Bestova viteliformní dystrofiekomplikovaná neovaskulárnímembránou a krvácením

Best s Vitelliform Dystrophy Complicated by a Neovascular Membrane and Haemorrhage

The objective of the paper is to draw attention to an uncommon picture of Bests vitelliform dystrophy of the macula (BVD)complicated by a choroidal neovas-cular membrane (CNVM)and haemorrhage.In an 8-year-old boy where incidentally at the age of 5 years cystic changes in bothmaculas were found and a reduced central visual acuity (CVA)of the left eye,theCVA of the so far asymptomatic right eye suddenly deteriorated.In the right maculaa vitelliform focus was present the lower half of which was formed by a vascularizedscar with subretinal haemorrhage on the margin.In the left macula was a roundchorioretinal scar with hyperplasia of the pigmented epithelium (RPE)and rem-nants of macular yellow.Fluoroangiographic examination (FAG)visualized classicalCNVM,optical coherence tomography (OCT)ablation of the RPE.The electrooculo-gram (EOG)was bilaterally reduced,CVA of the right eye was 6/60,of the left eye6/36.Examination of the mother revealed though the ophthalmological finding wasnormal,a significantly reduced EOG and in an older brother there was a smallvitelliform focus in both maculae and reduced EOG with CVA of 6/6.Conclusion:CNVM with haemorrhage is a rare complication of BVD.This compli-cation markedly extends the polymorphism of findings,is a risk of the CVA andmakes the diagnosis more difficult.EOG examination is decisive for the diagnosisof less common forms of BVD.

Key words:
Best ’s vitelliform dystrophy,choroidal neovascular membrane.