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Journal of Child Neurology
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Choroid Plexus Papilloma Expansion Over 7 Years in Aicardi Syndrome

Richard E. Frye, MD, PhD, FAAP

Department of Pediatrics, Division of Pediatric Neurology, University of Texas Health Science Center, Houston, Texas, drfryemdphd{at}gmail.com

Jon S. Polling, MD, PhD

Athens Neurological Associates, Athens, Georgia

Louis C. K. Ma, MBChB, MRCP, MRCPCH, FHKAM (Paed), FHKCPaed

Department of Pediatrics, United Christian Hospital, Hong Kong, China

Choroid plexus papillomas have been reported in Aicardi syndrome. Management of these tumors is controversial because their natural progression in Aicardi syndrome has only been rarely documented. This report describes the progression of such a tumor over 7 years in a girl with Aicardi syndrome. A magnetic resonance imaging study at 2 months of age demonstrated a right ventricular mass that was consistent with a unilateral choroid plexus papilloma. The mass enlarged over the next 7 years without causing any clinically apparent symptoms, ventricular enlargement, hydrocephalus, or mass effect. The tumor was removed without change in behavior or development. The known cases of Aicardi syndrome associated with choroid plexus papillomas are reviewed. The heterogeneous nature of this lesion is highlighted.

Key Words: Aicardi syndrome • choroid plexus papilloma

Journal of Child Neurology, Vol. 22, No. 4, 484-487 (2007)
DOI: 10.1177/0883073807302759
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