizzi syndrome.
Abbreviations: CT, computed tomography; ERCP, endoscopic retrograde cholangiopancreatography; IOC, intraoperative
cholangiogram; MRCP, magnetic resonance cholangiopancreatography; USG, ultrasonography.
INTRODUCTION
Mirizzi syndrome was reported in 0.3–3% of patients undergoing
cholecystectomy.1–6 Mirizzi syndrome is a spectrum of disease
process evolving from gallstone impaction with biliary obstruc-
tion to cholecystocholedochal fistula to complete erosion of com-
mon hepatic duct.1 The distortion of anatomy and the presence of
cholecystocholedochal fistula increase the risk of bile duct injury
during cholecystectomy. Preoperative diagnosis of Mirizzi syn-
drome followed by good surgical planning is very important. If
preoperative diagnosis is not made, intraoperative recognition is
essential. Inadequate recognition of this condition leads to high
operative morbidity and mortality.2
This article reviews the history, classification, diagnosis and
management of Mirizzi syndrome as well as the role of laparo-
scopic management of Mirizzi syndrome.
METHODS
A Medline search was undertaken to identify articles that were
published from 1974 to 2004 using the keywords ‘Mirizzi syn-
drome’, ‘cholecystocholedochal fistula’, ‘laparoscopic cholecys-
tectomy’ and ‘bile duct injury’. Additional papers were identified
by a manual search of the references from the key articles.
HISTORY AND CLASSIFICATION
Partial ductal obstruction secondary to impacted stone and
inflammation was first described by Kehr in 1905 and Ruge in
1908.7,8 In 1948, Mirizzi described a functional hepatic syn-
drome, which consisted of a common hepatic duct obstruction
secondary to compression by the gallstone impacted at the gall
bladder neck or cystic duct, surrounding inflammation, recurrent
cholangitis and spasm of the circular muscular sphincter in the
hepatic duct.9 Mirizzi postulated that a number of factors might
trigger or predispose to the contraction of this sphincter such as
inflammation, aberrant vessels or stones impacted in the cystic
duct. Nowadays, we all know that there is no sphincter in the
common hepatic duct. Mirizzi syndrome now denotes the narrow-
ing of the common hepatic duct by gallstone impacted in the
cystic duct or in the neck of the gall bladder.
For cholecystobiliary fistula, Puestow described the first case
in 1942.10 Subsequently, more cases were reported.11,12 Mirizzi
also described four cases of cholecystocholedochal fistula in
1952.13 Corlette and Bismuth presented 24 cases of fistula in
1975.14 They classified these fistulas as type I, with a fistula
between the gall bladder and the common hepatic duct, and type
II, with a large fistula between the gall bladder and the common
duct in the ‘trajectory of the cystic duct’, such that no cystic duct
was found. The pathogenesis of cholecystocholedochal fistula
starts with a long-standing history of gallstones impacted at
the gall bladder neck or cystic duct and inflammation of the
gall bladder.15 The inflamed gall bladder adheres or even fuses
to the adjacent bile duct. The gallstone impaction causes pres-
sure necrosis of the intervening wall and further inflammation.
The stone may then erode through the wall into the adjacent
bile duct.
E. C. H. Lai MB ChB, MRCSEd; W. Y. Lau MD, FRCS, FACS,
FRACS(Hon).
Correspondence: Professor W. Y. Lau, Department of Surgery, Prince of
Wales Hospital, The Chinese University of Hong Kong, Shatin, New Territo-
ries, Hong Kong.
E-mail: josephlau@cuhk.edu.hk
Accepted for publication 24 May 2005.
ANZ J. Surg. 2006; 76: 251–257 doi: 10.1111/j.1445-2197.2006.03690.x
Ó 2006 Royal Australasian College of Surgeons
In the 1980s, the original type of Mirizzi syndrome and the
cholecystocholedochal fistula became recognized as different
evolving stages of the same disease process. In 1982, McSherry
et al. classified Mirizzi syndrome into two types based on endo-
scopic retrograde cholangiopancreatography (ERCP) findings
(Fig. 1).16 Type I involves the external compression of the com-
mon hepatic duct by a large stone impacted in the cystic duct or
the Hartmann’s pouch, without any lesion in the gall bladder or
the common hepatic duct wall. In type II, a cholecystcholedochal
fistula is present. It is caused by a calculus, which has eroded
partly or completely into the common duct. In 1989, Csendes
et al. classified Mirizzi syndrome into four types (Fig. 1). Their
classification further categorized the cholecystocholedochal fis-
tula according to its extent of destruction.1 Type I lesion is the
external compression of the common duct because of a stone
impacted at the neck of the gall bladder or at the cystic duct. Type
II lesion is a cholecystobiliary fistula (cholecystohepatic or chol-
ecystocholedochal) that results from the erosion of the anterior or
lateral wall of the common duct by the impacted stones, the fistula
involving less than one-third of the circumference of the common
duct. Type III lesion is a cholecystobiliary fistula with erosion of
the wall of the common duct that involves up to two-thirds of its
circumference. Type IV lesion is a cholecystobiliary fistula, with
complete destruction of the entire wall of the common duct. In the
past 20 years other authors have described different classification
systems, such as acute versus chronic, anatomic variant of cystic
duct versus no anatomic variant of cystic duct and obstruction due
to gallstones versus obstruction due to inflammation.17–19 In pub-
lished reports and in clinical practice, most clinicians would use
either the McSherry classification or the Csendes classification.
These classifications are more useful to guide surgical man-
agement. The reported incidence of McSherry type I varied from
11 to 45%, whereas that of McSherry type II (i.e. Csendes type II,
III and IV) varied from 55 to 89%. Only less than 6% of patients
with Mirizzi syndrome had Csendes type IV.
