portal biliopathy is defined as the development of extensive venous collateral vessels , due to obstruction of the extrahepatic portal vein , causing impediment to portal venous drainage .
it develops from morphological changes of dilatation and stenosis of intrahepatic , extrahepatic and pancreatic ducts .
it is also designated as cholangiopathy associated with portal hypertension , or portal cavernoma associated cholangiopathy .
majority of patients are asymptomatic , but rarely some experience symptoms such as abdominal pain , jaundice , and fever .
magnetic resonance cholangiopancreatography ( mrcp ) and endoscopic retrograde cholangiopancreatography ( ercp ) are considered to be the diagnostic tools of choice , once symptoms are manifested [ 1 - 3 ] .
proper treatment is required to prevent complication such as biliary sepsis in cases of symptomatic portal biliopathy .
there are few reports on radiologic findings of portal biliopathy in korea , however , a symptomatic case was not previously reported .
a 56-year - old man visited the emergency room with fever , chill , and abdominal pain .
the past medical history indicated that he was diagnosed with hepatitis - related liver cirrhosis and was started on an anti - viral agent since 15 years ago .
he was diagnosed with a small hepatocellular carcinoma in s2 of the liver 8 years ago and performed trans - catheter arterial chemoembolization twice .
he had been suffered from chronic portal veous thrombosis in main portal vein and portal hypertension .
he had a history of embolization of a splenic arterial pseudoaneurysm with severe splenomegaly and pancytopenia 5 years ago .
he had multiple hospital admissions for esophageal and gastric variceal bleeding , hepatic encephalopathy and spontaneous bacterial peritonitis .
on presentation at our emergency room , he was in a febrile state and looked acutely ill with icteric sclera and abdominal tenderness and palpable abdominal mass on the left upper quadrant were found on physical examination .
the vital signs on initial presentation were as follows : blood pressure of 104/66 mmhg , heart rate of 79 beats per minute , and body temperature of 38.2. the initial blood test showed hemoglobin of 10.2 g / dl , white blood cell count of 3,240/l , platelet count of 20,000/l , prothrombin time international normalized ratio of 1.95 , albumin of 2.7 g / dl , total bilirubin of 29.4 mg / dl , direct bilirubin of 20.1 mg / dl , aspartate aminotransferase of 58 iu / l , alanine aminotransferase of 32 iu / l , alkaline phosphatase of 229 iu / l , c reactive protein of 15.2 mg / dl , and creatinine of 1.5 mg / dl .
hepatitis b e ( hbe ) antigen , anti - hbe antibody and hbv dna were not detected .
alpha - fetoprotein was 2.3 ng / ml . a plain radiograph of the chest showed no active lung lesion .
abdominal ct scan showed chronic thrombosis in the main portal vein , cavernous transformation , extensive splenic varix , large gall stones and pericholecystic infiltration of the gallbladder ( fig .
additionally , cirrhotic change of the liver , mild ascites and mild bile duct dilatation were seen ( fig .
irregular filling defect in the cbd on mrcp by cavernous transformation of the portal vein indicated portal bilopathy ( fig .
therapeutic endoscopic sphincterostomy and drainage were performed to widen the obstruction of biliary tract caused by compression .
more specifically , a double pigtail stent of 7 fr . in diameter and 10 cm in length was inserted through endoscopic retrograde biliary drainage ( erbd ) ( fig .
3b ) . fever , jaundice and abdominal pain subsided on the 2nd day after erbd .
there was a significant decrease in total bilirubin level from 29.4 to 8.9 mg / dl , 7 days after erbd .
4 ) . there were no symptoms including fever , chill , jaundice , abdominal pain during the follow up .
total bilirubin ranged from 1.5 to 2.0 mg / dl for 6 months until stent removal .
however , esophageal variceal bleeding and hepatic encephalopathy were developed during the 6 months follow - up period .
furthermore , gastrointestinal bleeding from gastric antral vascular ectasia was treated with argon plasma coagulation under hospitalization .
portal biliopathy is defined as the development of extensive venous collateral vessels , due to the obstruction of extra- and intrahepatic ducts , causing impediment to portal venous drainage .
the underlying pathogenesis of the disease starts with the compression of venous collaterals on the bile duct and prolonged portal vein thrombosis .
this induces sclerosis on veins around the bile duct leading to a series of events explained by the ischemic and infective theory .
approximately 5 - 17% of cases advance to the symptomatic stage , experiencing abdominal pain , jaundice , recurrent fever , and chilling [ 1 - 3 ] .
patients with such symptoms are known to be comorbid with cholethiasis approximately upto 84 % .
the production of the stone is explained by cholestasis due to increased fibrosis and decreased motility of the biliary tract in portal biliopathy .
increase in serum bilirubin , alkaline phosphate and transaminase is caused by insufficient biliary drainage of portal biliopathy .
