A 65-year-old woman presented with a complaint of right upper quadrant pain.
Three years prior, the patient was incidentally diagnosed with idiopathic BCS during health checkup; Doppler ultrasound (US) examination showed obstruction of the middle and left hepatic veins, and investigations for underlying thrombophilia were unremarkable.
She was treated with warfarin to maintain an international normalized ratio between 2 and 3, but discontinued after at least 1 year due to non-compliance; the patient did not consult with any doctors for 2 years.
She has remained asymptomatic for the past 3 years until recently she started experiencing steadily worsening, intermittent pain in the right upper quadrant of her abdomen, with no associated or alleviating factors.
Physical examination was only notable for mild right upper quadrant tenderness.
Laboratory tests revealed mildly elevated bilirubin (2.2 mg/dL) and albumin deficit (2.38 g/dL).
Her Child-Pugh (CP) score was 8, Model for End-Stage Liver Disease (MELD) score was 11, Rotterdam score was 0.15, and BCS-TIPS prognostic index (BCS-TIPS PI) score was 6.3.
A contrast-enhanced computed tomography (CT) scan showed non-visualized middle and left hepatic veins, hepatomegaly with hypertrophy of the caudate lobe, splenomegaly, hepatic venous collaterals, and a saccular aneurysm located at the extrahepatic portal vein main branch measuring 3.2 cm in height and 2.5 cm × 2.4 cm in diameter (Figure ​1A).
The aneurysm was thought to be associated with BCS as there was no preceding history of trauma and it had not been present on Doppler US examination performed 3 years previously.
Although the patient had no ascites or variceal bleeding, the decision was made to create a TIPS to relieve hepatic venous outflow obstruction because of increasing pain and concern for complications due to aneurysm size; direct approach to the aneurysm was not considered due to risk of associated complications in the setting of portal hypertension, and stent-grafting was not considered due to concerns about patency in the settings of hepatic venous outflow obstruction and underlying thrombophilia.
After informed consent was obtained, the patient was brought to the interventional radiology suite.
The procedure was performed with the patient under conscious sedation.
Portal vein access was obtained via the right hepatic vein under fluoroscopy, and portography showed a saccular aneurysm located at the main portal vein, extensive intrahepatic portal vein thrombosis, and small splenorenal varices (Figure ​2A).
A 10-mm diameter expanded polytetrafluoroethylene (ePTFE) covered stent-graft (Fluency Plus; Bard Peripheral Vascular, Tempe, Arizona, United States) was deployed across the liver parenchymal tract; deploying the stent-graft into the main portal vein trunk to cover the aneurysm neck was not considered because it would obstruct blood flow to the portal vein branches.
Completion portography showed a widely patent shunt and markedly decreased aneurysm filling (Figure 2B).
The portosystemic pressure gradient (PPG) was decreased from 19 mmHg to 8 mmHg.
The patient recovered uneventfully and had complete resolution of her abdominal pain in 2 d.
She was discharged on long-term warfarin and remained asymptomatic after 1 year of follow-up.
Most recent laboratory tests revealed normal liver function, and Doppler US examination performed at 1, 3, 6, and 12 mo postprocedure confirmed a patent shunt.
Her CP score was 5, MELD score was 8, Rotterdam score was 0.12, and TIPS-BCS PI score was 6.1.
CT scans showed that the aneurysm had decreased in size to 2.4 cm in height and 2.0 cm × 1.9 cm in diameter at 3 mo after TIPS (Figure 1B), and had further decreased to 1.9 cm in height and 1.6 cm × 1.5 cm in diameter at 1 year (Figure ​1C).