Table of contents
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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.
- Primary: Thrombosis
- 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)
- Demonstrate obstructed hepatic venous outflow tract.
- 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)
- Dopper U/S or Contrast CT: Hyperechoic material in vessel lumen --> distension of portal vein.
- 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.
- Acute PVT:
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