Cholangioscopic Lithotripsy by Using Self-Expanding Metal Stent for Intrahepatic Stones in Portal Cavernoma Cholangiopathy After Failed Biliary Bypass

CASE REPORT Extrahepatic portal venous obstruction is the second most common cause of portal hypertension globally, often (80%–100%) leading to portal cavernoma cholangiopathy (PCC). Though frequently asymptomatic, prolonged PCC can cause obstructive jaundice. Definitive treatment involves endoscopic retrograde cholangiopancreatography (ERCP), followed by shunt surgery and hepaticojejunostomy. In the absence of shuntable vein, long-term biliary stenting is required. A 28-year-old man presented with obstructive jaundice and was diagnosed with PCC complicated by bile duct and intrahepatic stones, as seen on magnetic resonance cholangiopancreatography (Figure 1, asterisks: stones). Initial management included ERCP with placement of a 4 cm fully covered self-expanding metal stent (FCSEMS) (Figure 1: endoscopic ultrasound showing portal biliopathy and stent in situ). However, the patient experienced recurrent cholangitis and cholecystitis requiring percutaneous cholecystostomy and multiple plastic stent placements (Figure 2). Subsequent surgical exploration revealed a small renal vein (7 mm), preventing shunt surgery, and torrential bleeding during attempted hepaticojejunostomy led to abandonment of the procedure after cholecystectomy.Figure 1.: Magnetic resonance cholangiopancreatography (MRCP) and endoscopic ultasound (EUS) findings: (A) MRCP showing common bile duct and right posterior hepatic duct stones (asterisks: stones), (B) left hepatic duct stones on MRCP, (C) righr posterior hepatic duct stones, (D) EUS showing pericholedochal collaterals and stent in situ.Figure 2.: Interventions after placement of 4 cm fully covered self-expanding metal stent (FCSEMS). (A) Plastic stent and nasobiliary tube placed through FCSEMS, (B) plastic stent and nasobiliary tube in both biliary systems, (C) percutaneous cholecystostomy placed, (D) three biliary stents placed.Given the failure of surgical intervention, a 6 cm FCSEMS was placed following per-oral cholangioscopy (POC)-guided electrohydraulic lithotripsy (EHL). Despite this, the patient experienced persistent cholangitis necessitating 2 sessions of POC-guided EHL to clear intrahepatic and impacted stones (Figure 3: impacted stone over SEMS, 3B: hilar stones, 3C: cleared stones, 3D: cholangiogram postclearance).Figure 3.: Cholangioscopic lithotripsy for intrahepatic and bile duct stones, (A) lithotripsy being done for stone just above the fully coverd metal stent, (B) lithotripsy for hilar stones, (C) post lithotripsy cholangioscopic picture, (D) post lithotripsy cholangiogram showing cleared bile duct.Two months later, the patient developed another episode of cholangitis due to a partially migrated FCSEMS. A third session of EHL was performed, leading to complete stone clearance (Figure 4). During this session, an intraductal varix was identified (Figure 4: asterisk). Finally, the 6 cm FCSEMS was replaced with an anchoring plastic stent to address the persistent distal common bile duct stricture (Figure 4). Subsequently, another surgical attempt was done for permanent biliary bypass, which also failed due to extensive collaterals around bile duct. At 4 months of follow-up, the patient presented with intermittent cholangitis, not requiring antibiotics or admission. Ultrasound showed pneumobilia and on ERCP, earlier FCSEMS was removed. Cholangiogram showed persistent mid-common bile duct stricture for which 6 cm FCSEMS was replaced along with an anchoring plastic stent. The long-term treatment plan is to exchange the FCSEMS with multiple plastic stents/FCSEMS every 6-12 months with or without cholangioscopic lithotripsy (if recurrent stone) until biliary stricture resolves.Figure 4.: Cholangioscopy during 3rd session of electrohydraulic lithotripsy (EHL). (A) EHL being done, (B) post EHL cleared biliary system, (C) intraductal varix (asterisk) on cholangioscopy, (D) fully covered metal stent with anchoring plastic stent.This case underscores the efficacy of POC-guided EHL through FCSEMS for managing complex intrahepatic stones and strictures in PCC, especially when conventional surgical options repeatedly fail. The advanced endoscopic approach provided complete stone clearance and restored biliary patency (Video 1). {"href":"Single Video Player","role":"media-player-id","content-type":"play-in-place","position":"float","orientation":"portrait","label":"Video 1","caption":"Per oral cholangioscopic lithotripsy through self-expanding metal stent for intrahepatic stones in portal cavernoma cholangiopathy.","object-id":[{"pub-id-type":"doi","id":""},{"pub-id-type":"other","content-type":"media-stream-id","id":"1_7a6wds8o"},{"pub-id-type":"other","content-type":"media-source","id":"Kaltura"}]} DISCLOSURES Author contributions: P. Pal: Conceptualization, formal analysis, investigation, visualization, writing original draft and revision; A. Kaipa: Data curation, investigation, methodology, writing review and editing; R. Gupta and M. Tandan: Supervision, writing review and editing; DN Reddy: conceptualization, supervision, writing review and editing. P. Pal is the article guarantor. Financial disclosure: None to report. Informed consent was obtained for this case report.