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Mirizzi syndrome

Authors
Renuka Umashanker, MD
Douglas Smink, MD, MPH
Section Editors
Sanjiv Chopra, MD, MACP
Stanley W Ashley, MD
Deputy Editors
Shilpa Grover, MD, MPH, AGAF
Wenliang Chen, MD, PhD

INTRODUCTION

Mirizzi syndrome is defined as common hepatic duct obstruction caused by an extrinsic compression from an impacted stone in the cystic duct or Hartmann's pouch of the gallbladder [1-3]. Patients with Mirizzi syndrome can present with jaundice, fever, and right upper quadrant pain. Mirizzi syndrome is often not recognized preoperatively in patients undergoing cholecystectomy and can lead to significant morbidity and biliary injury, particularly with laparoscopic surgery [4].

This topic reviews the epidemiology, clinical manifestations, diagnosis, and management of Mirizzi syndrome. Other complications of gallstone disease including choledocholithiasis, acute cholangitis, and acute cholecystitis are discussed separately. (See "Choledocholithiasis: Clinical manifestations, diagnosis, and management" and "Acute cholangitis" and "Acute cholecystitis: Pathogenesis, clinical features, and diagnosis".)

EPIDEMIOLOGY

Prevalence — Mirizzi syndrome is estimated to occur in 0.05 to 4 percent of patients undergoing surgery for cholelithiasis [5-8]. Approximately 50 to 77 percent of patients with Mirizzi syndrome are women, which may in part be due to a higher incidence of gallstones in women. (See "Approach to the patient with incidental gallstones", section on 'Epidemiology and risk factors'.)

Association with gallbladder cancer — Mirizzi syndrome has been associated with gallbladder cancer [7,9]. It has been hypothesized that recurrent inflammation and biliary stasis may predispose to both conditions. The reported prevalence of gallbladder cancer in patients with Mirizzi syndrome undergoing cholecystectomy ranges from 5 to 28 percent [7,9]. In a retrospective study of 4800 patients who underwent cholecystectomy, Mirizzi syndrome was present in 133 patients, of whom seven (5 percent) had gallbladder cancer [9]. A preoperative diagnosis of gallbladder cancer was made in only one of seven patients. Gallbladder cancer was detected intraoperatively in one patient and only on pathologic examination of the gallbladder in five patients.

PATHOPHYSIOLOGY

The gallbladder consists of the fundus, body, infundibulum, and neck. The body extends from the fundus into the tapered portion, or neck. The neck usually forms a gentle curve, the convexity of which forms the infundibulum, or Hartmann’s pouch. The gallbladder is connected at its neck to the cystic duct which empties into the common bile duct. Large gallstones can become impacted in the cystic duct or in Hartmann's pouch (figure 1). These stones can produce common hepatic duct obstruction by mechanical obstruction of the hepatic duct because of the proximity of the cystic duct and the common hepatic duct, and secondary inflammation with frequent episodes of cholangitis [10-12]. In rare cases, chronic inflammation may result in bile duct wall necrosis and erosion of the anterior or lateral wall of the common bile duct by impacted stones leading to cholecystobiliary (cholecystohepatic or cholecystocholedochal) fistula.

             

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Literature review current through: May 2017. | This topic last updated: Feb 28, 2017.
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