Extrahepatic portal vein occlusion (EHPVO) from portal vein thrombosis is a rare condition associated with substantial morbidity and mortality. The purpose of this study is to investigate the efficacy of TIPS for the treatment of chronic EHPVO, cavernomatosis and mesenteric venous thrombosis in non-cirrhotic adults refractory to standard of care therapy. 39 patients with chronic EHPVO received TIPS. Laboratory parameters and follow-up were assessed at 1, 3, 6, 12, 24 and every 6 months thereafter. Two hepatologists adjudicated symptom improvement attributable to mesenteric thrombosis and EHPVO pre/post TIPS. Kaplan-Meier was used to assess primary and overall TIPS patency assessing procedural success. Adverse events, radiation exposure, hospital length-of-stay and patency were recorded. Cavernoma was present in 100%, with TIPS being successful in all cases using splenic, mesenteric and trans-hepatic approaches. Symptom improvement was noted in 26/30 (87%) at 6-month follow-up. 12 patients (31%) experienced TIPS thrombosis. There were no significant long-term laboratory adverse events or deaths. At 36 months, freedom from primary TIPS thrombosis was 63%; following secondary interventions, overall patency was increased to 81%. CONCLUSION: TIPS in chronic, non-cirrhotic EHPVO with cavernomas and mesenteric venous thrombosis is technically feasible, and does not adversely affect liver function. Most patients demonstrate subjective and objective benefit from TIPS. Improvement in patency rates are needed with proper timing of adjuvant anticoagulation.
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