97th DOG Annual Meeting 1999
P574
PRESENTATION OF A PATIENT WITH BEST'S DISEASE AND MULTIPLE VITELLIFORM CYSTS AND NORMAL ELECTRORETINOGRAM AND ELECTROOCULOGRAM
O. Arend, A. Remky, C. Dahlke
A male patient (47 years) was referred with reduced vision in the left eye. The right eye has known amblyopia since childhood. The best corrected visual acuity was 20/200 OD (corrective error +6,75 dpt) and 20/30 OS (corrective error +6,25). The anterior segment examination was unremarkable and the perimetry (Humphrey, 24-2) showed no defects. The fundus examination revealed central, yellowish vitelliform cysts OU and additional cysts in the temporal superior vessel formation OU and another formation temporal of the macula in the left eye. Photopic and scotopic ERG were normal. The EOG was normal (Arden ratio: OD 1.78, OS 2.34). The family history revealed no other affected siblings.
At the follow-up visit one year later the ERG tests were unchanged and the EOG showed again normal responses (Arden ratio OD: 2.6; OS: 1.96). The fundus examination showed different stages of the vitelliform cysts. The fluorescein angiography showed blocked fluorescence or slight hyperfluorescence depending on the stage of the vitelliform cysts.
In summary, the patient represents a rare case of juvenile Best with multiple vitelliform cysts. The differential diagnosis of central serous retinopathy or adult Best can be excluded from the clinic and the presentation at the follow up visit. Best's disease represents a generalized disorder of the retinal pigment epithelium which normally can be characterized with the EOG, however, not present in this patient.
Dept. of Ophthalmology, RWTH Aachen
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