Classification and etiology
1. Prehepatic pre-sinusoidalPortal vein thrombosis due to sepsis (umbilical, portal pyaemia) or procoagulopathy or secondary to cirrhosis
Portal v. thrombosis
Splenic v. thrombosis (only splenomegaly, gastric varices)
Abdominal trauma including surgery
Increased incidence in pediatrics
Congenital atresia of the portal vein
External compression - Wilms tumor
2. Intrahepatic pre-sinusoidalSchistosomiasis
Inherited causes: Wilson's, hemochromatosis, CF, Congenital hepatic fibrosis
Drugs
Vinyl chloride
Sarcoidosis
3. SinusoidalCirrhosis (most common)
Alcoholic (most common)
Chronic hepatitis B, C
Autoimmune hepatitis (AIH)
PBC
PSC
NASH
Biliary atresia
Cryptogenic
Polycystic liver disease
Nodular regenerative hyperplasia
Metastatic malignant
4. Intrahepatic post-sinusoidal - Veno-occlusive disease
5. Post-hepatic post-sinusoidal
Budd-Chiari syndrome:
Primary Budd-Chiari
Secondary Budd-Chiari
Polycythemia rubra vera
pregnancy/ post-partum
OCP use
PNH
Hepatocellular carcinoma
Inherited/acquired hypercoagulability
Constrictive pericarditis
right-sided heart failure
Budd-Chiari syndrome: occlusion of the hepatic veins - classical triad: abd pain, ascites, hepatomegaly.
Complications
Hepatic encephalopathy ensues because suffcient blood is no longer being detoxified by the liver (and the liver is usually diseased to begin with.) Ammonium levels increase. Sx: asterixis (wrist flap), confusion, psychiatric changes, lethargy, coma
Splenomegaly occurs as a direct result of increased venous pressure.
Ascites occurs due to increased hydrostatic pressure in the abdominal circulation. This fluid is pushed into the peritoneal space.
Malnutrition may co-present with portal hypertension as a symptom of liver disease.
Hemorrhoids occur as a direct result of increased venous pressure.
GI bleeding, namely from esophageal varices, occurs because blood is shunted through the esophageal veins. These can rupture and result in massive hematemesis. Gastric or rectal varices may form, too.
Management
non-selective β-blockers (propanolol, nadolol) decrease risk of bleeding from varices
transjugular intrahepatic portosystemic shunt (TIPS): to ↓ portal venous pressure
shunt between portal and hepatic vein via transjugular vein catheterization and percutaneous puncture of portal vein
can be used to stop acute bleeding or prevent rebleeding or treat ascites
shunt usually remains open for <1 yr
complications: hepatic encephalopathy, deterioration of hepatic function
contraindicated with severe liver dysfunction
most commonly used as a "bridge" to liver transplant
other surgically created shunts (rare): portacaval, distal spleno-renal (Warren shunt)
Tx
β-blockers
Nitrates
Shunts [e.g. transjugular intrahepatic portosystemic shunt (TIPS)]