Long-Term Outcomes of Endoscopic Management of Patients with Symptomatic Portal Cavernoma Cholangiopathy with No Shuntable Veins for Surgery or Failed Surgery

Abstract

Background and aim: Shunt surgery is the definitive treatment for symptomatic patients with portal cavernoma cholangiopathy (PCC), but few patients are non-surgical candidates or fail to improve even after surgery. This study aims to analyze the long-term outcomes of endoscopic therapy in these patients.

Methods: Retrospective review of a prospectively maintained database of all patients with symptomatic PCC managed with endoscopic retrograde cholangiography (ERC) followed by stent placement. Outcomes studied included number of biliary interventions, complications, resolution of stricture, development of decompensation and mortality.

Results: Thirty-five patients (68.6% males, median age = 35 years) with a median follow-up duration of 46 months (12-112) were included in the analysis. Presentation was only jaundice in 51.4% cases while one-third (37.1%) of the patients presented with cholangitis. Patients underwent a total of 363 endoscopic sessions with a median of 9 procedures (3-29) per patient. Hemobilia was the most common complication of the procedure (6.06%). Ten (28.5%) patients required frequent stent exchanges. Patients who required frequent stent exchanges had higher number of cholangitis episodes and hospitalization. Secondary biliary cirrhosis developed in 4 (11.4%) patients and 2 (5.7%) patients had mortality. Of the 5 (14.3%) patients who were given a stent free trial, 3 patients required restenting due to redevelopment of symptoms.

Conclusion: Patients with PCC without shuntable veins for surgery or those who failed to improve after surgery can be managed long-term with repeated endoscopic intervention with a slightly increased risk of non-fatal hemobilia.

Keywords: BBS, Benign biliary strictures; CBD, Common bile duct; CSC, Chandra and Sarin classification; CT, computed tomography; EHPVO, Extrahepatic portal vein obstruction; ERC, Endoscopic retrograde cholangiography; ERCP; FCSEMS, Fully covered self-expandable metal stent; IHBR, Intrahepatic biliary radicles; INAS, Indian National Association for Study of Liver; MPS, Multiple plastic stents; MRCP, Magnetic resonance cholangiopancreatography; PCC, Portal cavernoma cholangiopathy; PVT, Portal vein thrombosis; TIPS, Transjugular intrahepatic portosystemic shunt; UDCA, Ursodeoxycholic acid; US, Ultrasound; acute cholangitis; choledochal varices; extrahepatic portal venous obstruction; portal cavernoma cholangiopathy.

Figure 1

Cholangiographic grading of portal cavernoma cholangiopathy (PCC) equivalent to the magnetic resonance cholangiography grading as proposed by Llop et al. A. grade I PCC: irregularities or angulations of the biliary tree, B. grade II PCC: strictures in CHD (small arrow) without dilation, C. grade III PCC: strictures with upstream dilation (large arrow); biliary dilation was considered when the intrahepatic duct was ≥4 mm or when the extrahepatic duct was ≥7 mm.

Figure 3

(A) The presence of stricture in proximal CBD (arrow) with upstream dilatation and non-opacification of distal CBD (B) Multiple plastic stent placement (C) Resolution of stricture (arrow head) after repeated stent exchange with normal drainage of contrast.

Figure 2

Treatment course and outcome of the patients in the study.