Endoscopic ultrasound evaluation of portal cavernoma cholangiopathy and endoscopic management of choledochal variceal rupture during ERCP

Portal cavernoma cholangiopathy (PCC) is characterized by pathological alterations in the biliary system in patients with extrahepatic portal vein obstruction and portal cavernoma [1]. Choledocholithiasis, found in 17% of these patients, is attributed to biliary stasis related to PCC [2]. The biliary alterations in PCC consist of reversible components, caused by extrinsic compression and varices formation, and fixed components, due to fibrosis secondary to ischemic-inflammatory damage from chronic portal vein thrombosis and portal cavernoma [3] [4]. This case demonstrates the diagnostic utility of endoscopic ultrasound (EUS) in PCC, the endoscopic treatment of stenosis and choledocholithiasis, and the management of hemobilia resulting from biliary varices rupture ([Video 1]).

Endoscopic ultrasound evaluation of portal cavernoma cholangiopathy with choledocholithiasis, followed by endoscopic retrograde cholangiopancreatography for choledocholithiasis treatment, complicated by massive bleeding, and managed with placement of a self-expandable metal stent.Video 1

An asymptomatic 60-year-old man with noncirrhotic chronic portal vein thrombosis associated with controlled human immunodeficiency virus infection was referred due to alteration of liver function tests. An abdominal ultrasound showed lithiasis of the common bile duct (CBD). EUS confirmed the presence of collateral vessels ([Fig. 1]), gallstones in the CBD ([Fig. 2]), and CBD stenosis consistent with PCC. Endoscopic retrograde cholangiopancreatography (ERCP) was performed for gallstone extraction and stenosis evaluation. Sphincterotomy was uneventful, but balloon sweeping caused significant hemobilia due to CBD varices rupture ([Fig. 3] a), which was successfully managed with placement of a fully covered self-expandable metal stent (FC-SEMS) ([Fig. 3] b).

Fig. 1 Endoscopic ultrasound image showing the main bile duct and cystic duct, with a gallstone within the common bile duct. Collateral vessels surrounding the biliary tract are also discernible. CBD, common bile duct.

Fig. 2 Endoscopic retrograde cholangiopancreatography demonstrated narrowing of the distal common bile duct secondary to portal cavernoma cholangiopathy. CBD, common bile duct.

Fig. 3 Endoscopic images. a Massive bleeding through the biliary tract, caused by rupture of choledochal varices, was observed. b Placement of a self-expandable metal stent resulted in good bile drainage and bleeding control.

Portal vein recanalization associated with transjugular intrahepatic portosystemic shunt was performed to complete PCC treatment. Six months after the CBD varices rupture, the previously placed biliary stent was removed during another ERCP, and biliary duct clearance was confirmed, with no active bleeding ([Fig. 4]). The patient’s condition improved, with preserved liver function and no recurrent bleeding.

Fig. 4 Cholangiography after removal of the self-expandable biliary stent, showing resolution of distal common bile duct stenosis.

This case demonstrates the value of EUS in evaluating PCC and associated choledocholithiasis. It also highlights a rare complication during ERCP and the efficacy of therapeutic interventions employed. Multidisciplinary collaboration among gastroenterologists, hepatologists, and interventional radiologists is crucial for optimizing outcomes in complex PCC cases. These findings contribute to clinical decision making, patient management strategies, and future research in PCC and its associated complications.

Endoscopy_UCTN_Code_CPL_1AK_2AF

Conflict of Interest

A. Poujol-Robert is a consultant for Biotest. R. Leenhardt is cofounder and shareholder of Augmented Endoscopy, and has given a lecture for Abbvie. M. Camus is a consultant for Cook Medical, Ambu, and Medtronic. X. Dray is a founder and shareholder of Augmented Endoscopy, has acted as a consultant for Boston Scientific and Norgine, and has given lectures for Fujifilm, Medtronic, MSD, and Pentax. U. Chaput is a consultant for Boston Scientific. A.P.S.T. Kotinda, A. Payancé, and R. Leenhardt declare that they have no conflict of interest.

• References

• 1 Franceschet I, Zanetto A, Ferrarese A. et al. Therapeutic approaches for portal biliopathy: a systematic review. World J Gastroenterol 2016; 22: 9909-9920
• 2 Chandra R, Kapoor D, Tharakan A. et al. Portal biliopathy. J Gastroenterol Hepatol 2001; 16: 1086-1092
• 3 Puri P. Pathogenesis of portal cavernoma cholangiopathy: is it compression by collaterals or ischemic injury to bile ducts during portal vein thrombosis?. J Clin Exp Hepatol 2014; 4: 27-33
• 4 Elkrief L, Houssel-Debry P, Ackermann O. et al. Portal cavernoma or chronic non cirrhotic extrahepatic portal vein obstruction. Clin Res Hepatol Gastroenterol 2020; 44: 491-496

Publication History

Article published online:
09 January 2024