Classification of Hepatic Venous Outflow Obstruction: Ambiguous Terminology of the Budd-Chiari Syndrome

doi:10.1016/S0025-6196(12)62109-0

Mayo Clinic Proceedings

Volume 65, Issue 1, January 1990, Pages 51-55

https://doi.org/10.1016/S0025-6196(12)62109-0 Get rights and content

Severe hepatic venous outflow obstruction and its manifestations often are recorded under the label “Budd-Chiari syndrome.” Unfortunately, this label is ambiguous; it does not clearly identify the site of the lesion (hepatic veins versus inferior vena cava), its morphologic features (thrombotic versus nonthrombotic), or its cause. In the literature, implied or expressed definitions vary. Use of a standardized topographic and pathogenetic classification of hepatic venous outflow obstruction would enable investigators to group patients with comparable conditions, as required for therapeutic trials, prognostic evaluations, and studies of pathogenetic pathways. Review of our own cases revealed that hepatic venous outflow obstruction involving large hepatic veins is usually thrombotic and that isolated obstruction of the inferior vena cava or of small hepatic veins is usually nonthrombotic. Application of such a classification seems feasible and may yield useful results.

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)

FX Solano et al.
Constrictive pericarditis mimicking Budd-Chiari syndrome
Am J Med
(1986)
BJ Rollins
Hepatic veno-occlusive disease
Am J Med
(1986)
LI Alpert
Veno-occlusive disease of the liver associated with oral contraceptives: case report and review of literature
Hum Pathol
(1976)
B Langer et al.
Clinical spectrum of the Budd-Chiari syndrome and its surgical management
Am J Surg
(1975)
F Sevenet et al.
Membranous obstruction of the inferior vena cava associated with a myeloproliferative disorder: a clue to membrane formation?
Gastroenterology
(1989)
RR Brinson et al.
Recovery from hepatic vein thrombosis (Budd-Chiari syndrome) complicating ulcerative colitis
Dig Dis Sci
(1988)
S McClure et al.
Budd-Chiari syndrome and antithrombin III deficiency
Am J Clin Pathol
(1982)
TF Disney et al.
Budd-Chiari syndrome with inferior vena cava obstruction associated with systemic lupus erythematosus
J Clin Gastroenterol
(1984)

There are more references available in the full text version of this article.

Cited by (193)

