Open Access Case

Report DOI: 10.7759/cureus.37029

Mirizzi Syndrome Type I: A Case Presentation

Michelle N. Won 1 , Dylon P. Collins 1 , Stephanie Bouchard 1 , Christopher Finley 2

Received 02/18/2023 1. Dr. Kiran C. Patel College of Osteopathic Medicine, Nova Southeastern University, Fort Lauderdale, USA 2. Bariatric
Review began 03/19/2023 and Minimally Invasive Surgery, Fawcett Florida Memorial Hospital, Port Charlotte, USA
Review ended 03/30/2023
Published 04/02/2023
Corresponding author: Dylon P. Collins, dc2273@mynsu.nova.edu
© Copyright 2023
Won et al. This is an open access article
distributed under the terms of the Creative
Commons Attribution License CC-BY 4.0.,
which permits unrestricted use, distribution, Abstract
and reproduction in any medium, provided
Mirizzi syndrome (MS) is a rare complication of chronic cholelithiasis. The syndrome describes gallstone
the original author and source are credited.
obstruction of Hartmann’s pouch or the cystic duct that extrinsically compresses the common hepatic duct,
causing obstructive jaundice. In advanced cases, the gallstones may erode into the biliary tree creating a
fistula, requiring prompt diagnosis and careful surgical management. We present a case of an 82-year-old
female who presented with upper abdominal pain and jaundice, later diagnosed with suspected MS type I,
and managed surgically. We aim to highlight MS type I because of the potential progression and damage to
the bile duct, creating complications that may affect overall patient outcome.

Categories: Gastroenterology, General Surgery

Keywords: obstructive jaundice, cholecystectomy, gallbladder, cholelithiasis, mirizzi syndrome

Introduction
The gallbladder is a pear-shaped organ attached to and located on the inferior right lobe of the liver and,
when fully distended, measures roughly 7 x 4 cm. Bile is formed in the liver and stored in the gallbladder.
Hardening bile in the gallbladder leads to the formation of gallstones, also known as cholelithiasis. There are
three methods by which gallstones form: cholesterol supersaturation, excess bilirubin, and gallbladder
hypomotility or impaired contractility. Cholelithiasis is relatively common, found in approximately 6% of
men and 9% of women in the United States [1]. In some instances, gallstones can grow so large that they
become impacted in the gallbladder’s Hartmann’s pouch or cystic duct, causing external compression on the
common hepatic duct (CHD). This condition is known as Mirizzi syndrome (MS), a rare complication of
cholelithiasis.

MS was first described in the early 1900s and later named in 1948 by physician Pablo Luis Mirizzi [2]. MS
describes the impaction of gallstones in the neck of the gallbladder that erodes into the bile duct causing
obstructive symptoms. The incidence has been estimated to fall between 0.7% and 2.9% of all
cholecystectomies [2]. In chronic cholelithiasis, the stones may ulcerate and create a cholecystocholedochal
or cholecystohepatic fistula. Therefore, it is essential to be aware of MS because of the complications that
can arise with bile duct injury. Here, we present a case of an 82-year-old female suffering from unrelievable
epigastric pain and obstructive biliary symptoms who underwent definitive treatment with a
cholecystectomy due to suspected MS type 1.

Case Presentation
An 82-year-old caucasian female presented to the emergency department at a local hospital with upper back
and upper abdominal pain. Her past medical history consisted of diabetes and obesity, both resolved by
gastric bypass surgery eight years prior to her presentation. She had previous epigastric pain that was
relieved by antacids. However, this pain was constant and unremitting. She denied vomiting, fever, and
lower abdominal pain. On examination, she was found to have jaundice with localized right upper quadrant
tenderness and a positive Murphy's sign.

Pertinent labs on admission were increased total bilirubin, direct bilirubin, aspartate aminotransferase
(AST), alanine aminotransferase (ALT), alkaline phosphatase and lipase, and a normal level of WBCs (Table
1).

How to cite this article

Won M N, Collins D P, Bouchard S, et al. (April 02, 2023) Mirizzi Syndrome Type I: A Case Presentation. Cureus 15(4): e37029. DOI
10.7759/cureus.37029
 Value Reference range

Total bilirubin (pre-surgery) 5.38 mg/dL 0.2-1.0 mg/dL

Total bilirubin (post-surgery) 1.76 mg/dL 0.2-1.0 mg/dL

Direct bilirubin 4.64 mg/dL 0.0-0.20 mg/dL

AST 59 U/L 15-37 U/L

ALT 87 U/L 12-78 U/L

Alkaline phosphatase 319 U/L 45-117 U/L

Lipase 462 U/L 73-393 U/L

WBCs 6.5 U/L 4-10.5 U/L

TABLE 1: Patient lab values

AST: aspartate aminotransferase, ALT: alanine aminotransferase

A CT scan of the abdomen and pelvis with contrast showed a distended gallbladder with multiple
noncalcified gallstones. There was no wall edema or pericholecystic fluid and no intrahepatic or
extrahepatic biliary ductal dilation. A magnetic resonance cholangiopancreatography (MRCP) was utilized
and confirmed a distended gallbladder with cholelithiasis and demonstrated no biliary filling defect,
dilation, or strictures (Figure 1). The patient was referred to general surgery for laparoscopic
cholecystectomy.

