Date: 01 May 2012

Choroidal neovascularization and bevacizumab therapy in Aicardi syndrome

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Dear Editor,

Aicardi syndrome is an uncommon neurodevelopmental disorder originally defined by a triad of infantile spasms, agenesis of the corpus callosum, and pathognomonic chorioretinal lacunae [1]. Previous reports have noted increased pigmentation or fading of lacunae over time [2, 3], but the development of new lesions has not been described. We report the first case of choroidal neovascularization (CNV) arising in a patient with Aicardi syndrome and its successful treatment with bevacizumab.

A 7-month-old girl was referred for chorioretinal lesions. She had been born at term by normal spontaneous vaginal delivery without complications and had developed normally until the onset of infantile spasms at the age of 3 months. These were treated successfully with oral prednisolone. Serum chemistries, complete blood count, CSF analysis, and MRI of the brain were all normal. Serologic tests for toxoplasmosis, syphilis, rubella, cytomegalovirus, herpes simplex, and Lyme disease were all neg ...