Treatment of Chronic PVT
Anticoagulation
Anticoagulant therapy is generally not recommended in patients with chronic PVT/portal cavernoma, however, in those patients with a persistent documented prothrombotic state, anticoagulant therapy can be considered. In these patients anticoagulation has been shown to prevent the progression and recurrence of thrombosis. There seems to be no increased risk of gastrointestinal bleeding provided adequate prophylactic measures to prevent variceal bleeding or rebleeding have been adopted before starting anticoagulation. End-stage liver disease patients with chronic PVT, who are on transplant waiting list, may require anticoagulation especially if they have documented prothrombotic state, to prevent worsening of thrombosis which would imply drop-out from the list due to technical unfeasibility. However, major risk factors for bleeding, such as oesophageal varices or a low platelet count, are frequently present in these patients. Evidence to drive treatment decisions is limited because no randomised controlled trials have been carried out in these patients. Thus, the risks associated with anticoagulant therapy should be carefully assessed against its benefits.
Interventional Therapies
There is insufficient evidence in favour of interventional therapy such as local thrombolysis or thrombectomy. A recent pilot study indicates that thrombolytic treatment of recent PVT with intravenous r-tPA and LMWH in patients with cirrhosis maybe a promising therapy. Transjugular intrahepatic portosystemic shunt for portal cavernoma with symptomatic portal hypertension in noncirrhotic patients appears to be feasible and safe in selected patients may decrease the incidence of variceal rebleeding.
Treatment and Prophylaxis of Variceal Bleeding
Because of the lack of data in this specific population, variceal bleeding in patients with noncirrhotic PVT is managed as in cirrhotic patients. A combination of early administration of vasoconstrictor agents (somatostatin, terlipressin or octreotide), endoscopic band ligation of the varices, and prophylactic antibiotics is the treatment of choice. Endoscopic injection of cyanoacrylate glue should be utilised for the control of active bleeding of gastric varices and to prevent rebleeding.
In patients with large oesophageal varices primary prophylaxis of variceal bleeding is recommended which can be achieved by either pharmacological or endoscopic therapy. For secondary prophylaxis of rebleeding, endoscopic therapy has been shown to be effective however in a recent study beta-blockers were shown to be equally effective. Decompressive surgery should only be considered for patients with failure of endoscopic therapy. The surgical procedure must be individualised and depends on experience and expertise available in the centre. Many centres prefer distal splenorenal shunts or 'H' graft mesocaval shunts. Other procedures such as oesophageal transection, with or without splenectomy and ligation of the varices, are less useful because of the relatively high risk of late rebleeding due to reappearance of the varices. In paediatric patients mesenteric-left portal vein bypass (Rex bypass) is a good treatment option for management of bleeding.
Treatment of Portal Biliopathy
Most patients with portal biliopathy are asymptomatic. Treatment is indicated only for symptomatic cases and the treatment decision is on case-to-case basis. For patients with mild symptoms with abnormal cholestatic tests treatment with ursodeoxycholic acid maybe tried, however, experience is limited and there are no clinical trials in support of this therapy. Choledocholithiasis might be present complicating a bile duct stenosis. If so, an ERCP with sphincterotomy, extraction with balloon catheter and stent placement are indicated. Stone extraction by dormia basket should be avoided to prevent inadvertent rupture of bile duct varices. When there is no evidence of common bile duct stones contributing to the symptoms, sphincterotomy might be useless and hazardous. For common bile duct strictures endoscopic stenting with periodic replacement should be tried first, if not successful porto-systemic shunt surgery aimed at decompressing the collaterals should be considered. If symptoms are not sufficiently relieved as happens in one-third of patients, a hepatico-jejunostomy may have to be done to relieve the patient of symptomatic biliary obstruction and prevent secondary biliary cirrhosis. Portosystemic shunts are the treatment of choice for severe portal biliopathy. In the majority of patients, the changes caused by biliopathy resolve after shunt surgery, however, 15–20% patients require a subsequent bilio-enteric bypass or endoscopic management for persistent biliopathy.