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.