it exhibits increased portal vein diameter , hyper - echogenicity on tissue of the hilum and distended paracholedochal veins .
however , it was difficult to explore on the cbd using this modality , especially when there was no extrinsic compression by enlarged paracholedochal veins .
abdominal ct has the advantage of visualizing collateral circulation , enabling investigation on cavernous formation of the portal vein and the compressive effect of collateral circulation on the bile duct and gallbladder varices .
recently , mrcp is more often used and has become the choice non - invasive diagnostic modality for evaluating the biliary tract ; it not only shows the nature and changes of intra- and extra - hepatic bile duct over time , but is also useful to rule out malignancy .
ercp is recommended as an invasive diagnostic tool , since it can show the irregularity , length and degree of narrowed bile duct , segmental upstream dilatation , ductal displacement , calculi , pruning of intrahepatic ducts , and the filling defects in cbd .
it is also useful for differential diagnosis of recurrent pyogenic cholangitis , cbd stones with stricture , and biliary ascariasis [ 8 - 10 ] .
some studies have suggested that it is advantageous for the differential diagnoses of diseases including bile duct stone , pericholedochal varices and tumor .
endoscopic ultrasound is used for the diagnosis of portal biliopathy , but it is not recommended as a routine exam .
he suffered from variceal bleeding due to portal hypertension but had no biliary symptoms before admission .
symptoms including recurrent fever , chill , jaundice , and abdominal pain developed over time .
ct revealed typical development of multiple collateral vessels , gallstone and portal cavernoma presumably caused by portal vein thrombosis , however cbd stone was not observed .
mrcp was followed immediately and showed typical filling defects on the distal bile duct due to compression from extrinsic cavernomatous transformation without cbd stone .
mrcp is a non - invasive diagnostic modality for detection of small biliary stone with high sensitivity and specificity ( > 90% ) .
asymptomatic portal biliopathy does not require treatment . on the other hand , treatment to reduce symptoms and to prevent complication such as cholecystitis , cholangitis , chronic obstructive jaundice , biliary sepsis and biliary cirrhosis ,
endoscopic treatment should be considered as an initial therapeutic modality when there are symptoms caused by biliary stricture or obstruction , since it effectively alleviates symptoms of biliary obstruction .
endoscopic treatment also has some disadvantages ; it requires regular follow - up exams for monitoring stent migration and for a replacement of stent when occluded .
a case study on endoscopic stent indicated that patients with prolonged indwelling stent suffered from recurrent cholangitis .
though endoscopic treatment is the first choice for portal biliopathy , a favorable outcome may not always be achieved .
transjugular intrahepatic portosystemic shunt ( tips ) could be considered when symptoms persist even after endoscopic biliary decompression .
biliary intestinal bypass is the next treatment choice , when tips fails to significantly reduce symptoms .
surgical intervention should be considered in patients if the symptoms from the stricture or obstruction of bile duct continue , or when regular follow - up exams is expected to be difficult for a patient . in this case , endoscopic treatment was considered as the initial therapeutic modality since our patient experienced symptoms of biliary obstruction .
the subject successfully received balloon catheter dilation , endoscopic papillotomy and stent insertion and was discharged from the hospital .
there was no complication from the procedure itself , such as recurrent cholangitis or stent obstruction .
the stent was removed 6 months after the erbd insertion . despite successful endoscopic treatment on portal biliopathy
, complications from portal hypertension including hepatic encephalopathy , and variceal bleeding and gave bleeding could not be avoided in the patient .
complications of portal hypertension or cirrhosis should be carefully monitored and promptly managed during the follow - up in portal biliopathy patients . in conclusion , when a patient with portal hypertension and prominent extrahepatic collaterals experiences jaundice , fever , and abdominal pain , portal biliopathy should be considered in the differential diagnosis .
once portal biliopathy is confirmed , the treatment plan should be established according to each patient s clinical symptoms and disease status .
however , this treatment is limited since it does not radically resolve primary portal hypertension and complications of hepatic decompensation .
 portal biliopathy is defined as abnormalities in the extra- and intrahepatic ducts and gallbladder of patients with portal hypertension . 
 this condition is associated with extrahepatic venous obstruction and dilatation of the venous plexus of the common bile duct , resulting in mural irregularities and compression of the biliary tree . 
 most patients with portal biliopathy remain asymptomatic , but approximately 10% of them advance to symptomatic abdominal pain , jaundice , and fever . 
 magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography are currently used as diagnostic tools because they are noninvasive and can be used to assess the regularity , length , and degree of bile duct narrowing . 
 management of portal biliopathy is aimed at biliary decompression and reducing the portal pressure . 
 portal biliopathy has rarely been reported in korea . 
 we present a symptomatic case of portal biliopathy that was complicated by cholangitis and successfully treated with biliary endoscopic procedures . 
INTRODUCTION
CASE REPORT
DISCUSSION