Título / Title

CENTRAL AREOLAR DYSTROPHY: THE CHALLENGE IN DIAGNOSIS OF MACULAR DYSTROPHIES

Introdução / Purpose

The Central Areolar Dystrophy is rare and affect the macula, leading to changes in the retinal pigment epithelium (RPE) with structural losses that includes choriocapillaris. The disease usually shows an autosomal dominant genetical inherited with progressive, slow and bilateral evolution.

Material e Método / Methods

Case report of a patient with Central Areolar Dystrophy and association of literature review, review of medical records, interview and photographic register.

Resultados / Results

Man, 60 years, brazilian, complaining of progressive low visual acuity over the past 20 years. He denies use of eye drops, he denies night blindness (nyctalopia).
Medical history: treating schizophrenia.
Best Corrected Visual Acuity: OD: < 20/400 (hand moviments), OS: < 20/400 (hand moviments) without any improvement with correction.
Slit Lamp examination: mild nuclear cataract bilaterally
Goldmann Visual Fields: large central absolut scotoma
Dilated fundus examination: Physiological cups, area of well – circumscribed retinal pigment epithelium atrophy and rarification of the choriocapillaris with geographic atrophy enabling the view of the large choroidal vessels in macula, bilaterally.
Optical Coherence Tomography : OD: Atrophy of all retinal external segments, retinal pigment epithelium atrophy, area of possible subfoveolar fibrosis. Macular Thinckness OD: 119 OS: Atrophy retinal diffuse, severe atrophy retinal pigment epithelium. Macular Thinckness OS: 143

Discussão e Conclusões / Conclusion

The case reported shows a patient with Central Areolar Dystrophy in advanced phase. The case is yet relevant when shows the difficult relationship of the ophthalmologists and their patients with non-cure or treatment diseases and which sometimes compromises the patient's adherence to the adaptation required for future visual impairment.

Palavras Chave

Central Areolar Dystrophy, Macular Dystrophies, Geographic Atrophy

Area

CLINICAL RETINA