DIAGNOSIS
The preoperative diagnosis of Mirizzi syndrome is very import-
ant.20,21 In the series reported by Tan et al., bile duct injuries were
observed in four (16.7%) out of 24 patients operated. All the four
bile duct injuries occurred in patients who did not have a preop-
erative diagnosis.21
In Mirizzi syndrome, patients present with jaundice (60–100%)
and abdominal pain (50–100%).1,5,22–27 Ultrasonography (USG)
of the abdomen is widely used for the initial screening.28,29 A
typical USG finding of Mirizzi syndrome is a large, immovable
stone in the region of the neck of a shrunken gall bladder, with
dilatation of bile ducts above it and a common bile duct of normal
calibre below it. Computed tomography (CT) scan of the abdo-
men may show an irregular cavity near the gall bladder neck and
calculi outside the viscus. However, the radiological signs are not
specific.29–31 The main role of CT scan is to differentiate Mirizzi
syndrome from malignancy of the extrahepatic biliary system.
Direct cholangiography (ERCP or percutaneous transhepatic
cholangiography) is usually carried out after USG or CT scan in
order to delineate the cause, level and extent of biliary obstruc-
tion. Direct cholangiography is valuable in showing ductal ab-
normalities and fistula.32–34 Cholangiographic features of Mirizzi
syndrome are narrowing or curvilinear extrinsic compression,
usually involving the lateral portion of the distal common hepatic
duct with proximal dilatation. Magnetic resonance cholangiopan-
creatography (MRCP) has been shown to have a high sensitivity
and specificity in the detection of gallstone and bile duct steno-
sis.35,36 Magnetic resonance cholangiopancreatography can show
the typical features in Mirizzi syndrome, such as the extrinsic nar-
rowing of the common hepatic duct, a gallstone in the cystic duct,
the dilatation of the intrahepatic and common hepatic ducts and
a normal common bile duct. Magnetic resonance imaging can also
show the extent of inflammation around the gall bladder, and this
helps to differentiate this condition from other gall bladder dis-
eases.36,37 Magnetic resonance cholangiopancreatography has the
advantage of avoiding the complications of direct cholangiography.
Unfortunately, a preoperative diagnosis can be made only in
8–62.5% of patients.1,5,6,24–26 Therefore, intraoperative recognition
is essential.1,2,5,6,22–26 The presence of a shrunken gall bladder,
an obliterated Calot’s triangle, a dense fibrotic mass at the Calot’s
triangle and a dense adhesion at the subhepatic space should raise
the suspicion of Mirizzi syndrome. Baer et al. noted that if there
is a gush of bile after the removal of gall bladder stones, it
is suggestive of the presence of cholecystocholedochal fistula as
the cystic duct is always completely obliterated as a result of
chronic inflammation in patients without a fistula.2 Intraoperative
Fig. 1. Pathology and classifications of Mirizzi syndrome.
252 LAI AND LAU
Ó 2006 Royal Australasian College of Surgeons
cholangiogram (IOC) helps to confirm the diagnosis, determine
the location and size of the fistula, detect ductal stones and detect
whether there is any loss of integrity of the bile duct wall.1,2
However, it is sometimes difficult to have an IOC if the cystic
duct is obliterated, and persistent dissection in the Calot’s triangle
in trying to do a cholangiogram may result in bile duct injury.
Another useful investigatory tool is laparoscopic ultrasonography.
It provides real-time multiplanar images of the bile ducts from
various angles. It is useful in identifying the anatomy of the bil-
iary tree and in showing the relation of the common hepatic duct
to the cystic duct stone during dissection.38–41
MANAGEMENT
An accurate preoperative diagnosis with a careful surgical plan-
ning is very important in the management of Mirizzi syndrome.
The severe inflammatory process with dense adhesions and
oedematous tissues distort the anatomy. In addition, there may
be the presence of a cholecystocholedochal fistula. During the
operation, dissection of the Calot’s triangle may lead to bile duct
injury or excessive blood loss. Inadequate surgical planning may
lead to surgical morbidity, such as bile duct injury, delayed bile
duct stricture, secondary biliary cirrhosis of the liver, sepsis and
bleeding. In the published works in medicine, open surgical man-
agement of Mirizzi syndrome gives good short-term and long-
term results (Table 1).1,2,5,6,22–26,42,43 In some case series, there
were 0% operative morbidity and mortality.2,22–24,26,42
Mirizzi syndrome without cholecystocholedochal fistula
Conventional antegrade cholecystectomy is likely to damage the
biliary tree. Retrograde fundus first cholecystectomy cannot com-
pletely solve the problem. It can lead to a high transection of the
biliary tree if the pathology is not recognized. Subtotal cholecys-
tectomy was described by Bornman and Terblanche in 1985 to be
an easy, safe and definitive operation for a ‘difficult gall bladder’,
particularly in the presence of portal hypertension.44 This
approach is used to choose a convenient site, preferably the Hart-
mann’s pouch, to open the gall bladder. The gall bladder content is
then emptied. The gall bladder wall is partly removed by excision,
leaving a rim of wall attached to the liver. The origin of the cystic
duct and its course are identified from inside the gall bladder. Any
residual stones are removed. The cystic duct is secured without
dissection. This approach obviates the need for dangerous dissec-
tion in the Calot’s triangle, which has severe fibrosis and inflam-
mation. Since then, subtotal cholecystectomy has been used more
in the treatment of a ‘difficult gall bladder’, for example, portal
hypertension, Mirizzi syndrome.1,2,45–47 A lesser degree of chole-
cystectomy, that is, partial cholecystectomy, has also been shown
to be effective for type I Mirizzi syndrome.1,2 The common bile
duct should be investigated if necessary to exclude other causes of
obstructive jaundice. T-tube insertion may be needed for tempo-
rary decompression. Most inflammatory strictures return to nor-
mal when the inflammatory process resolves.
