The purpose is to show echographic presentation of retinal astrocytoma in Bourneville's disease and the possibilities of clinical and echographic diagnosis and follow-up. In our patient, partial and generalized seizures were the first sign of the disease at the age of 3.5 years. Computed tomography showed hyperdense cerebral lesions and Sabryl medication was efficient in controlling the seizures. At the age of 7, typical retinal mulberry lesions were seen on the fundus bilaterally. Echography revealed solid epiretinal masses of high surface and internal echogenicity casting a shadow on distal structures. There were two parapapillary lesions and one lesion in the periphery on the left eye. Due to permanent Sabryl therapy, perimetry and visual evoked potentials were monitored to show reduced retinal sensitivity in the periphery of both eyes. Facial angiofibrosis developed at the age of 8 years. Regular yearly controls up to the age of 12 were without significant changes in clinical and echographic characteristics. In conclusion, typical echographic presentation of retinal astrocytoma is of great help in differential diagnosis to other intrabulbar massive lesions in childhood, such as retinoblastoma.

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