Supported by Dr. Portal biliopathy refers to cholangiographic abnormalities which occur in patients with portal cavernoma. These changes occur as a result of pressure on bile ducts from bridging tortuous paracholedochal, epicholedochal and cholecystic veins. Bile duct ischemia may occur due prolonged venous pressure effect or result from insufficient blood supply. In addition, encasement of ducts may occur due fibrotic cavernoma.
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Portal biliopathy PB is a rare disorder, characterized by biliary ductal and gallbladder wall abnormalities seen in patients with portal hypertension. The abnormalities consist mainly of bile duct compression, stenoses, fibrotic strictures and dilation of both extrahepatic and intrahepatic bile ducts, as well as gallbladder varices.
PB may mimic cholangiocarcinoma, sclerosing cholangitis, or choledocholithiasis. Misdiagnosis can be avoided using appropriate imaging modalities to prevent complications. Portal biliopathy PB is defined as abnormalities of the extrahepatic and intrahepatic bile ducts, gallbladder and cystic duct, observed in patients with portal hypertension [ 1 , 2 ].
Although jaundice and common bile duct CBD compression, associated with portal hypertension, was described by Fraser et al. After a portal vein thrombosis, collaterals develop to bypass the obstruction, resulting in portal cavernoma. The peribiliary collateral vessels cause extrinsic compression, lead to reduction of the diameter of the intrahepatic and extrahepatic bile ducts. There are also underlying inflammatory changes, resulting in peribiliary fibrosis [ 4 ].
PB may mimic cholangiocarcinoma, sclerosing cholangitis, or choledocholithiasis [ 5 , 6 ]. Misdiagnosis can be avoided by using appropriate imaging modalities to prevent complications. Magnetic resonance imaging MRI and magnetic resonance cholangiography MRCP can be used for diagnosis and differential diagnosis, and for determining the type of treatment required. A year-old man with a history of hyperhomocysteinemia, and who had portal vein thrombosis for 2 years, presented with abdominal pain, fever, nausea and vomiting for the previous 3—4 weeks.
His physical examination was unremarkable. He had jaundice but signs of decompensated liver disease were absent. Viral profiles, including hepatitis B and C, were negative. The laboratory test results were total protein 6. Ultrasonography revealed dilation of the intrahepatic bile ducts; the portal vein could not be seen and was replaced by anechogenic tubular structures.
Doppler ultrasound confirmed cavernomatous transformation of the portal vein not shown. MRI demonstrated intrahepatic biliary dilation Figure 1 ; the portal vein was not visualized separately.
The portal vein showed cavernomatous transformation that encircled the CBD, proximal intrahepatic bile ducts and the gallbladder was characterized by focal and circumferential wall thickening, suggesting epicholedochal veins and fibrosis.
Biliary ductal wall thickening indicated hypointense on fat-saturated T1-weighted imaging Figure 1 C and enhancement on fat-saturated imaging Figures 1 B and 1 D. There were no focal lesions or changes suggestive of chronic liver disease. The patient was followed-up in outpatient clinics. The patient was re-admitted on several occasions because of recurrent cholangitis.
Focal and circumferential wall thickening with enhancing foci, suggesting epicholedochal veins and fibrosis thick arrows , gallbladder varices thin arrows appear as low signal intensity on fat saturated T2-weighted imaging A and as enhancing on contrast-enhanced fat-saturated T1-weighted image B.
Biliary ductal wall thickening indicates hypointensity on fat-saturated T1-weighted imaging C and enhancement on fat-saturated imaging B, D thick arrows. MRCP images E, F show multiple biliary stenoses involving the common bile duct CBD and proximal right hepatic duct are seen with proximal bile duct dilation open arrows. A year-old man, with a one-year history of myeloproliferative disorder, presented to our hospital with abdominal pain. Physical examination was unremarkable.
Laboratory tests showed mild liver abnormalities. Ultrasound subsequently revealed thrombosed portal and splenic veins, with multiple anechogenic tubular structures, at the porta hepatis and moderate intrahepatic biliary dilation not shown.
