Portal Hypertension

Portal Hypertension Portal hypertension is characterized by: Prolonged elevation of of the portal venous pressure (normally 2-5 mm Hg). Patients developing clinical features or complications usually have portal venous pressure > 12 mm Hg.

Causes Extrahepatic post-sinusoidal: Intrahepatic post-sinusoidal: Budd-Chiari syndrome Intrahepatic post-sinusoidal: Veno-occlusive disease Sinusoidal: Cirrhosis Cystic liver disease Partial nodular transformation of the liver Metastatic malignant disease.

Causes Extra-hepatic pre-sinusoidal: Intra-hepatic pre-sinusoidal: Portal vein thrombosis due to: Sepsis (umbilical, portal pyemia) Procoagulopathy (thrombotic disease, OC pills, pregnancy, secondar – cirrhosis) Abdominal trauma including surgery. Malignant disease of pancreas or liver Pancreatitis Congenital Intra-hepatic pre-sinusoidal: Schistosomiasis Sarcoidosis Congenital hepatic fibrosis Vinyl chloride Drugs

Clinical Features Clinical features result from: Splenomegaly: Portal venous congestion Collateral vessels formation. Splenomegaly: If splenomegaly can not be detected by examination or by US, diagnosis of portal hypertension is unlikely. Rarely enlarged > 5 cm below the left costal margin in adults. More marked in children and adolescents. Results in: Thrombocytopenia. Leukopenia Anemia (hardly attributed to hypersplenism. Collateral vessels: Anterior abdominal wall, occasionally form caput medusae and rarely blood flow cause venous hum on auscultation (Cruveilhier-Baumgarten syndrome)

Clinical Features collateral vessels (cont.) Fetor hepaticus: Esophageal Gastric Rectal Fetor hepaticus: Result from portosystemic shunting of blood This allows mercaptans to pass directly to the lungs.

Investigations: Radiological & endoscopic examination of upper GIT: It can show varices This establishes the presence of portal hypertension but not its cause. Imaging (particularly ultrasonography): Show features of portal hypertension (splenomegaly & collateral vessels) Sometimes can indicate the cause (liver disease, portal vein thrombosis)

Investigations: Portal venography: It demonstrates the site and often the cause. It is performed prior to surgical intervention. Portal venous pressure measurements: Are rarely needed Can be used to: Confirm the presence of portal hypertension Differentiate sinusoidal and pre-sinusoidal forms.

Complications: Variceal bleeding: Congestive gastropathy Hypersplenism Esophageal Gastric Other (rare) Congestive gastropathy Hypersplenism Ascites Renal failure Hepatic encephalopathy

Variceal Bleeding: It usually occurs from: Predisposing factors: Esophageal varices (3-5 cm from esophago-gastric junction) Gastric varices Predisposing factors: Size of the varices Endoscopic variceal features including: Red spots Red strips High portal pressure Liver failure Use of drugs capable of causing mucosal erosion e.g. NSAIDs & Aspirin Variceal bleeding is often severe Recurrent bleeding occurs if preventative treatment is not given

VARICEAL BLEEDING

general factors that predispose to bleeding Variceal bleeding occurs from oesophageal varices that are usually located within 3-5 cm of the oesophago-gastric junction, or from gastric varices. The mortality from bleeding oesophageal varices is high (up to 50% in those with advanced liver disease) general factors that predispose to bleeding The size of the varices, endoscopic variceal features such as red spots, high portal pressure liver failure. Drugs capable of causing mucosal erosion, such as salicylates and other NSAIDs, can also precipitate bleeding.

Management of acute variceal bleeding The priority in acute bleeding from oesophageal varices is to restore the circulation with blood and plasma. Even in patients with known varices, the source of bleeding should always be confirmed by endoscopy because about 20% of such patients are found to be bleeding from some other lesion, especially acute gastric erosions.

Resuscitation Assess the general condition of the patient - pulse and blood pressure. Insert an intravenous line and obtain blood for grouping and cross matching, haemoglobin, PT/INR, urea, electrolytes, creatinine, liver biochemistry and blood cultures. Restore blood volume with plasma expanders or, if possible, blood transfusion. All patients with cirrhosis and gastrointestinal bleeding should receive broad-spectrum antibiotics such as ciprofloxacin because sepsis is common and treatment with antibiotics has been shown to improve outcome.

Local measures The measures used to control acute variceal bleeding include endoscopic therapy (banding or sclerotherapy), balloon tamponade oesophageal transection.

Banding or sclerotherapy This is the most widely used initial treatment and is undertaken if possible at the time of diagnostic endoscopy. It stops variceal bleeding in 80% of patients and can be repeated if bleeding recurs. Active bleeding at endoscopy may make endoscopic therapy difficult; in such cases bleeding should be controlled by balloon tamponade prior to endoscopic therapy.

Balloon tamponade This technique employs a Sengstaken-Blakemore tube possessing two balloons which exert pressure in the fundus of the stomach and in the lower oesophagus respectively. Balloon tamponade will almost always stop oesophageal and gastric fundal variceal bleeding, but only creates time for the use of more definitive therapy. Particular care should be taken to avoid pulmonary aspiration whilst inserting the tube; patients unable to protect their airway should be intubated.

Oesophageal transection Transection of the varices can be performed with a stapling gun, although it carries some risk of subsequent oesophageal stenosis, and is normally combined with splenectomy.

Reduction of portal venous pressure 1) Pharmacological treatment Terlipressin is the current drug of choice and releases the vasoconstrictor (vasopressin), over several hours in amounts sufficient to reduce the portal pressure without producing systemic effects. Octreotide, the synthetic form of somatostatin, reduces the portal pressure and can stop variceal bleeding. It has few side-effects.

2) TIPSS and shunt surgery Transjugular intrahepatic portosystemic stent shunt, can be used for acute bleeding not responding to sclerotherapy or banding. Emergency portosystemic shunt surgery has a mortality of 50% or more and is now virtually never used for treating active variceal bleeding.

Prevention of recurrent bleeding Band ligation The technique is generally more effective than sclerotherapy, has fewer side-effects and is now the treatment of choice. Prophylactic acid suppression with proton pump inhibitors may reduce the risk of secondary bleeding from banding-induced ulceration.

Sclerotherapy Sclerotherapy, a technique in which varices are injected with a sclerosing agent, has now been largely abandoned in preference to banding ligation. The treatment could cause transient chest or abdominal pain, fever, transient dysphagia and occasionally oesophageal perforation. Oesophageal strictures may also develop.

TIPSS This technique uses a stent placed between the portal vein and the hepatic vein in the liver to provide a portosystemic shunt and therefore reduce portal pressure. Hepatic encephalopathy may occur following TIPSS and is managed by reducing the shunt diameter.

Portosystemic shunt surgery Although surgery prevents recurrent bleeding, it carries a high mortality and often leads to encephalopathy. portosystemic shunts are now reserved for patients in whom other treatments have not been successful and are offered only to those with good liver function.

Beta-adrenoceptor antagonists (ß-blockers) Propranolol (80-160 mg/day) or nadolol reduces the portal venous pressure in portal hypertension and has been used to prevent recurrent variceal bleeding.