Best's vitelliform macular dystrophy complicated with choroidal neovascularization is rare in children. We report three children from a Malay family of five siblings with Best's vitelliform macular dystrophy, in which two of them subsequently developed choroidal neovascularization. The possible pathogenesis of this rare condition is described and highlighted in this report.

Psychophysical dark adaptation studies were performed in six patients with Best vitelliform macular dystrophy (BVMD) using a Goldmann-Weekers dark adaptometer. Prebleach thresholds were determined before obtaining a postbleach full recovery curve. Unlike patients with Stargardt macular dystrophy, all patients with BVMD showed a normal time to reach their baseline dark adapted thresholds after b...

vitelliform lesions: correlation of clinical findings and multiple imaging analyses. Retina. 2011;31(1):13-25. 2. Querques G, Forte R, Querques L, Massamba N, Souied EH. Natural course of adult-onset foveomacular vitelliform dystrophy: a spectral-domain optical coherence tomography analysis. Am J Ophthalmol. 2011;152(2):304-313. 3. Schachat AP, de la Cruz Z, Green WR, Patz A. Macular hole and r...

OBJECTIVES To report a child with early-onset autosomal recessive Best vitelliform macular dystrophy and compound heterozygous BEST1 mutations, the management of a choroidal neovascular membrane with intravitreal bevacizumab in the proband, the benefits of amblyopia therapy in the fellow eye, and the findings in the parents, carriers of heterozygous BEST1 mutations. METHODS A 5-year-old white...

BACKGROUND Vitelliform macular dystrophy is an autosomal-dominant disease and has two clinical variants: Best's (VMD2) and adult onset vitelliform macular dystrophy (AOVMD). We report an atypical presentation of VMD2. CASE A 50-year-old male presented with history of blurring of vision in left eye since two year. On fundus examination, left eye revealed a single, circular, yellow-opaque egg y...

A family with vitelliform macular dystrophy is presented in which the proband became symptomatic at age 51 rather than during the more typical first or second decade. Elderly patients with vitelliform macular dystrophy may have clinical findings resembling age-related degenerative choroidopathy of the macula.

OBJECTIVE To report a novel de novo vitelliform macular dystrophy (VMD2) mutation in a patient with Best macular dystrophy. METHODS Best-corrected visual acuity, dilated fundus examination, and electro-oculography were performed in a patient with Best macular dystrophy and his parents. Both the patient and his parents also had blood samples drawn, and their DNA was analyzed by direct genomic ...