Clinical, Angiographic, Optic Coherence Tomographic and Electrophysiological Findings in Bietti’s Crystalline Dystrophy

Abstract

A AB BS ST TR RA AC CT T O Ob bj je ec ct ti iv ve e: : To present the characteristic clinical, angiographic, electrophysiologic and optic coherence tomographic findings in eight patients with Bietti's crystalline dystrophy. M Ma at te er ri ia al l a an nd d M Me et th ho od ds s: : All patients had detailed ophthalmologic examination. Visual field examination, fluorescein angiography, electroretinography, electrooculography recordings, and optic coherence tomography were performed in all patients. R Re es su ul lt ts s: : 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. C Co on nc cl lu us si io on n: : Common characteristics of clinical, angiographic, electrophysiologic and optic coherence tomographic findings in patients with Bietti's crystalline dystrophy were described. K Ke ey y W Wo or rd ds s: : Electrooculography; fluorescein angiography; electroretinography; retinal degeneration; tomography, optical coherence Ö ÖZ ZE ET T A Am ma aç ç: : Bietti'nin kristalin distrofisi olan sekiz hastada karakteristik klinik, anjiyografik, elektrofizyolojik ve optik koherens tomografi bulgularını sunmak. G Ge er re eç ç v ve e Y Yö ön nt te em ml le er r: : Tüm hastalar tam bir göz muayenesinden geçti. Tüm hastalara görme alanı muayenesi, floresan anjiyografi, elektroretinografi, elektrookülografi kayıtları ve optik koherens tomografi yapıldı. B Bu ul lg gu ul la ar r: : En iyi düzeltilmiş görme keskinliği 2/10 ile 10/10 arasındaydı. Fundoskopik muayenelerde yansıyan sarı birikintiler özellikle retinanın arka kutbunda yerleşmişti, tüm retinalarda retina pigment epitelinin beneklenmesi görülüyordu, oysa olguların bazılarında pigment birikmesi ve retina pigment epiteli, koriyokapiller atrofi de fark edildi. Üç olguda limbusta korneal kristaller görüldü. Perimetrik muayenede santral ve parasantral skotomlar saptandı. Elektrookülografideki Arden oranları ve elektroretinografi kayıtlarının sonuçları farklıydı. Floresan anjiyografide retinal pigment epitelinin coğrafik al...