FN: Budd chiari syndrome

Question 1

Def

Answer 1

􏰀 Hepatic vein obstruction → ischaemia and hepatocyte damage → liver failure or insidious cirrhosis.

Question 2

Causes

Answer 2

􏰀 Hypercoagulable states: myeloproliferative disorders
(PV = commonest cause), PNH, anti-phospholipid, OCP
􏰀 Local Tumour: HCC
􏰀 Congenital: membranous obstruction of IVC

Question 3

Presentation

Answer 3

􏰀 RUQ pain: stretching of Glisson’s capsule 􏰀 Hepatomegaly
􏰀 Ascites: SAAG ≥1.1g/dL
􏰀 Jaundice (and other features of liver failure)

Question 4

Ix

Answer 4

􏰀 Bloods: FBC, clotting, LFTs
􏰀 US + hepatic vein Doppler
􏰀 Ascitic tap: ↑↑ protein (>2.5g/dL) ̄c ↑SAAG (≥1.1g/dL) 􏰀 Other: JAK2 mutation analysis, RBC CD55 and CD59

Question 5

Rx

Answer 5

􏰀 Anticoagulate unless there are varices
􏰀 Ascites: fluid and salt restrict, spiro, fruse, tap, daily wt 􏰀 Other options: thrombolysis, angioplasty, TIPSS
􏰀 Transplant if fulminant hepatic failure or cirrhosis
􏰀 Rx underlying cause