Mirizzi syndrome with cholecystocholedochal fistula
It is important to recognize the presence of a cholecystochole-
dochal fistula before operation. Corlette and Bismuth recom-
mended the initial removal of gallstones, followed by a partial
cholecystectomy, with a remnant of gall bladder left around the
Table1.ResultsofopensurgicalmanagementforMirizzisyndrome
StudynTypeITypeIIFollow-up
duration
Postoperative
haemorrhage
Residual
stone
Bile
leakage
Acutebile
ductinjury
External
biliaryfistula
Delaybile
ductstricture
In-hospital
mortality
Fanetal.1985224044–14months0000000
VenkateshRao
etal.198823
9092years0000000
Csendesetal.1989121923II,90;III,97;IV,9†1–13years
(mean,5.7)
42160161215
Baeretal.19902404ND0000000
Yipetal.1991245056–36months0000000
Ibrarullahetal.19935144II,7;III,3†1–27months
(mean,14)
0210000
Curetetal.199425175121–41months
(mean,30)
0221000
Xiaodongetal.199926164II,8;III,3;IV,1†78months
(mean,78)
0000000
Khanetal.19994233017–33months0000000
Shahetal.2001433482612–59months0230010
Johnsonetal.20016115II,3;III,3†1–20years1120100
†CsendesClassificationwasused;ND,nodataobtained.
MIRIZZI SYNDROME 253
Ó 2006 Royal Australasian College of Surgeons
fistula margins to aid in the closure of the fistula with its associ-
ated loss of part of the circumference of the bile duct wall.14
Successful choledochoplasty using the gall bladder or cystic duct
as pedicle graft has been well described in several series.48–51
A gall bladder flap has the advantage of having an independent
blood supply and a related mucosal lining. This technique has
been shown to be effective in an animal study.52 It has been widely
used in the past 20 years. Choledochoplasty using ligamentum
teres and vein patch has also been reported, but the available
outcome data were very limited.53,54 After exploring bile ducts
through a choledochotomy distal to the fistula, a T-tube is usually
placed into the bile duct through the fistula, and the gall bladder
remnant is closed around the tube. In case of a large fistula,
hepaticojejunostomy is recommended.
In the large series of 219 patients with Mirizzi syndrome
reported by Csendes et al., the authors recommended partial
cholecystectomy to remove the stones, to see the common duct,
and to define the type and location of the fistula with operative
cholangiogram.1 They recommended placing the T-tube distal to
the fistula rather than through the fistula as there were risks of
bile leakage and bile duct stricture. In Csendes’ type II Mirizzi
syndrome, suturing of the fistula using absorbable material or
choledochoplasty using the remnant of gall bladder was carried
out. In Csendes’ type III Mirizzi syndrome, choledochoplasty
rather than suturing was recommended. In Csendes’ type IV
Mirizzi syndrome, bilioenteric anastomosis was preferred. They
found that the operative mortality and morbidity increased with
the severity of the lesion. However, in the series reported by Baer
et al., high operative morbidity and mortality rates were found in
patients with cholecystocholedochal fistula managed with direct
suturing and flap closure technique.2 Baer et al. proposed that
direct repair using gall bladder flap was prone to failure because
the tissues were damaged by the inflammatory process, thus
resulting in a high rate of bile duct stenosis. Instead, they
suggested using biliary-enteric bypass to the wall of the chole-
cystocholedochal defect by using either a duodenostomy or a
Roux-en-Y jejunostomy. This technique has also been described
in other series.55,56
There is still no prospective randomized trial or large retrospec-
tive comparative trial to define whether choledochoplasty with
a gall bladder flap or choledochoduodenostomy bypass should
be used in the treatment of small-sized to moderate-sized chole-
cystocholedochal fistula and whether a T-tube should be placed at
or distal to the fistula site.
Based on the available data in the published works in medi-
cine, most authors suggest partial cholecystectomy with gall-
stone removal and exploration of the common duct through the
defect or a separate choledochotomy site. A gall bladder flap
is used to close the fistula site. A T-tube is inserted at the fistula
site or distal to the fistula site. If the gall bladder remnant tissue
is too inflamed, biliary-enteric bypass to the wall of the chole-
cystocholedochal defect in the fistula site or Roux-en-Y
hepaticojejunostomy is required. For the management of a
large cholecystocholedochal fistula with extensive destruction
of the common hepatic duct, Roux-en-Y hepaticojejunostomy is
recommended.
Non-surgical treatment of Mirizzi syndrome
Endoscopic treatment of Mirizzi syndrome comprises biliary
drainage by stent insertion and gallstone removal by using a basket
or a balloon. The alternatives are mechanical, electrohydraulic or
extracorporeal lithotripsy, or dissolution therapy.57–64 Percutane-
ous transhepatic management is reserved for patients who have
failed endoscopic treatment.65,66
The limitations of non-surgical treatment include: the need of
expertise, time-consuming procedures, multiple sessions of treat-
ment, cost of equipment and risk of complications. Complications
of non-surgical treatment include bile leakage from cystic or fis-
tula stump, sepsis and residual stones. Thus, non-surgical treat-
ment of Mirizzi syndrome should be used only in patients with
poor surgical risks. It can also be used as a temporary measure
during the preparation of patients for an elective operation.20,57,67
Mirizzi syndrome and carcinoma of gall bladder
It is difficult to differentiate Mirizzi syndrome from carcinoma of
gall bladder and from xanthogranulomatous cholecystitis.68–73 In
approximately 6–27.8% of patients with a preoperative diagnosis
of Mirizzi syndrome, carcinoma of gall bladder turned out to be
the final diagnosis. Redaelli et al. reported a high association
(27.8%) of carcinoma of gall bladder and Mirizzi syndrome.71
Computed tomography scan before an operation plays an impor-
tant role in the detection of any suspicious signs of carcinoma of
gall bladder.29 Frozen section should be obtained for all patients
with Mirizzi syndrome undergoing surgery. If an operable carci-
noma of gall bladder is diagnosed intraoperatively, open radical
cholecystectomy with porta hepatis lymph node dissection should
be carried out.74 We should not leave the diagnosis of gall bladder
carcinoma undetected at the time of surgery because of the fol-
lowing reasons. First, radical reoperation for incidental carcinoma
of gall bladder diagnosed after cholecystectomy has a poorer out-
come than one-stage radical operation.75,76 Second, for patients
who have been diagnosed to have incidental carcinoma of gall
bladder after laparoscopic cholecystectomy, port site recurrence
and peritoneal seeding are common and have been reported in 5–
20% of patients.77,78 Third, gall bladder perforation during chole-
cystectomy is associated with a high incidence of recurrence
(40%).77 All these would jeopardize the patient’s survival.79
Development in laparoscopic treatment
The incidence of iatrogenic bile duct injury is significantly higher
in laparoscopic cholecystectomy than in open cholecystectomy
for gall bladder pathologies.80–85 Three conditions predispose lap-
aroscopic cholecystectomy to an increased risk of bile duct inju-
ries: dangerous pathologies, difficult anatomy and dangerous
technique. Mirizzi syndrome is one of the dangerous pathologies.
