Three choroid plexus papillomas in a patient with Aicardi syndrome - A case report

Aicardi syndrome is an X-linked dominant disorder primarily defined by the triad of corpus callosum agenesis, infantile spasms and a pathognomonic lac unar chorioretinopathy. Papillomas of the choroid plexus have been reported in affected patients. We report an Aicardi syndrome patient who had three separate choroid plexus papiilomas and associated hydrocephalus, A dizygoti c twin was unaffected, Staged resection of the tumors was safely accomplish ed, with improvement in seizure control. imaging between procedures reveale d rapid tumor growth. Copyright (C) 2000 S. Karger AG, Basel.