Elementos sugestivos do diagnóstico
Não há critérios de diagnóstico formais para a síndrome de Budd-Chiari (SBC); no entanto, os achados a seguir respaldam o diagnóstico:
Clínica[1]Northup PG, Garcia-Pagan JC, Garcia-Tsao G, et al. Vascular liver disorders, portal vein thrombosis, and procedural bleeding in patients with liver disease: 2020 practice guidance by the American Association for the Study of Liver Diseases. Hepatology. 2021 Jan;73(1):366-413.
https://journals.lww.com/hep/fulltext/2021/01000/vascular_liver_disorders,_portal_vein_thrombosis,.26.aspx
http://www.ncbi.nlm.nih.gov/pubmed/33219529?tool=bestpractice.com
[6]Mitchell MC, Boitnott JK, Kaufman S, et al. Budd-Chiari syndrome: etiology, diagnosis and management. Medicine (Baltimore). 1982;61:199-218.
http://www.ncbi.nlm.nih.gov/pubmed/7045569?tool=bestpractice.com
[7]Hemming AW, Langer B, Greig P, et al. Treatment of Budd-Chiari syndrome with portosystemic shunt or liver transplantation. Am J Surg. 1996;171:176-180.
http://www.ncbi.nlm.nih.gov/pubmed/8554136?tool=bestpractice.com
[9]Murad SD, Valla DC, de Groen PC, et al. Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology. 2004;39:500-508.
http://www.ncbi.nlm.nih.gov/pubmed/14768004?tool=bestpractice.com
Exames por imagem[51]Bargalló X, Gilabert R, Nicolau C, et al. Sonography of Budd-Chiari syndrome. Am J Roentgenol. 2006;187:W33-W41.
http://www.ajronline.org/doi/full/10.2214/AJR.04.0918
http://www.ncbi.nlm.nih.gov/pubmed/16794137?tool=bestpractice.com
Venografia[1]Northup PG, Garcia-Pagan JC, Garcia-Tsao G, et al. Vascular liver disorders, portal vein thrombosis, and procedural bleeding in patients with liver disease: 2020 practice guidance by the American Association for the Study of Liver Diseases. Hepatology. 2021 Jan;73(1):366-413.
https://journals.lww.com/hep/fulltext/2021/01000/vascular_liver_disorders,_portal_vein_thrombosis,.26.aspx
http://www.ncbi.nlm.nih.gov/pubmed/33219529?tool=bestpractice.com
[52]Frank JW, Kamath PS, Stanson AW. Budd-Chiari syndrome: early intervention with angioplasty and thrombolytic therapy. Mayo Clin Proc. 1994;69:877-881.
http://www.ncbi.nlm.nih.gov/pubmed/8065191?tool=bestpractice.com
Patologia[46]Tanaka M, Wanless IR. Pathology of the liver in Budd-Chiari syndrome: portal vein thrombosis and the histogenesis of veno-centric cirrhosis, veno-portal cirrhosis, and large regenerative nodules. Hepatology. 1998;27:488-496.
http://onlinelibrary.wiley.com/doi/10.1002/hep.510270224/pdf
http://www.ncbi.nlm.nih.gov/pubmed/9462648?tool=bestpractice.com
Na SBC, o fígado pode desenvolver congestão centrolobular grave, necrose de hepatócitos e fibrose e, às vezes, apresentar cirrose coexistente.
Critérios de gravidade
Para avaliar a gravidade da forma crônica (cirrose) da SBC, pode-se usar a classificação de Child-Pugh.[53]Child CG, Turcotte JG. Surgery and portal hypertension. Major Probl Clin Surg. 1964;1:1-85.
http://www.ncbi.nlm.nih.gov/pubmed/4950264?tool=bestpractice.com
O escore de Child-Pugh usa 5 medidas clínicas de doença hepática. Cada medida é classificada como entre 1 e 3 pontos, com 3 indicando o desequilíbrio mais grave. As medidas clínicas são:
Encefalopatia
Nenhuma: 1 ponto
Grau 1 a 2: 2 pontos
Grau 3 a 4: 3 pontos.
Ascite
Nenhuma: 1 ponto
Leve/moderada: 2 pontos
Tensa: 3 pontos.
Bilirrubina (mg/dL)
<2: 1 point
2 ou 3 = 2 pontos
Mais de 3: 3 pontos
Albumina (g/dL)
> 3.5: 1 ponto
2.8 a 3.5: 2 pontos
< 2.8: 3 pontos
razão normalizada internacional (INR)
< 1.7: 1 ponto
1.7 a 2.3: 2 pontos
> 2.3: 3 pontos
A doença hepática crônica é classificada na classe de Child-Pugh A a C usando os escores conforme acima:
Classe A: 5 a 6 pontos
Classe B: 7 a 9 pontos
Classe C: 10 a 15 pontos