Severe inflammation of the Calot’s triangle makes dissection of
the cystic duct and artery hazardous. The role of laparoscopic
surgery in the treatment of Mirizzi syndrome is still controversial.
Mirizzi syndrome is generally considered as a contraindication to
laparoscopic cholecystectomy.86–88 Laparoscopic subtotal chole-
cystectomy and laparoscopic fundus first cholecystectomy have
been described to decrease the chance of bile duct injury and
conversion rate to open surgery.89–93 There has been no retrospec-
tive comparative or prospective randomized trials to compare lap-
aroscopic with open surgical approach for Mirizzi syndrome.
Only small case series or case reports of laparoscopic treatment
of Mirizzi syndrome were found in the published works in medi-
cine. All these reports focused on the technical aspects and the
short-term outcomes (Table 2).41,87,88,94–103 Conversion to open
surgery rate was reported to range from 0 to 100%. If only patients
with type II Mirizzi syndrome were analysed, the conversion rate
254 LAI AND LAU
Ó 2006 Royal Australasian College of Surgeons
could be as high as 100%. Overall complication rate was 0–60%.
Bile duct injury rate ranged from 0 to 22.2%. In-hospital mortality
rate was reported to range from 0 to 25%.
It is evident that technically it is feasible to carry out laparo-
scopic treatment in highly selected patients with Mirizzi syn-
drome. However, the evidence in published reports showed that
laparoscopic treatment for Mirizzi syndrome is associated with
high morbidity and mortality rates. Mirizzi syndrome should
still be considered as a contraindication to laparoscopic surgery
in most centres, possibly with the exception of highly experi-
enced centres using laparoscopic treatment as a treatment under
investigation.
CONCLUSIONS
A high index of clinical suspicion is required to make a preop-
erative diagnosis of Mirizzi syndrome, which leads to good
surgical planning to treat the condition. Failing a preoperative
diagnosis, intraoperative recognition of Mirizzi syndrome is
important to avoid a high rate of bile duct injury. Intraoperative
cholangiography and ultrasound can help in the diagnosis. Open
surgical treatment is the gold standard. Mirizzi syndrome
should still be considered as a contraindication for laparoscopic
surgery.
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Table 2. Results of laparoscopic management for Mirizzi syndrome
Study n Type I Type II Follow-up
duration
Conversion
rate (%)
Overall complication
rate (%)
Residual
stone
Bile
leakage
Acute bile
duct injury
In-hospital
mortality
Rust et al. 199187 1 1 0 ND 100 0 0 0 0 0
Paul et al. 199294 1 1 0 ND 0 0 0 0 0 0
Binnie et al. 199295 1 0 1 ND 0 0 0 0 0 0
Meng et al. 199541 1 1 0 ND 0 0 0 0 0 0
Posta et al. 199588 1 0 1 ND 100 0 0 0 0 0
Targarona et al. 199796 4 0 4 3–36 months
(mean, 19)
100 0 0 0 0 0
Sare et al. 199897 4 3 1 ND 25† 25 0 1 0 1
Kok et al. 199898 6 3 3 8–17 months
(mean, 12)
16.7 0 0 0 0 0
Chowbey et al. 200099 27 12 15 2.1 years
(mean, 2.1)
22 0 0 0 0 0
Vezakis et al. 2000100 5 2 3 26–61 months 0 60 3 0 0 1
Bagia et al. 2001101 9 8 1 ND 22.2 33.3 1 1 1 0
Schafer et al. 2003102 39 34 5 ND 74† 18 0 1 0 0
Yeh et al. 2003103 11 10 1 ND 36.4† 9.1 1 0 0 0
†All patients were Mirizzi type II; ND, no data obtained.
MIRIZZI SYNDROME 255
Ó 2006 Royal Australasian College of Surgeons
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30. Pedrosa CS, Casanova R, de la Torre S, Villacorta J. CT findings
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MIRIZZI SYNDROME 257
Ó 2006 Royal Australasian College of Surgeons

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Mirizzi Syndrome Diagnosis and Treatment Review

  • 1. REVIEW ARTICLE MIRIZZI SYNDROME: HISTORY, PRESENT AND FUTURE DEVELOPMENT ERIC C. H. LAI AND WAN YEE LAU Department of Surgery, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, New Territories, Hong Kong Background: Mirizzi syndrome was reported in 0.3–3% of patients undergoing cholecystectomy. The distortion of anatomy and the presence of cholecystocholedochal fistula increase the risk of bile duct injury during cholecystectomy. Methods: A Medline search was undertaken to identify articles that were published from 1974 to 2004. Additional papers were identified by a manual search of the references from the key articles. Results: A preoperative diagnosis was made in 8–62.5% of cases. Open surgical treatment gave good short-term and long-term results. There was a lack of good data in laparoscopic treatment. Conversion to open surgery rates was high, and bile duct injury rate varied from 0 to 22.2%. Conclusion: A high index of clinical suspicion is required to make a preoperative or intraoperative diagnosis, which leads to good surgical planning to treat the condition. Open surgery is the gold standard. Mirizzi syndrome should still be considered as a contra- indication for laparoscopic surgery. Key words: bile duct injury, cholecystectomy, gall bladder neoplasm, laparoscopy, Mirizzi syndrome. Abbreviations: CT, computed tomography; ERCP, endoscopic retrograde cholangiopancreatography; IOC, intraoperative cholangiogram; MRCP, magnetic resonance cholangiopancreatography; USG, ultrasonography. INTRODUCTION Mirizzi syndrome was reported in 0.3–3% of patients undergoing cholecystectomy.1–6 Mirizzi syndrome is a spectrum of disease process evolving from gallstone impaction with biliary obstruc- tion to cholecystocholedochal fistula to complete erosion of com- mon hepatic duct.1 The distortion of anatomy and the presence of cholecystocholedochal fistula increase the risk of bile duct injury during cholecystectomy. Preoperative diagnosis of Mirizzi syn- drome followed by good surgical planning is very important. If preoperative diagnosis is not made, intraoperative recognition is essential. Inadequate recognition of this condition leads to high operative morbidity and mortality.2 This article reviews the history, classification, diagnosis and management of Mirizzi syndrome as well as the role of laparo- scopic management of Mirizzi syndrome. METHODS A Medline