The primary goals are to alleviate acute bleeding and to prevent further bleeding. In the acute setting, these goals are best accomplished with variceal banding or sclerotherapy, often requiring several sessions to obliterate the varices. This has a success rate of 95% for controlling an acute bleed.
Octreotide infusion has also been used in acute bleeding, with control of the acute bleed in 85% of patients. The rate of recurrent bleeding with this approach is 16-28%.
In the setting of portal vein obstruction, the role of propranolol to prevent rebleeding has not been studied, though it is used routinely.
After endoscopic therapy of bleeding varices, propranolol is instituted with a goal of reducing the heart rate by 25% below baseline.
Treatment of underlying etiology
Anticoagulation in patients with acute/recent portal vein thrombosis, studied only retrospectively, has been shown to recanalize the thrombosed vessel in more than 80% of cases. This is essential to prevent the advancement of thrombosis or rethrombosis in patients with inherited coagulation disorders in which lifelong anticoagulation therapy is recommended once variceal control has been achieved. Anticoagulation therapy has also been recommended after shunt surgery to prevent rethrombosis. In one study in which 84 of 136 nonmalignant, noncirrhotic patients with portal vein thrombosis were anticoagulated with similar bleeding risks but less risk for thrombotic propagation. Debate remains regarding the risk-to-benefit ratio of anticoagulation in chronic portal vein thrombosis and should be decided on a case-by-case approach at this time.
Thrombolysis is recommended in acute portal vein thrombosis through the transhepatic route, which avoids the need for systemic thrombolysis.
Tissue-type plasminogen activator (tPA) has been used for this purpose, followed by prolonged anticoagulation therapy with Coumadin for at least 3 months (indefinitely in patients with inherited coagulation disorders).
In the setting of acute portal vein thrombosis with symptoms, shunt surgery with subsequent anticoagulation therapy is an alternative.
In a study consisting of 65 children (median age, 12.5 y) with extrahepatic portal vein obstruction, a stepwise treatment protocol involving medical, endoscopic, and surgical approaches resulted in good outcomes.  The protocol took into consideration the severity of the portal hypertension and the feasibility of mesoportal bypass. The children underwent surveillance endoscopies: Those with large varices received nonselective beta blockers (NSBBs) or underwent variceal obliteration (EVO). For patients with disease refractory to NSBBs and EVO, mesoportal bypass was the first-line treatment, and transjugular intrahepatic portosystemic shunt surgery was second-line therapy. There was a 93% survival with bleeding control, and 82% had a patent conduit. 
For variceal bleeding refractory to endoscopic therapy or recurrent bleeding after endoscopic therapy, transfer to a center experienced in TIPS placement or shunt surgery is recommended.
Transfer to a liver transplantation center is recommended for patients with hepatic dysfunction related to cirrhosis in the setting of portal vein thrombosis.
Patients that had endoscopic ligation of varices should have repeat endoscopies every 2-4 weeks with the goal of complete eradication of varies.
After TIPS placement, repeat ultrasonography is recommended (initially, 4 wk after placement, then every 3 mo) to check patency of the stent. If significant occlusion is discovered, revision can be performed, usually in the outpatient setting.
In portal vein obstruction, the place for shunt surgery in the treatment of variceal bleeding is debated. Some authors recommend endoscopic treatment and propranolol as first-line treatment to prevent recurrent bleeding. Others recommend shunt surgery after the first variceal bleed to prevent further rebleeding.
In general, shunt surgery should only be attempted when endoscopic treatment fails.
A distal splenorenal shunt is usually the preferred surgical shunt. For patients in whom the splenic vein is also thrombosed and surgery is undertaken, splenectomy and other shunt procedures (eg, the Sugiura procedure) have been performed. A more recent salvage operation showing success is the right and left mesogonadal shunt. In patients who are critically ill, esophagogastrectomies have been used as a last resort.
In the presence of cirrhosis, the operative mortality rate has been reported to be 18%. In the absence of cirrhosis, operative mortality is approximately 2%. The postoperative complication rate is approximately 30%.
The presence of liver nodules has been reported following portal systemic shunt surgery in animal models and in humans with liver cirrhosis. In a small retrospective study of 45 children without liver disease, Guerin et al examined the incidence of liver nodules following surgical intervention for extrahepatic portal vein obstruction.  Using ultrasonography, the investigators noted 7 (15%) of the children had liver nodules (median 80 months' follow-up), all of which occurred following portal systemic shunt surgery and 5 of which demonstrated either liver cell adenomas (2 nodules) or focal nodular hyperplasias (3 nodules).  Guerin et al recommended keeping in mind the possible presence of liver nodules during follow-up of children post portal systemic shunt surgery for extrahepatic portal vein obstruction. 
Previously considered a relative contraindication in portal vein thrombosis, TIPS has been successfully used in this condition. Stent placement requires an aspiration thrombectomy through a sheath with subsequent angioplasty of the tract and stent placement. Some centers have obtained good results by performing an embolectomy and then using local thrombolytic therapy through the TIPS after deployment.
In portal vein obstruction, TIPS is indicated in uncontrollable variceal bleeding in a patient with cirrhosis, usually as a bridge to transplant. The choice of TIPS over shunt surgery depends upon the expertise of the center in these techniques and the distance from skilled health care because TIPS is more likely to occlude and require revision. However, TIPS has the advantage of being less invasive than shunt surgery.
In the setting of portal vein obstruction and cirrhosis, TIPS has a success rate of 69% in controlling variceal bleeding and a complication rate of 22%, including a mortality rate of 11% in one series.
In patients referred for orthotopic liver transplantation (OLT), portal vein thrombosis complicates 5-15% of cases.
Although traditionally viewed as a relative contraindication to OLT, recent innovative surgical techniques (eg, thrombectomy, venous jump grafts, use of portal vein tributaries) have resulted in improved results post-OLT in end-stage liver disease with portal vein thrombosis.
In patients with associated portal vein thrombosis, the 5- and 10-year survival rates after OLT are approximately 63% and 53%, respectively, whereas, in patients without thrombosis, the 5- and 10-year survival rates after OLT are 67% and 59%, respectively. Moreover, a higher incidence (5%) of primary nonfunction, renal failure, and recurrent portal vein thrombosis exists.
In young, otherwise healthy patients with extension of thrombus to the splenic and mesenteric venous systems, eliminating surgical shunt options, should be considered for multivisceral transplantation.
Gastroenterologists and hepatologists: Seek a consultation for management of acute variceal bleeding and to coordinate further management.
Interventional radiologists: Seek a consultation for consideration of TIPS, especially if recurrent variceal bleeding exists in a transplant candidate.
General surgeons and transplant surgeons: Seek a consultation for consideration of shunt surgery and to assess transplant suitability in patients with underlying cirrhosis.
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