Mesh : Humans Visual Fields Nystagmus, Congenital Scotoma / diagnosis etiology Albinism Retina Genetic Diseases, X-Linked

来  源:   DOI:10.1167/iovs.65.2.13   PDF(Pubmed)

Abstract:
UNASSIGNED: This is the first systematic comparison of visual field (VF) deficits in people with albinism (PwA) and idiopathic infantile nystagmus (PwIIN) using static perimetry. We also compare best-corrected visual acuity (BCVA) and optical coherence tomography measures of the fovea, parafovea, and circumpapillary retinal nerve fiber layer in PwA.
UNASSIGNED: VF testing was performed on 62 PwA and 36 PwIIN using a Humphrey Field Analyzer (SITA FAST 24-2). Mean detection thresholds for each eye were calculated, along with quadrants and central measures. Retinal layers were manually segmented in the macular region.
UNASSIGNED: Mean detection thresholds were significantly lower than normative values for PwA (-3.10 ± 1.67 dB, P << 0.0001) and PwIIN (-1.70 ± 1.54 dB, P < 0.0001). Mean detection thresholds were significantly lower in PwA compared to PwIIN (P < 0.0001) and significantly worse for left compared to right eyes in PwA (P = 0.0002) but not in PwIIN (P = 0.37). In PwA, the superior nasal VF was significantly worse than other quadrants (P < 0.05). PwIIN appeared to show a mild relative arcuate scotoma. In PwA, central detection thresholds were correlated with foveal changes in the inner and outer retina. VF was strongly correlated to BCVA in both groups.
UNASSIGNED: Clear peripheral and central VF deficits exist in PwA and PwIIN, and static VF results need to be interpreted with caution clinically. Since PwA exhibit considerably lower detection thresholds compared to PwIIN, VF defects are unlikely to be due to nystagmus in PwA. In addition to horizontal VF asymmetry, PwA exhibit both vertical and interocular asymmetries, which needs further exploration.
摘要:
这是首次使用静态视野检查法对白化病(PwA)和特发性婴儿眼球震颤(PwIIN)患者的视野(VF)缺陷进行系统比较。我们还比较了最佳矫正视力(BCVA)和中央凹的光学相干断层扫描测量值,Parafovea,PwA中的周围乳头视网膜神经纤维层。
使用Humphrey场分析仪(SITAFAST24-2)对62PwA和36PwIIN进行VF测试。计算每只眼睛的平均检测阈值,以及象限和中心措施。在黄斑区域手动分割视网膜层。
平均检测阈值显着低于PwA的标准值(-3.10±1.67dB,P<<0.0001)和PwIIN(-1.70±1.54dB,P<0.0001)。与PwIIN相比,PwA的平均检测阈值显着降低(P<0.0001),而与PwA的右眼相比,左眼的平均检测阈值显着降低(P=0.0002),但在PwIIN中则没有(P=0.37)。在PwA,上鼻VF明显低于其他象限(P<0.05)。PwIIN似乎显示出轻度的相对弓形阴点。在PwA,中心检测阈值与视网膜内外的中央凹变化相关.两组VF均与BCVA密切相关。
PwA和PwIIN中存在明显的外周和中枢VF缺陷,和静态VF结果需要在临床上谨慎解释。由于与PwIIN相比,PwA表现出相当低的检测阈值,VF缺陷不太可能是由于PwA中的眼球震颤。除了水平VF不对称,PwA表现出垂直和两眼不对称性,这需要进一步探索。
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