Padhy, Srikanta KumarParameswarappa, Deepika CAgarwal, KomalTakkar, BrijeshBehera, ShashwatPanchal, BhavikRamappa, MuralidharPadhi, Tapas RanjanJalali, Subhadra2023-08-252023-08-252022-07Padhy Srikanta Kumar, Parameswarappa Deepika C, Agarwal Komal, Takkar Brijesh, Behera Shashwat, Panchal Bhavik, Ramappa Muralidhar, Padhi Tapas Ranjan, Jalali Subhadra. Clinical and visual electrophysiological characteristics of vitelliform macular dystrophies in the first decade of life. Indian Journal of Ophthalmology. 2022 Jul; 70(7): 2516-25251998-36890301-4738http://imsear.searo.who.int/handle/123456789/224424Purpose: To evaluate patterns of pediatric vitelliform macular dystrophy (PVMD). Methods: This is a retrospective analysis of Indian children with vitelliform macular dystrophy (VMD) presenting within the first decade of life. Records were evaluated for clinical findings, family screening, and investigative findings including optical coherence tomography (OCT), fundus autofluorescence (FAF), full?field electroretinogram (ERG) and electrooculogram (EOG). Electrophysiology was scrutinized and audited for acquisition and interpretation errors. Findings on follow?up were also recorded. Results: 46 eyes of 24 patients were included. Mean age at presentation was 7.17 ± 2.17 years. Mean follow?up duration was 1.55 ± 1.69 years. Best disease was the commonest type of VMD detected (21 patients), while autosomal recessive bestrophinopathy was seen in three cases. Mean logMAR BCVA was 0.364 which decreased to 0.402 on follow?up. Hyperopia was noted in 29 out of 46 eyes (mean being +3.87 D, range ebing +0.75 to +8.75 D). Four eyes of four children had choroidal neovascular membrane at presentation, while another child developed while in follow?up. Solid type subretinal deposit was the commonest OCT finding (n = 29/38) and central hyper FAF was the commonest pattern (n = 18/32). EOG was available for review in 32 eyes, but was unreliable in 11 eyes. Seven eyes demonstrated complete absence of light rise on EOG. Conclusion: PVMD can present in advanced forms. Progression to complications with loss of visual acuity can happen within the first decade of life. EOG shows grossly suppressed waveforms in the light phase in a large number of such childrenAutosomal recessive bestrophinopathybest diseasebest vitelliform macular dystrophyelectrooculogrampediatric retinal dystrophyJournal ArticleIndiaVitreoretina and Uveitis Services, Mithu Tulasi Chanrai Campus, L V Prasad Eye Institute, Bhubaneswar, Odisha, IndiaSrimati Kanuri Santamma Centre for Vitreoretinal Diseases, L V Prasad Eye Institute, Hyderabad, Telangana, IndiaThe Centre of Excellence for Rare Eye Diseases, L V Prasad Eye Institute, Hyderabad, Telangana, IndiaIndian Health Outcomes, Public Health and Economics (IHOPE) Research Centre, L V Prasad Eye Institute, Hyderabad, Telangana, IndiaVitreoretina and Uveitis Services, Kode Venkatadri Chowdary Campus, L V Prasad Eye Institute, Vijayawada, Andhra Pradesh, IndiaVitreoretina and Uveitis Services, GMR Varalalakshmi Campus, L V Prasad Eye Institute, Visakhapatnam, Andhra Pradesh, IndiaJasti V Ramanamma Children’s Eye Care Centre, L V Prasad Eye Institute, Hyderabad, Telangana, India