portal hypertension

Caput medusae
bei Pfortaderhochdruck bei Leberzirrhose in der Computertomographie. (sagittal)

Caput medusae
mit Durchtritt oberhalb des Nabels über Verbindungen der rekanalisierten Nabelvene zu den epigastrischen Gefäßen.

Caput medusae
bei Pfortaderhochdruck bei Leberzirrhose in der Computertomographie. (axial)

Fundusvarizen
bei Leberzirrhose mit portaler Hypertension. Computertomographie axial.

Großkalibrige
portosystemische Umgehungskreisläufe über Venen der Bauchwand. Computertomographie: Rekonstruktion mit Volume-rendering-Technik

Großkalibrige
portosystemische Umgehungskreisläufe über Venen der Bauchwand. Computertomographie: Rekonstruktion mit Volume-rendering-Technik

Großkalibrige
portosystemische Umgehungskreisläufe über Venen der Bauchwand. Computertomographie: Rekonstruktion mit Volume-rendering-Technik

Hepatofugal
flow in portal vein in ultrasound - patient has known cirrhosis
Portal hypertension is defined as hepatic venous pressure gradient (HVPG) greater than 5 mmHg. HVPG is a surrogate for the portosystemic pressure gradient. Clinically significant portal hypertension is defined as a gradient greater than 10 mmHg and variceal bleeding may occur at a gradient greater than 12 mmHg.
Pathology
Causes can be split by their relation to the hepatic sinusoids:
Prehepatic causes
- portal vein thrombosis
- extrinsic compression of portal vein
- congenital portal vein stenosis
- arteriovenous fistula
- SVC obstruction (downhill varices)
Hepatic / sinusoidal causes
- cirrhosis
- viral hepatitis
- Schistosomiasis (S. mansoni or S. japonicum)
- congenital hepatic fibrosis
- infiltrative liver diseases
- polycystic liver disease
- nodular regenerative hyperplasia
- idiopathic portal hypertension
Posthepatic causes
- Budd-Chiari syndrome
- sinusoidal obstruction syndrome
- congestive cardiac failure
- constrictive pericarditis
Subtypes
Radiographic features
Ultrasound
- dilated portal vein (>13 mm): non-specific
- biphasic or reverse flow in portal vein (late stage): pathognomonic
- recanalization of paraumbilical vein: pathognomonic
- portal-systemic collateral pathways (collateral vessels/varices)
- splenomegaly
- ascites
- cause of portal hypertension often identified, most commonly cirrhosis
CT and MRI
- dilated portal vein +/- mesenteric veins
- contrast enhancement of paraumbilical vein: pathognomonic
- collateral vessels/varices: these are many and can include
- coronary venous collaterals: considered one of the commonest
- esophageal collaterals
- paraumbilical collaterals
- abdominal wall collaterals
- perisplenic collaterals
- retrogastric collaterals
- paraesophageal collaterals
- omental collaterals
- retroperitoneal collaterals
- mesenteric collaterals
- splenorenal collaterals
- gastrorenal collaterals
- splenomegaly
- ascites
- cause of portal hypertension often identified, most commonly liver cirrhosis
Treatment and prognosis
- dietary restriction
- medication: propranolol, diuretics
- interventional procedures
- transjugular intrahepatic portosystemic shunt (TIPS)
- surgical portosystemic shunt
- surgical splenorenal shunt
- balloon dilatation of hepatic vein (in case of thrombosis/web in hepatic vein)
- transhepatic clot thrombolysis (in portal vein thrombosis)
- splenic artery embolization
- liver transplantation
- treatment of complications
Complications
- ascites
- esophageal varices and gastric varices
- variceal bleeding (30-50% mortality with each bleed)
- portal hypertensive gastropathy/enteropathy/colopathy
- hepatic encephalopathy
- hepatorenal syndrome
- hepatopulmonary syndrome
- congestive splenomegaly and hypersplenism
Differential diagnosis
Dilatation of splenic veins at the splenic hilum without splenomegaly may occur in situations such as state of increased perfusion of splenic tissue associated with an immune response .
Siehe auch:
- Ascites
- Leberzirrhose
- Budd-Chiari syndrome
- portosystemische Umgehungskreisläufe
- Splenomegalie
- Pfortaderthrombose
- portal hypertensive gastropathy / enteropathy / colopathy
- transjugulärer intrahepatischer portosystemischer Shunt
- Hepatische Enzephalopathie
- hepatic veno-occlusive disease
- Cruveilhier-Baumgarten-Syndrom
und weiter:
- esophageal varices
- kavernöse Transformation Pfortader
- abdominal manifestations of cystic fibrosis
- hepatopetal
- Arterielle Hypertonie
- Fundusvarizen
- hepatofugal
- COACH syndrome
- Morbus Osler-Weber-Rendu
- duodenal varices
- Caput medusae
- splenic hydatid infection
- misty mesentery
- Milzarterienaneurysma
- portale Biliopathie
- Gamma-Gandy bodies
- pathological conditions of hepatic vascularization
- renal vein varices
- Wassermelonenmagen
- abdominal manifestations of sickle cell disease
- superior mesenteric venous (SMV) varices
- Hypertonus
- portalvenöse Verkalkungen
- erweiterte Vena azygos
- enlarged azygos vein from portal hypertension
- Caput medusae Zeichen bei DVA (developmental venous anomaly)
- high signal intensity in the basal ganglia in portal hypertension
- portopulmonale Hypertension
- splenic varices
