Budd-Chiari syndrome
= Hepatic venous obstruction syndrome
hepatic vein obstruction으로 인하여 hepatomegaly, abd pain, ascites, zone 3 sinusoidal
distension이 생기는 병이다.
1. 원인
① myeloproliferative disease 예) polycythemia vera : 60%에서 관련, young female
② SLE, antiphospholipid syndrome(primary or secondary to SLE)
③ circulating anticoagulant
④ DIC
⑤ Idiopathic granulomatous venulitis
⑥ PNH
⑦ anticoagulant factor deficiency: AT III deficiency
⑧ Behcet's disease Cx
⑨ oral contraceptive, pregnancy로 인한 hepatic vein thrombosis
⑩ trauma
⑪ polycystic liver disease에서 mechanical compression
⑫ IVC obstruction d/t malignant ds
⑬ RA myxoma
⑭ alcoholic & veno-occlusive ds에서 central hepatic vein involve
⑮ liver transplantation후 veno-occlusive disease
2. 병리
large hepatic vein : thrombotic
Isolated IVC or small hepatic vein obstruction : 흔히 non-thrombotic
Liver : enlarge, purplish, smooth
venous congestion - cut surface "nutmeg" change
* chronic- caudate lobe enlarge, IVC compress
splenomegaly, portal-systemic collateral circulation발생
mesenteric vessel thrombosis
* histology : zone3 venous dilatation
congestion with hemorrhage, necrosis
3. 임상특징
fulminant course때 encephalopathy발생하며 2-3주내 사망
chronic hepatocellular disease발생
-> 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 occlusion발생시 delirium, coma with hepatocellular failure
-> 수일내 사망
2) chronic form
1-6개월에 걸쳐서 발생
pain, enlarged tender liver, ascites
portal hypertension -> spleen palpation
caudate lobe enlargement
IVC block -> leg edema
3) LFT
Bil 2 mg/dL이상은 잘 넘지 않는다.
s-ALP, Alb감소
PT prolongation, protein↓(∵ protein-losing enteropathy)
4) liver Bx: 필수적, zone 3 congestion
5) hepatic venography : tortous, lase-like spider-web pattern
6) IVC venography : IVC patency확인
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 metastasis와 혼동됨
4. 진단
liver cirrhosis와 감별위해 liver Bx시행
상기 영상진단시행
5. 예후
원인에 따라 다르다.
6. 치료
Acute : fibrinolytic therapy - uncertain
Chronic : anticoagulant - underlying thrombosing disease환자에서 사용
원인에 따른 치료를 한다.