Differential Diagnosis for Acute Hepatic Insufficiency with Portal Vein Thrombosis and Fever

Single Most Likely Diagnosis

• Portal Vein Thrombosis due to Cirrhosis: This is the most likely diagnosis because cirrhosis is a common cause of portal vein thrombosis, and the presence of acute hepatic insufficiency and fever suggests a possible complication of cirrhosis, such as spontaneous bacterial peritonitis or hepatocellular carcinoma.

Other Likely Diagnoses

• Budd-Chiari Syndrome: This condition, caused by hepatic vein thrombosis, can lead to acute hepatic insufficiency and may be associated with portal vein thrombosis. Fever can occur due to associated complications.
• Pyogenic Liver Abscess: A liver abscess can cause fever and hepatic insufficiency, and portal vein thrombosis can occur as a complication. The presence of an abscess would explain the fever.
• Cholangitis: Infection of the biliary tract can lead to fever, hepatic insufficiency, and potentially portal vein thrombosis, especially if the infection is severe and involves the portal vein.

Do Not Miss Diagnoses

• Septic Thrombophlebitis of the Portal Vein (Pylephlebitis): Although less common, this condition is life-threatening and can present with fever, hepatic insufficiency, and portal vein thrombosis. It is usually associated with intra-abdominal infections.
• Hepatic Artery Thrombosis: In patients with pre-existing liver disease or after liver transplantation, hepatic artery thrombosis can lead to acute hepatic insufficiency and may be associated with portal vein thrombosis. Fever can be present due to ischemic liver injury or superimposed infection.

Rare Diagnoses

• Veno-occlusive Disease (Sinusoidal Obstruction Syndrome): This rare condition, often associated with hematopoietic stem cell transplantation or certain medications, can cause hepatic insufficiency and may lead to portal vein thrombosis. Fever can occur due to the underlying condition or superimposed infection.
• Paroxysmal Nocturnal Hemoglobinuria (PNH): A rare, acquired, life-threatening disease of the blood characterized by the destruction of red blood cells, activation of the coagulation system, and potential for thrombosis, including portal vein thrombosis. Hepatic insufficiency and fever can be part of the clinical presentation.
• Inherited Thrombophilias: Conditions such as Factor V Leiden or antithrombin deficiency can increase the risk of portal vein thrombosis. While less common, they should be considered, especially in younger patients without other risk factors for cirrhosis or liver disease.