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Mirizzi Syndrome: A Review of the Literature

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March 2006 KUWAIT MEDICAL JOURNAL

Review Article

Mirizzi Syndrome: A Review of the Literature

George J Xeroulis, Ward Davies
Department of Surgery, University of Western Ontario, London, Ontario, Canada

Kuwait Medical Journal 2006, 38 (1): 3-6

INTRODUCTION
Mirizzi syndrome is a rare cause of obstructive
jaundice. This entity should be considered in the
differential diagnosis of all patients with obstructive
jaundice. Failure to recognize the condition
preoperatively can result in a major bile duct injury,
particularly during laparoscopic surgery[1]. The
syndrome refers to obstruction of the common
hepatic duct by extrinsic compression usually from
a gallstone impacted in Hartmanns pouch or the
cystic duct. Large gallstones that become impacted
in this area produce common hepatic duct
obstruction by two mechanisms: mechanical
obstruction by direct compression of the common
hepatic duct, or they can cause obstruction
secondary to repeated bouts of local inflammation.
In 1948, Argentinean surgeon Pablo Luis
Mirizzi, first described a syndrome of common
hepatic duct obstruction in the setting of
longstanding cholelithiasis and cholecystitis[2]. The
classic description of the disease includes four
components: (a) a close parallel course of the cystic
duct and the common hepatic duct, (b) an impacted
stone in the cystic duct or the neck of the
gallbladder, (c) common hepatic duct obstruction
secondary to external compression by the cystic
duct stone (and the surrounding inflammation),
and (d) jaundice, with or without cholangitis.
Mirizzis syndrome is a rare complication of
cholelithiasis, with an estimated incidence of 0.05-
2.7%[1,3,4]. It presents as a spectrum of disease that
varies from extrinsic compression of the common
hepatic duct to the presence of a cholecystobiliary
fistula. Often, this dangerous alteration to anatomy
is not recognized preoperatively, and has the
potential to lead to significant morbidity and
biliary injury, particularly in the laparoscopic era.
CLASSIFICATION
There are three classifications which have been
proposed to describe variants of Mirizzi syndrome,
and to aid in selecting the appropriate therapeutic
procedure. The original classification, by McSherry
Address correspondence to:
George J Xeroulis, Department of Surgery, University of Western Ontario London, Ontario, Canada. E-mail: wardd@rogers.com
et al[5], described two types. Type I referred to
compression of the common hepatic duct by a stone
impacted in the cystic duct or Hartmanns pouch.
Type II referred to erosion of the calculus from the
cystic duct into the common hepatic duct,
producing a cholecystocholedochal fistula.
Csendes et al[6] created a second classification
taking into account the extent of fistula. Type I
remained the same, external compression of the
common hepatic duct due to a stone impacted at
the neck of the gallbladder or at the cystic duct.
Types II to IV lesion referred to the presence and
extent of a cholecystobiliary (cholecystohepatic or
cholecystocholedochal) fistula, due to erosion of
the anterior or lateral wall of the common hepatic
duct by impacted stones. The fistula involved less
than one-third of the circumference of the common
hepatic duct in type II. Involvement of between
one-third and two-thirds of the circumference of
the common hepatic duct was called a type III
lesion, while destruction of the entire wall of the
common hepatic duct was called a type IV lesion.
In their original paper, a total of 219 patients were
identified with Mirizzis syndrome. The incidence
of type I lesions was 11 per cent, type II, 41 per cent,
type III, 44 per cent and type IV, four per cent. The
majority had obstructive jaundice.
The third classification, proposed by Nagakawa
and colleagues[7], expanded upon the definition of
the Mirizzi syndrome. Type I referred to a stone
impacted in the cystic duct or gallbladder neck.
Type II was characterized by a fistula of the
common duct. Type III was defined by hepatic duct
stenosis due to a stone at the confluence of the
hepatic and cystic ducts. Type IV was characterized
by hepatic duct stenosis as a complication of
cholecystitis in the absence of calculi impacted in
the cystic duct or gallbladder neck.
In one series of 30 patients, the frequency of
these four types as described by Nagakawa et al
was 14, 2, 6, and 8%, respectively[8].
4 Mirizzi Syndrome: A Review of the Literature March 2006

