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 papillomas 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.
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Division of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA. arnold-menezes@uiowa.edu
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D061085:Agenesis of Corpus Callosum; D016545:Choroid Plexus Neoplasms / Q000175:diagnosis / Q000601:surgery*; D003399:Craniotomy; D004827:Epilepsy / Q000209:etiology / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis / Q000601:surgery*; D020288:Papilloma, Choroid Plexus / Q000175:diagnosis / Q000601:surgery*; D012008:Recurrence; D012086:Reoperation; D013577:Syndrome; D016896:Treatment Outcome; D014429:Twins, Dizygotic