OBJECTIVE: There are different therapeutic approaches for biliary strictures and reducing portal hypertension in patients with symptomatic portal cavernoma cholangiopathy (PCC). Endoscopic treatment includes endoscopic biliary sphincterotomy (EST), dilation of stricture with a biliary balloon, placement of plastic stent(s) and stone extraction. Fully covered self-expandable metal stent (FCSEMS) is placed as a rescuer in case of haemobilia seen after EST, dilation of stricture and removal of plastic stent rather than the stricture treatment itself. In this retrospective observational study, we sought to assess the clinical outcomes of FCSEMS as the initial treatment for PCC-related biliary strictures.
METHODS: Twelve symptomatic patients with PCC both clinically and radiologically between July 2009 and February 2019 were examined. Magnetic resonance cholangiopancreatography (MRCP) and cholangiography were employed as the diagnostic imaging methods. Chandra-Sarin classification was used to distinguish between biliary abnormalities in terms of localization. Llop classification was used to group biliary abnormalities associated with PCC. Endoscopic partial sphincterotomy was performed in all the patients. If patients with dominant strictures 6-8-mm balloon dilation was first performed. This was followed by removal of the stones if exist. Finally, FCSEMS placed. The stents were removed 6-12 weeks later.
RESULTS: The mean age of the patients was 40.9 ± 10.3 years, and 91.6% of the patients were male. Majority of the patients (n = 9) were noncirrhotic. Endoscopic retrograde cholangiopancreatography (ERCP) findings showed that 11 of the 12 patients were Chandra Type I and one was Chandra Type IIIa. All the 12 patients were Llop Grade 3. All patients had biliary involvement in the form of strictures. Stent placement was successful in all patients. FCSEMSs were retained for a median period of 45 days (30-60). Seven (58.3%) patients developed acute cholecystitis. There was no occurrence of bleeding or other complications associated with FCSEMS replacement or removal. All patients were asymptomatic during median 3 years (1-10) follow up period.
CONCLUSIONS: FCSEMS placement is an effective method in biliary strictures in case of PCC. Acute cholecystitis is encountered frequently after FCSEMS, but majority of patients respond to the medical treatment. Patients should be followed in terms of the relapse of biliary strictures.

BACKGROUND: Portal hypertensive biliopathy (PHB) was caused by anatomical and functional abnormalities in the intrahepatic and extrahepatic bile ducts secondary to portal hypertension. Currently, there is no consensus regarding to the optimal treatment for PHB. Transjugular intrahepatic portosystemic shunt (TIPS) is the treatment choice for the management of symptomatic PHB, however, it could be very difficult in patients with PHB and cavernous transformation of portal vein.
METHODS: We report a case of PHB, successfully managed with TIPS. A 23-year-old man with liver cirrhosis presented with jaundice. Magnetic resonance cholangiopancreatography (MRCP) showed multiple tortuous hepatopetal collateral vessels compressing the common bile duct (CBD) and leading to the dilated proximal bile duct. He was diagnosed with PHB and treated with TIPS. A guidewire was inserted into the appropriate collateral vessel through transsplenic approach to guide intrahepatic puncture and TIPS was performed successfully. After the operation, portal vein pressure decreased and the symptoms of biliary obstruction were relieved significantly. In addition, the patient showed no jaundice at a follow-up of one year.
CONCLUSIONS: For PHB patients presenting for cavernous transformation of the portal vein, which precludes the technical feasibility of TIPS, a combined transjugular/transsplenic approach could be an alternative option.

暂无摘要。

Portal biliopathy (PB) refers to the biliary abnormalities of the biliary ducts observed in patients with extrahepatic portal hypertension. Although majority of patients are asymptomatic, approximately 20% of these patients present with biliary symptoms (pain, pruritus, jaundice, cholangitis). The pathogenesis of PB is uncertain but compression by dilated veins into or around common bile duct may play the main role. CT-scan, MR cholangiopancreatography with MR portography should be the initial investigations in the evaluation of PB. Treatment is limited to symptomatic cases and is dictated by clinical manifestations and complications of the disease. Treatment of PB could be done by endoscopy (sphincterotomy, stone extraction or biliary stenting of the common bile duct) or surgery (definitive decompression by porto-systemic shunt followed by bilioenteric anastomosis, if necessary). This review describes pathogenesis, clinical features, investigation and management of portal biliopathy.

