Table of ContentsDefinition / general | Essential features | Terminology | Etiology | Clinical features | Diagnosis | Microscopic (histologic) description | Microscopic (histologic) images | Sample pathology report | Board review style question #1 | Board review style answer #1
Cite this page: Gonzalez R. Portal hypertension. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/liverportalhypertension.html. Accessed May 15th, 2021.
Definition / general
- Increased blood pressure within portal venous system
- Causes are numerous and can be divided into prehepatic, intrahepatic and posthepatic
- Cirrhosis is most common cause
- Patients without cirrhosis typically are labeled with "noncirrhotic portal hypertension"
- Prehepatic causes include obstructive thrombosis / narrowing of portal vein and shunting of blood into splenic vein via splenomegaly
- Intrahepatic causes include cirrhosis, schistosomiasis (most common noncirrhotic cause in the world), nodular regenerative hyperplasia, granulomatous disease, congenital hepatic fibrosis, hepatoportal sclerosis, severe fatty liver disease including NASH
- Posthepatic causes include congestive heart failure, constrictive pericarditis, hepatic vein outflow obstruction (e.g., Budd-Chiari syndrome), myeloproliferative disorders
- Some cases may be idiopathic (Orphanet J Rare Dis 2015;10:67)
- Clinical complications include ascites, portosystemic venous shunts (hemorrhoidal veins, esophageal varices, etc.), congestive splenomegaly, hepatic encephalopathy
- Gold standard is measurement of hepatic venous pressure gradient via portal vein catheterization
- Ultrasound is noninvasive and can detect signs of portal hypertension
Microscopic (histologic) description
- Findings vary depending on causative disease; changes may be those of cirrhosis, schistosomiasis, nodular regenerative hyperplasia, hepatoportal sclerosis, granulomatous disease, outflow obstruction or others
- Collapsed portal vein radicles may be seen in pediatric patients (Pediatr Dev Pathol 2003;6:421)
Microscopic (histologic) images
Sample pathology report
- Liver, biopsy:
- Liver with prominent nodular regenerative hyperplasia (see comment)
- Comment: The finding likely explains the patient’s reported portal hypertension. A reticulin stain confirms nodular regenerative hyperplasia. Trichrome and iron stains are unremarkable.
Board review style question #1
- A patient is found to have portal hypertension in the absence of cirrhosis. Considering the most common overall cause of noncirrhotic portal hypertension, which of the following is most likely to be seen on liver biopsy?
- Herniated portal veins
- Nodular hepatocyte parenchyma
- Parasite eggs
- Sinusoidal fibrosis