Budd-Chiari syndrome (BCS) is a rare clinical condition characterized by obstruction of the hepatic venous outflow tract between hepatic veins and the junction of the inferior vena cava with the right atrium. Despite the use of anticoagulation, many patients may need additional Interventional Radiology (I.R) treatment strategies. Algorithms consisting of local Venoplasty, Hepatic Vein Stenting (HVS), Catheter Directed Thrombolysis (CDT), Trans-jugular Intra-hepatic Porto-systemic Shunt (TIPS), Direct Intrahepatic Portacaval Shunt (DIPS) as bridge to Liver Transplantation (LT) have been proposed with treatment choice dictated by a lack of response to a less-invasive treatment regimen and clinical response. Endovascular management has emerged to play an important role in the treatment of Budd-Chiari Syndrome and offers minimally invasive and highly effective methods to restore adequate venous outflow required to overcome portal hypertension, and in turn helps to mitigate long term complications i.e. delay progression to hepatic failure and cirrhosis by salvage as alternative outflow and decongestion.
Budd-Chiari syndrome (BCS) is a rare clinical condition characterized by obstruction of the hepatic venous outflow tract between hepatic veins and the junction of the inferior vena cava with the right atrium; which was described by George Budd (1808-1882) in 1845 long before Hans Chiari (1851-1916) mentioned in his pathological observations as “obliterating endo-phlebitis of the hepatic veins" in the later years in 1899. [1, 12, 13]
Despite the use of anticoagulation, many patients may need additional Interventional Radiology (I.R) treatment strategies. Algorithms consisting of local Venoplasty, Hepatic Vein Stenting (HVS), Catheter Directed Thrombolysis (CDT), Trans-jugular Intra-hepatic Porto-systemic Shunt (TIPS), Direct Intrahepatic Portacaval Shunt (DIPS) [9] as bridge to Liver Transplantation (LT) have been proposed with treatment choice dictated by a lack of response to a less-invasive treatment regimen and clinical response. [1,10,11,12].
Select List of cases with analytics:
Age / Sex |
Clinical Profile |
Risk factors |
Hepatic Vein |
IVC |
Ascitis |
Intervention recommended |
48yr / Male |
Abdominal distension Jaundice |
K/c/o Chronic DVT (2-3 yrs back) |
Involved |
Partially involved |
Present |
IVC Venoplasty with Hepatic vein stenting and Venoplasty |
45yr / Male |
Abdominal distension Post Covid - AKI Post dialysis via femoral catheter - DVT Sub-segmental pulmonary embolism |
Alcoholic
|
Involved |
stenosis |
Present |
DIPSS |
27yr / Male |
Abdominal discomfort Deranged Liver function tests Jaundice Loss of appetite Anorexia Abdominal distension |
Occupation farmer inorganic pesticide exposure longterm |
Involved |
Narrowed |
Present |
Hepatic Vein Stenting with IVC Venoplasty |
27yr / female |
Infertility underwent dianostic laproscopy with gynec , eventful bleeding during laproscopy; revealed nodular appearance of liver and underwent cect ; flank veins & collaterals ++
Childhood history of jaundice
|
Not known |
Involved |
Narrowed - IVC membrane with Chronic stenosis |
Present |
DIPS with IVC stenting |
19yr / female |
Abdominal distension Malena Thrombocytopenia |
Not known |
Involved |
Stenosis |
Present |
DIPS |
Fig 1: USG showing no flow across Hepatic veins.
Fig 2: CECT showing the classic nutmeg appearance.