search was undertaken to identify articles that were published from 1974 to 2004 using the keywords ‘Mirizzi syn- drome’, ‘cholecystocholedochal fistula’, ‘laparoscopic cholecys- tectomy’ and ‘bile duct injury’. Additional papers were identified by a manual search of the references from the key articles. HISTORY AND CLASSIFICATION Partial ductal obstruction secondary to impacted stone and inflammation was first described by Kehr in 1905 and Ruge in 1908.7,8 In 1948, Mirizzi described a functional hepatic syn- drome, which consisted of a common hepatic duct obstruction secondary to compression by the gallstone impacted at the gall bladder neck or cystic duct, surrounding inflammation, recurrent cholangitis and spasm of the circular muscular sphincter in the hepatic duct.9 Mirizzi postulated that a number of factors might trigger or predispose to the contraction of this sphincter such as inflammation, aberrant vessels or stones impacted in the cystic duct. Nowadays, we all know that there is no sphincter in the common hepatic duct. Mirizzi syndrome now denotes the narrow- ing of the common hepatic duct by gallstone impacted in the cystic duct or in the neck of the gall bladder. For cholecystobiliary fistula, Puestow described the first case in 1942.10 Subsequently, more cases were reported.11,12 Mirizzi also described four cases of cholecystocholedochal fistula in 1952.13 Corlette and Bismuth presented 24 cases of fistula in 1975.14 They classified these fistulas as type I, with a fistula between the gall bladder and the common hepatic duct, and type II, with a large fistula between the gall bladder and the common duct in the ‘trajectory of the cystic duct’, such that no cystic duct was found. The pathogenesis of cholecystocholedochal fistula starts with a long-standing history of gallstones impacted at the gall bladder neck or cystic duct and inflammation of the gall bladder.15 The inflamed gall bladder adheres or even fuses to the adjacent bile duct. The gallstone impaction causes pres- sure necrosis of the intervening wall and further inflammation. The stone may then erode through the wall into the adjacent bile duct. E. C. H. Lai MB ChB, MRCSEd; W. Y. Lau MD, FRCS, FACS, FRACS(Hon). Correspondence: Professor W. Y. Lau, Department of Surgery, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, New Territo- ries, Hong Kong. E-mail: josephlau@cuhk.edu.hk Accepted for publication 24 May 2005. ANZ J. Surg. 2006; 76: 251–257 doi: 10.1111/j.1445-2197.2006.03690.x Ó 2006 Royal Australasian College of Surgeons
  • 2. In the 1980s, the original type of Mirizzi syndrome and the cholecystocholedochal fistula became recognized as different evolving stages of the same disease process. In 1982, McSherry et al. classified Mirizzi syndrome into two types based on endo- scopic retrograde cholangiopancreatography (ERCP) findings (Fig. 1).16 Type I involves the external compression of the com- mon hepatic duct by a large stone impacted in the cystic duct or the Hartmann’s pouch, without any lesion in the gall bladder or the common hepatic duct wall. In type II, a cholecystcholedochal fistula is present. It is caused by a calculus, which has eroded partly or completely into the common duct. In 1989, Csendes et al. classified Mirizzi syndrome into four types (Fig. 1). Their classification further categorized the cholecystocholedochal fis- tula according to its extent of destruction.1 Type I lesion is the external compression of the common duct because of a stone impacted at the neck of the gall bladder or at the cystic duct. Type II lesion is a cholecystobiliary fistula (cholecystohepatic or chol- ecystocholedochal) that results from the erosion of the anterior or lateral wall of the common duct by the impacted stones, the fistula involving less than one-third of the circumference of the common duct. Type III lesion is a cholecystobiliary fistula with erosion of the wall of the common duct that involves up to two-thirds of its circumference. Type IV lesion is a cholecystobiliary fistula, with complete destruction of the entire wall of the common duct. In the past 20 years other authors have described different classification systems, such as acute versus chronic, anatomic variant of cystic duct versus no anatomic variant of cystic duct and obstruction due to gallstones versus obstruction due to inflammation.17–19 In pub- lished reports and in clinical practice, most clinicians would use either the McSherry classification or the Csendes classification. These classifications are more useful to guide surgical man- agement. The reported incidence of McSherry type I varied from 11 to 45%, whereas that of McSherry type II (i.e. Csendes type II, III and IV) varied from 55 to 89%. Only less than 6% of patients with Mirizzi syndrome had Csendes type IV. DIAGNOSIS The preoperative diagnosis of Mirizzi syndrome is very import- ant.20,21 In the series reported by Tan et al., bile duct injuries were observed in four (16.7%) out of 24 patients operated. All the four bile duct injuries occurred in patients who did not have a preop- erative diagnosis.21 In Mirizzi syndrome, patients present with jaundice (60–100%) and abdominal pain (50–100%).1,5,22–27 Ultrasonography (USG) of the abdomen is widely used for the initial screening.28,29 A typical USG finding of Mirizzi syndrome is a large, immovable stone in the region of the neck of a shrunken gall bladder, with dilatation of bile ducts above it and a common bile duct of normal calibre below it. Computed tomography (CT) scan of the abdo- men may show an irregular cavity near the gall bladder neck and calculi outside the viscus. However, the radiological signs are not specific.29–31 The main role of CT scan is to differentiate Mirizzi syndrome from malignancy of the extrahepatic biliary system. Direct cholangiography (ERCP or percutaneous transhepatic cholangiography) is usually carried out after USG or CT scan in order to delineate the cause, level and extent of biliary obstruc- tion. Direct cholangiography is valuable in showing ductal ab- normalities and fistula.32–34 Cholangiographic