Portal Hypertension


Definition

Portal HTN is characterized by prolonged elevation of portal venous pressure.
Normal portal venous pressure = 2-5mmHg.
In Portal HTN portal venous pressure > 12mmHg.

PORTAL HTN
PHTN

ETIOLOGY OF PORTAL HTN

  • Extrahepatic post-sinusoidal -Budd-Chiari syndrome.
  • Intrahepatic post-sinusoidal -Veno-occlusive disease.
  • Sinusoidal
    -Cirrhosis (most common )
    -Cystic liver disease.
    -Partial nodular transformation of liver.
    -Metastatic malignant disease.
  • Intrahepatic pre-sinusoidal -Schistosomiasis (common)
    -Sarcoidosis.
    -Congenital hepatic fibrosis.
    -Vinyl chloride.
    -Drugs.
  • Extrahepatic pre-sinusoidal
    -Portal vein thrombosis.
    -Abdominal trauma, including surgery.
    -Malignant disease of pancreas or liver.
    -Pancreatitis.
    -Congenital.

PATHOGENESIS OF PORTAL HTN

  • -Extrahepatic portal vein obstruction common cause in childhood & adolescence while cirrhosis in adults.
    -Schistosomiasis common cause of portal HTN world wide.
    -Portal venous pressure determined by
    -Portal blood flow.
    -Portal vascular resistance Increased portal vascular resistance is main factor producing portal HTN .
    -Increased portal vascular resistance – flow of portal blood to liver – development of collateral vessels – portal blood enters the systemic circulation directly.
    -Collateral vessels formation sites :- esophagus, stomach, rectum & anterior abdominal wall.
    -Increased portal blood flow contributing to HTN is not dominating factor.

CLINICAL FEATURES

  • Splenomegaly is cardinal feature. Usually less than 5cm below costal margin. Manifesting as thrombocytopenia & leucopenia.
  • Collateral vessels visible as caput medusae.
  • Hematemesis & melaena.
  • Fetor hepaticus due to portosystemic shunting of blood.
  • Cruveilhier-Baumgarten syndrome association of dilated abdominal wall veins & loud venous hum at umbilicus.
  • Liver may be enlarged or shrunken.
    a)small, contracted, fibrotic liver – very high portal HTN
    b)soft liver – extrahepatic portal vein obstruction
    c)firm liver – cirrhosis.
  • Hemorrhoid’s may occur.
  • Ascites occurs partly.

INVESTIGATIONS

  • Barium swallow -varices as feeling defect.
  • Upper GIT Endoscopy.
  • USG :- detects size of liver & spleen.
  • Portal venography :- demonstrates site & cause of portal venous obstruction.
  • Portal venous pressure measurement :- wedge hepatic venous pressure (WHVP) – free hepatic venous pressure (FHVP)= hepatic venous pressure gradient (HUPG).
    Low HUPG indicates pre-sinusoidal portal pressure.

COMPLICATIONS

  • Variceal bleeding :- esophageal, gastric, other (rare ).
  • Congestive gastropathy.
  • Hypersplenism.
  • Ascites.
  • Renal failure.
  • Hepatic encephalopathy.

TREATMENT

  • Absolute bed rest.
  • Salt free high protein diet. Protein restricted at onset of encephalopathy.
  • Rx of infections by proper AMA.
  • Rx of variceal hemorrhage by
    • Blood transfusion.
    • Vasopressin, octreotide or infusion of somatostatin.
    • Sengstaken tube.
    • Endoscopic sclerotherapy.
    • TIPSS.
  • Rx of hepatic encephalopathy.