MRI demonstrated mild intrahepatic bile duct dilation Figure 2. The splenic and portal veins were not visualized separately. Cavernomatous transformation was detected. Collateral vascularization encircled the CBD, common hepatic and cystic ducts and the gallbladder, characterized by enhancing focal and circumferential wall thickening Figure 2 C suggesting epicholedochal veins and fibrosis.
Biliary ductal wall thickening showed as enhancement on fat-saturated imaging Figure 2 E. Cystic ductus and gallbladder contour irregularities were demonstrated. The patient was treated for his primary disease. He is currently asymptomatic. Focal and circumferential wall thickening with enhancing foci, suggesting epicholedochal veins and fibrosis thick arrows , gallbladder varices thin arrows appear as low signal intensity on axial T2-weighted A and fat-saturated T2-weighted images B , and as enhancing on contrast-enhanced fat-saturated T-1 weighted imaging C.
Biliary ductal wall thickening indicates hyperintensity on T2-weighted imaging D and enhancement on fat-saturated T1-weighted imaging E thick arrows.
MRCP imaging F shows multiple stenoses, and the gallbladder wall irregularities open arrows. Axial T2-weighted imaging A , axial fat-saturated T2-weighted imaging B , axial contrast-enhanced fat saturated T1-weighted imaging C at the same level; axial T2-weighted D , axial contrast-enhanced fat saturated T1-weighted imaging E at the same level; and MRCP imaging F.
A year-old girl presented to our hospital, with haematemesis and melena. Physical examination and laboratory findings were unremarkable.
She had developed melena when she was 3 years old. She had a history of umbilical venous catheterization in the neonatal period. Her developmental history was also reported to be normal until the age of seven, when she started to show growth retardation. There was no family history of such a problem. Laboratory findings were total protein 7. Ultrasound at that time showed features of chronic liver disease with cavernous transformation not shown.
MRI showed a concentric low-signal intensity area surrounding the bile ducts on axial fat-saturated T2-weighted imaging, suggesting epicholedochal veins and fibrosis, and signal-void collateral vessels on axial fat-saturated T2-weighted and coronal T2-weighted imaging Figures 3 A and 3 B. Features of chronic liver disease with splenomegaly were also noted. Follow-up of the patient was not available as the patient continued her theraphy in another hospital. A concentric low—signal intensity area is seen surrounding the bile ducts, suggesting epicholedochal veins and fibrosis on axial fat-saturated T2-weighted imaging A thick arrows , corresponding to signal-void collateral vessels thin arrows on axial fat-saturated T2-weighted A and coronal T2-weighted images B.
PB is an uncommon manifestation, used to describe changes in the bile ducts due to cavernous transformation in patients with portal hypertension [ 2 ]. PB may lead to hepatic dysfunction if not treated [ 7 ]. In a small proportion of patients, the condition has also been described in non-cirrhotic portal fibrosis and cirrhosis, [ 5 ]. Causes of EHPVO include clotting and myeloproliferative disorders, neonatal umbilical vein catheterization, abdominal post-operative complications, dehydration, intra-abdominal inflammatory diseases, and direct invasion or extrinsic compression by tumours.
Our cases had known risk factors, as mentioned above. The drainage veins ascend along the course of the CBD and form epicholedochal and paracholedochal venous plexuses. The epicholedocal plexus lies on the surface of the bile duct and forms a fine reticular venous plexus not greater than 1 mm in diameter. The paracholedochal plexus runs parallel to the CBD. After portal vein thrombosis, new collaterals develop, resulting in portal cavernoma.
The dilated collateral plexuses cause extrinsic compression—smooth indentations in the biliary ducts—which can progress to narrowing and stenosis. There are also inflammatory and ischaemic changes underlying the portal thrombosis, resulting in peribiliary fibrosis [ 1 , 4 ].
