Junqueira Daniela Laura Melo, Lopes Flavio Siqueira Santos, Biteli Luís Gustavo, Prata Tiago Santos
Arq Bras Oftalmol. 2013 Nov-Dec;76(6):380-2. doi: 10.1590/s0004-27492013000600014.
Pigmented paravenous retinochoroidal atrophy is an ocular disease characterized by outer retina and choroidal atrophy often with overlying intraretinal bone spicule pigment deposition along the retinal veins. As a rare condition, there is scant information in the literature regarding the pattern of inner retinal layers involvement. We present a case of a 41-year-old white man initially referred for a glaucoma evaluation. Fundoscopy revealed patches of retinochoroidal atrophy and light pigmentation extending from the optic nerve head along the inferior-temporal retinal veins in both eyes. Using different spectral-domain optical coherence tomography (SD-OCT) protocols we identified a significant thinning of the inner retinal layers along the inferior-temporal veins, but with a lucid interval surrounding the optic nerve head. Standard automated perimetry revealed a superior absolute arcuate scotoma sparing the central fixation (good structure-functional correlation). This pattern of inner retinal layers involvement was not previously described. We believe SD-OCT added significantly to the anatomical description of this case. Physicians should consider these new anatomical findings and correlate them with functional status while assessing these patients.
色素性静脉旁视网膜脉络膜萎缩是一种眼部疾病,其特征为视网膜外层和脉络膜萎缩,通常在视网膜静脉沿线有视网膜内骨针状色素沉着。作为一种罕见疾病,文献中关于视网膜内层受累模式的信息很少。我们报告一例41岁白人男性病例,最初因青光眼评估前来就诊。眼底检查发现双眼视网膜脉络膜萎缩斑和淡色素沉着,从视盘沿颞下视网膜静脉延伸。使用不同的光谱域光学相干断层扫描(SD-OCT)方案,我们发现沿颞下静脉的视网膜内层显著变薄,但视盘周围有一个清晰的间隔。标准自动视野检查显示上方绝对弓形暗点,中央注视点不受影响(结构-功能相关性良好)。这种视网膜内层受累模式此前未被描述过。我们认为SD-OCT显著增加了对该病例的解剖学描述。医生在评估这些患者时应考虑这些新的解剖学发现,并将它们与功能状态相关联。