Título / Title

Bietti's Crystalline Dystrophy

Introdução / Purpose

The purpose of the present work will be to present a rare clinical case of bietti crystal dystrophy in a male patient, 46-years-old and asymptomatic.

Material e Método / Methods

Detailed description of the clinical case

Resultados / Results

Examination of both eyes revealed retinal mapping with numerous, thin, yellowish-white crystals scattered throughout the posterior pole with rarefaction and atrophy of the retinal pigment epithelium. On computerized visual field examination, localized loss of sensitivity with absolute and relative peripheral scotomas. Fluorescein angiography shows initial hypofluorescence due to non-perfusion of the choriocapillaris and on posterior segment OCT hyperreflectivity spots corresponding to crystalline deposits throughout the sensorineural retina and retinal pigment epithelium with irregularity of the outer retina and retinal pigment epithelium and disruption of the ellipsoid zone.

Discussão e Conclusões / Conclusion

Patients with bietti crystal dystrophy may be asymptomatic until adulthood. Symptoms usually start between the 3rd and 4th decade of life with progressive decrease in visual acuity and nyctalopia. There is no treatment for this type of dystrophy, but early diagnosis is important for guidance on patient and family follow-up, rehabilitation when necessary, and treatment of complications such as choroidal neovascularization.

Palavras Chave

bietti dystrophy, crystals, retinal atrophy

Area

CLINICAL RETINA