Redefining the Management of Portal Cholangiopathy: Therapeutic Strategies and Long- Term Outcomes

Background:
Portal cholangiopathy is a frequent but incompletely characterized complication of extrahepatic portal vein thrombosis, predominantly affecting non-cirrhotic patients. While biliary abnormalities are commonly detected on imaging, clinically relevant disease is largely restricted to grade III portal cholangiopathy. Evidence regarding the natural history and optimal management of symptomatic disease remains limited, and therapeutic strategies are heterogeneous. In recent years, interventional radiology techniques, particularly portal vein recanalization with or without transjugular intrahepatic portosystemic shunt placement, have increasingly been incorporated into the management of selected patients. However, the absence of validated treatment algorithms and predictors of response represents a major unmet clinical need.
Study design and data collection:
This is an observational, retrospective, multicenter study. Data will be retrospectively collected from medical records using a dedicated case report form at predefined time points following the diagnosis of grade III portal cholangiopathy. All data will be pseudonymized, and patients will be assigned unique center-specific identifiers.
Inclusion criteria:
Patients aged ≥ 15 years with non-tumoral, non-cirrhotic portal vein thrombosis and either: (a) MR colangiopathy showing portal colangiopathy grade III according to the definition of Llop et al or (b) contrast-enhanced abdominal CT scan showing features consistent with grade III portal cholangiopathy, defined as: intrahepatic bile duct dilatation, with or without visible biliary strictures, after exclusion of other causes of bile duct dilatation