OBJECTIVE: The purpose was to investigate magnetic resonance imaging (MRI) features of biliary collateral veins and associated biliary abnormalities of portal hypertensive biliopathy (PHB).
METHODS: Thirty-six patients including 18 patients with abnormal biliary changes and 18 patients as control group were involved in this study. MRI features of biliary collateral veins were analyzed.
RESULTS: Stenosis with dilated proximal bile ducts occurred in 33.3% of patients, 27.8% of patients had irregular ductal walls, 22.2% of patients had thickened ductal walls, 16.7% of patients had angulated ductal walls, and 44.4% of patients had thickened gallbladder walls.
CONCLUSIONS: Biliary collateral veins and associated biliary abnormalities of PHB can be detected by MRI.

Portal cavernoma cholangiopathy (PCC) is the presence of typical cholangiographic changes in patients with a portal cavernoma due to chronic portal vein thrombosis, in the absence of other biliary tract diseases. Probably due to biliary stasis related to the cavernoma, there is a high incidence of biliary sludge and calculi in PCC, which trigger symptoms that resolve with appropriate interventions. Persistent and troublesome symptoms are usually due to biliary stenoses or strictures, which may occur with or without biliary calculi and may be short or long, solitary or multifocal, extrahepatic or intrahepatic. Experience with endoscopic interventions in PCC over the last twenty years has shown that it is the procedure of choice for bile duct calculi. Plastic stenting with repeated, timely, stent exchanges is the first line intervention for jaundice or cholangitis due to biliary strictures. If biliary obstruction does not resolve, portosystemic shunt surgery (PSS) or transjugular intrahepatic portosystemic stent shunt (TIPS) is performed to decompress the portal cavernoma. However, for patients with non-shuntable veins or blocked shunts, repeated plastic stent exchanges are the only option though there are reports of the use of biliary self-expandable metal stents in this situation. If symptomatic biliary obstruction persists after successful PSS or TIPS, second stage biliary surgery may be necessary. Recent experience suggests that treating biliary strictures in PCC on the lines of postoperative benign biliary strictures with balloon dilatation and repeated exchanges of plastic stent bundles may be effective therapy. Endoscopic management appears to be associated with an increased frequency of hemobilia, which usually responds to standard management. Recurrent cholangitis with formation of sludge and concretions may be a problem with repeated stent exchanges, especially if patient compliance is poor. In conclusion, the current understanding is that symptomatic PCC is best managed jointly by the endoscopist and surgeon with sequential interventions designed initially to establish and maintain biliary drainage, then to decompress the portal cavernoma and, finally, if required, second stage biliary surgery or endotherapy for biliary strictures. Endoscopic therapy occupies a central role in management before, during and after surgical therapy. Paradigms of endoscopic therapy continue to evolve as knowledge of pathogenesis and natural history improves and newer approaches and techniques are applied.

Portal cavernoma cholangiopathy (PCC) is defined as abnormalities in the extrahepatic biliary system including the cystic duct and gallbladder with or without abnormalities in the 1st and 2nd generation biliary ducts in a patient with portal cavernoma. Presence of a portal cavernoma, typical cholangiographic changes on endoscopic or magnetic resonance cholangiography and the absence of other causes of these biliary changes like bile duct injury, primary sclerosing cholangitis, cholangiocarcinoma etc are mandatory to arrive a diagnosis. Compression by porto-portal collateral veins involving the paracholedochal and epicholedochal venous plexuses and cholecystic veins and ischemic insult due to deficient portal blood supply or prolonged compression by collaterals bring about biliary changes. While the former are reversible after porto-systemic shunt surgery, the latter are not. Majority of the patients with PCC are asymptomatic and approximately 21% are symptomatic. Symptoms in PCC could be in the form of long standing jaundice due to chronic cholestasis, or biliary pain with or without cholangitis due to biliary stones. Endoscopic retrograde cholangiography has no diagnostic role because it is invasive and is associated with risk of complications, hence it is reserved for therapeutic procedures. Magnetic resonance cholangiography and portovenography is a noninvasive and comprehensive imaging technique, and is the modality of choice for mapping of the biliary and vascular abnormalities in these patients. PCC is a progressive condition and symptoms develop late in the course of portal hypertension only in patients with severe or advanced changes of cholangiopathy. Asymptomatic patients with PCC do not require any treatment. Treatment of symptomatic PCC can be approached in a phased manner, coping first with biliary clearance by nasobiliary or biliary stent placement for acute cholangitis and endoscopic biliary sphincterotomy for biliary stone removal; second, with portal decompression by creating portosystemic shunt; and third, with persistent biliary obstruction by performing second-stage biliary drainage surgery such as hepaticojejunostomy or choledochoduodenostomy. Patients with symptomatic PCC have good prognosis after successful endoscopic biliary drainage and after successful shunt surgery.