Redefining Budd-Chiari syndrome: A systematic review

Abstract

Aim: To re-examine whether hepatic vein thrombosis (HVT) (classical Budd-Chiari syndrome) and hepatic vena cava-Budd Chiari syndrome (HVC-BCS) are the same disorder.

Methods: A systematic review of observational studies conducted in adult subjects with primary BCS, hepatic vein outflow tract obstruction, membranous obstruction of the inferior vena cava (IVC), obliterative hepatocavopathy, or HVT during the period of January 2000 until February 2015 was conducted using the following databases: Cochrane Library, CINAHL, MEDLINE, PubMed and Scopus.

Results: Of 1299 articles identified, 26 were included in this study. Classical BCS is more common in women with a pure hepatic vein obstruction (49%-74%). HVC-BCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins (14%-84%). Classical BCS presents with acute abdominal pain, ascites, and hepatomegaly. HVC-BCS presents with chronic abdominal pain and abdominal wall varices. Myeloproliferative neoplasms (MPN) are the most common etiology of classical BCS (16%-62%) with the JAK2V617-F mutation found in 26%-52%. In HVC-BCS, MPN are found in 4%-5%, and the JAK2V617-F mutation in 2%-5%. Classical BCS responds well to medical management alone and 1(st) line management of HVC-BCS involves percutaneous recanalization, with few managed with medical management alone.

Conclusion: Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.

Keywords: Budd-Chiari; Hepatic vein outflow tract obstruction; Hepatic vein thrombosis; Membranous obstruction of the inferior vena cava; Obliterative hepatocavopathy.

Figure 1

Flow diagram of studies selection. ¹Searches conducted with MEDLINE results removed; ²Studies missing key clinical information including clear inclusion and exclusion criteria, clear diagnostic parameters, etc., and studies that investigated subpopulations (e.g., BCS patients requiring liver transplantation, BCS patients without MPN, etc.); ³Studies focused on other categories (e.g., causes of liver failure). BCS: Budd-Chiari syndrome; MPN: Myeloproliferative neoplasms.

Figure 2

Classical Budd-Chiari syndrome - Occlusions are within the hepatic veins themselves and usually thrombi. RHV: Right hepatic vein; MHV: Middle hepatic vein; LHV: Left hepatic vein; IVC: Inferior vena cava.

Figure 3

Hepatic vena cava-Budd Chiari syndrome - Occlusions are thin or thick (membranous or segmental) and within the inferior vena cava and occlusion can extend into the hepatic veins and generally involve the ostia to the inferior vena cava. RHV: Right hepatic vein; MHV: Middle hepatic vein; LHV: Left hepatic vein; IVC: Inferior vena cava.