Title
BEST DISEASE: OPPOSITE FINAL EVOLUTIONARY STAGES IN THE SAME PATIENT AND EARLY IDENTIFICATION OF ASYMPTOMATIC CHOROIDAL NEOVASCULARIZATION
Introduction
Purpose: To present a case of Best Disease with distinct final evolutionary stages in the same patient.
Methods
Case Report
Results
A 34 years old white female patient being followed up at another institution, attend for ophthalmological evaluation in the retinal subspecialty. No ophthalmic complaints. Past medical and ophthalmic history: Best disease diagnosed 16 years ago. No comorbidities, use of medications or drug abuse. Best corrected visual acuity was 20/20 in the right eye and counting fingers in the left eye. Anterior segment and intraocular pressure were normal. Fundus examination revealed (fundus photography) showed an egg-yolk like round or oval yellow smooth elevated lesion with a well-defined margin at the macula in the right eye and an area of atrophy and pigmentation without visible vitelliform deposit. Optical Coherence Tomography showed vitelliform lesion with subretinal fluid associated in the right eye and an atrophic lesion with loss of outer retina in the left eye. OCT Angiography of the right eye evidenced a vascular trunk arising from the choroid with extension to the subretinal space. The left eye exam could not be performed due to the poor visual acuity. Based on clinical history and ophthalmological examination, the hypothesis of choroidal neovascularization in the right eye and atrophic stage in the left eye was suggested. Injection of anti-vascular endotelial growth fator was proposed as treatment to the right eye and the bad prognosis of the left eye was explained to the patient.
Discussion
Best disease is a rare autosomal dominant disorder. The disease usually goes through several stages and eyes without choroidal neovascularization needs no treatment. With the advent of new imaging technologies, the understanding and classification of best disease no longer depends only on the fundoscopic appearance of vitelliform lesions and makes their classification more precise, allowing the earlier identification of neovascular membrane whose early treatment changes prognosis.
Keywords
Best Disease; Choroidal Neovascularization
Area
CLINICAL CASE
Authors
IURI CARDOSO DA SILVA, DELMA REGINA GOMES HUARACHI, VICENTE HIDALGO RODRIGUES FERNANDES, ERICK CARNEIRO HOLANDA, ANDREA MARA SIMOES TORIGOE, HEITOR PANETTA, FERNANDO RODRIGO PEREIRA CHAVES, MAURICIO ABUJAMRA NASCIMENTO