PB is characterized by bile duct and GB wall abnormalities associated with portal hypertension (PH). PB is often associated with idiopathic extrahepatic portal venous occlusion [1] [2] . The abnormalities recorded in PB are bile duct compression, stenoses, and strictures interspaced by dilated ducts both intrahepatic and extrahepatic are affected. GB varices may present as a thickened wall, elegantly demonstrated by Doppler interrogation. PB may mimic a cholangiocarcinoma, sclerosing cholangitis, or bile duct calculi.

The association of jaundice and common bile duct compression and PH were first described by Fraser et al. [3] in 1944. The term “portal biliopathy” was coined in the early 1990s [1] .

Accurate diagnosis is achieved by using appropriate imaging, which includes ERCP, PTC, CT and MRI/MRCP. Presently there is no role for intravenous cholangiography. Shin SM in their study of MRI features of PB showed that MR cholangiography and 3D gradient-echo imaging not only detect portal vein occlusion, but cavernous transformation, and GB varices and other associated bile duct abnormalities [4] - [11] .

The aim of this study has been to present a series of cases evaluated, treated and followed at a tertiary care public institution. Patients with PB were exposed to different therapeutic approaches focused on the management of portal hypertension and biliary decompression. They were followed for ~5 years. Three cases achieved a favorable outcome with symptom remission, but one patient died while attempting dilatation of the bile duct another died because of multiple underlying pathologies. Finally, we carried out a literature review of portal hypertensive biliopathy therapeutics. Presently there is no consensus on the optimal treatment PB. The ultimate goal decompression of the biliary tree should be individually evaluated to choose the best treatment option. The literature was reviewed supported by data from our series, with a view to offering the best possible option for the management of this rare complication of PVT. The liver function abnormalities in PB are highly non-specific, and only imaging provides guidance for the most appropriate management. Imaging feature in 9 cases illustrates the later statement. What the imaging features from our case series is discussed individually in the images (please see images of the nine cases).

PB is associated with PH, particularly in patients with extrahepatic portal vein obstruction. The signs of PB include bile duct and GB abnormalities. Portal cavernoma formation, choledochal varices and ischemic injury of the bile duct have been implicated in the causation of PB. The majority of the patients with PB remain asymptomatic while a minority have a raised alkaline phosphatase, abdominal pain, fever, and cholangitis. Choledocholithiasis is a known complication, causing obstructive jaundice with or without cholangitis. Endoscopic sphincterotomy and stone extraction can alleviate cholangitis and jaundice associated CBD calculi. Definitive treatment by decompressive shunt surgery is sometimes required with progressive disease [2] .

Several mechanisms are involved in the genesis of incomplete PB. The mechanisms include induction of fibrosis in the biliary tract related either directly to dilated peribiliary portal venous collaterals and recurrent cholangitis, loss of bile duct motility and chronic cholestasis secondary to fibrosis or bile calculi. The management of biliary calculi in patients with portal vein cavernous transformation is complex. Moreover, a heterogeneous clinical presentation and concomitant pathophysiological changes that take place in biliary anatomy, diagnosis and therapy can become very complicated. Conventional treatment of cholelithiasis, such as a sphincterotomy and balloon dilatation of bile ducts can cause complications such as cholangitis, biliary strictures, and hemobilia. Harmanci & Bayraktar reviewed the management bile calculi related to bile duct obstruction in non-cirrhotic portal vein thrombosis and present evidence in the current literature [12] .

Rarely PB is only diagnosed at laparotomy. Colle et al. [13] reported a man, 51-year of age with a history of PVT that presented with elevated liver enzymes and mass at the liver hilum. CT and MRI confirmed a hilar mass and compression of the CBD. ERCP showed irregular narrowing of the mid-CBD. The patient underwent an explorative laparotomy, which revealed a hypervascular mass in the porta hepatis surrounded by dilated by portal vein tributaries. The diagnosis of portal cavernoma was made. Further hematological tests showed an anti-phospholipid syndrome and myeloproliferative disorder as a cause of PVT. The patient was started on oral anticoagulants. In conclusion, the authors reported a patient with a biliary stricture secondary to a portal venous thrombosis and a cavernoma (PB), not diagnosed preoperatively [13] .

