PORTAL HYPERTENSION

Portal hypertension is a condition characterised by prolonged elevation of the portal venous

pressure (normally 2-5 mm Hg). Patients developing clinical features or complications of portal
hypertension usually have portal venous pressure above 12 mm Hg. As portal hypertension
produces no symptoms it is usually diagnosed following presentation with decompensated chronic
liver disease and encephalopathy, ascites or varical bleeding.

ANATOMICAL AND PHYSIOLOGICAL CONSIDERATIONS

The liver has a dual supply from both hepatic artery and the portal vein. 80% of blood supply is
from the portal vein and 20% from the hepatic artery.
Hepatic artery arises from coeliac trunk of the aorta, along with Spleenic artery. After branching to
firm the gastroduodinal artery it branches to produce right and left hepatic arteries.
Portal vein is formed by the union of superior mesentric and spleenic veins, which in tern drain the
splachnic and spleenic beds. Coronary or left gastric vein which drains the lesser curve of the
stomach and the lower oesphagus, enter into the origin of portal vein. The inferior mesentric vein,
which drains the left hemicolon, and most of the rectum, and joins the spleenic vein just before its
confluence with the superior mesentric vein. After division into left and right branches, hepatic
artery parallels the portal system, and subsequently divides into branches corresponding to the
segmental anatomy of the liver.
The portal venous system is entirely devoid of valves. Numerous small tributaries connect the portal
and systemic venous systems, and there can evolve into major collateral channels when portal
hypertension superveins formation of such collaterals is triggered when portal pressure rises above
the normal level of 5 to 10 mm Hg.
The most important of these portal - systemic channel is the
Left gastric or coronary vein, which connect the oesophageo-cardiac venous plexus with the spleenic
or portal vein.
The short gastric and left gastroepiploic vein, connecting oesophageal and gastric plexus with the
spleenic vein.
Numerous retroperitoneal portal radicles, which connect to the left renal via the left adrenal vein.
The umbilical and periumbilical veins connecting to the left portal vein.
The inferior mesentric vein connecting via the superior haemarrhoidal veins of the systemic
circulation.
When flow to the sinusoids is significantly reduced, a compensatory increase in hepatic arterial
inflow takes place to provoide the liver O2 requirements.

AETIOLOGY AND PATHOGENESIS

Portal venous pressure is determined by the portal blood flow and by the portal vascular resistance.
Increased vascular resistance is usually the main factor producing portal hypertension irrespective
of its cause.

Causes of portal hypertension according to site of portal venous obstruction.

I Presinusoidal obstruction
a) Portal vein thrombosis
Neonal umbilical sepsis
Dehydration
Portal pyaemia
Hypercoagulable states (polycythacmia, oral contraceptives)
Periportal inflammation (pancreatitis)
Trauma (accidental, iatrogenic)

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

III Post - Sinusoidal obstruction

a) Intrahepatic
Cirrhosis (alcoholic, post necrotic, secondary biliary cirrhosis)
Alcoholic hepatitis
Viral hepatitis
Haemochromatosis
Budd - chiari syndrom (hyper coagulable state veno - ocelusive disease)
b) Extrahepatic
Budd-chiari syndrome (hepatic vein and inferior venacavathrombosis, congenital superheptic inferior
venacava webs)
Extrisic inferior vena cava compression (hepatic, renal & adrenal tumour)
Chronic congestive cardiac failure
Constrictive pericarditis

IV. Increased flow portal hypertension

Hepatic arterial-portal venous fistula (congenital, traumatic or malignant)
Spleenic or mesenteric arteriovenous fistula
Portal-hepatic venous shunts
Massive spleenomegaly (tropical spleenomegaly syndrome)
Extrahepatic portal vein obstruction is frequently the cause of portal hypertension in childhood &
adolescence, while cirrhosis causes 90% or more of portal hypertension in adults. Schistosomiasis is
the most common cause in the world, but it is infrequent outside endemic areas.
Collateral vessel formation is widespread but occurs particularly in the gastrointestinal tract,
espacially the oesophagus, stomach and rectum, in the anerior abdominal wall, and in the renal,
lumbar, ovarian and testicular vasculature

ROLE OF VASOACTIVE SUBSTANCES IN PORTAL HYPERTENSION

Several substances have been implicated as hormonal factors which act on both splachnic and
systemic circulation to produce hyperaemia. The most important of these are bile acids, serotonin,
glucagon, and prostaglandins in particular prostacyclin. Elevated levels of bile acids have been
demonstrated in patients with liver disease as promoters of splanchnic hyperaemia. Serotonin has a
powerful vasoconstrictor effect on all vascular smooth muscles, and is normally released into the
portal circulation by the enterochromaffin cells of GIP. Glucogon a patent splachnic vasodilator is
elevated in patients with portal hypertension, and accounts for 30% of the increased splanchnic
blood flow. Prostacyclin is another naturally occuring powerful vasodilator. Cirrhotic patients have
extremely high levels of urinary 6-aceto prostaglandin, a stable metabolite of prostacyclin
production reduces portal pressure in these patients.
There substances will remain vasoactive since they have avoided deactivation by the hepatocytes.
They provide a likely explanation for the systamic hyperaemia of portal hypertension.

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

Heapatic encephalopathy is a neuropsychiatric syndrome caused by liver disease.

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