LAUSR

© BMJ Publishing Group Limited 2021. No commercial reuse. See rights and permissions. Published by BMJ. DESCRIPTION A 35yearold woman presented to us with a sudden onset diminution of vision in the right eye. She had reduced vision in both eyes since childhood. At presentation, her best corrected visual acuity (BCVA) was 20/120 OD and 20/40 OS. Anterior segment examination was within the normal limits. Dilated fundus examination showed yellow subretinal deposits under the macula in both eyes. OD showed dense flameshaped intraretinal haemorrhages along the superotemporal (ST) arcade extending to the fovea with dilated and tortuous ST veins. Optical coherence tomography revealed foveal thinning with cystoid macular oedema and minimal subretinal fluid OD and OS showed subretinal hyperreflective deposits with subretinal fluid (figure 1). A diagnosis of Best vitelliform maculopathy with right superotemporal branch retinal vein occlusion (BRVO) was made. Intravitreal ranibizumab (0.5 mg/0.05 mL) was injected in the right eye and vision improved to 20/60 at 1 month followup. The patient later developed retinal neovascularisation in the ST quadrant with minimal dispersed vitreous haemorrhage. Sectoral laser photocoagulation was performed and the retina was stable for more than a year without vitreous haemorrhage or macular oedema and BCVA of 20/60. However, after 14 months, the patient developed sudden onset of loss of vision preceded by floaters OD. On examination, vitreous haemorrhage obscuring a major part of the retina was noted. Patient was advised observation with a propped up position. The haemorrhage did not clear for >4 weeks and pars plana vitrectomy with additional laser photocoagulation was performed. Vision improved to 20/40 and has remained stable for more than a year till last followup (figure 2). Best vitelliform macular dystrophy or Best disease is a hereditary macular dystrophy which comes under the spectrum of bestrophinopathies which involves mutation of the gene BEST1 present in the long arm of chromosome 11 (11q12). The disease usually presents itself in childhood and young adults and is characterised by typical egg yolk appearance in the macula. As the disease progresses, it takes the appearance of pseudohypopyon, scrambled egg, atrophic and may even lead to the appearance of choroidal neovascularisation