Episode 288: WDx #23: Clinical Unknown Discussion with Dr Rebecca Berger
The Clinical Problem Solvers
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Acute on Chronic Blood Chiari Syndrome
The patient most likely had congenital hepatic vein anatomical abnormalities that resulted in this severe stenosis at the intra hepatic IBC and like hepatic vein juncture. And because of that, it created a scenario of chronic partial bed Chiari like process with congestion in the liver over time manifesting as like portal hypertension. Her initial presentation of thrombus edipenia was most likely due to the splenomegaly that was secondary to the portal hypertension and the splenic sequestration caused by the sloped vasodilation. She then manifested as acute bud Chiari syndrome that then turned into acute liver injury for which she was transferred to a transplant center. Just an exceptional case
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