Classification of Hepatic Venous Outflow Obstruction: Ambiguous Terminology of the Budd-Chiari Syndrome
Section snippets
DEFINITION OF BCS
Review of the literature and our own experiences suggests that the following definition of BCS currently would be the most useful:
BCS consists of hepatic venous outflow obstruction and its manifestations, regardless of cause, the obstruction being either within the liver or in the IVC between the liver and the right atrium. Functional hepatic venous outflow obstruction caused by congestive heart failure is not considered BCS.
Commonly, heart disease other than constrictive pericarditis is
DIAGNOSIS OF BCS
In our opinion, the diagnosis of BCS should be used sparingly; this label is appropriate primarily for cases of HVOO before the type and location of the obstruction are known. For the treatment of individual patients, for therapeutic trials, and for other retrospective and prospective studies of HVOO, a more detailed pathogenetic classification is needed. For any such classification, the components of the hepatic venous outflow tract must be well defined by radiologic and other
APPLICATION OF CLASSIFICATION
For preparation of Table 3 (and thus testing of the proposed classification), we retrieved all Mayo Clinic cases of BCS and HVOO that had been recorded during the 12-year period from 1976 to 1987. We identified 62 patients who fulfilled the previously stated definition of BCS; some patients from this group had already been described in a previous publication from this institution.17 In 21 instances, morphologic data were unobtainable or insufficient; therefore, these patients were excluded from
DISCUSSION
Although most clinicians implicitly use in their publications a classification similar to the one described herein, that information rarely is communicated. We believe that use of a standardized classification would facilitate therapeutic trials, prognostic evaluations, and study of pathogenetic pathways. Obviously, for patient management, many other factors should be considered, such as the degree of liver dysfunction and the duration of the disease. For diagnostic considerations, the data
CONCLUSION
We prefer not to use the term BCS or, if that cannot be achieved, to apply it only to the symptom complex of noncardiogenic HVOO either (1) before complete diagnostic workup of a patient or (2) as a collective term for all cases in a study. For patient management and all other purposes—in particular, publication of therapeutic trials and prognostic evaluations—specific etiologic and pathogenetic designations should be used. For example, a diagnostic group could be described as “HVOO, IVC,
REFERENCES (17)
- et al.
Constrictive pericarditis mimicking Budd-Chiari syndrome
Am J Med
(1986) Hepatic veno-occlusive disease
Am J Med
(1986)Veno-occlusive disease of the liver associated with oral contraceptives: case report and review of literature
Hum Pathol
(1976)- et al.
Clinical spectrum of the Budd-Chiari syndrome and its surgical management
Am J Surg
(1975) - et al.
Membranous obstruction of the inferior vena cava associated with a myeloproliferative disorder: a clue to membrane formation?
Gastroenterology
(1989) - et al.
Recovery from hepatic vein thrombosis (Budd-Chiari syndrome) complicating ulcerative colitis
Dig Dis Sci
(1988) - et al.
Budd-Chiari syndrome and antithrombin III deficiency
Am J Clin Pathol
(1982) - et al.
Budd-Chiari syndrome with inferior vena cava obstruction associated with systemic lupus erythematosus
J Clin Gastroenterol
(1984)
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