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Portal Vein Obstruction Treatment & Management

  • Author: Adnan Said, MD, MSPH; Chief Editor: BS Anand, MD  more...
 
Updated: Dec 28, 2015
 

Medical Care

Acute bleeding

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 the 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 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

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.[9] 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.[9]

Transfer 

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.

Outpatient monitoring

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.

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Surgical Care

Shunt surgery

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 or 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.[10] 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).[10] 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.[10]

TIPS

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.

Liver transplantation

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.

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Consultations

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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Contributor Information and Disclosures
Author

Adnan Said, MD, MSPH Assistant Professor, Department of Medicine, Section of Gastroenterology and Hepatology, University of Wisconsin School of Medicine and Public Health at Madison; Consulting Staff, Department of Medicine, William S Middleton Memorial Veterans Hospital

Adnan Said, MD, MSPH is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, American Society of Transplantation

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Noel Williams, MD, FRCPC FACP, MACG, Professor Emeritus, Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada; Professor, Department of Internal Medicine, Division of Gastroenterology, University of Alberta, Edmonton, Alberta, Canada

Noel Williams, MD, FRCPC is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

BS Anand, MD Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine

BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Additional Contributors

Ann Ouyang, MBBS Professor, Department of Internal Medicine, Pennsylvania State University College of Medicine; Attending Physician, Division of Gastroenterology and Hepatology, Milton S Hershey Medical Center

Disclosure: Nothing to disclose.

Acknowledgements

Mark Reichelderfer, MD

Disclosure: Nothing to disclose.

Andrew Taylor, MD Professor, Department of Radiology, University of Wisconsin Hospitals and Clinics

Disclosure: Nothing to disclose.

Jennifer T Wells, MD Fellow, Department of Gastroenterology and Hepatology, University of Wisconsin Hospitals and Clinics, Madison

Jennifer T Wells, MD is a member of the following medical societies: American Medical Association and Phi Beta Kappa

Disclosure: Nothing to disclose.

References
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  3. Sharma P, Sharma BC, Puri V, Sarin SK. Natural history of minimal hepatic encephalopathy in patients with extrahepatic portal vein obstruction. Am J Gastroenterol. 2009 Apr. 104(4):885-90. [Medline].

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  5. Nihal L, Bapat MR, Rathi P, Shah NS, Karvat A, Abraham P, et al. Relation of insulin-like growth factor-1 and insulin-like growth factor binding protein-3 levels to growth retardation in extrahepatic portal vein obstruction. Hepatol Int. 2009 Mar. 3(1):305-9. [Medline]. [Full Text].

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  9. Alberti D, Colusso M, Cheli M, Ravelli P, Indriolo A, Signorelli S, et al. Results of a stepwise approach to extrahepatic portal vein obstruction in children. J Pediatr Gastroenterol Nutr. 2013 Nov. 57(5):619-26. [Medline].

  10. Guérin F, Porras J, Fabre M, Guettier C, Pariente D, Bernard O, et al. Liver nodules after portal systemic shunt surgery for extrahepatic portal vein obstruction in children. J Pediatr Surg. 2009 Jul. 44(7):1337-43. [Medline].

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  14. Ha TY, Kim KM, Ko GY, et al. Variant meso-Rex bypass with transposition of abdominal autogenous vein for the management of idiopathic extrahepatic portal vein obstruction: a retrospective observational study. BMC Surg. 2015 Oct 17. 15:116. [Medline].

  15. Arrive L, Hodoul M, Arbache A, Slavikova-Boucher L, Menu Y, El Mouhadi S. Magnetic resonance cholangiography: Current and future perspectives. Clin Res Hepatol Gastroenterol. 2015 Dec. 39 (6):659-64. [Medline].

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The etiology of portal vein obstruction.
Coagulation disorders in portal vein thrombosis.
Portal vein thrombosis with cavernous transformation. The long arrow indicates the splenic vein at the junction with the superior mesenteric vein just below the site of thrombosis. The short arrow points to a serpiginous mass consistent with periportal collaterals, the so-called cavernous transformation of the portal vein.
Hepatocellular carcinoma with portal vein thrombosis. The short arrow indicates the tumor thrombus with an abrupt cut off of the portal vein. The long arrow points to a compensatory, prominent left hepatic arterial branch.
 
 
 
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