Vascular diseases of the liver are relatively uncommon when compared with the aforementioned conditions, and their diagnoses are unlikely to be made during an evaluation in the emergency department. However, these conditions warrant brief inclusion because their consideration in the evaluation of the acutely ill patient is key to timely diagnosis, treatment, and ultimately improved outcome. These conditions include portal vein thrombosis, hepatic vein thrombosis (Budd-Chiari syndrome), and nonthrombotic venoocclusive disease.
Portal vein thrombosis can result as a late complication of abdominal trauma, sepsis, pancreatitis, and hypercoagulable states and in neonates with umbilical vein infection. Portal hypertension and related complications develop in a subacute manner. Splenomegaly may occur in the absence of hepatomegaly, and liver histology is normal. The diagnosis is made by angiography, and therapy is surgical. 32
Hepatic vein thrombosis, or Budd-Chiari syndrome, has both acute and chronic presentations that include abdominal pain, hepatomegaly, ascites, and usually mild alterations in liver serologies. Causes and associations include a history of abdominal trauma, oral contraceptive use, polycythemia vera, paroxysmal nocturnal hemoglobinuria, hypercoagulable states, and congenital webs of the vena cava. Diagnosis can be made by Doppler ultrasound of the hepatic veins, and therapy includes anticoagulation.33
Nonthrombotic occlusion of hepatic venules creating a small-vessel variant of Budd-Chiari syndrome is associated with the ingestion of certain medicinal teas containing pyrrolozidine alkaloids. These alkaloids are found in Senecio and Crotalia genera of plants.34 Venoocclusive hepatic disease also occurs rarely as a complication of chemotherapy and bone marrow transplantation.
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