Long Term Outcomes of Patients with Significant Biliary Obstruction Due to Portal Cavernoma Cholangiopathy and Extra-Hepatic Portal Vein Obstruction (EHPVO) With No Shuntable Veins.

AIMS: The natural history of portal cavernoma cholangiopathy (PCC) in patients with significant biliary obstruction (SBO) who cannot undergo shunt surgery, is not known. We therefore, analyzed data of patients of extra-hepatic portal venous obstruction (EHPVO) with PCC. METHODS: Prospectively recorded details of 620 (age 21.2 [11.4] years; 400 [65%] males) patients with primary EHPVO were reviewed. Outcomes (hepatic decompensation/mortality) of patients with PCC and SBO without shuntable veins were noted at follow up of 7 [4-11] years. RESULTS: Ninety-seven of 620 (15.6% [60 men]) EHPVO patients had PCC-SBO. Of these 57 did not have shuntable veins. The median duration from any index symptom to symptomatic PCC was 7 (0-24) years and from index bleed to symptomatic PCC was and 12 (5-24) years, respectively. Thirteen patients underwent endoscopic retrograde cholangiography; nine repeatedly over 7 (4-10) years. Decompensation was seen in 5 patients. Presentation other than variceal bleed was associated with hepatic decompensation (5/19 versus 0/38, P = 0.003). CONCLUSIONS: Majority of patients with PCC-SBO do not have shuntable veins, and may have good long-term outcomes. Patients presenting with variceal bleed have low chance of decompensation. Symptomatic PCC appears to be a late event in EHPVO.

Portal vein thrombosis.

Portal vein thrombosis is an important cause of portal hypertension. PVT occurs in association with cirrhosis or as a result of malignant invasion by hepatocellular carcinoma or even in the absence of associated liver disease. With the current research into its genesis, majority now have an underlying prothrombotic state detectable. Endothelial activation and stagnant portal blood flow also contribute to formation of the thrombus. Acute non-cirrhotic PVT, chronic PVT (EHPVO), and portal vein thrombosis in cirrhosis are the three main variants of portal vein thrombosis with varying etiological factors and variability in presentation and management. Procoagulant state should be actively investigated. Anticoagulation is the mainstay of therapy for acute non-cirrhotic PVT, with supporting evidence for its use in cirrhotic population as well. Chronic PVT (EHPVO) on the other hand requires the management of portal hypertension as such and with role for anticoagulation in the setting of underlying prothrombotic state, however data is awaited in those with no underlying prothrombotic states. TIPS and liver transplant may be feasible even in the setting of PVT however proper selection of candidates and type of surgery is warranted. Thrombolysis and thrombectomy have some role. TARE is a new modality for management of HCC with portal vein invasion.

Endoscopic retrograde cholangiography in portal cavernoma cholangiopathy - results from different studies and proposal for uniform terminology.

Portal cavernoma cholangiopathy (PCC) refers to a constellation of secondary changes in the biliary tree in patients with chronic portal vein (PV) thrombosis and portal cavernoma formation. These findings of PCC are seen in the extra-hepatic bile duct(s), with or without involvement of the 1st or 2nd degree intra-hepatic bile ducts. Of all patients with chronic PV thrombosis, cholangiographic features of PCC are found in 80%-100%. The biliary changes are symptomatic in a smaller proportion of 5%-38% patients. Choledocholithiasis and hepatolithiasis occur in 5%-20%, independent of the occurrence of cholelithiasis. We review the published literature on cholangiographic description of PCC. We also propose standardized nomenclature for the cholangiographic findings, namely: extrinsic impressions/indentations, shallow impressions, irregular ductal contour, stricture (s), upstream dilatation, filling defects, bile duct angulation, and ectasia.

Natural history of portal cavernoma cholangiopathy.

The natural history of portal cavernoma cholangiopathy (PCC) is poorly defined and poorly understood. It develops early after acute portal vein thrombosis (PVT) if there is failure of recanalization. In PCC, the likelihood of progression of biliary abnormalities after 1 year is extremely low. The natural history of PCC is conveniently divided into asymptomatic and symptomatic stages. The majority of patients with PCC are asymptomatic and are detected incidentally on imaging. Limited data suggest that asymptomatic PCC is static or only slowly progressive in the initial stages. However, most workers agree that, overall, PCC is a slowly progressive disease. Symptomatic PCC represents a late stage in its natural history. Finding strictures with dilatation at cholangiography is associated with a higher risk of developing symptoms of PCC. Onset of symptoms is often precipitated by the development of biliary sludge or calculi and treating calculi usually relieves symptoms for prolonged periods of time. Clinical presentations include biliary pain, obstructive jaundice, acute cholangitis, acute cholecystitis, or other presentations of gallstone disease. Progressive liver dysfunction and secondary biliary cirrhosis can develop in a minority of patients.