Imaging features of portal biliopathy. Frequency of involvement patterns with emphasis on MRCP

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. ( J CLIN EXP HEPATOL 2014;4:S2-S14)

Section: Computed Tomography (Ct) or Magnetic Resonance Imaging (Mri)mentioning

confidence: 91%

Section: Computed Tomography (Ct) or Magnetic Resonance Imaging (Mri)mentioning

confidence: 97%

Portal Cavernoma Cholangiopathy: Consensus Statement of a Working Party of the Indian National Association for Study of the Liver

Dhiman

Saraswat

Valla

et al. 2014

“…8 On MRI, PCD collaterals and ECD collaterals are identified separately. 17 PCD collaterals and gallbladder varices appear as low signal intensity channels on T2-weighted images and as enhancing tortuous collaterals on dynamic 3D gradient-echo images. ECD collaterals appear as dot like enhancing structures in the bile duct wall.…”

Section: Role Of Imaging For Different Type Of Collateralsmentioning

confidence: 94%

Portal Cavernoma Cholangiopathy: An Endoscopic Ultrasound Based Imaging Approach

Sharma

Rameshbabu²

2014

In patients with portal cavernoma cholangiopathy (PCC), appearance and location of collateral channels depends on extent and location of occlusive thrombus in the porto-mesenteric venous system. If the porto-mesenteric venous system is occluded near the formation of portal vein, blood tends to flow through collateral channels that form varices in and around the common bile duct. Though endoscopic ultrasound (EUS) is considered the investigative modality of choice for evaluating common bile duct obstruction, its role in evaluating collateral pathways in and around the common bile duct is poorly defined. This article reviews the anatomy, genesis and appearance of these collateral pathways in PCC. EUS identifies different layers of the common bile duct (CBD) wall and, in PCC, where varices are in close contact with or part of these different layers, can establish the relationship between them. Thus, EUS appears to be the investigation of choice for tracing the origin and course of collaterals in PCC. Careful study of varices in the common bile duct wall prior to ERCP for bile duct stones or biliary strictures may help to plan the procedure and to manage anticipated complications such as hemobilia. ( J CLIN EXP HEPATOL 2014;4:S53-S61) B oth porto-systemic and porto-portal collaterals can develop in response to chronic occlusion of the portal venous system. The location and extent of occlusive thrombus in the porto-mesenteric venous system determine the type of collaterals that eventually develop and the direction of collateral flow.

“…18 Recent reports of MRCP imaging in PCC show that either type I or type III are the most frequent, with the extrahepatic bile ducts being the most common location of narrowing. 15 It is important to note that MRCP can overestimate bile duct stenosis compared to conventional cholangiographic studies as was seen in a retrospective study of 10 patients of EHPVO with direct cholangiographic comparison in 3 patients. 19 Llop et al 20 classified cholangiographic findings of 67 patients with portal vein thrombosis using MRCP.…”

Section: Magnetic Resonance Imagingmentioning

confidence: 95%

“…Condat et al in their prospective study of 25 patients showed that findings at MRCP were similar to direct cholangiographic images in 4 patients in whom both the studies were done. 14 In a retrospective study of 16 patients, 15 biliary findings on MRCP included biliary stenosis in 93.7%, upstream dilatation in 68.7%, wavy appearance of the biliary tree in 87.5% and angulation of the CBD in 75% patients. None of the patients had choledocholithiasis.…”

Section: Magnetic Resonance Imagingmentioning

confidence: 99%

Imaging of Portal Cavernoma Cholangiopathy

Kalra

Shankar

Khandelwal

2014

Portal cavernoma cholangiopathy (PCC) refers to the biliary changes which occur in the setting of extrahepatic portal vein obstruction and secondary portal cavernoma formation. The main radiological findings include the vascular changes in the form of portosystemic collaterals and biliary changes in the form of extrinsic impressions and strictures. Till date, conventional cholangiography has been the gold standard for the diagnosis of PCC. However, it is an invasive procedure and is associated with complications. At present there is a transition towards non-invasive modalities like ultrasound (US), computed tomography (CT) and magnetic resonance imaging (MRI). The recent advances in these modalities provide an excellent delineation of both the vascular and the biliary changes non-invasively in a short time. The findings of PCC using these newer modalities are not so well described in literature. The findings of PCC also overlap with malignant conditions of biliary tract such as cholangiocarcinoma and compression of biliary tract by malignant adenopathies. In this article we describe the vascular and biliary changes associated with PCC on US, CT and MRI. We also describe the imaging findings using each modality along with their advantages and disadvantages. ( J CLIN EXP HEPATOL 2014;4:S44-S52)