What is the recommended management for a patient with Extrahepatic Portal Vein Obstruction (EHPVO)?

Management of Extrahepatic Portal Vein Obstruction (EHPVO)

The recommended management for patients with Extrahepatic Portal Vein Obstruction (EHPVO) should include anticoagulation therapy for prevention of thrombotic extension, combined with endoscopic management of varices and treatment of underlying prothrombotic conditions. 1

Diagnostic Approach

• First-line investigation: Doppler ultrasound for initial diagnosis 1, 2
• Confirmatory imaging: CT scan with vascular contrast agents to assess:
  • Absence of visible portal vein lumen
  • Presence of serpiginous vascular channels in porta hepatis (cavernoma)
  • Extension of thrombosis 1
• Additional testing: Screen for underlying prothrombotic disorders, particularly myeloproliferative neoplasms and antiphospholipid syndrome 1, 3
• Liver biopsy: Only indicated if persistent liver test abnormalities or dysmorphic liver appearance to rule out cirrhosis or obliterative portal venopathy 1

Management Algorithm

1. Acute Management of Portal Vein Thrombosis

• Initiate immediate anticoagulation with low molecular weight heparin (LMWH) in the absence of major contraindications 1
• Monitor anti-Xa activity in overweight patients, pregnancy, and poor kidney function, targeting 0.5-0.8 IU/ml 1
• Transition to oral vitamin K antagonists (VKA) for long-term treatment with target INR 2-3 1
• Continue anticoagulation for at least 6 months 1
• Assess recanalisation with CT scan at 6-12 months follow-up 1

2. Management of Portal Hypertension Complications

• Screen for gastroesophageal varices in all patients, especially those without recanalisation 1
• Prophylaxis for variceal bleeding:
  • Non-selective beta-blockers (propranolol) as first-line therapy 1, 4
  • Endoscopic band ligation is superior to sclerotherapy for primary prophylaxis 1, 4
• Acute variceal bleeding:
  • Endoscopic therapy (band ligation preferred over sclerotherapy) 1, 4
  • For uncontrolled bleeding, consider TIPS (transjugular intrahepatic portosystemic shunt) when intrahepatic portal veins are visible 1
  • Surgical portosystemic shunting may be considered in selected patients 1, 5

3. Management of Portal Biliopathy

• Screen with MR cholangiography in patients with persistent cholestasis or biliary tract abnormalities 1
• Treatment approach:
  • Only treat symptomatic portal biliopathy 1
  • Endoscopic management for bile stones and biliary strictures 1, 6
  • Surgical shunt followed by biliary bypass for persistent symptoms 1, 6
  • Be aware of high risk of hemorrhage during biliary procedures due to varices 6

4. Long-term Management

• Consider permanent anticoagulation in patients with:
  • Strong prothrombotic conditions (especially myeloproliferative neoplasms)
  • History suggesting intestinal ischemia
  • Recurrent thrombosis on follow-up 1
• Regular surveillance:
  • Endoscopic monitoring for varices
  • Imaging to assess portal vein status
  • Monitor for development of portal biliopathy 1

Special Considerations

Children with EHPVO

• More commonly presents with variceal bleeding and splenomegaly 7, 5
• Growth retardation may occur due to decreased insulin-like growth factor production 5
• Consider mesenterico-Rex shunt (bypass between superior mesenteric and left portal veins) which can:
  • Prevent gastrointestinal bleeding
  • Improve mental status and coagulation factors
  • Potentially improve growth 1, 5

Patients with Myeloproliferative Disorders

• Screen for JAK2V617F mutation and other markers of myeloproliferative neoplasms 3
• Coordinate management with hematology for cytoreduction therapy 3
• Long-term anticoagulation is indicated in these cases 1

Pitfalls and Caveats

• Bleeding risk: Carefully balance anticoagulation benefits against bleeding risk; implement variceal prophylaxis before starting anticoagulation 1, 3
• Biliary procedures: High risk of hemobilia from ruptured intrabiliary varices during endobiliary interventions 1, 6
• Surgical complications: Significant morbidity and mortality with primary biliary tract surgery; consider staged procedures (portosystemic shunt followed by biliary surgery) when endoscopic management fails 6
• Underlying conditions: Always screen for and treat underlying prothrombotic conditions to prevent recurrence 1, 3

The overall prognosis for patients with EHPVO without cirrhosis or malignancy is relatively good, with five-year survival rates above 70% 1.