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DEFINITION, ETIOLOGY, PATHOGENESIS
Idiopathic noncirrhotic portal hypertension (INCPH) is a disease of unknown etiology, characterized by the presence of portal hypertension due to presinusoidal hepatic vascular obstruction in a person with no histologic evidence of cirrhosis and no impairment of blood flow in the large vessels supplying blood to and draining blood from the liver. The condition may be associated with:
1) Chronic or recurrent gastrointestinal infections in childhood.
2) Impaired immune response, as suggested by frequent co-occurrence of immune-related diseases (systemic connective tissue diseases, acquired or congenital immunodeficiency).
3) Hematologic disorders and hypercoagulable states: Congenital thrombophilias, antiphospholipid syndrome, myeloproliferative neoplasms (essential thrombocythemia, polycythemia vera).
CLINICAL FEATURES AND NATURAL HISTORY
INCPH manifests with complications such as splenomegaly with thrombocytopenia and bleeding varicose veins in the esophagus or stomach (often the presenting symptom). In Asia, INCPH accounts for 10% to 30% of bleeding esophageal varices. The course of the disease is usually slow; portal vein thrombosis (PVT) is common (especially in patients with HIV infection); in 20% to 30% of cases, ischemia leads to a gradual parenchymal loss and liver failure after years. As the disease progresses, ascites may appear (rarely).
1. Laboratory tests: Thrombocytopenia, leukopenia, and anemia (due to hypersplenism and possibly portal gastropathy) are common; liver chemistry is usually normal.
2. Imaging studies: The appearance of the liver may be unremarkable or resemble that seen in cirrhosis. Splenomegaly is observed. Perform Doppler ultrasonography to exclude PVT and Budd-Chiari syndrome (PVT secondary to INCPH is possible).
3. Ultrasound elastography shows increased liver stiffness, but not as significant as in cirrhosis (usually <13 kPa).
4. Histologic examination shows no cirrhosis or advanced fibrosis, but findings may include hepatic sinusoidal dilatation, a reduced number of hepatic venules with thickened walls, intravascular clots, microcollaterals, and regenerative nodules with coexisting atrophic or hypertrophic hepatocytes.
There are no abnormalities specific for INCPH. The coexistence of portal hypertension with preserved liver function (normal international normalized ratio [INR] values and serum bilirubin and albumin levels) should raise a suspicion. Despite clinically significant portal hypertension, the pressure gradient in the hepatic veins is normal or only slightly increased.
The diagnosis requires exclusion of other liver diseases. Diagnostic criteria (all 5 must be met):
1) At least 1 of the following features of portal hypertension:
a) Splenomegaly, hypersplenism, or both, accompanied by another symptom of portal hypertension.
b) Varicose veins of the esophagus, stomach, or both.
c) Nonneoplastic ascites.
d) Presence of portosystemic collaterals.
2) Exclusion of cirrhosis on histologic examination of a liver biopsy specimen.
3) Exclusion of chronic liver diseases causing cirrhosis: Chronic hepatitis B or C, metabolic dysfunction–associated steatotic liver disease, alcoholic hepatitis, autoimmune hepatitis, hereditary hemochromatosis, Wilson disease, and primary cholangitis.
4) Exclusion of other diseases causing noncirrhotic portal hypertension (eg, congenital liver fibrosis, sarcoidosis, schistosomiasis).
5) Patent portal vein and hepatic veins.
1. Primary and secondary prophylaxis of bleeding from esophageal varices: Pharmacologic (nonselective beta-blocker) and/or endoscopic methods (see Cirrhosis).
2. Management of variceal bleeding: see Gastrointestinal Bleeding. In the case of persistent recurrences of bleeding, esophagogastric devascularization procedures combined with splenectomy are an alternative to portosystemic vascular anastomosis.
3. Anticoagulation: In patients with thrombophilia (see Thrombophilia (Hypercoagulable States)) or coexisting PVT. Vitamin K antagonists are preferred, but as the metabolic function of the liver and kidneys is usually preserved, the use of oral anticoagulants other than vitamin K antagonists (direct oral anticoagulants [DOACs], eg, rivaroxaban, dabigatran) is acceptable.
4. Liver transplant in the case of liver failure or recurrent bleeding that cannot be controlled by other means (very rare).
Doppler ultrasonography should be performed every 6 months as a screening test for PVT.
Bleeding esophageal or gastric varices; PVT (may be mistakenly considered the sole cause of portal hypertension). Renal (glomerulonephritis) and pulmonary (pulmonary hypertension, hepatopulmonary syndrome) complications are rare.
Prognosis is better than in patients with cirrhosis. After eradication of varicose veins by endoscopic or surgical methods, 80% to 100% of patients survive for years. Patients with severe comorbidities may have worse prognosis. No recurrence of INCPH in the transplanted liver has been observed.
