Mirizzi syndrome

Mirizzi syndrome is an uncommon hepatobiliary disorder caused by extrinsic compression of the common hepatic duct (CHD) or common bile duct (CBD) by an impacted gallstone, typically lodged in the cystic duct or Hartmann’s pouch. This mechanical obstruction can progress to inflammatory erosion and fistula formation between the gallbladder and the bile duct or adjacent structures.

Clinically, patients often present with right upper quadrant pain, jaundice, fever, and signs consistent with acute or chronic cholecystitis. Diagnostic confusion with choledocholithiasis or cholangiocarcinoma is common, and preoperative recognition remains low, often leading to significant surgical morbidity.

Mirizzi syndrome is classically associated with longstanding gallstone disease, affecting approximately 0.1% of gallstone patients and seen in up to 1% of cholecystectomy specimens.

Current Classification (Csendes Modified)

The Csendes classification is the most widely used and provides a stratified system based on the extent of bile duct involvement:

• Type I: External compression of the CHD by a stone in the gallbladder neck or cystic duct.
  • Ia: Cystic duct present
  • Ib: Cystic duct obliterated
• Type II: Fistula formation eroding <1/3 of the CHD wall.
• Type III: Fistula involving up to 2/3 of the CHD wall.
• Type IV: Complete erosion of the CHD wall.
• Type V: Any of the above + cholecystoenteric or cholecystocolonic fistula.
  • Va: Without gallstone ileus
  • Vb: With gallstone ileus.

Complications and Associations

A significant subset of patients may develop biliary-enteric fistulae, most commonly cholecystoduodenal. In Type Vb, these fistulae can lead to gallstone ileus, representing a complex and life-threatening complication.

Other complications include:

• Recurrent cholangitis
• Biliary cirrhosis
• Iatrogenic bile duct injury (due to misidentification intraoperatively)

Diagnosis and Imaging

The condition is often missed preoperatively. Modalities such as MRCP, ERCP, and CT cholangiography are pivotal for pre-surgical identification:

• ERCP may reveal a characteristic smooth, right-concave stricture.
• MRCP can show a large stone in Hartmann’s pouch and intrahepatic biliary dilatation with tapering at the site of compression.

Summary Points:

• Rare complication of chronic cholelithiasis
• Results from external compression or erosion of bile duct
• Requires high index of suspicion in patients with obstructive jaundice and long-standing gallstones
• Pre-op diagnosis essential to avoid bile duct injury
• Multiple classification systems, with Csendes system most commonly used
• Surgical treatment varies with type — ranging from cholecystectomy to biliary reconstruction

---

History

1890 – Ludwig Courvoisier (1843-1918) in his analysis of biliary tract diseases, describes the topography and variants of the extrahepatic biliary tree, including anomalies.

1905 – Hans Kehr (1862-1916) publishes Die in meiner Klinik geübte Technik der Gallensteinoperationen, describing in detail the consequences of impacted gallstones in the cystic duct and infundibulum, including chronic inflammation, adhesions, and fistula formation. No direct mention of a hepatocystic duct compression syndrome, but Kehr is often cited (e.g., in Ruge 1908) for his detailed anatomical observations and operative cholangiographic findings relevant to the eventual Mirizzi construct.

1908 – Ernst Rüge (1878–1953) publishes Beitrage zur chirurgischen Anatomie der grossen Gallenwege with highly detailed illustrations of the surgical anatomy of the extrahepatic biliary tract. Though not clinical in focus, he describes variants in bile duct anatomy and their relation to lithiasis but does not propose a distinct syndrome. His work influences future interpretations of ductal compression and variants as pathophysiological entities.

1932 – Pablo Luis Mirizzi (1893-1964) was the first make a practical application for operative cholangiography. He publishes Cholangiografi durante las operaciones de las vias biliares and begins exploring the functional sphincteric mechanisms of the hepatic duct system. He hypothesizes a physiological sphincteric zone in the hepatic duct that regulates bile flow and protects against retrograde infection.

1940 – Mirizzi introduces the concept of a “physiologic sphincter” in the common hepatic duct and discusses the presence of muscular fibres in a spiral or annular pattern. He begins to frame a functional model of biliary regulation with protective implications in disease.

1942 – Mirizzi publishes El sistema del hepático: Su fisiopatología and expands the functional-anatomical model. He suggests the hepatic duct’s contractile properties form a “hepatocystic sphincter”.

1948 – Review in J. Internat. de Chir. (translated abstract):

• Discusses the impaired function of this sphincter during stasis and inflammation (e.g., cholangitis), leading to the so-called “hepatic syndrome”.
• Indicates that bile duct dilation and spasm are central to this process.
• Reflects mounting evidence for a distinct clinical entity, still functional in emphasis, not yet anatomical obstruction by stone.

