Fundus findings and longitudinal study of visual acuity loss in patients with X-linked retinoschisis
Fundus findings and longitudinal study of visual acuity loss in patients with X-linked retinoschisis
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DOI：
10.1097/00006982-200507000-00012
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发表时间：
2005-07-01
影响因子：
3.3
通讯作者：
Rajagopalan, AS
中科院分区：
文献类型：
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作者：
Apushkin, MA;Fishman, GA;Rajagopalan, AS
目的：探讨青少年X连锁视网膜劈裂症(XLRS)的眼底表现和视力变化的自然病程。方法：对38例9~65岁的青少年XLRS患者进行回顾性研究。所有患者均获得最佳矫正视力、Goldmann视野、眼前节裂隙灯生物显微镜检查和扩张眼底检查结果。比较了最近一次就诊和初次就诊时的视力结果。术后随访1年~28年，平均10.2年。25例患者观察了5年，11例患者观察了15年。结果：黄斑中心凹病变从主要的放射状条纹(3%)、微囊性病变(34%)、蜂窝状囊肿(8%)或它们的组合(31%)到非囊性中心凹病变，如色素斑点(8%)、中心凹反射消失(8%)或萎缩样病变(8%)。视网膜血管倒位12例(32%)。我们观察到，即使在临床上没有明显的周围性视网膜劈裂的情况下，我们也观察到外周视野中的鼻部明显受限。在38例多次检查的患者中，使用最小分辨角度(LogMAR)比较的对数，4例视力下降了>；0.1logMAR，相当于ETDRS图表上的>；1线，视力下降了3例，视力下降了>；0.2logMAR。结论：我们队列中的38例XLRS患者，即使在较长的时间内，视力也有有限的变化。然而，与那些中心凹内有囊性改变的患者相比，那些在中心凹内有非囊性改变的患者，包括色素斑驳或萎缩外观损害，往往具有更明显的视力损害程度。
Purpose: To determine the presence of fundus findings and natural course of visual acuity change in patients with juvenile X-linked retinoschisis (XLRS).Methods: A retrospective longitudinal study of 38 patients with juvenile XLRS (age range, 9-65 years) was conducted. Best-corrected visual acuity, Goldmann visual fields, and results of slit-lamp biomicroscopy of the anterior segment and dilated fundus examination were obtained for all patients. Visual acuity findings at the most recent and initial visits were compared. Follow-up ranged from 1 year to 28 years (mean, 10.2 years). Twenty-five patients were observed for >5 years, and 11, for >= 15 years.Results: Foveal lesions varied from predominantly radial striations (3%), microcystic lesions (34%), honeycomblike cysts (8%), or their combinations (31%) to non-cystic-appearing foveal changes, such as pigment mottling (8%), loss of the foveal reflex (8%), or an atrophic-appearing lesion (8%). Twelve patients (32%) had situs inversus of their retinal vessels. We observed a superior nasal restriction in the peripheral visual field even in the absence of clinically apparent peripheral retinoschisis. Of the 38 patients who were seen more than once, using logarithm of the minimum angle of resolution (logMAR) comparison, 4 had a decrease in visual acuity of >0.1 logMAR, equivalent to >1 line on an ETDRS chart, in their better seeing eye, and 3 had a reduction in visual acuity of >0.2 logMAR in their better eye.Conclusions: A limited change in visual acuity was observed in our cohort of 38 patients with XLRS even over an extended period. However, those patients with non-cystic-appearing changes within the fovea, including pigment mottling or an atrophic-appearing lesion, tended to have a more appreciable degree of visual acuity impairment compared with those patients with a cystic-appearing foveal change.