What is the management of Non-Cirrhotic Portal Fibrosis (NCPF)?

Management of Non-Cirrhotic Portal Fibrosis (NCPF)

Endoscopic variceal ligation is the first-line treatment for acute variceal bleeding in NCPF, combined with non-selective beta-blockers for prevention of rebleeding, with TIPS reserved for refractory cases. 1, 2

Initial Assessment and Diagnosis

• NCPF presents with portal hypertension, massive splenomegaly, and preserved liver function in the absence of cirrhosis 1, 3, 4
• Screen for portal vein thrombosis at baseline and every 6 months, as PVT incidence is higher in NCPF compared to cirrhosis 1, 2
• Hepatic venous pressure gradient (HVPG) is typically low (mean 4.9 mmHg) despite significant portal hypertension, distinguishing NCPF from cirrhosis 5

Management of Variceal Bleeding

Acute Bleeding Episode

• Initiate vasoactive drugs (somatostatin/octreotide or terlipressin) immediately when variceal bleeding is suspected 6, 7
• Perform endoscopic variceal ligation (EVL) within 12 hours once hemodynamically stable—EVL is superior to sclerotherapy with 90-95% effectiveness 1, 3, 4, 8
• Administer short-term antibiotic prophylaxis (maximum 7 days); intravenous ceftriaxone 1 g/24h is preferred 7
• Transfuse red blood cells conservatively: start when hemoglobin reaches 7 g/dL with goal of 7-9 g/dL, as excessive transfusion paradoxically increases portal pressure 6, 7
• Continue vasoactive drugs for 2-5 days post-endoscopy 7

Prevention of First Bleeding (Primary Prophylaxis)

• Start non-selective beta-blockers (NSBBs) for patients with high-risk varices (large varices or those with red signs) 1, 2, 7
• NSBBs reduce portal pressure by decreasing cardiac output and causing splanchnic vasoconstriction 1, 2
• Target HVPG reduction of 10-12% or to <12 mmHg protects against variceal bleeding 1, 2, 7
• Carvedilol 12.5 mg/day is more effective than traditional NSBBs (propranolol/nadolol), achieving hemodynamic response in 50-75% of patients 7

Prevention of Rebleeding (Secondary Prophylaxis)

• Combination therapy with NSBBs plus endoscopic band ligation is recommended for secondary prophylaxis 1
• Continue NSBBs long-term after transitioning from vasoactive drugs 7
• Repeat EVL sessions until variceal eradication is achieved 3, 4, 8

Management of Refractory Cases

TIPS Placement

• TIPS should be considered for uncontrolled bleeding despite endoscopic and pharmacological therapy 1, 2
• TIPS demonstrates good technical success in NCPF with 5-year survival of 60-89% 1, 2
• Hepatic encephalopathy rates may exceed 35% after TIPS, though risk is lower than in cirrhosis due to preserved liver function 1, 2
• TIPS surveillance is mandatory: imaging at 1-6 months post-procedure, then every 6-12 months to detect stenosis/occlusion 2

Surgical Options

• Surgical shunts (proximal splenorenal shunt) are effective in preventing variceal bleeding but carry 47% delayed morbidity including encephalopathy (18%), glomerulonephritis, and ascites 9
• Surgery is now reserved for failure of endoscopic therapy, symptomatic hypersplenism, or gastric varices not amenable to endoscopic treatment 3, 4, 8

Management of Portal Vein Thrombosis

• Early anticoagulation leads to recanalization in 54% of PVT cases when initiated promptly 1, 2
• Anticoagulation should be considered for patients with prothrombotic conditions or acute PVT, though balanced against bleeding risk 1, 2
• TIPS can be considered for extensive PVT with bowel ischemia 2

Management of Other Complications

Portal Hypertensive Gastropathy

• Treat bleeding from portal hypertensive gastropathy with portal pressure-lowering measures (NSBBs) rather than hemostatic correction 6, 2, 7
• TIPS should be considered for refractory bleeding when medical therapy fails 2, 7

Hypersplenism

• Symptomatic hypersplenism with severe cytopenias may require surgical intervention (splenectomy with or without devascularization) 9, 4, 8

Advanced Interventions

• Liver transplantation should be considered for unmanageable portal hypertension complications or progressive liver failure, though indications are limited since liver function is typically preserved in NCPF 1, 2

Critical Pitfalls to Avoid

• Do NOT routinely correct coagulation abnormalities before prophylactic band ligation in stable NCPF patients, as this provides no benefit 7
• Do NOT use tranexamic acid in active variceal bleeding—it is contraindicated and increases thrombotic risk 6, 7
• Avoid large-volume blood product transfusion as it paradoxically increases portal pressure and worsens bleeding 6, 7
• Temporarily suspend NSBBs during acute bleeding with hypotension (systolic BP <90 mmHg or MAP <65 mmHg) 7

Prognosis

• NCPF has excellent prognosis with 5-year survival >95% when variceal bleeding is controlled 4, 8
• Spontaneous portosystemic shunts develop in 15.9% of patients and protect against variceal bleeding 3
• Liver failure, jaundice, ascites, and hepatic encephalopathy are rare in NCPF 3, 4, 8