Table 1: Various Classification Systems of Mirizzis Syndrome

McSherry Csendes Nagakawa

Type I Extrinsic compression of Type I
the common hepatic duct
by stones generally impac-
ted in the cystic duct or in
the infundibulum of the
gallbladder
Type II Presence of cholecystobiliary Type II
Type II Presence of cholecystobi-
liary fistula
Extrinsic compression of the Type I
common hepatic duct by stones
generally impacted in the cystic
duct or in the infundibulum of
the gallbladder
fistula with diameter one third
of circumference of the common
hepatic duct wall
Extrinsic compression (stenosis) of
the common hepatic duct by stones
generally impacted in the cystic
duct or in the infundibulum of the
gallbladder
Fistulization of common hepatic
duct from a stone impacted in the
cystic duct or in the infundibulum
of the gall bladder

Type III Presence of cholecystobiliary Type III Common hepatic duct stone at the
fistula with diameter two third cystic duct-hepatic duct confluence
of circumference of the common
hepatic duct wall
Type IV Common hepatic duct stenosis
caused by cholecystitis without
stones in the cystic duct or
Type IV Presence of cholecystobiliary
infundibulum of the gallbladder
fistula which involves the entire
circumference of the common
hepatic duct wall

Mirizzi syndrome is part of the differential
diagnosis of all patients with obstructive jaundice,
and requires a high index of suspicion. Most
patients present with jaundice, and right upper
quadrant pain[1]. Elevations in the serum concentra-
tions of alkaline phosphatase and bilirubin are
present in over 90 per cent of patients[8,9]. The
clinical and laboratory findings are similar to
patients who present with obstructive jaundice
secondary to choledocholithiasis. Once a diagnosis
of obstructive jaundice has been made an
abdominal ultrasound is often the first imaging test
preformed. Imaging generally reveals gallstones,
dilated intrahepatic ducts, with a long parallel
cystic duct and a contracted gallbladder[10]. The
presence of a stone impacted in the gallbladder
neck and an abrupt change to a normal width of the
common duct below the level of the stone are also
very suggestive of Mirrizis syndrome. The
sensitivity of ultrasound in detecting Mirizzis
syndrome is 23-46%[3,4]. In Csendes series,
ultrasound revealed dilated ducts in 81% of
patients and raised suspicion of Mirizzis syndrome
in only 27% of cases. CT scanning has a similar
sensitivity to ultrasound, but can be helpful in
diagnosing other causes of obstructive jaundice
such as gallbladder cancer, cholangiocarcinoma, or
metastatic tumor [11].
CHOLANGIOGRAPHY
Direct cholangiography is usually necessary to
establish the correct diagnosis and to delineate the
hepatic duct anatomy[10]. Pre-operative diagnosis is
essential in avoiding CBD injuries[12,13,14]. If it was
unexpectedly encountered at the time of surgery, a
cautious approach should be taken. Periductal
inflammation and the potential for a cholecysto-
choledochal fistula make a trial dissection
particularly challenging and should only be
undertaken by an experienced surgeon. Additional
imaging is often needed to obtain details of the
biliary anatomy. Intraoperative cholangiogram or
closing and obtaining a postoperative ERCP or
MRCP should be considered. Cholangiography
(intraoperative or ERCP) as well as MRCP will
allow for an accurate assessment of anatomy and
classification of the type of Mirizzis syndrome (Fig. 1).
The possibility of stone retrieval and biliary
stenting during ERCP is an added advantage in
improving surgical outcome, and stenting also
facilitates identification of the CBD during
operative dissection [7,15,16]. When ERCP is
unsuccessful or difficult, percutaneous transhepatic
cholangiography (PTC) is a viable alternative.
MRCP and ERCP are equivalent in their ability
to diagnosis and to delineate details of biliary
strictures, and to detect a cholecystocholedochal
fistula [4]. In addition, T2 weighted images can
differentiate a neoplastic mass from an
inflammatory one which US or CT scan may not be
capable of [17]. Early ERCP is preferred when biliary
sepsis is the dominant clinical issue and where a
beneficial endoscopic therapeutic procedure can be
instituted at the same time. By contrast, MRCP is
used in the non-septic patient to corroborate the
suspicion of malignancy or stones after initial
imaging with US or CT scans[4].
March 2006 KUWAIT MEDICAL JOURNAL
Fig. 1: Endoscopic retrograde cholangiopancreatography (ERCP) of
patient with obstructive jaundice and Mirizzis syndrome. Notice
impacted stone in cystic duct causing obstruction of common hepatic
duct. Adapted from UptoDate Mirizzi Syndrome James B McGee.
TREATMENT
Surgery is the mainstay of therapy of Mirizzi
syndrome, the dense inflammatory reaction in
Calots triangle, as well as the frequent aberrant
