Budd–Chiari Syndrome Resulting from an Obstructive Membranous Web: Diagnosis and Percutaneous Transhepatic Angioplasty Intervention
22 March 2018 (online)
Introduction To highlight the imaging diagnosis and intervention for primary Budd–Chiari syndrome secondary to an obstructing congenital web based on a case presentation.
Case Report A 30-year-old male with abdominal pain and weight gain had normal liver function tests (LFTs). Computed tomography (CT) findings demonstrating heterogeneous and mildly enlarged liver with narrowing of intrahepatic inferior vena cava (IVC). Non-visualization of hepatic veins and moderate ascites.
Ultrasound examination was performed showing an attenuated right hepatic vein. The middle and left hepatic veins were draining into the IVC via a common ostium. Subsequent IVC cavogram and hepatic venogram demonstrated narrowing of the intrahepatic IVC. No gradient between right atrium and infrahepatic IVC was found, and access into the hepatic veins was not feasible.
Ultrasound examination was repeated, now showing a web at the confluence of the left and middle hepatic veins, thus explaining the turbulent flow at the junction of the middle and left hepatic veins with the IVC, consistent with Budd–Chiari syndrome secondary to membranous obstruction of the hepatic veins.
Subsequent intervention using a percutaneous transhepatic approach with access of the middle hepatic vein percutaneously using US guidance. The obstructing web was confirmed with high velocity flow of contrast through the pinhole opening. Successful percutaneous transhepatic balloon angioplasty (PTA) was performed with no residual gradient. Absence of web was confirmed on post PTA venogram and ultrasound examination.
The liver appeared normal on a CT obtained 6 months later along with clinical improvement, demonstrating weight loss, and resolution of ascites.
Importance of meticulous ultrasound in the diagnosis of membranous obstruction as a cause of Budd–Chiari syndrome.
Utility of transhepatic intervention as an option for endovascular treatment.