FIGURE 1: MRCP showing large triangular gallstones contained within

the gallbladder

A markedly enlarged gallbladder was visualized during the surgery, 100 ccs of bilious fluid were drained from
the gallbladder, and the head of the gallbladder was floppy and found to be impacted with large stones. A
critical view of safety was obtained. The cystic duct and artery were clipped and transected, and the
gallbladder was removed using an Endo Catch bag through the 12 mm umbilical port with great difficulty
due to the size of multiple stones. Postoperatively, the gallbladder, measuring 10.2 x 5 x 3.4 cm, was opened

2023 Won et al. Cureus 15(4): e37029. DOI 10.7759/cureus.37029 2 of 5

 and four large, brown, triangular calculi were present, with the largest measuring 3.4 cm in greatest
dimension (Figures 2, 3).

FIGURE 2: Postoperative gallbladder and gallstones

FIGURE 3: Four large gallstones removed from the gallbladder post-

operatively

No polyps or masses were observed. Labs were drawn the following day, and the patient’s total bilirubin had
significantly decreased (Table 1). The patient was discharged after her post-acute care recovery. At her one-
week follow-up, she had no complaints with complete resolution of symptoms, and the histology reports
showed no signs of malignancy.

Discussion

2023 Won et al. Cureus 15(4): e37029. DOI 10.7759/cureus.37029 3 of 5

 Initially, McSherry et al. broadly classified MS into type I and type II [3]. Type I, also considered classic MS,
demonstrates external compression of the common bile duct or CHD, and Type II describes the formation of
a cholecystobiliary fistula. Csendes proposed the currently accepted classification system of MS in 1989,
which further divided type II by the extent of damage and erosion into the common bile duct [4]. Type II
lesions form a cholecystobiliary fistula with erosion into the bile duct less than one-third of its
circumference. In type III lesions, the fistula involves up to two-thirds of the duct circumference, and type IV
lesions show complete destruction of the bile duct.

Common symptoms of biliary obstruction are jaundice, fever, and right upper quadrant pain, but most
patients do not present with all three. MS is often diagnosed intraoperatively and postoperatively due to
similar imaging findings and lab results as other causes of obstructive jaundice, such as cholelithiasis,
choledocholithiasis, or biliary strictures. Additionally, an extrahepatic tumor, such as a Klatskin tumor, may
show similar clinical signs and stricture findings as MS. The surgical management of MS requires an
experienced hepatobiliary surgeon, as the bile ducts may be injured if a cholecystobiliary fistula is present.
Preoperative diagnosis has decreased the risks of open conversion, procedure-related complications, and
reoperation compared to a low preoperative diagnosis [5]. Understanding the clinical signs, symptoms, and
operative findings of MS is crucial to avoiding a diagnostic error.

Preoperative imaging, such as ultrasound, CT, endoscopic retrograde cholangiopancreatography (ERCP), and
MRCP, are often essential to differentiate the cause of the obstructive process [6]. MRCP was utilized in this
patient. ERCP is considered the gold standard for diagnosing MS due to its high sensitivity rate of 76.2% [7].
Furthermore, ERCPs are not only diagnostic but also considered therapeutic because of the ability to
visualize fistula formation and place biliary stents to alleviate obstructive symptoms [7]. An ERCP was not
feasible as the patient had altered anatomy due to a previous gastric bypass. A cholangiogram was
unavailable due to the constraints of a small community hospital setting. However, prior to surgery, biliary
obstruction was ruled out on CT and MRCP.

The case presented is highly suspicious of MS type I due to the large size of multiple gallstones, obstructive
signs and symptoms-elevated direct bilirubin and jaundice, and resolution postoperatively. Additionally, CT
and MRCP revealed patent common hepatic and common bile ducts, ruling out choledocholelithiasis.
Although the patient did not present with an expected dilated CHD, this case demonstrates suspected MS
before it reaches chronic inflammatory compression leading to erosion and fistula formation into the bile
duct.

The mainstay of definitive treatment for MS is surgery. Laparoscopic cholecystectomies are commonly
performed on patients with MS type I, which resolves the biliary duct compression and inflammation.
However, the risks for open conversion and surgical complications are similar for type I and type II [5]. If MS
is discovered intraoperatively, a cholangiogram is needed during surgery to assess the extent of biliary
damage. Commonly, surgeons will find anatomical difficulties during the dissection of Calot's triangle due to
extensive inflammation and large stone compression. In MS types II-IV, surgical procedures, such as
choledochoplasty with gallbladder flap and T-tube placement or a preferred bilioenteric anastomosis for
complex bile duct erosion, are often required [8]. Overall, this further illustrates why preoperative suspicion
and diagnosis are crucial for optimal surgical management and outcome.

Conclusions
MS type I is typically benign due to transient compression of the bile duct, but chronic compression can lead
to erosion and fistula formation. It is imperative to have raised suspicion in patients presenting with
obstructive jaundice and to investigate its causes adequately. Early diagnosis of MS will lead to the best
surgical and post-surgical outcomes.

Additional Information
Disclosures
Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In
compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services
info: All authors have declared that no financial support was received from any organization for the
submitted work. Financial relationships: All authors have declared that they have no financial
relationships at present or within the previous three years with any organizations that might have an
interest in the submitted work. Other relationships: All authors have declared that there are no other
relationships or activities that could appear to have influenced the submitted work.

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