biliary anatomy, pose a difficult challenge to the
unsuspecting surgeon when dealing with a Mirizzi
syndrome. The two principal aims are (a) the safe
completion of cholecystectomy without injuring
the biliary system and (b) the appr opriate
management of the cholecystocholedochal fistula.
Meticulous dissection and vigilance are essential in
order to avoid inadvertent bile duct injury. If the
diagnosis of Mirizzi syndrome is made preoperatively,
an operative strategy that minimizes the risk of
injury to the biliary tract can be carried out.
However, a preoperative diagnosis of Mirizzi
syndrome is seldom made because ERCPand direct
cholangiography are not widely used. ERCP, direct
cholangiography, or magnetic resonance cholangio-
graphy should be performed in patients with
clinical jaundice and signs and symptoms
suggestive of biliary obstruction.
A standardized surgical approach has been
recommended based on the Csendes classification
of the variants of Mirizzi syndrome [6]:
5
l Type I - Cholecystectomy plus common bile
duct exploration with T-tube placement.
Exploration should be performed only if the CBD is
easily exposed.
l Type II - Suture of the fistula with absorbable
material or choledochoplasty with the remnant
gallbladder.
l Type III - Choledochoplasty; suture of the
fistula is not indicated.
l Type IV - Bilio-enteric anastomosis is
preferred since the entire wall of the common bile
duct has been destroyed.
l The approach may vary with the type of
fistula present; both the operative mortality and
postoperative morbidity increase according to the
severity of the lesion [6].
LAPAROSCOPIC SURGERY
The Mirizzi syndrome presents a difficult
challenge for laparoscopic surgery because the
dense adhesions and edematous inflammatory
tissue cause distortion of the normal anatomy and
increase the risk for biliary injury. While it appears
to be feasible, especially for type I anatomy[18,19], the
routine use of laparoscopic surgery as the primary
treatment of Mirizzi syndrome is controversial[20,21].
It has been suggested, that a prudent approach for
type 1 Mirizzi syndrome is to perform a trial
laparoscopic dissection, but to have a low threshold
to convert to an open procedure. This approach
should be undertaken only by experienced
laparoscopic surgeons[18,20].
ENDOSCOPIC THERAPY
Endoscopic treatment with or without
electrohydraulic lithotripsy (EHL) can be effective
as a temporizing measure before surgery and can
be definitive treatment for unsuitable surgical
candidates[9,22,23]. One report described the
experience with 14 patients with Mirizzi syndrome
treated with EHL[9]. Twelve patients had a single
stone and complete clearance was achieved with
one treatment session; two had multiple stones and
required an additional treatment session.
Asymptomatic leakage of contrast medium from
the cystic duct into the peritoneal cavity was
observed in one patient after removal of a large
impacted cystic duct stone. This patient recovered
with conservative therapy and suffered no adverse
events. In another series of 25 patients with
cholangiographic evidence of Mirizzi syndrome, 12
were referred for surgery after preliminary
endoscopic therapy and 13 were treated solely with
endoscopy[23]. Stones were completely removed in
three and nine were treated with long-term stents;
complications occurred in four patients [23].
6 Mirizzi Syndrome: A Review of the Literature
Endoscopic treatment of Mirizzi syndrome
should be used as a temporizing measure before
surgery. It can serve as a definitive treatment for
those patients who are unsuitable surgical
candidates when further endoscopic attempts can
be made to disimpact and remove the stones. Long-
term success appears to be most likely in patients
with type II disease who do not have residual
gallbladder stones[24].
CONCLUSION
Mirizzi syndrome is a rare complication of
cholelithiasis and requires a high index of suspicion
in the setting of obstructive jaundice. Diagnosis
preoperatively may be elusive with bloodwork, US
and CT alone. Cholangiography (intraoperative
and ERCP) as well as MRCP aids in both the
diagnosis and identification of anatomy and may
prevent serious biliary injury. Surgery is the
mainstay of therapy of Mirizzi syndrome, and
requires the safe completion of cholecystectomy
without injuring the biliary system and the
appropriate management of the cholecysto-
choledochal fistula. The aberrant anatomy intrinsic
to this syndrome presents a difficult challenge to
surgeons and the laparoscopic approach should be
undertaken with caution and probably left to
specialized minimally invasive centres. Endoscopic
treatment may be effective as a temporizing
measure before surgery and can be definitive
treatment for unsuitable surgical candidates.
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