Transhepatic inferior vena cava recanalization in a case of Budd Chiari syndrome: A novel approach
2023, Radiology Case Reports
Sharp recanalization for short-segment intravascular occlusion, using an endovascular route, has been described for inferior vena cava (IVC) occlusion. Often, the technical challenge to the endovascular management of Budd-Chiari syndrome (BCS) is the recanalization of the occluded hepatic vein or suprahepatic IVC. Presented here, the challenge was the level of occlusion of the suprahepatic IVC, with the resultant separation of both the patent IVC segments in a horizontal plane, making it technically challenging for sharp recanalization. We describe the use of percutaneous transhepatic access into the suprahepatic IVC via the middle hepatic vein under ultrasound guidance with eventual sharp recanalization of the occluded segment of the IVC, in a woman with BCS. This novel approach has not been described in the literature and can serve as an important addition to guide complex suprahepatic IVC recanalization.
Breaking down tumor thrombus: Current strategies for medical management
2023, Thrombosis Research
Tumor thrombus, the intravascular extension of tumor into adjacent blood vessels, is frequently encountered in patients with renal cell carcinoma and hepatocellular carcinoma, and often involves the abdominal vasculature including the renal vein, portal vein, and the inferior vena cava. While a bland thrombus is composed of platelets and fibrin, in contrast, a tumor thrombus refers to an organized collection of tumor cells. Though oftentimes detected incidentally on imaging, tumor thrombus may have significant clinical implications and can be challenging to differentiate from bland thrombus. Additionally, the optimal management of tumor thrombus, including the use of anticoagulation, remains poorly described. This review summarizes common causes of tumor thrombus, as well as its impact on staging, prognosis, and treatment.
Vascular Disorders
2023, MacSween's Pathology of the Liver, Eighth Edition
Vascular diseases of the liver are less common than other liver diseases, such as hepatitides, cholestatic diseases and alcoholic/nonalcoholic fatty liver diseases, but are gaining increasing recognition because of the improved diagnosis of these other diseases, as well as the secondary involvement of vessels in the course of almost all liver diseases. The dramatic decrease in chronic viral hepatitis C due to effective antiviral regimens in recent years also highlights the increased proportion of vascular diseases as pathology in liver seen by practitioners in daily practice. This chapter focuses mainly on primary vascular diseases of the liver using a classification based on the type of blood vessels involved, since different aetiologies of vascular liver disease affect respective aspects of the hepatic vasculature.
Large Regenerative Nodules and Focal Nodular Hyperplasia-Like Lesions: Definition, Pathogenesis, and Imaging Findings
2022, Radiologic Clinics of North America
Radical surgical treatment of Budd-Chiari syndrome through entire exposure of hepatic inferior vena cava
2019, Journal of Vascular Surgery: Venous and Lymphatic Disorders
Therapies for Budd-Chiari syndrome (BCS) can be divided into three main categories: medical, endovascular, and surgical. Surgery is applicable to the disease when other therapeutic options have failed. We introduce a surgical method of recanalization through exposure of the entire hepatic inferior vena cava (IVC) and hepatic vein (HV) outflow tract for BCS and investigate the long-term outcomes.
From July 2002 to December 2015 in our center, 83 consecutive symptomatic BCS patients with failure of endovascular therapy were treated by radical surgical recanalization. IVC recanalization was the first goal for all patients, and recanalization of at least one HV was the second goal for selected patients at the same surgical operation. Patients were followed up, and data on technical and clinical success, survival, and patency of target vessels were analyzed.
Technical success of surgical recanalization was achieved in 80 patients (96.4%), with relief of clinical symptoms and improvement of liver function. During a mean follow-up of 84 ± 25.9 months, the cumulative 1-, 3-, and 5-year primary patency rates of the HV were 96.7%, 90.0%, and 83.3%, respectively. The cumulative 1-, 3-, and 5-year primary patency of the IVC was 86.7%, 71.7%, and 68.3%, respectively. No factor demonstrated significant association with recurrence of obstruction. During follow-up, 10 patients died, 8 of end-stage hepatic disease and 2 of unknown causes. The cumulative 1-, 3-, and 5-year all-cause survival rates were 91%, 90%, and 87%, respectively. Female sex, encephalopathy, severe ascites, and hypersplenism had an impact on survival in univariate analysis. With Cox regression, encephalopathy was the only independent determining factor for surgical survival.
Surgical recanalization through exposure of the entire hepatic IVC for BCS is suitable for most primary BCS patients after failure of endovascular therapies.
Pattern of vascular involvement in egyptian patients with budd-chiari syndrome: Relation to etiology and impact on clinical presentation
2018, Annals of Hepatology
Introduction and aim. Budd-Chiari syndrome (BCS) is caused by hepatic venous outflow obstruction. This work aims to analyze the pattern of vascular involvement in Egyptian patients with BCS, demonstrates its relation to etiology and shows its impact on clinical presentation.
Material and methods. The current retrospective study was conducted at The Tropical Medicine Department, Ain Shams University on one hundred Egyptian patients with confirmed diagnosis of primary BCS who were presented to the Budd-Chiari Study Group (BCSG) from April 2014 to May 2016 by collecting clinical, laboratory and radiological data from their medical records.
Results. Isolated hepatic vein occlusion (HVO) was the most common pattern of vascular involvement (43%), followed by combined HVO and inferior vena cava (IVC) compression by enlarged caudate lobe (32%), then combined HVO and IVC stenosis/webs (21%), and lastly isolated IVC occlusion (4%). Ascites was more significantly encountered in BCS patients with HVO than in those with isolated inferior vena cava (IVC) occlusion and patent HVs (P = 0.005). Abdominal pain was significantly encountered in patients with occluded three major HVs (P = 0.044). Behcet’s disease was significantly detected in isolated IVC occlusion. Protein C deficiency was significantly detected in patients with combined HVO and IVC compression.
Conclusion. Isolated HVs occlusion was the most common pattern of vascular involvement in Egyptian patients with primary BCS. Vascular pattern of involvement affected the clinical presentation and was related to the underlying thrombophilia in those patients.

View all citing articles on Scopus

View full text

Classification of Hepatic Venous Outflow Obstruction: Ambiguous Terminology of the Budd-Chiari Syndrome

Section snippets

DEFINITION OF BCS

DIAGNOSIS OF BCS

APPLICATION OF CLASSIFICATION

DISCUSSION

CONCLUSION

Am J Med

Am J Med

Hum Pathol

Am J Surg

Gastroenterology

Recovery from hepatic vein thrombosis (Budd-Chiari syndrome) complicating ulcerative colitis

Dig Dis Sci

Budd-Chiari syndrome and antithrombin III deficiency

Am J Clin Pathol

Budd-Chiari syndrome with inferior vena cava obstruction associated with systemic lupus erythematosus

J Clin Gastroenterol