Demonstrationof CicatricialStagewith Optical CoherenceTomography in A Child with Best VitelliformMacularDystrophy

Abstract

Best vitelliform macular dystrophy (BVMD) is a rare hereditary macular dystrophy. Various staging systems are performed in the clinical evaluation of BVMD. In this case report, it is aimed to demonstrate the presence of cicatricial stage in a 9-year-old boy. A 9-year-old boy was applied to our Eskisehir YunusEmre State Hospital OphthalmologyClinic with decreased visual acuity in his right eye. When the family history of the patient was questioned, it was learned that the other family members were also followed in another center due to loss of vision. BMVD diagnosis was confirmed by using electrophysiological tests and additional clinical studies. In optical coherence tomography, a hyporeflective lesion consistent with subretinal fluid was observed for both eyes. Additionally, a hump-shaped subretinalhyporeflective lesion was found in the right eye. Visual acuity is generally stable in the early stages of the disease for patients with BVMD, but the presence of a visual impairment is usually thought as a sign of the development of a choroidal neovascular membrane. Although rare, however, cicatricial stage, which is observed in the advanced stages of the disease, can be seen in small children. Distinguishing choroidal neovascular membrane from the cicatricial stage is necessary especially in children due to the risk of amblyopia and to make an accurate treatment planning.