features of Mirizzi syndrome are narrowing or curvilinear extrinsic compression, usually involving the lateral portion of the distal common hepatic duct with proximal dilatation. Magnetic resonance cholangiopan- creatography (MRCP) has been shown to have a high sensitivity and specificity in the detection of gallstone and bile duct steno- sis.35,36 Magnetic resonance cholangiopancreatography can show the typical features in Mirizzi syndrome, such as the extrinsic nar- rowing of the common hepatic duct, a gallstone in the cystic duct, the dilatation of the intrahepatic and common hepatic ducts and a normal common bile duct. Magnetic resonance imaging can also show the extent of inflammation around the gall bladder, and this helps to differentiate this condition from other gall bladder dis- eases.36,37 Magnetic resonance cholangiopancreatography has the advantage of avoiding the complications of direct cholangiography. Unfortunately, a preoperative diagnosis can be made only in 8–62.5% of patients.1,5,6,24–26 Therefore, intraoperative recognition is essential.1,2,5,6,22–26 The presence of a shrunken gall bladder, an obliterated Calot’s triangle, a dense fibrotic mass at the Calot’s triangle and a dense adhesion at the subhepatic space should raise the suspicion of Mirizzi syndrome. Baer et al. noted that if there is a gush of bile after the removal of gall bladder stones, it is suggestive of the presence of cholecystocholedochal fistula as the cystic duct is always completely obliterated as a result of chronic inflammation in patients without a fistula.2 Intraoperative Fig. 1. Pathology and classifications of Mirizzi syndrome. 252 LAI AND LAU Ó 2006 Royal Australasian College of Surgeons
  • 3. cholangiogram (IOC) helps to confirm the diagnosis, determine the location and size of the fistula, detect ductal stones and detect whether there is any loss of integrity of the bile duct wall.1,2 However, it is sometimes difficult to have an IOC if the cystic duct is obliterated, and persistent dissection in the Calot’s triangle in trying to do a cholangiogram may result in bile duct injury. Another useful investigatory tool is laparoscopic ultrasonography. It provides real-time multiplanar images of the bile ducts from various angles. It is useful in identifying the anatomy of the bil- iary tree and in showing the relation of the common hepatic duct to the cystic duct stone during dissection.38–41 MANAGEMENT An accurate preoperative diagnosis with a careful surgical plan- ning is very important in the management of Mirizzi syndrome. The severe inflammatory process with dense adhesions and oedematous tissues distort the anatomy. In addition, there may be the presence of a cholecystocholedochal fistula. During the operation, dissection of the Calot’s triangle may lead to bile duct injury or excessive blood loss. Inadequate surgical planning may lead to surgical morbidity, such as bile duct injury, delayed bile duct stricture, secondary biliary cirrhosis of the liver, sepsis and bleeding. In the published works in medicine, open surgical man- agement of Mirizzi syndrome gives good short-term and long- term results (Table 1).1,2,5,6,22–26,42,43 In some case series, there were 0% operative morbidity and mortality.2,22–24,26,42 Mirizzi syndrome without cholecystocholedochal fistula Conventional antegrade cholecystectomy is likely to damage the biliary tree. Retrograde fundus first cholecystectomy cannot com- pletely solve the problem. It can lead to a high transection of the biliary tree if the pathology is not recognized. Subtotal cholecys- tectomy was described by Bornman and Terblanche in 1985 to be an easy, safe and definitive operation for a ‘difficult gall bladder’, particularly in the presence of portal hypertension.44 This approach is used to choose a convenient site, preferably the Hart- mann’s pouch, to open the gall bladder. The gall bladder content is then emptied. The gall bladder wall is partly removed by excision, leaving a rim of wall attached to the liver. The origin of the cystic duct and its course are identified from inside the gall bladder. Any residual stones are removed. The cystic duct is secured without dissection. This approach obviates the need for dangerous dissec- tion in the Calot’s triangle, which has severe fibrosis and inflam- mation. Since then, subtotal cholecystectomy has been used more in the treatment of a ‘difficult gall bladder’, for example, portal hypertension, Mirizzi syndrome.1,2,45–47 A lesser degree of chole- cystectomy, that is, partial cholecystectomy, has also been shown to be effective for type I Mirizzi syndrome.1,2 The common bile duct should be investigated if necessary to exclude other causes of obstructive jaundice. T-tube insertion may be needed for tempo- rary decompression. Most inflammatory strictures return to nor- mal when the inflammatory process resolves. Mirizzi syndrome with cholecystocholedochal fistula It is important to recognize the presence of a cholecystochole- dochal fistula before operation. Corlette and Bismuth recom- mended the initial removal of gallstones, followed by a partial cholecystectomy, with a remnant of gall bladder left around the Table1.ResultsofopensurgicalmanagementforMirizzisyndrome StudynTypeITypeIIFollow-up duration Postoperative haemorrhage Residual stone Bile leakage Acutebile ductinjury External biliaryfistula Delaybile ductstricture In-hospital mortality Fanetal.1985224044–14months0000000 VenkateshRao etal.198823 9092years0000000 Csendesetal.1989121923II,90;III,97;IV,9†1–13years (mean,5.7) 42160161215 Baeretal.19902404ND0000000 Yipetal.1991245056–36months0000000 Ibrarullahetal.19935144II,7;III,3†1–27months (mean,14) 0210000 Curetetal.199425175121–41months (mean,30) 0221000 Xiaodongetal.199926164II,8;III,3;IV,1†78months (mean,78) 0000000 Khanetal.19994233017–33months0000000 Shahetal.2001433482612–59months0230010 Johnsonetal.20016115II,3;III,3†1–20years1120100 †CsendesClassificationwasused;ND,nodataobtained. MIRIZZI SYNDROME 253 Ó 2006 Royal Australasian College of Surgeons
