[Best's vitelliform dystrophy with complications of a neovascular membrane and hemorrhage]

The objective of the paper is to draw attention to an uncommon picture of Best s vitelliform dystrophy of the macula (BVD) complicated by a choroidal neovascular membrane (CNVM) and haemorrhage. In an 8-year-old boy where incidentally at the age of 5 years cystic changes in both maculars were found and a reduced central visual acuity (CVA) of the left eye, the CVA of the so far asymptomatic right eye suddenly deteriorated. In the right macula a vitelliform focus was present the lower half of which was formed by a vascularized scar with subretinal haemorrhage on the margin. In the left macula was a round chorioretinal scar with hyperplasia of the pigmented epithelium (RPE) and remnants of macular yellow. Fluoroangiographic examination (FAG) visualized classical CNVM, optical coherence tomography (OCT) ablation of the RPE. The electrooculogram (EOG) was bilaterally reduced, CVA of the right eye was 6/60, of the left eye 6/36. Examination of the mother revealed though the ophthalmological finding was normal, a significantly reduced EOG and in an older brother there was a small vitelliform focus in both maculae and reduced EOG with CVA of 6/6.

CNVM with haemorrhage is a rare complication of BVD. This complication markedly extends the polymorphism of findings, is a risk of the CVA and makes the diagnosis more difficult. EOG examination is decisive for the diagnosis of less common forms of BVD.