S1676 The Great Escape: The Rare Type V Mirizzi Syndrome

Abstract

Introduction: Mirizzi syndrome is a rare complication of chronic gallstone disease, defined as the obstruction of the common bile duct (CBD) or common hepatic duct (CHD) secondary to compression from gallstone impaction Hartmann’s pouch. It is categorized based on the presence and degree of cholecystobiliary fistula formation; however resultant cholocystoenteric fistulas are rare. We discuss the case of a patient who presented to the outpatient clinic with evidence of painless jaundice and on further evaluation, was found to have type Vb Mirizzi syndrome. Case Description/Methods: A 44-year-old male currently on suboxone for previous opioid use disorder presented for evaluation of painless jaundice. He denied fevers, chills, abdominal pain, nausea, or vomiting. Laboratory investigations noted AST 65 IU/L, ALT 46 IU/L, ALP 347 IU/L, total bilirubin 16.9 mg/dL (direct bilirubin 13 mg/dL), albumin 3.4 g/dL, INR 1.1, negative viral serologies and acetaminophen level < 5 mcg/mL. CT abdomen with contrast showed pneumobilia, smooth hepatic contour, and splenomegaly [Figure 1A]. Endoscopic retrograde cholangiopancreatography (ERCP) showed spontaneous air in the biliary tree with bile emanating from a fistulous tract in the duodenal bulb [Figure 1B]. Contrast dye injection filled up the gallbladder thus confirming the presence of cholecystoduodenal fistula. Cholangiogram demonstrated an obstruction in the mid-CBD caused by a 22 mm stone in the gallbladder, protruding into the bile duct [Figure 1C]. The bile duct was stented and the patient underwent open cholecystectomy with CBD stone removal and repair, along with cholecsytoduodenal fistula disconnection and closure. Discussion: Mirizzi syndrome is uncommon with an incidence of < 1% per year. While previously classified as type I (extrinsic compression of the CHD or CBD without a cholecystobiliary fistula) or type II-IV (including a cholecystobiliary fistula), more recently, an extended classification has introduced type V Mirizzi syndrome encompassing types I-IV with the concomitant identification of a cholecystoenteric fistula. It is further subclassified as type Va and Vb (i.e., without and with gallstone ileus, respectively). Given that it encompasses the other subtypes, the treatment option primarily depends on the obstructing biliary lesion type. It is crucial to carefully evaluate and correctly identify patients' chronic gallstone disease complications as they can affect further endoscopic and surgical management.Figure 1.: Figure 1A: CT abdomen with contrast showing pneumobilia (red arrows); Figure 1B: Fistulous tract noted in the duodenal bulb on direct visualization (yellow arrow); Figure 1C: Cholangiogram demonstrating an obstruction in the mid common bile duct consistent with a large, approximately 22 mm bile duct stone in the gallbladder, protruding into the bile duct (red circle).