Bietti’nin Kristalin Distrofisinde Klinik, Anjiyografik, Optik Koherans Tomografik ve Elektrofizyolojik Bulgular

Abstract

Objective: To present the characteristic clinical, angiographic, electrophysiologic and optic coherence tomographic findings in eight patients with Bietti's crystalline dystrophy. Material and Methods: All patients had detailed ophthalmologic examination. Visual field examination, fluorescein angiography, electroretinography, electrooculography recordings, and optic coherence tomography were performed in all patients. Results: Best-corrected visual acuity ranged from 2/10 to 10/10. On fundoscopic examination, the reflective yellow deposits located especially in the posterior pole of the retina with the mottling of retina pigment epithelium were seen in all patients whereas in some of the cases pigment deposition, retina pigment epithelium and choriocapillaris atrophy were also noted. Corneal crystals were observed in the limbus in three cases . Central and paracentral scotomas were detected in perimetric examination. Arden ratios in electrooculography and the results of electroretinography recordings were different. Fluorescein angiography showed island like hypoflourescence corresponding to the geographic areas of retinal pigment epithelium and choriocapillary atrophy in the posterior pole. In addition to this fluorescein angiographic findings, in diffuse type cases, diffuse hypofluorescence that extended to the midperiphery was detected. Optic coherence tomographic examination disclosed multiple hyperreflective lesions in the retina and acoustic shadowing behind these hyperreflective lesions and a hyperreflective white band. Conclusion: Common characteristics of clinical, angiographic, electrophysiologic and optic coherence tomographic findings in patients with Bietti's crystalline dystrophy were described.