Progressive Porto-Sinusoidal Vascular Disorder Complicated by Portal Biliopathy Requiring Liver Transplantation: A Case Report
DOI:
https://doi.org/10.60591/crspmi.562Keywords:
Hepatic Veno-Occlusive Disease, Hypertension, Portal, Liver Transplantation, Portal Vein, Portasystemic Shunt, Transjugular IntrahepaticAbstract
Porto-sinusoidal vascular disorder (PSVD) is a rare cause of non-cirrhotic portal hypertension that may be complicated by portal biliopathy (PB). We report a 16-year follow-up of a male patient who presented at age 4 with splenomegaly and extensive collateralization of the portal vein, despite patency, subsequently diagnosed with PSVD on transjugular liver biopsy. Despite multiple endoscopic treatments for variceal bleeding and transjugular intrahepatic portosystemic shunt (TIPS) placement, the patient developed progressive symptomatic PB, presenting with jaundice and abdominal pain. Imaging showed massive portal vein dilation (40 mm) and grade III biliary changes according to the Llop classification, unresponsive to percutaneous drainage and endoscopic stenting. Liver transplantation was performed, with an excellent outcome at 3-year follow-up. This case illustrates the progressive nature of PSVD complicated by portal hypertension and PB over nearly two decades, highlighting the complexity of therapeutic decision-making in these settings.
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Copyright (c) 2026 Elisa Pinto, Kymentie Ferdinande, Alberto Zanetto, Umberto Cillo, Marco Senzolo
This work is licensed under a Creative Commons Attribution 4.0 International License.
