Pleurobiliary Fistula and Refractory Choledocholithiasis in Extrahepatic Portal Vein Obstruction with Portal Cavernoma Cholangiopathy: A Multimodality Odyssey

Portal cavernoma cholangiopathy (PCC), the preferred term for the biliary changes previously labelled “portal biliopathy,” represents the spectrum of biliary strictures and irregularities caused by cavernomatous transformation of the portal vein. Symptomatic PCC poses major technical challenges for endoscopic retrograde cholangiopancreatography (ERCP), especially when associated with common bile duct (CBD) stones and sepsis. We describe a 23-year-old man with long-standing extrahepatic portal vein obstruction and portal cavernoma who presented with fever and right hypochondrial pain. Imaging revealed a solitary hepatic abscess (6.5 cm × 5.0 cm, segments VII–VIII) and a 1.2-cm CBD stone with a short, tight distal CBD stricture on a background of PCC. Culture of the abscess aspirate grew multidrug-resistant Escherichia coli alone, confirming a monomicrobial Gram-negative abscess, whereas a later blood culture yielded Gram-positive cocci, indicating a separate bacteremic episode rather than polymicrobial abscess. Multiple ERCP attempts (including cholangioscopy) were hampered by a sharply angulated “hairpin” distal CBD and dense paracholedochal collaterals; only partial ductal clearance was achieved. A percutaneous catheter drain (PCD) placed into the abscess initially improved sepsis, but cranial migration led to a pleurobiliary/bronchobiliary fistula with bilious cough and right pleural effusion. The PCD was repositioned into the liver under imaging guidance; the pleural collection was thin and nonloculated, and the patient improved without the need for an intercostal drain. Because of ongoing cholestasis and recurrent fever, percutaneous transhepatic biliary drainage (PTBD) was instituted as a salvage measure to decompress the ducts, but percutaneous cholangioscopy-guided lithotripsy was not performed due to slender ducts encased in collaterals, complex fistulous anatomy and lack of local facilities. Despite ERCP, PCD and PTBD, the patient remained symptomatic with persistent obstruction. In the absence of readily available shunt surgery or Transjugular Intrahepatic Portosystemic Shunt, and after multidisciplinary discussion, high-risk open cholecystectomy with choledochotomy, stone extraction and T-tube drainage was undertaken, fully acknowledging the bleeding and bile-duct-injury risk in septic PCC. Postoperative T-tube cholangiography demonstrated a short distal CBD stricture with small residual lower-CBD stones and contrast reflux into the previous abscess cavity. The patient is currently stable on external drainage and is being evaluated for staged portal decompression followed by definitive ductal clearance (advanced endoscopic therapy or surgical hepaticojejunostomy). This case underscores the anatomical and hemodynamic barriers to conventional ERCP in PCC, illustrates a catheter-related pleurobiliary fistula, highlights the role and limitations of PTBD and endoscopic ultrasound-guided biliary drainage in the presence of collaterals, and explains why high-risk cholecystectomy without prior portal decompression may occasionally be required as a damage-control strategy in resource-limited settings.