PH and PT is a rare cause of PB in children with extrahepatic portal vein obstruction with PH. Children may be asymptomatic or could lead to cholestatic liver biochemistry. El-Matary et al. reported a child that developed a cholestatic picture with PH some, time after having neonatal surgery for duodenal atresia. Symptomatic PB is rare in children but should be suspected in children patients with PH and features of bile duct obstruction. If endoscopic or percutaneous biliary intervention is unsuccessful, and there is no response to portal-systemic shunt, hepaticojejunostomy may have a therapeutic role in selected children [14] .

PB is predominantly related to a combination of PH and extra-hepatic portal vein thrombosis. A rare case of partial intrahepatic venous thrombosis associated with acute pancreatitis, probably due to kissing gastric ulcers perforation has been described. The patient developed a portal cavernoma in addition to partial PV thrombosis [15] . A literature search showed a case where multiple duodenal ulcers in hypertensive portal duodenopathy associated with upper GI bleeding and cirrhosis was described [16] .

Imaging of the bile ducts is the most important consideration in the diagnosis of PB. Arrivé et al. [7] studied and described several limitations of MRI of cholangitis. The authors suggest a systematic approach, which should include evaluation of bile duct stenoses, duct dilatation, intrahepatic calculi and inhomogeneous liver parenchyma and PH enhancement of the liver parenchyma and any enhancement and bile duct walls.

Ozkavukcu and associates [8] studied 16 patients to evaluate the frequency MRCP findings in PB. Described in the order of frequency was as follows: biliary stenosis, a wavy appearance of the bile ducts, angulation of the CBD, and upstream dilatation of the bile ducts.

Chevallier et al. [4] studied 10 patients with PB in a retrospective and monocentric study and concluded that MRC constituted an accurate imaging modality to investigate noninvasively patients with PB.

Aguilar-Olivos et al. [9] studied 4 patients with PB from a tertiary referral center and suggested that PB is an underdiagnosed condition because only some patients have symptoms.

MRI has certain limitations. Matsuo et al. [10] reported a pseudo PV thrombosis on MRI in a 65-year-old woman with severe cirrhosis. Unenhanced T2-weighted fast spin-echo images showed an area of high signal intensity occupying the left second-order portal vein branch, suggesting portal vein thrombosis in cirrhosis. Doppler sonography, showed no flow in the involved portal vein, supporting the diagnosis. Gadolinium-enhanced MRI subsequently revealed the patency of the vessel. Matsuo et al. [10] concluded that an extremely slow portal venous flow might thus cause a false-positive finding venous thrombosis with unenhanced MRI and Doppler sonography.

Nunoi H et al. [11] described a patient with PB-associated with the morphological abnormality of the biliary duct and GB associated with PH that presented with essential thrombocythemia, initially diagnosed with extrahepatic portal vein obstruction. The contrast-enhanced CT findings were similar to those of cholangiocarcinoma or sclerosing cholangitis. However, color Doppler and contrast-enhanced ultrasound (US) were more precise, leading to a diagnosis PB, which prevented the patient, resulting in the avoidance of risky procedures such as bile duct biopsies.

Concerning management, Khan et al. [17] made the ensuing iterations:

The vast majority of patients with PB are usually asymptomatic and found incidentally, but rarely the patients present with abdominal pain, jaundice, weight loss and pyrexia; Treatment is instituted in symptomatic patients only, modified by the clinical picture and the stage of the disease process; The presence of severe PH, endoscopic biliary intervention is usually the first line of treatment, considered safe and often sufficient; in patients that fail to respond to endoscopic intervention, surgery may be considered; When surgery is indicated, a portosystemic shunt prior to biliary bypass provides early symptomatic relief and may preclude biliary bypass surgery.

Chaudhary et al. [18] retrospectively reviewed the surgical management of symptomatic PB in 9 patients. Eight of the 9 patients had jaundice, two presented with abdominal pain and one with recurrent cholangitis. Bile duct strictures and bile duct abnormalities were found in 8 patients, and two patients had choledocholithiasis on Endoscopic retrograde cholangiography (ERCP). Placement of a Portal-systemic shunt relieved jaundice in five of seven patients, and in two patients required a second-stage hepatico-jejunostomy. The authors suggested that a direct approach to the biliary tract without a preliminary shunt may be hazardous and is frequently not necessary.