Development of varices in the gallbladder wall as a collateral pathway via the cystic vein is also a characteristic feature of PB [ 2 ] Figures 1 and 2. PB has a slowly progressive character. Symptoms may occur if high-grade obstruction of the bile ducts develops, and include right upper quadrant pain, jaundice, pruritus, cholestasis, and cholangitis.
Recent advances in the spatial and temporal resolution of MRI permit excellent demonstration of biliary system anatomy. MRI has replaced endoscopic or direct cholangiography DC as the diagnostic procedure of choice for numerous conditions involving the biliary system, including PB.
This has limited the role of direct or endoscopic cholangiography for therapeutic purposes [ 9 ]. Dynamic, contrast-enhanced MRI allows anatomical relation of biliary strictures to peribiliary enlarged vessels, which provides additional information beyond cholangiographic studies. MRI is also helpful in differential diagnosis, for example cholangiocarcinoma. The imaging features of PB on MRCP include biliary strictures, dilations, a wavy appearance of the bile ducts and varicose veins located at the ductal walls and gallbladder.
Shin et al. The investigators in these studies proposed that varicoid PB is a biliary obstruction by large collateral paracholedochal veins that compress and distort the extrahepatic bile duct, while fibrotic PB results from smaller intramural epicholedochal collaterals visible as narrowed, thickened, and densely enhancing bile ducts. The importance of this distinction is that varicoid PB may be reversible with decompression of the collateral veins, while the fibrotic type is not [ 10 ].
In our case series, MRI and MRCP findings of the case 1 and 2 were consistent with fibrotic type Figures 1 and 2 and the findings of the third case were consistent with the mixed type Figure 3. Cavernous transformation of the portal vein due to EHPVO is not infrequent, but biliary obstruction in association with this disorder is distinctly uncommon.
Early diagnosis and treatment are very important for these patients, because a prolonged biliary duct obstruction can lead to the development of secondary biliary cirrhosis. Google Scholar. Oxford University Press is a department of the University of Oxford. It furthers the University's objective of excellence in research, scholarship, and education by publishing worldwide. Sign In or Create an Account. Sign In. Advanced Search.
Search Menu. Article Navigation. Close mobile search navigation Article Navigation. Volume 4. Article Contents Abstract. Oxford Academic. Cengiz Erol. Yusuf Sahingoz.
Select Format Select format. Permissions Icon Permissions. Abstract Portal biliopathy PB is a rare disorder, characterized by biliary ductal and gallbladder wall abnormalities seen in patients with portal hypertension.
Therapeutic approaches for portal biliopathy: A systematic review
Clinical presentation includes jaundice, cholangitis, cholecystitis, abdominal pain, and cholelithiasis. In this subset of patients is required a specific treatment. Different therapeutic approaches aimed to diminish portal hypertension and treat biliary strictures are available. In order to decompress PC, surgical porto-systemic shunt or transjugular intrahepatic porto-systemic shunt can be performed, and treatment on the biliary stenosis includes endoscopic Endoscopic retrograde cholangiopancreatography with endoscopic sphincterotomy, balloon dilation, stone extraction, stent placement and surgical bilioenteric anastomosis, cholecystectomy approaches. Definitive treatment of PB often requires multiple and combined interventions both on vascular and biliary system. Liver transplantation can be considered in patients with secondary biliary cirrhosis, recurrent cholangitis or unsuccessful control of portal hypertension.
Portal biliopathy PB is a rare disorder, characterized by biliary ductal and gallbladder wall abnormalities seen in patients with portal hypertension. The abnormalities consist mainly of bile duct compression, stenoses, fibrotic strictures and dilation of both extrahepatic and intrahepatic bile ducts, as well as gallbladder varices. PB may mimic cholangiocarcinoma, sclerosing cholangitis, or choledocholithiasis. Misdiagnosis can be avoided using appropriate imaging modalities to prevent complications. Portal biliopathy PB is defined as abnormalities of the extrahepatic and intrahepatic bile ducts, gallbladder and cystic duct, observed in patients with portal hypertension [ 1 , 2 ].