MIRIZZI SYNDROME, DESCRIPTION, ETIOLOGY, EPIDEMIOLOGY, PATHOPHYSIOLOGY, CLASSIFICATION, HISTOPATHOLOGY, PRESENTATION, DIAGNOSIS, TREATMENT, PROGNOSIS AND COMPLICATIONS

Abstract

Introduction: Mirizzi's syndrome, named after the Argentine surgeon Pablo Luis Mirizzi who first reported the syndrome in 1948, when he showed a history of a patient who presented a calculus of considerable size impacted in the infundibulum of the gallbladder, which generated jaundice due to extrinsic compression of the common bile duct. We speak of Mirizzi syndrome when there is an impaction of a calculus in the infundibulum of the gallbladder or the cystic duct that crushes the common hepatic duct, weakening it and forming a cholecystocholedochal fistula.

Objective: to detail the current information related to Mirizzi syndrome, description, etiology, epidemiology, pathophysiology, classification, histopathology, presentation, diagnosis, treatment, prognosis and complications.

Methodology: a total of 45 articles were analyzed in this review, including review and original articles, as well as clinical cases, of which 33 bibliographies were used because the other articles were not relevant to this study. The sources of information were PubMed, Google Scholar and Cochrane; the terms used to search for information in Spanish, Portuguese and English were: Mirizzi Syndrome, obstruction, common bile duct, gallstone, hepatic duct.

Results: Mirizzi syndrome is relatively infrequent, occurring in only 0.1% of patients with gallstones with findings in 0.7% to 25% of patients who have undergone cholecystectomy. Between 5% to 28% of individuals with Mirizzi syndrome had gallbladder cancer following cholecystectomy. The modification of the anatomy and the cholecystocoledocian fistula increase the risk of damage to the biliary tract in the surgical act of cholecystectomy. Regarding classification type I was present in 40% of cases, type II, type III and type IV in 20% each.

Conclusions: Mirizzi syndrome, also called extrinsic biliary compression syndrome, is an infrequent complication of chronic cholecystitis and cholelithiasis secondary to obliteration of the cystic duct or gallbladder infundibulum (Hartmann's pouch) generated due to the impact of one or more calculi. The impacted stone plus the inflammatory response produces the obstruction of the external biliary tract, with which the mucosa will erode and form a cholecystohepatic or cholecystocoledocian fistula. The risk of presenting gallbladder cancer increases with Mirizzi syndrome. The most common presentation is obstructive jaundice and right upper quadrant pain accompanied by epigastric pain, dark urine, nausea, tachycardia, vomiting, anorexia, fever and chills. The beginning of the diagnosis should be made with the usual tests for cholecystitis.Right upper quadrant abdominal ultrasound is currently the first-line study for the diagnosis of gallstones and acute cholecystitis. If a gallstone in the common bile duct is suspected by ultrasound, magnetic resonance cholangiopancreatography should be performed. This is followed by endoscopic retrograde cholangiopancreatography (ERCP) performed by a trained gastroenterologist. Conventionally the treatment of Mirizzi syndrome is surgical. Cholecystectomy is the first-line treatment; in case of fistula, open cholecystectomy with bilioenteric anastomosis, possibly with a Roux-en-Y is effective. In individuals without fistula, the prognosis is usually positive. In elderly patients with multiple comorbidities and high risk of surgical complications, non-surgical methods should be considered to minimize the morbidity associated with surgery.