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REVIEW

Mirizzi syndrome: A pictorial gazette


JC Kloppers1, SR Thomson2
HPB Surgery1, Medical Gastroenterology2, Groote Schuur Hospital & University of Cape Town, Cape Town, South Africa

Introduction
Mirizzi syndrome is a rare complication of longstanding
gallstones disease causing extra hepatic bile duct
obstruction by a stone in the cystic duct or Hartmanns
pouch.1 Pablo Luis Mirizzi, an Argentinean surgeons, main
contribution to modern biliary surgery was the performance
of first operative cholangiography in 1931. He published the
syndrome synonymous with his name in 1948.1,2
Classifications of Mirizzi Syndrome
Classification systems have evolved as the pathology
progressed giving rise to fistulation into the common
hepatic duct of varying degrees and how that may
influence management options.
These classifications are amalgamated schematically in
Figure 2. McSherry et al who were the first to recognise
fistulation based on ERCP findings, divided Mirizzi
syndrome into type I & II. Type I is the external
compression of the bile duct by a large stone impacted in
the cystic duct or in the Hartmanns pouch. Type II consist
of a cholecystobiliary fistula, caused by a gallstone or
gallstones that have eroded into the common hepatic duct.3
Csendes and colleagues initially further subdivided the
McSherry type II cholecystobiliary fistula according to the

extent of bile duct destruction to correlate with a


management strategy.4 Csendes type II is a
cholecystobiliary fistula with erosion of less than a third of
the circumference of the bile duct. Type III is erosion of up
to two thirds circumference of the bile duct and type IV is
the complete destruction of the common bile duct wall by
the offending stone. In 2007, eighteen years after his
original classification, Csendes added a type V to his
classification. Type V represents a cholecystoenteric fistula
in addition to any of the other types. Type V is further
subdivided in Va, without gallstone ileus and type Vb,
complicated by gallstone ileus.5
Beltrn recently proposed a new classification to
correlated surgical treatment with type.1 The fistulising
type is divided in type II as less and more than 50% ductal
destruction. This is not yet universally used and for the
purpose of this article we have used the Csendes
classification.
Figure 2: Mirizzi Classification Systems

Figure 1: Professor Pablo Luis Mirizzi (Picture by Sociedad


Argentina de Radiologia)

Correspondence
Christo Kloppers
email: jckloppers@gmail.com

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Diagnosis
The Mirizzi syndrome will generally present with features
of biliary obstruction and cholestatic liver function tests.
There are no pathognomonic clinical features on
presentation to distinguish between choledocholithaisis
or malignant biliary obstruction.6 Tumour markers are not
helpful in discriminating between malignancy and
Mirizzi, as there are numerous reports of Mirizzi
syndrome and very high CA 19-9 values.7 Ultrasound is
the initial imaging investigation performed on most
patients presented with jaundice or right upper quadrant
pain.8 It often reveals gallstones and cholecystitis and
findings suggestive of Mirizzi syndrome, a shrunken
gallbladder, a dilated common hepatic duct (CHD) with
normal-sized common bile duct (CBD) may be reported.5
The main utility of the radiological signs elicited by
computer tomography (CT) is the exclusion of malignancy.
Magnetic resonance cholangiopancreatography
(MRCP) and endoscopic retrograde
cholangiopancreatography (ERCP) are equivalent in their
ability to diagnose and delineate details of biliary
strictures.9 However, the diagnostic accuracy for MRCP in
Mirizzi syndrome is 50%.1 T2 weighted sections can
differentiate a neoplastic mass from an inflammatory one,
which cannot be detected by ultrasound or CT scan.6
ERCP is an invasive procedure with associated risk,
but is often indicated in suspected Mirizzi syndrome. It is
useful to confirm the presence of Mirizzi syndrome with
or without cholecystobiliary or cholecystoenteric fistulae.
Furthermore it may allow stone removal and at least
placement of a biliary stent to alleviate the jaundice.2,10
The diagnostic accuracy of ERCP for Mirizzi ranging from
55% - 90%. Mirizzi syndrome can often be diagnosed de
novo on an ERCP, as the indication for the procedure
would be endoscopic retrieval of suspected CBD stones.
MRCP and ERCP multiplanar imaging and
reconstruction may be helpful in determining the site of

Figure 3: ERCPs of two different patients, both with type I


Mirizzi syndrome. Arrow in A indicates a radio lucent stone as a
filling defect and in B a radio opaque stone with upstream
biliary dilatation

the Hartmanns/cystic duct or the CHD/CBD in Type 1 but


when fistulation occurs interpretation may be difficult.
Percutaneous trans hepatic cholangiography (PTC) is
not routinely indicated in suspected Mirizzi syndrome,
but could be helpful in diagnostic conundrums or failed
endoscopic management of drainage for sepsis. The PTC
catheter further helps in identification of the bile duct at
surgery in these challenging cases.
Figure 4: PTC demonstrated Mirizzi type V with the arrow
indicating a cholecysto-colonic fistula and intra-operatively
bilateral PTC catheters visible through fistula

