What is the treatment for non-cirrhotic portal fibrosis (NCPF)?

Treatment of Non-Cirrhotic Portal Fibrosis (NCPF)

The treatment of non-cirrhotic portal fibrosis should focus on managing portal hypertension and its complications, primarily through endoscopic therapy for variceal bleeding and pharmacological management with non-selective beta blockers. 1

Clinical Presentation and Diagnosis

• NCPF (also called idiopathic portal hypertension or hepatoportal sclerosis) presents with signs of portal hypertension in the absence of cirrhosis 1
• Diagnosis requires exclusion of cirrhosis and other causes of non-cirrhotic portal hypertension 1
• Clinical features typically include:
  • Massive splenomegaly with anemia 2
  • Preserved liver function 2
  • Esophageal and gastric varices 2
  • Portal hypertension with patent portal and hepatic veins 1

Management Approach

1. Management of Variceal Bleeding

• First-line treatment: Endoscopic therapy with band ligation or sclerotherapy for acute variceal bleeding 1

  • Endoscopic therapy is effective in controlling acute bleeding in approximately 90-95% of patients 3
  • Endoscopic band ligation is preferable to sclerotherapy due to superior outcomes 1

• Pharmacological therapy: Non-selective beta blockers (NSBBs) should be used for prevention of variceal bleeding 1, 4

  • NSBBs reduce portal pressure by decreasing cardiac output and causing splanchnic vasoconstriction 4
  • A reduction of hepatic venous pressure gradient by 10-12% with beta blockers protects against variceal bleeding 4

• Combination therapy: For secondary prophylaxis (prevention of rebleeding), combined therapy with NSBBs plus endoscopic band ligation is recommended 1

2. Management of Refractory Cases

• Transjugular Intrahepatic Portosystemic Shunt (TIPS): Consider for uncontrolled bleeding despite endoscopic and pharmacological therapy 1

  • TIPS has shown good clinical outcomes in NCPF with good control of ascites and 5-year survival of 60-89% 1
  • However, hepatic encephalopathy rates may be high (>35%) 1

• Surgical shunts: Consider when endoscopic therapy fails or for symptomatic hypersplenism 3

  • Side-to-side lienorenal shunt has been used historically 2
  • However, prophylactic surgical shunts have high delayed morbidity (47%) including portosystemic encephalopathy 5

3. Management of Other Complications

• Gastric varices: Present in about 25% of patients 6

  • Can be managed with cyanoacrylate glue injection or surgery 3

• Hypersplenism: Consider splenectomy for symptomatic hypersplenism when other treatments fail 3

• Portal vein thrombosis (PVT): Higher incidence in NCPF compared to cirrhosis 1

  • Screen for PVT at least every 6 months 1
  • Early anticoagulation therapy leads to recanalization in 54% of patients 1

Special Considerations

• Liver transplantation: Consider for patients who develop liver failure or unmanageable portal hypertension-related complications 1

  • Although rare, a minority of patients may develop progressive liver failure over time 1

• Anticoagulation: Consider in patients with NCPF who have underlying prothrombotic conditions or who develop PVT 1

  • However, this remains debated due to the risk of gastrointestinal bleeding 1

• Children with NCPF: Presentation with variceal bleeding is less common than in adults 7

  • Endoscopic sclerotherapy is effective in pediatric patients 7

Prognosis

• Overall prognosis is good with 5-year survival rates >95% in patients where variceal bleeding can be controlled 3, 6
• Mortality is primarily related to variceal bleeding rather than liver failure 5