Patients with the Budd-Chiari Syndrome develop different patterns of vascular fibrosis in the liver. The pattern of fibrosis correlates with the pattern of parenchymal fibrosis or cirrhosis that develops. The authors are from the Toronto Hospital and the University of Toronto in Canada.
Blood vessels evaluated:
(1) hepatic veins
(2) portal veins
Types of hepatic or portal veins
(1) small veins (< 0.2 mm in diameter)
(2) medium veins (0.2 to 3.0 mm in diameter)
(3) large veins (> 3.0 mm in diameter)
Rules for reporting vascular lesions:
(1) The grade for small and medium veins is the median grade for all available histologic slides.
(2) The grade for large veins is the most severe narrowing recorded in all available histologic slides.
|
Lesion and Vessel Size |
Extent of Involvement |
Grade |
|
obliteration (blocking) of small hepatic or portal vein |
< 25% of veins |
0 |
|
|
25 - 49% of veins |
1 |
|
|
50 - 75% of veins |
2 |
|
|
> 75% of veins |
3 |
|
fibrous intimal thickening in medium hepatic or portal vein |
< 25% of veins |
0 |
|
|
25 - 49% of veins |
1 |
|
|
50 - 75% of veins |
2 |
|
|
> 75% of veins |
3 |
|
fibrous intimal thickening in large hepatic vein |
< 1% narrowing |
0 |
|
|
1 - 19% narrowing |
1 |
|
|
20 - 80% narrowing |
2 |
|
|
> 80% narrowing |
3 |
|
fibrous intimal thickening in large portal vein |
< 1% narrowing |
0 |
|
|
1 - 9% narrowing |
1 |
|
|
10 - 20% narrowing |
2 |
|
|
> 20% narrowing |
3 |
If hepatic vein disease is prominent, then patients develop veno-centric cirrhosis.
If portal vein disease is prominent, then patients develop nodular regenerative hyperplasia.
If both hepatic and portal vein disease is prominent, then veno-portal cirrhosis develops, which can progress to total parenchymal extinction in the region or lobe.
Specialty: Gastroenterology, Hematology Oncology