Treatment
Endoscopic therapy is unlikely to result in definitive care.
In frail, poor surgical candidates ERCP placed biliary stent
with exchanges could be a strategy. To capture a Mirizzi
stone with conventional baskets and balloons are
exceedingly difficult. With the advent and availability of
cholangioscopy and directed manipulation and lithotripsy
this might change.11,12
The surgical treatment of Mirizzi syndrome requires
an individualized approach depending on the stage of the
disease and the expertise of the surgical team. It can
constitute a formidable challenge.8,13 In the absence of
definite preoperative diagnosis, awareness and careful
dissection on the part of the operating surgeon may
unmask a few unsuspected cases. The findings of dense
adhesions, shrunken gallbladder with or without
cholecystoenteric fistula, obliterated Calots triangle
should arouse suspicion of this entity.9,14
The surgical technique depends on the type of Mirizzi
syndrome. If the type has not been classified preoperatively, retrograde dissection is contra-indicated due
to the risk of injury to the structures in Calots triangle in
the presence of inflammation resulting in adhesions and
distorted anatomy.15 The best way forward is to open the
gallbladder from the fundus, and remove the stone this
will reveal the fistula and the degree of destruction of the
CHD.15
It is well established that the treatment for type I
Mirizzi is cholecystectomy or sub-total cholecystectomy.
This entity was initially considered an absolute
contraindication to laparoscopic surgery, but can be
attempted laparoscopically in experience hands. A high
incidence of conversion to open surgery could be
expected and a low threshold for conversion should be
maintained.16 All other types of Mirizzi should be
managed with open surgery.

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Figure 5: Operative picture of Type III Mirizzi and stone


delivered though fistula

Figure 6: Choledochoplasty performed with interrupted sutures


after removal of stones

Csendes type II & III can be treated with


choledochoplasty. A biliary reconstruction with a bilioenteric anastomosis must be reserved for selected cases
and those with distal obstructions. The gallbladder
should be removed leaving a remnant of gallbladder wall
measuring about 5mm around the cholecystobiliary fistula
in order to aid the closure of the defect in the bile duct.8
Drainage of the biliary tract via a t-tube or an ante grade
placed biliary stent can be used if the ampulla is still
intact to prevent a pressure build up in the bile duct with
it attendant risk of anastomotic breakdown. These
manoeuvres are probably not necessary if
choledochoscopy and intra-operative cholangiography
confirm ductal clearance and free drainage into the
duodenum.
In Csendes type II fistulas the stone within the bile
duct might be too large to easily deliver through the fistula
tract. The temptation to enlarge the fistula defect must be
avoided as this could compromise the common bile ducts
blood supply and are likely to heal with stricturing.17 The
surgical approach to best serve this situation is to perform
a separate choledochotomy. (See figure 7)

Figure 7: Csendes II, yellow arrow indicating fistula, blue


arrow indicating separate longitudinal choledochotomy for
removal of stone

The treatment of type IV Mirizzi with extensive


destruction of the bile duct consists of a bilio-enteric
anastomosis. A Roux-en-Y hepaticojejunostomy is the
preferred reconstruction, although many alternatives
has been described.8 In a surgical frail patient with
difficult dissection a Roux-en-Y cholecysto-choledochojejunostomy has been described.6 (See figure 8) Type
V Mirizzi provides a different surgical challenge and
the bilio-enteric fistula should be treated on its own
merit, mostly by simple closure of the implicated
viscera.
Conclusion
Mirrizzi syndrome and its fistulating forms remain a
diagnostic challenge as choledocholithiasis and malignant
biliary obstruction are far commoner causes of biliary
obstruction. Preoperative biliary imaging is often
suggestive if not diagnostic and can direct therapy
accordingly. ERCP may be valuable in further delineating
the anatomy or providing drainage for cholangitis. The
gallbladder is usually grossly inflamed making
laparoscopic surgery for the non fistulating variety an
Figure 8: Roux-en-Y cholecysto-choledocho-jejunostomy

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operation likely to result in conversion. In those where
there is a high clinical suspicion of a fistula open surgery
with a ductal reconstruction tailored to the situation is
the preferred surgical strategy.
References
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learnt from 169 patients at a single center. Korean J Hepatobiliary
Pancreat Surg. 2016;20(1):17-22.
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