Chronic non-cirrhotic extrahepatic portal vein obstruction (CNC-EHPVO) is a rare condition that can lead to severe portal hypertension and its associated complications, including gastrointestinal bleeding, portal cholangiopathy, ascites, and extension or recurrence of portal thrombosis. Managing these complications is particularly challenging due to the lack of standardized treatment protocols.
Recanalization of CNC-EHPVO has emerged as a strategy for managing severe portal hypertension in these patients, offering significant long-term benefits with a good safety profile. Additionally, a combined approach with transjugular intrahepatic portosystemic shunt (TIPS) placement may be considered in cases of advanced liver fibrosis, although this carries some risks such as hepatic encephalopathy, heart failure, or liver insufficiency.
Clinical and radiological follow-up after portal vein recanalisation is essential to manage stent patency and clinical complications, yet it remains less codified.
A recent study have shown the performance of SSM by VCTE as a non-invasive predictor for portal hypertension and presence of high-risk varices (HRV) in patients with CNC-EHPVO.
However, the role of SSM in predicting complications post recanalization in patients with CNC-EHPVO who have undergone portal vein recanalization is not known.
Design:
Part I: Derivation Cohort
The learning cohort will include patients with CNC-EHPVO from French centers who underwent portal vein recanalization and who had spleen stiffness measurement by VCTE before and after recanalization. Diagnosis of CNC-EHPVO will be based on VALDIG criteria, using both clinical and imaging features.
Part II: Validation Cohort
The validation cohort will include patients from the other VALDIG centers who underwent portal vein recanalization for CNC-EHPVO and meet the same inclusion criteria.
These patients will be followed longitudinally to evaluate the predictive value of spleen stiffness for re-thrombosis and liver-related outcomes.
Inclusion Criteria:
- Patients ≥18 years old, with CNC-EHPVO and signs of portal hypertension according to VALDIG criteria
- Available spleen stiffness measurement by VCTE using FibroScan® performed before recanalization and after stent placement
Non-Inclusion Criteria:
- Other causes of portal hypertension (Budd-Chiari syndrome, cardiac insufficiency, hereditary hemorrhagic telangiectasia etc)
- Portal vein malignant invasion;
- Cirrhosis (Exclusion of underlying cirrhosis can be based on imaging findings and/or liver stiffness measurement <10 kPa.
- Tense ascites at the time of SSM – VCTE.
Data Collection:
Data will be collected from medical records in a dedicated case report form by investigators participating in the study.:
- Demographics , etiological data, clinical presentation, upper endoscopic data and laboratory data at baseline and during follow up
- Imaging results
- Baseline and follow-up liver and spleen stiffness measurements by VCTE
- Liver-related events during follow-up, including re-thrombosis and clinical decompensation
Data will be pseudonymized, and each patient will be assigned a unique identifier based on the center and patient number.