How are non-cirrhotic causes of portal hypertension managed?

Management of Non-Cirrhotic Causes of Portal Hypertension

Non-cirrhotic portal hypertension should be managed according to the same guidelines established for cirrhotic portal hypertension, with treatment focused on addressing the underlying cause and preventing complications related to portal hypertension. 1

Diagnostic Approach

• Non-cirrhotic portal hypertension (NCPH) comprises a heterogeneous group of conditions characterized by increased portal pressure in the absence of cirrhosis 2
• Diagnosis requires exclusion of cirrhosis and other causes of portal hypertension through liver biopsy 1
• Clinical signs include splenomegaly/hypersplenism, esophageal varices, ascites, increased hepatic venous pressure gradient, and portovenous collaterals 1
• Doppler ultrasound is the first-line investigation, with CT used for diagnostic confirmation and extension assessment 1
• MR cholangiography should be performed in patients with persistent cholestasis or biliary tract abnormalities suggesting portal biliopathy 1

Classification of Non-Cirrhotic Portal Hypertension

• Anatomical classification includes prehepatic (e.g., portal vein thrombosis), intrahepatic (e.g., idiopathic non-cirrhotic portal hypertension), and posthepatic (e.g., Budd-Chiari syndrome) causes 3
• Common causes include:
  • Extrahepatic portal vein obstruction (EHPVO) 1
  • Idiopathic non-cirrhotic portal hypertension (INCPH) 1
  • Budd-Chiari syndrome 1
  • Schistosomiasis, congenital hepatic fibrosis, and sarcoidosis 1

Management Principles

General Approach

• Management of portal hypertension should follow the same guidelines established for cirrhosis 1
• Treatment should address both the underlying cause and portal hypertension-related complications 2
• Screen for portal vein thrombosis at least every 6 months in patients with INCPH 1

Variceal Bleeding Management

• Endoscopic therapy is effective in controlling acute variceal bleeding in 95% of INCPH patients 1
• Endoscopic band ligation is preferred over sclerotherapy for both acute bleeding and secondary prophylaxis 1
• Non-selective beta-blockers should be used for primary and secondary prophylaxis of variceal bleeding, following the same approach as in cirrhosis 1, 4
• For uncontrolled bleeding, transjugular intrahepatic portosystemic shunt (TIPS) is recommended over emergency surgical shunting 1, 2

Specific Management Based on Etiology

Idiopathic Non-Cirrhotic Portal Hypertension (INCPH)

• Anticoagulation should be considered only in patients with underlying prothrombotic conditions or those who develop portal vein thrombosis 1
• Liver transplantation should be considered for patients who develop liver failure or have unmanageable portal hypertension-related complications 1

Budd-Chiari Syndrome (BCS)

• TIPS should be considered in patients not responsive to medical therapy 1
• Liver transplantation is beneficial for patients with acute liver failure or advanced chronic disease from BCS 1
• Most patients transplanted for BCS require prolonged anticoagulation 1

Extrahepatic Portal Vein Obstruction (EHPVO)

• After implementing prophylaxis for gastrointestinal bleeding:
  • Treat underlying prothrombotic conditions according to corresponding guidelines 1
  • Consider permanent anticoagulation in patients with strong prothrombotic conditions or history of intestinal ischemia 1
  • Long-term anticoagulation is indicated in cases of underlying myeloproliferative neoplasms 1

Interventional Options

• TIPS placement is effective for refractory variceal bleeding or ascites in NCPH 2
• Surgical options include portosystemic shunts and splenectomy for patients with uncontrolled variceal bleeding or symptomatic hypersplenism 4
• The meso-Rex bypass is an excellent option for children with accessible intrahepatic left portal vein 1
• Liver transplantation should be considered for patients with progressive liver failure or unmanageable portal hypertension-related complications 1, 2

Prognosis

• Patients with NCPH generally have better prognosis than those with cirrhotic portal hypertension due to preserved liver function 5, 6
• Five-year survival in patients with non-cirrhotic portal fibrosis in whom variceal bleeding can be controlled is approximately 95-100% 5
• Complications of portosystemic shunting such as hepatic encephalopathy are rare in NCPH patients due to preserved liver function 1

Monitoring and Follow-up

• Regular endoscopic surveillance for varices is essential 4
• Monitor for development of portal vein thrombosis, especially in patients with INCPH 1
• Assess for cardiopulmonary complications such as hepatopulmonary syndrome, which may develop in patients with NCPH 1