Vascular Disorders

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    Budd-Chiari Syndrome

    • Hepatic venous outflow tract obstruction including suprahepatic IVC in absence of R-sided heart failure or constrictive pericarditis.
    • Obstruction is caused by:
      • Primary: Thrombosis
        • Risk factors found in 80%, myeloproliferative diseases in 50%.
        • Other Risk Factors:  Behçet syndrome, antiphospholipid antibody syndrome, and OCP use.
      • Secondary: From malignancy or compression.
    • Clinical Features:
      • Asymptomatic in 15% (incidentally find intra+extra-hepatic collaterals on imaging)
      • Symptoms: Abdo pain, ascites, liver/spleen enlargement.
      • Marked dilatation of superficial veins on strunk = IVC obstruction.
    • Labs:
      • Liver chemistry NOT helpful.
      • Ascites protein > 2.5 g/dL (25 g/L) and SAAG ≥ 1.1 g/dL (≥ 11 g/L) is suggestive
    • Diagnosis:
      • Demonstrate obstructed hepatic venous outflow tract.
        • Doppler U/S, triphasic CT, or MRI.
        • Hepatic venography is Gold Standard (helps find precise place of stenosis)
    • Management
      • Poor prognosis if untreated: 90% mortality in 3 years (end-stage liver disease complication).
      • Anticoagulate!! (even if prothrombotic disorder found)
      • OCP are contraindicated.
      • Hepatic Vein or IVC angioplasty +/- stent is recommended in symptomatic BCS and refractory to anticoagulation.
      • Complications:
        • Portal Hypertension: manage same as other causes
        • TIPS may be required
        • If unresponsive to TIPS --> Liver Transplant  (5 yr survival 70%)
        • Lifelong anticoagulation post-transplant

    Portal Vein Thrombosis

    • Acute PVT is characterized by sudden thrombus in portal vein.
      • Can involve segments of mesenteric or splenic veins.
    • Chronic: Stasis from reduced blood flow (i.e. cirrhosis, portal HTN)
    • Clinical Features:
      • Sudden Onset: Fever, abdominal pain
      • Intestinal ischemia and infarction can develop (mesenteric extension).
    • Diagnosis:
      • Dopper U/S or Contrast CT: Hyperechoic material in vessel lumen --> distension of portal vein.
        • "Cavernous transformation" with porto-systemic collaterals often seen (chronic thrombosis)
    • Management:
      • Acute PVT:
        • Anticoagulation and surgery to r/o intestinal infarction
        • Thrombolytic therapy: No Data.
      • Chronic PVT: found incidentally (assd with cirrhosis and portal HTN)
        • Anticoagulation suggested (safety/efficacy not proven)
        • Expectant management recommended.
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