Best's Vitelliform Macular Dystrophy

Maggon, Rakesh; Parihar, Jitendra Kumar Singh; Vats, DP; Mathur, Vijay

doi:10.1016/s0377-1237(08)80035-4

Cited by 4 publications

(2 citation statements)

References 5 publications

(6 reference statements)

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“…Peripheral retinal drusen are harbingers of AMDs, vere impairment of vision [17,22]. In comparison with the presentation of this index patient, there was no reduction of vision as the lesion did not involve the macula.…”

Section: Resultsmentioning

confidence: 65%

Atypical Bilateral Peripheral Drusen in a Forty-Year-Old Man: Case Report and Review of Literature

Ejike¹,

Chidi²,

Rex³

et al. 2020

Int J Ophthalmol Clin Res

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Section: Resultsmentioning

confidence: 65%

Atypical Bilateral Peripheral Drusen in a Forty-Year-Old Man: Case Report and Review of Literature

Ejike¹,

Chidi²,

Rex³

et al. 2020

Int J Ophthalmol Clin Res

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“…Fundus image of the vitelliform disease was divided into six stages: Stage I (Previtelliform), normal or only subtle RPE changes (tiny, central honeycomb structure centrally); Stage II (Vitelliform), classic “egg-yolk” lesion; Stage III (Pseudohypopyon), layering of lipofuscin; Stage IV (Vitelleruptive), breakup of material giving “scrambled-egg” appearance; Stage V (Atrophic), central RPE and retinal atrophy; and Stage VI, choroidal neovascular membrane (CNVM). [ 11 ] Cases with multifocal and extramacular hyper autofluorescent deposits and corresponding sub-normal electrophysiology with autosomal recessive inheritance were considered as ARB.…”

Section: Methodsmentioning

confidence: 99%

Clinical and visual electrophysiological characteristics of vitelliform macular dystrophies in the first decade of life

Padhy

Parameswarappa

Agarwal

et al. 2022

Indian J Ophthalmol

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Purpose: 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 children.

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