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 lacunar 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 dizygotic twin was unaffected, Staged resection of the tumors was safely accomplished, with improvement in seizure control. imaging between procedures revealed rapid tumor growth. Copyright (C) 2000 S. Karger AG, Basel.