  • 4. fistula margins to aid in the closure of the fistula with its associ- ated loss of part of the circumference of the bile duct wall.14 Successful choledochoplasty using the gall bladder or cystic duct as pedicle graft has been well described in several series.48–51 A gall bladder flap has the advantage of having an independent blood supply and a related mucosal lining. This technique has been shown to be effective in an animal study.52 It has been widely used in the past 20 years. Choledochoplasty using ligamentum teres and vein patch has also been reported, but the available outcome data were very limited.53,54 After exploring bile ducts through a choledochotomy distal to the fistula, a T-tube is usually placed into the bile duct through the fistula, and the gall bladder remnant is closed around the tube. In case of a large fistula, hepaticojejunostomy is recommended. In the large series of 219 patients with Mirizzi syndrome reported by Csendes et al., the authors recommended partial cholecystectomy to remove the stones, to see the common duct, and to define the type and location of the fistula with operative cholangiogram.1 They recommended placing the T-tube distal to the fistula rather than through the fistula as there were risks of bile leakage and bile duct stricture. In Csendes’ type II Mirizzi syndrome, suturing of the fistula using absorbable material or choledochoplasty using the remnant of gall bladder was carried out. In Csendes’ type III Mirizzi syndrome, choledochoplasty rather than suturing was recommended. In Csendes’ type IV Mirizzi syndrome, bilioenteric anastomosis was preferred. They found that the operative mortality and morbidity increased with the severity of the lesion. However, in the series reported by Baer et al., high operative morbidity and mortality rates were found in patients with cholecystocholedochal fistula managed with direct suturing and flap closure technique.2 Baer et al. proposed that direct repair using gall bladder flap was prone to failure because the tissues were damaged by the inflammatory process, thus resulting in a high rate of bile duct stenosis. Instead, they suggested using biliary-enteric bypass to the wall of the chole- cystocholedochal defect by using either a duodenostomy or a Roux-en-Y jejunostomy. This technique has also been described in other series.55,56 There is still no prospective randomized trial or large retrospec- tive comparative trial to define whether choledochoplasty with a gall bladder flap or choledochoduodenostomy bypass should be used in the treatment of small-sized to moderate-sized chole- cystocholedochal fistula and whether a T-tube should be placed at or distal to the fistula site. Based on the available data in the published works in medi- cine, most authors suggest partial cholecystectomy with gall- stone removal and exploration of the common duct through the defect or a separate choledochotomy site. A gall bladder flap is used to close the fistula site. A T-tube is inserted at the fistula site or distal to the fistula site. If the gall bladder remnant tissue is too inflamed, biliary-enteric bypass to the wall of the chole- cystocholedochal defect in the fistula site or Roux-en-Y hepaticojejunostomy is required. For the management of a large cholecystocholedochal fistula with extensive destruction of the common hepatic duct, Roux-en-Y hepaticojejunostomy is recommended. Non-surgical treatment of Mirizzi syndrome Endoscopic treatment of Mirizzi syndrome comprises biliary drainage by stent insertion and gallstone removal by using a basket or a balloon. The alternatives are mechanical, electrohydraulic or extracorporeal lithotripsy, or dissolution therapy.57–64 Percutane- ous transhepatic management is reserved for patients who have failed endoscopic treatment.65,66 The limitations of non-surgical treatment include: the need of expertise, time-consuming procedures, multiple sessions of treat- ment, cost of equipment and risk of complications. Complications of non-surgical treatment include bile leakage from cystic or fis- tula stump, sepsis and residual stones. Thus, non-surgical treat- ment of Mirizzi syndrome should be used only in patients with poor surgical risks. It can also be used as a temporary measure during the preparation of patients for an elective operation.20,57,67 Mirizzi syndrome and carcinoma of gall bladder It is difficult to differentiate Mirizzi syndrome from carcinoma of gall bladder and from xanthogranulomatous cholecystitis.68–73 In approximately 6–27.8% of patients with a preoperative diagnosis of Mirizzi syndrome, carcinoma of gall bladder turned out to be the final diagnosis. Redaelli et al. reported a high association (27.8%) of carcinoma of gall bladder and Mirizzi syndrome.71 Computed tomography scan before an operation plays an impor- tant role in the detection of any suspicious signs of carcinoma of gall bladder.29 Frozen section should be obtained for all patients with Mirizzi syndrome undergoing surgery. If an operable carci- noma of gall bladder is diagnosed intraoperatively, open radical cholecystectomy with porta hepatis lymph node dissection should be carried out.74 We should not leave the diagnosis of gall bladder carcinoma undetected at the time of surgery because of the fol- lowing reasons. First, radical reoperation for incidental carcinoma of gall bladder diagnosed after cholecystectomy has a poorer out- come than one-stage radical operation.75,76 Second, for patients who have been diagnosed to have incidental carcinoma of gall bladder after laparoscopic cholecystectomy, port site recurrence and peritoneal seeding are common and have been reported in 5– 20% of patients.77,78 Third, gall bladder perforation during chole- cystectomy is associated with a high incidence of recurrence (40%).77 All these would jeopardize the patient’s survival.79 Development in laparoscopic treatment The incidence of iatrogenic bile duct injury is significantly higher in laparoscopic cholecystectomy than in open cholecystectomy for gall bladder pathologies.80–85 Three conditions predispose lap- aroscopic cholecystectomy to an increased risk