1949 – Síndrome del Conducto Hepático, Cirugía y Cirujanos:

• This is the first comprehensive clinical-anatomical definition of what becomes known as Mirizzi Syndrome.
• Describes stone impaction in the cystic duct or Hartmann’s pouch causing compression of the common hepatic duct, leading to obstructive jaundice without choledocholithiasis.
• Emphasizes radiographic findings and cholangiographic diagnosis.
• Also outlines the protective role of the hepatic duct sphincter and the clinical-pathological picture

1940 – Pablo Mirizzi

• Mirizzi first described the syndrome in Spanish-language literature as “Síndrome del conducto hepático”, attributing jaundice to a functional spasm of the hepatic duct due to gallstone impaction. He incorrectly proposed the presence of a sphincter in the common hepatic duct.

1948 – Expanded Description

• Mirizzi published further clinical observations on bile duct obstruction due to gallstones and inflammation, consolidating the concept, although his proposed mechanism (spasm) was later refuted.

1949 – Original English Translation

• An English-language summary by Mirizzi in Annals of Surgery brought broader international attention to the syndrome.

1961 – Additional Cases by Mirizzi

• He published seven more cases from a cohort of 4,000 cholelithiasis patients, adding to his earlier findings and reinforcing the clinical pattern.

1982 – McSherry Classification

• McSherry et al. proposed the first structured classification:
  • Type I: External compression of the common hepatic duct.
  • Type II: Presence of a cholecystobiliary fistula.

1989 – Csendes Modification

• Csendes expanded this into a 4-type system based on the extent of bile duct erosion:
  • Type I: External compression without fistula
  • Type II: Fistula involving <33% of bile duct
  • Type III: Fistula involving 33–66%
  • Type IV: Fistula involving >66% or complete destruction
  • His study involved 219 patients from a surgical cohort of over 17,000.

2002 – Abou-Saif and Al-Kawas Review

• The American Journal of Gastroenterology review re-emphasized the two-stage pathogenesis: (1) extrinsic compression, (2) fistula formation. Also highlighted that cholecystocholedochal fistulas and Mirizzi syndrome lie on a spectrum.

2005–2008 – Beltrán Modifications

• Beltrán et al. proposed further classification refinements, including:
  • Type V: Mirizzi syndrome with associated cholecystoenteric fistula and gallstone ileus.

2019–2024 – Contemporary Reviews

• Emphasize imaging modalities (US, CT, MRCP, ERCP), the importance of preoperative diagnosis to avoid bile duct injury, and the relevance of laparoscopic vs open surgical approaches. Gallstone ileus is now clearly defined as a rare but related complication due to chronic fistulization.

---

Associated Persons

• Ludwig Courvoisier (1843-1918)
• Hans Kehr (1862-1916)
• Ernst Ludwig Richard Ruge (1878–1953)
• Pablo Luis Mirizzi (1893-1964)

---

References

Original articles

• Kehr H. Die in meiner Klinik geübte Technik der Gallensteinoperationen mit einem Hinweis auf die Indikationen und die Dauererfolge. 1905
• Ruge E. Beitrage zur chirurgischen Anatomie der grossen Gallenwege (Ductus hepaticus, choledochus und pancreaticus) Archiv für Klinische Chirurgie. 1908; 77: 47-77
• Mirizzi PL. Physiologic sphincter of hepatic bile duct. Archives of Surgery 1940; 41: 1325-1333.
• Mirizzi PL. Sindrome del conducto hepatico. Journal international de chirurgie. 1948; 8: 731–777.
• Mirizzi PL. Síndrome de Conducto Hepático. (Sindrome hepaticiano). Cirugía y Cirujanos 1949; 17(3): 111-133

Review articles

• Pemberton M, Wells AD. The Mirizzi syndrome. Postgrad Med J. 1997 Aug;73(862):487-90
• Abou-Saif A, Al-Kawas FH. Complications of gallstone disease: Mirizzi syndrome, cholecystocholedochal fistula, and gallstone ileus. Am J Gastroenterol. 2002 Feb;97(2):249-54.
• Becker CD, Hassler H, Terrier F. Preoperative diagnosis of the Mirizzi syndrome: limitations of sonography and computed tomography. AJR Am J Roentgenol. 1984 Sep;143(3):591-6.
• Lai EC, Lau WY. Mirizzi syndrome: history, present and future development. ANZ J Surg. 2006 Apr;76(4):251-7.
• Payá-Llorente C, Vázquez-Tarragón A, Alberola-Soler A, Martínez-Pérez A, Martínez-López E, Santarrufina-Martínez S, Ortiz-Tarín I, Armañanzas-Villena E. Mirizzi syndrome: a new insight provided by a novel classification. Ann Hepatobiliary Pancreat Surg. 2017 May;21(2):67-75.