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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
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
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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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
1. Beltran MA. Mirizzi syndrome: History, current knowledge and
proposal of a simplified classification. World J Gastroenterol.
2012;18(34):4639-4650.
2. Kumar A, Senthil G, Prakash A, et al. Mirizzi's syndrome: Lessons
learnt from 169 patients at a single center. Korean J Hepatobiliary
Pancreat Surg. 2016;20(1):17-22.
3. Gomez D, Rahman SH, Toogood GJ, et al. Mirizzi's syndrome-results from a large western experience. HPB (Oxford).
2006;8(6):474-479.
4. Csendes A, Diaz JC, Burdiles P, Maluenda F, Nava O. Mirizzi
syndrome and cholecystobiliary fistula: A unifying classification. Br
J Surg. 1989;76(11):1139-1143.
5. Cui Y, Liu Y, Li Z, Zhao E, Zhang H, Cui N. Appraisal of diagnosis
and surgical approach for mirizzi syndrome. ANZ J Surg.
2012;82(10):708-713.
6. Safioleas M, Stamatakos M, Revenas C, Chatziconstantinou C,
Safioleas C, Kostakis A. An alternative surgical approach to a
difficult case of mirizzi syndrome: A case report and review of the
literature. World J Gastroenterol. 2006;12(34):5579-5581.
7. Fontes PR, Teixeira UF, Waechter FL, Sampaio JA, Pereira-Lima L.
Mirizzi syndrome in association with serum CA 19-9 greater than
20.000U/mL: Is it possible? Arq Bras Cir Dig. 2012;25(1):69-70.
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