of bile duct inju- ries: dangerous pathologies, difficult anatomy and dangerous technique. Mirizzi syndrome is one of the dangerous pathologies. Severe inflammation of the Calot’s triangle makes dissection of the cystic duct and artery hazardous. The role of laparoscopic surgery in the treatment of Mirizzi syndrome is still controversial. Mirizzi syndrome is generally considered as a contraindication to laparoscopic cholecystectomy.86–88 Laparoscopic subtotal chole- cystectomy and laparoscopic fundus first cholecystectomy have been described to decrease the chance of bile duct injury and conversion rate to open surgery.89–93 There has been no retrospec- tive comparative or prospective randomized trials to compare lap- aroscopic with open surgical approach for Mirizzi syndrome. Only small case series or case reports of laparoscopic treatment of Mirizzi syndrome were found in the published works in medi- cine. All these reports focused on the technical aspects and the short-term outcomes (Table 2).41,87,88,94–103 Conversion to open surgery rate was reported to range from 0 to 100%. If only patients with type II Mirizzi syndrome were analysed, the conversion rate 254 LAI AND LAU Ó 2006 Royal Australasian College of Surgeons
  • 5. could be as high as 100%. Overall complication rate was 0–60%. Bile duct injury rate ranged from 0 to 22.2%. In-hospital mortality rate was reported to range from 0 to 25%. It is evident that technically it is feasible to carry out laparo- scopic treatment in highly selected patients with Mirizzi syn- drome. However, the evidence in published reports showed that laparoscopic treatment for Mirizzi syndrome is associated with high morbidity and mortality rates. Mirizzi syndrome should still be considered as a contraindication to laparoscopic surgery in most centres, possibly with the exception of highly experi- enced centres using laparoscopic treatment as a treatment under investigation. CONCLUSIONS A high index of clinical suspicion is required to make a preop- erative diagnosis of Mirizzi syndrome, which leads to good surgical planning to treat the condition. Failing a preoperative diagnosis, intraoperative recognition of Mirizzi syndrome is important to avoid a high rate of bile duct injury. Intraoperative cholangiography and ultrasound can help in the diagnosis. Open surgical treatment is the gold standard. Mirizzi syndrome should still be considered as a contraindication for laparoscopic surgery. REFERENCES 1. Csendes A, Diaz JC, Burdiles P, Maluenda F, Nava O. Mirizzi syndrome and cholecystobiliary fistula: a unifying classifica- tion. Br. J. Surg. 1989; 76: 1139–43. 2. Baer HU, Matthews JB, Schweizer WP, Gertsch P, Blumgart LH. Management of the Mirizzi syndrome and the surgical implication of cholecystocholedochal fistula. Br. J. Surg. 1990; 77: 743–5. 3. Mishra MC, Vashishitha S, Tandon R. Biliobiliary fistula: pre- operative diagnosis and management implications. Surgery 1990; 108: 835–9. 4. Pemberton M, Wells AD. The Mirizzi syndrome. Postgrad. Med. J. 1997; 73: 487–90. 5. Ibrarullah MD, Saxena R, Sikora SS, Kapoor VK, Saraswat VA, Kaushik SP. Mirizzi’s syndrome: identification and management strategy. Aust. N. Z. J. Surg. 1993; 63: 802–6. 6. Johnson LW, Sehon JK, Lee WC, Zibari GB, McDonald JC. Mirizzi’s syndrome: experience from a multi-institutional review. Am. Surg. 2001; 67: 11–14. 7. Kehr H. Die in neiner Klinik geubte Technik de Gallenstein Operationen, mit einen Hinweis Auf die Indikationen und die Dauerersolge. Munchen: JF Lehmann, 1905. 8. Ruge E. Deitrage Zur Chirurgischen Anatomie der grossen Gal- lenwege (Ductus hepaticus, choledochus und pancreaticus). Arch. Clin. Chir. 1908; 78: 47. 9. Mirizzi PL. Sindrome del conducto hepatico. J. Int. Chir. 1948; 8: 731–7. 10. Puestow CB. Spontaneous internal biliary fistula. Ann. Surg. 1942; 115: 1043–54. 11. Patt HH, Koontz AR. Cholecystocholedochal fistula; a report of two cases. Ann. Surg. 1951; 134: 1064–5. 12. Behrend A, Cullen ML. Cholecysto-choledochal fistula: an unusual internal biliary fistula. Ann. Surg. 1950; 132: 297–303. 13. Mirizzi PL. Les fistules biliobiliares internes spontanees. J. Chir. 1952; 68: 23–8. 14. Corlette MB, Bismuth H. Biliobiliary fistula. A trap in the sur- gery of cholelithiasis. Arch. Surg. 1975; 110: 377–83. 15. Tanaka N, Nobori M, Furuya T et al. Evolution of Mirizzi’s syndrome with biliobiliary fistula. J. Gastroenterol. 1995; 30: 117–21. 16. McSherry CK, Ferstenberg H, Virship M. The Mirizzi syn- drome: suggested classification and surgical therapy. Surg. Gas- troenterol. 1982; 1: 219–25. 17. Morelli A, Narducci F, Ciccone R. Can Mirizzi syndrome be classified into acute and chronic form? An endoscopic retro- grade cholangiography (ERC) study. Endoscopy 1978; 10: 109–12. 18. Starling JR, Matallana RH. Benign mechanical obstruction of the common hepatic duct (Mirizzi syndrome). Surgery 1980; 88: 737–40. 19. 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. 20. Gomez G. Mirizzi syndrome. Curr. Treat. Options Gastroen- terol. 2002; 5: 95–9. Table 2. Results of laparoscopic management for Mirizzi syndrome Study n Type I Type II Follow-up duration Conversion rate (%) Overall complication rate (%) Residual stone Bile leakage Acute bile duct injury In-hospital mortality Rust et al. 199187 1 1 0 ND 100 0 0 0 0 0 Paul et al. 199294 1 1 0 ND 0 0 0 0 0 0 Binnie et al. 199295 1 0 1 ND 0 0 0 0 0 0 Meng et al. 199541 1 1 0 ND 0 0 0 0 0 0 Posta et al. 199588 1 0 1 ND 100 0 0 0 0 0 Targarona et al. 199796 4 0 4 3–36 months (mean, 19) 100 0 0 0 0 0 Sare et al. 199897 4 3 1 ND 25† 25 0 1 0 1 Kok et al. 199898 6 3 3 8–17 months (mean, 12) 16.7 0 0 0 0 0 Chowbey et al. 200099 27 12 15 2.1 years (mean, 2.1) 22 0 0 0 0 0 Vezakis et al. 2000100 5 2 3 26–61 months 0 60 3 0 0 1 Bagia et al. 2001101 9 8 1 ND 22.2 33.3 1 1 1 0 Schafer et al. 2003102 39 34 5 ND 74† 18 0 1 0 0 Yeh et al. 2003103 11 10 1 ND 36.4† 9.1 1 0 0 0 †All patients were Mirizzi type II; ND, no data obtained. MIRIZZI SYNDROME 255 Ó 2006 Royal Australasian College of Surgeons
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