Budd-Chiari syndrome
= Hepatic venous obstruction syndrome
hepatic vein obstructionÀ¸·Î ÀÎÇÏ¿© hepatomegaly, abd pain, ascites, zone 3 sinusoidal
distensionÀÌ »ý±â´Â º´ÀÌ´Ù.
1. ¿øÀÎ
¨ç myeloproliferative disease ¿¹) polycythemia vera : 60%¿¡¼ °ü·Ã, young female
¨è SLE, antiphospholipid syndrome(primary or secondary to SLE)
¨é circulating anticoagulant
¨ê DIC
¨ë Idiopathic granulomatous venulitis
¨ì PNH
¨í anticoagulant factor deficiency: AT III deficiency
¨î Behcet's disease Cx
¨ï oral contraceptive, pregnancy·Î ÀÎÇÑ hepatic vein thrombosis
¨ð trauma
¨ñ polycystic liver disease¿¡¼ mechanical compression
¨ò IVC obstruction d/t malignant ds
¨ó RA myxoma
¨ô alcoholic & veno-occlusive ds¿¡¼ central hepatic vein involve
¨õ liver transplantationÈÄ veno-occlusive disease
2. º´¸®
large hepatic vein : thrombotic
Isolated IVC or small hepatic vein obstruction : ÈçÈ÷ non-thrombotic
Liver : enlarge, purplish, smooth
venous congestion - cut surface "nutmeg" change
* chronic- caudate lobe enlarge, IVC compress
splenomegaly, portal-systemic collateral circulation¹ß»ý
mesenteric vessel thrombosis
* histology : zone3 venous dilatation
congestion with hemorrhage, necrosis
3. ÀÓ»óƯ¡
fulminant course¶§ encephalopathy¹ß»ýÇϸç 2-3ÁÖ³» »ç¸Á
chronic hepatocellular disease¹ß»ý
-> slowly develp -> confusion, cirrhosis
1) acute form : ill patient
abdominal pain, vomiting, liver enlargement
ascites, mild icterus
watery diarrehea(mesenteric venous obstruction)
-> terminal, inconstant feature
total hepatic vein occlusion¹ß»ý½Ã delirium, coma with hepatocellular failure
-> ¼öÀϳ» »ç¸Á
2) chronic form
1-6°³¿ù¿¡ °ÉÃļ ¹ß»ý
pain, enlarged tender liver, ascites
portal hypertension -> spleen palpation
caudate lobe enlargement
IVC block -> leg edema
3) LFT
Bil 2 mg/dLÀÌ»óÀº Àß ³ÑÁö ¾Ê´Â´Ù.
s-ALP, Alb°¨¼Ò
PT prolongation, protein¡é(¡ñ protein-losing enteropathy)
4) liver Bx: ÇʼöÀû, zone 3 congestion
5) hepatic venography : tortous, lase-like spider-web pattern
6) IVC venography : IVC patencyÈ®ÀÎ
side-to-side narrowing, IVC distortion, extrinsic compression from Lt
7) USG : hepatic vein abnormality
caudate lobe hypertrophy
IVC compression
PW doppler : hepatic vein, IVC blood flow(-)
Color doppler : hepatic vein abnormality(+)
8) CT : liver enlargement, diffuse hypodensity
patchy enhancement after contrast
* hepatic metastasis¿Í È¥µ¿µÊ
4. Áø´Ü
liver cirrhosis¿Í °¨º°À§ÇØ liver Bx½ÃÇà
»ó±â ¿µ»óÁø´Ü½ÃÇà
5. ¿¹ÈÄ
¿øÀο¡ µû¶ó ´Ù¸£´Ù.
6. Ä¡·á
Acute : fibrinolytic therapy - uncertain
Chronic : anticoagulant - underlying thrombosing diseaseȯÀÚ¿¡¼ »ç¿ë
¿øÀο¡ µû¸¥ Ä¡·á¸¦ ÇÑ´Ù.