Noncirrhotic Portal Hypertension

Thank you, Polly, for presenting the case of a young man with portal hypertension without a definitive diagnosis of cirrhosis who presented with AKI and profound anemia. We had a great report discussing our approach to noncirrhotic portal hypertension. Love,

SamMy

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TOP PEARLS

  • Schistosomiasis is one of the most common causes of noncirrhotic portal hypertension world-wide!
  • Think about Non-cirrhotic portal hypertension in terms of anatomical causes: pre-hepatic vs intra-hepatic vs post-hepatic! Another way is to think about the pathophysiologic process (fibrotic vs infiltrative vs compressive vs obstructive processes)

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MORE ON NONCIRRHOTIC PORTAL HYPERTENSION  

Pre-Hepatic Causes of Noncirrhotic Portal Hypertension

  1. Portal vein thrombosis
  2. Splenic vein thrombosis: complication of pancreatitis, pancreatic tumor
  3. Splanchnic AV fistulas

Intrahepatic Causes of Noncirrhotic Portal Hypertension

  1. Presinusoidal – Developmental abnormalities (adult polycystic liver disease, congenital hepatic fibrosis, AV fistulas) – Biliary disease (biliary cirrhosis, PSC, toxic biliary injury from vinyl chloride) – Neoplastic occlusion of intrahepatic portal vein (lymphoma, CLL, epithelial malignancies) – Granulomatous processes (schistosomiasis, sarcoidosis) – Idiopathic noncirrhotic portal hypertension
  2. Sinusoidal – Think about processes that can infiltrate into the space of Disse: amyloid, light-chain deposition, mastocytosis, Gaucher disease – Think about processes that cause fibrosis of the space of Disse: nonalcoholic fatty liver, viral hepatitis, CMV, schistosomiasis, toxins (amiodarone, methotrexate, alcohol, vinyl chloride, copper) – Think about processes that cause compression of the space of Disse: hypertrophied hepatocytes
  3. Postsinusoidal – Budd-Chiari, vascular malignancies, granulomatous phlebitis (sarcoid, Mycobacterium avium), venoocclusive disease

Post-Hepatic Causes of Noncirrhotic Portal Hypertension

  1. IVC obstruction (Budd Chiari)
  2. Cardiac causes: constrictive pericarditis and restrictive cardiomyopathy

Schistosomiasis!

    • One of the most common causes of noncirrhotic portal hypertension world-wide!
  • Shout-out to a prior VA chief blog post on Schistosomiasis:

https://ucsfmed.wordpress.com/2016/10/04/schistosomiasis/

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Evernote Link: https://www.evernote.com/shard/s338/sh/0477960f-e237-47bc-81bf-2e6d0cb8dcd5/4eeea673da46531a6f9dd6bffbc609c8

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