b) Intrahepatic
Congenital heptic fibrosis
Idiopathic portal hypertension, non-cirrhiotic portal fibrosis
Schistosomiasis
Myeloproliferative disorder
Systemic mastocytosis
Primary biliary cirrhosis (early)
Toxic causes (Arsenic, vit - A intoxicion, Vinylchloride, Cytotoxins)
Sarcoidosis
Gaucher’s disease
c) Extrinsic compression
Periportal lymphadenopathy tumours
Pancreatitis
II Sinusoidal obstruction
Cirrhosis
Nodular regenerative hyperplasia
Fatty liver
Wilson’s disease
CLINICAL FEATURES
The clinical featuers of portal hypertension result principally from portal venous congestion and from
collateral vessel formation. Spleenomegaly is a cardinal finding. Hyperspleenism
is common and frequently results in thromboiytopenia. Platelet counts are usually around
100x109/l. Leucopenia occurs occasionally. Collateral vessels may be visible on the anterior
abdominal wall and several radiate from the umbilicus to form a capot medusae. Oesophageal
varices causes severe bleeding. Rectal & Varices also cause bleeding. Fetor hepaticus can also be
seen.
INVESTIGATIONS
1. Radiological and endoscopic examination of the upper Gap can show varies.
2. Ultrasonography can show spleenomegaly and collateral vessels.
3. Portal venography demonstrates the site of obstruction.
4. Portal venous pressure measurements are rarely needed.
Complications
1. Variceal bleeding
Oesophageal, gastric, other
2. Congestive gastropathy
3. Hyperpleenism
4. Ascites
5. Renal failure
6. Hepatic encephalopathy
HEPATIC ENCEPHALOPATHY
Aetiology
It is due to a biochemical disturbance of brain function. The nitrogenous substances produce in the
gut are thought to be the main cause.
Precipitating factors
1. Uraemia
2. Drugs
3. Gastrointestinal bleeding
4. Excess dietary protein
5. Constipation
6. Paracentesis
7. Hypokalaemia
8. Infection
9. Trauma
10. Portasystemic shunts.
Clinical Featuers
1. Changes of intellect, personality, emotions and consciousness.
2. Inability to concentrate, confusion, disorientation, drowsiness, slurring of speech and eventually
coma
3. Flapping tremor
4. Constructive apraxia
5. Hyper-reflexia and bilateral extensor plantar responses
6. Fetor-hepaticus (a sign of liver failure)
7. Cerebellar dysfunction
8. Parkinsonian syndromes
9. Spastic paraplegia & denentia
INVESTIGATIONS
EEG
Diffuse slowing of normal alpha waves with eventual development of delta waves
Management
1. Remove the precipitating factors
2. To reduce protein intake
3. Suppress the production of neutoxins in the bowel