Budd-Chiari syndrome

Incidence

Age

Sex

Geography

Aetiology

- occlusion of the hepatic vein -> obstruction to venous outflow of liver
- no cause is identified in1/3 of cases
- hypercoagulability states e.g. polycythaemia vera, OCP, leukaemia
- occlusion of hepatic vein due to posterior abdominal wall sarcoma
- renal/adrenal tumours
- HCC
- hepatic infections, e.g. hydatid cyst
- congenital venous webs
- radiotherapy
- trauma to liver

- caudate lobe has separate blood supply and venous drainage

Presentation

Acute
- abdominal pain
- nausea and vomiting
- tender hepatomegaly
- ascites

Crohnic
- enlargement of liver
- mild jaundice
- ascites
- negative hepatojugular reflex
- splenomegaly (2º to portal hypertension)

Investigations

Ascitic tap
- high protein content in fluid

Liver biopsy
- histology

USS/CT/MRI
- hepatic vein occlusion
- diffuse abnormal parenchyma; spares caudate lobe
- ± compression of IVC
Pulsed doppler sonography / doppler ultrasound can show direction of flow in the hepatic vein

Investigate cause
- e.g. FBC, U&Es, LFTs, clotting

Differential diagnosis
- IVC obstruction
- right heart failure
- constrictive pericarditis

Macro

Micro
- centrilobular congestion
- hepatocyte atrophy

Staging
 

Serum markers

Management

- treat underlying cause
- treat ascites
- side-to side portocaval or splenorenal anastomosis can be use to decompress congested liver
- peroitonesl-jugular LeVeen shunt may be useful

- liver transplantation is the treatment of choice

Prognosis
- depends on aeitology
- some patients can survive for several years

Complications

Hepatobiliary medicine

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