Portal HTN

Portal HTN is characterised by prolonged elevation of portal venous pressure .
   Normal portal venous pressure = 2-5mm Hg
   In Portal  HTN portal venous pressure > 12mm Hg
1. Extrahepatic post - sinusoidal     -Budd-Chiari syndrome
2. Intrahepatic post - sinusoidal     -Veno - occlusive disease
3. Sinusoidal
    -Cirrhosis (most common )
    -Cystic liver disease
    -Partial nodular transformation of liver
    -Metastatic malignant disease 4. Intrahepatic pre - sinusoidal      -Schistosomiasis (common)
    -Congenital hepatic fibrosis
    -Vinyl chloride
5. Extrahepatic presinusoidal
    -Portal vein thrombosis
    -Abdominal trauma ,including surgery
    -Malignant disease of pancrease  or liver
  Extrahepatic postal 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 produsing 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 :- oesophagus , stomach, rectum & anterior abdominal wall.
     Increased portal blood flow contributing to HTN is not dominating factor
1. Splenomegaly is cardinal feature. Usually less than 5cm below costal margin. Manifesting as thrombocytopenia & leucopenia
2. Collateral vessels visible as caput medusae
3. Haematemesis & melaena
4. Fetor hepaticus due to portosystemic shunting of blood 5. Cruveilhier-Baumgarten syndrome association of dilated abdominal wall veins & loud venous hum at umbilicus.  
6. 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
7. Haemorrhoids may occur
8. Ascites occurs partly
1. Barium swallow -varices as feeling defect
2. Upper GIT Endoscopy
3. USG :- detects size of liver & spleen
4. Portal venography :- demonstrates site & cause of portal venous obstruction
5. 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.
 1. Variceal bleeding :- oesophageal, gastric, other (rare ).
2. Congestive gastropathy    
3. Hypersplenism
4. Ascites
5. Renal failure
6. Hepatic encephalopathy
1. Absolute bed rest
2. Salt free high protein diet. Protein restricted at onset of encephalopathy
3. Rx of infections by proper AMA
4. Rx of variceal haemorrhage by
    - blood transfusion
   - vasopressin , octreotide or  infusion of somatostatin
    - Sengstaken tube
    - Endoscopic sclerotherapy
    - TIPSS
5. Rx of hepatic encephalopathy  

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