Budd Chiari Syndrome with Portal Vein Thrombosis Managed Successfully with Direct Intrahepatic Portosystemic Shunt: A Case Report and Review of the Literature

Abstract

Budd -Chiari syndrome (BCS) is a hepatic vascular disorder which affects hepatic veins or inferior vena cava. Portal vein thrombosis (PVT) occurs in around 15%-25% of patients with BCS. The presence of PVT in patients with BCS makes it more difficult to intervene radiologically. We present a case of a BCS-related chronic liver disease that presented with a history of variceal upper gastrointestinal bleeding and worsening ascites. The patient had thrombosed hepatic veins (HV) and partial right portal vein thrombosis. He was started on anticoagulation, and treatment for portal hypertension was initiated. Given the inaccessibility of all the HVs for trans-jugular intrahepatic portosystemic shunts (TIPS), the patient underwent direct intrahepatic portosystemic shunts (DIPS). Next-generation sequencing identified the factor V Leiden mutation. Following DIPS, the patient's ascites disappeared, and liver function tests improved. On a nine-month follow-up, the patient was symptom-free with a patent DIPS. DIPS has been widely used in patients with BCS with thrombosed hepatic veins, but there are only a few case reports on the feasibility of DIPS in BCS patients with PVT. This is one of the very few case reports where a patient with BCS-PVT was successfully managed with DIPS.

Keywords: Budd Chiari syndrome; Leiden mutation; cirrhosis; thrombophilia; venous thrombosis.