Vascular Disorders

Table of Contents

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

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.