# Successful Treatment of Portal Cavernoma Cholangiopathy With Portal Vein Recanalization and Transjugular Intrahepatic Portosystemic Shunt (TIPS)

## TL;DR

A rare liver condition called portal cavernoma cholangiopathy was successfully treated using a combination of portal vein recanalization and a shunt procedure.

## Contribution

The study demonstrates the effectiveness of PVR-TIPS as a primary treatment for non-cirrhotic patients with symptomatic PCC.

## Key findings

- Portal vein recanalization and TIPS via trans-splenic access restored portal flow and improved symptoms.
- Successful reintervention followed early stent thrombosis, leading to significant clinical improvement.
- PVR-TIPS is a viable treatment option for decompressing the portal system and relieving biliary obstruction.

## Abstract

Portal cavernoma cholangiopathy (PCC) is a rare but serious complication of chronic extrahepatic portal vein occlusion (EHPVO), resulting from cavernous transformation of the portal vein and extrinsic compression of the bile ducts. We present a 27-year-old male with a history of portal vein thrombosis and splenomegaly who developed symptomatic PCC secondary to chronic EHPVO. The patient underwent portal vein recanalization and transjugular intrahepatic portosystemic shunt (PVR-TIPS) via trans-splenic access. Despite early stent thrombosis, successful reintervention led to restoration of portal flow and significant symptom improvement. This case highlights the clinical utility of PVR-TIPS in managing symptomatic PCC in non-cirrhotic patients and supports its consideration as a primary treatment option for decompression of the portal system and relief of biliary obstruction.