Vleggaar and associates [19] described their experience in four adult patients with portal vein thrombosis that presented with symptoms or signs of biliary obstruction. Cholangiography revealed smooth extrinsic indentations CBD. The patients had a varied clinical course from spontaneous resolution and returned to normal liver function tests in two patients. Two patients had persistent cholestasis and required, surgical treatment failed due to the presence of numerous collateral veins.

Perego and associates [20] describe a case of obstructive jaundice and cholangitis secondary to calculi within the CBD associated with bile duct strictures after a cavernous transformation of the portal vein. Biliodigestive anastomosis was not appropriate as the patient suffered from a short bowel syndrome due to extensive ileal resection for mesenteric vein thrombosis. Repeated attempts were made at the removal of ductal calculi via ERCP, resulting in partial success. Finally, a percutaneous trans-hepatic approach, allowed stone extraction and balloon dilatation of the bile duct strictures. The patient responded well to this treatment remained asymptomatic 3 years after the procedure.

5% - 30% of patients with PB develop biliary obstruction. Bernon et al. [21] report on a patient with PB that was successfully managed with an intrahepatic segment 3 bypasses. The latter provides a definitive treatment of bile duct decompression and stone removal in a single procedure in appropriately selected patients.

PB is a rare complication of extra-portal venous obstruction associated with PH and extrahepatic PVT. Most patients remain asymptomatic, but some may present with features of cholangitis. Ischemic injury to the biliary tree is the postulated cause. Extrinsic compression of the common duct by dilated venous collaterals together with pericholedochal fibrosis from an inflammatory process can cause portal thrombosis, which may lead to biliary stricture and dilatation of the proximal biliary tree. These strictures set the scene for formation biliary stones and cholangitis. Imaging plays a vital role in the diagnosis and management of PB. A personal series of imaging of PB is presented. The causation, the course of disease and management particularly minimally invasive procedures is discussed. Imaging is the cornerstone of accurate diagnosis and directs further management. An illustrative atlas is provided supported by patients from our intuition seen over 10 - 15 years. See images with detailed explanations provided. These images are examples of personal series collected over years.

Retrospective study, archives from several hospitals, personal series. No consent is required as no direct patient data is published.

Ethical approval is not required, retrospective study.

Dr. Ali Nawaz Khan: Wrote, designed the study and the images are from personal archives;

Dr. Ken Uzoka: Wrote part of the study and contributed images and provided legends to the images;

Dr. Sumaira Macdonald: Contributed images, and revised the paper study retrieved all relevant documents pertaining to the paper;

Dr. Abeeku Afedzi Hammond: Worked on the images, labelled the images and revised the paper;

Mr. Anthony Kodzo-Grey Venyo: Read the paper and also made changes to the original drafted paper.

Khan, A.N., Uzoka, K., Macdonald, S., Hammond, A.A. and Venyo, A.K.-G. (2017) Portal Biliopathy: A Review of Imaging Features of Nine Patients. International Journal of Clinical Medicine, 8, 604-617. https://doi.org/10.4236/ijcm.2017.811057

Case 1: Portal biliopathy associated with PVT

Case 2: PB-associated with Leber optic atrophy (hereditary optic neuroretinopathy) and alcohol abuse

Case 3: PB and PVT

Case 4: PB and PVT

Case 5: PB-associated with liver transplant

Case 6: PVT

Case 7: PB associated with a splenorenal shunt

Case 8: Portal biliopathy associated with Budd-Chiari and PVT

The diagnosis of PVT associated with a Budd-Chiari syndrome cannot be achieved without imaging, as liver function test abnormalities are non-specific.

Case 9: Portal biliopathy associated with protein C & S deficiency

The diagnosis of PB in the elderly particularly in those with Protein C & S deficiency cannot be achieved by liver function tests, and imaging is vital. Figure 9 shows the value of imaging in such cases.