Wilmer Eye Institute, Johns Hopkins Hospital, Baltimore, Maryland.
Wilmer Biostatistics Consulting Center, The Johns Hopkins University, Baltimore, Maryland3Dana Center for Preventive Ophthalmology, The Johns Hopkins University, Baltimore, Maryland.
JAMA Ophthalmol. 2015 May;133(5):542-8. doi: 10.1001/jamaophthalmol.2015.22.
Extended follow-up of treated and untreated retinopathy in incontinentia pigmenti (IP) has not previously been documented, to our knowledge.
To determine which eyes with IP are at risk for retinal detachment.
DESIGN, SETTING, AND PARTICIPANTS: Observational cohort study of patients with IP who were retrospectively identified at a tertiary care academic center between 1976 and 2013. Fifty eyes of 25 female participants meeting clinical criteria for IP were followed up for at least 6 months. The last year of follow-up was between 1987 and 2014.
Progression of retinopathy or the development of retinal detachment was assessed with fluorescein angiography, clinical examination, or both.
The median duration of follow-up was 9.3 years (range, 0.5-22.8 years). Over this period, 11 eyes (22%; 95% CI, 11%-33%) developed retinal detachment. The odds of retinal detachment were increased if there was retinal neovascularization (odds ratio, 11.61; 95% CI, 1.34-100.56; P = .03) or ischemic optic neuropathy (odds ratio, 5.27; 95% CI, 1.61-17.23; P = .006) on initial examination. A bimodal distribution of retinal detachments was observed, with most tractional detachments (7 eyes) occurring by age 2.5 years (median, 1.5 years; range, 14 days-7.0 years) and most rhegmatogenous detachments (4 eyes) occurring in adults (median age, 31.5 years; range, 14.0-47.0 years). Three eyes of young patients (≤2.5 years) developed tractional detachment, despite prophylactic ablation in 4 eyes; only one eye of older patients (≥14.0 years) developed retinal detachment following prophylactic ablation in 6 eyes. Persistent fetal vasculature appears to occur more commonly in IP (14%; 95% CI, 4%-25%) than in the general population.
All eyes with retinopathy due to IP should be monitored throughout adulthood for the development of retinal complications. During infancy and early childhood, ophthalmoscopic examination should be performed frequently so that prompt treatment can be initiated if there is progressive disease. Because of the nonrandomized nature of this study, the indications for prophylactic ablation and its success rate remain uncertain. Patients with less than 6 months of follow-up were excluded from the analysis, which could have biased this study cohort toward patients with more severe or less severe disease.
据我们所知，此前尚未有研究对色素失禁症（IP）患者接受和未接受治疗的视网膜病变进行过长期随访。
确定哪些 IP 患者的眼睛有发生视网膜脱离的风险。
设计、地点和参与者：对 1976 年至 2013 年期间在一家三级保健学术中心通过回顾性方法确定的符合 IP 临床标准的患者进行了观察性队列研究。对符合条件的 25 名女性患者的 50 只眼进行了至少 6 个月的随访。最后一年的随访时间为 1987 年至 2014 年。
通过荧光素血管造影、临床检查或两者结合来评估视网膜病变的进展或视网膜脱离的发生。
中位随访时间为 9.3 年（范围，0.5-22.8 年）。在此期间，有 11 只眼（22%；95%CI，11%-33%）发生了视网膜脱离。如果初始检查发现视网膜新生血管（比值比，11.61；95%CI，1.34-100.56；P =.03）或缺血性视神经病变（比值比，5.27；95%CI，1.61-17.23；P =.006），则发生视网膜脱离的可能性会增加。观察到视网膜脱离呈双峰分布，大多数牵拉性脱离（7 只眼）发生在 2.5 岁之前（中位数为 1.5 岁；范围为 14 天至 7.0 岁），大多数孔源性脱离（4 只眼）发生在成年人（中位数年龄为 31.5 岁；范围为 14.0-47.0 岁）。尽管对 4 只眼进行了预防性消融，但仍有 3 只年轻患者（≤2.5 岁）的眼发生了牵拉性脱离；只有 1 只年龄较大的患者（≥14.0 岁）在对 6 只眼进行预防性消融后发生了视网膜脱离。持续性胎儿血管似乎在 IP 中更为常见（14%；95%CI，4%-25%），而在普通人群中则不常见。
所有因 IP 而患有视网膜病变的眼都应在整个成年期监测视网膜并发症的发生情况。在婴儿期和幼儿期，应经常进行眼底检查，如果疾病进展，以便及时开始治疗。由于本研究为非随机研究，预防性消融的适应证及其成功率仍不确定。由于排除了随访时间少于 6 个月的患者，因此本分析可能使该研究队列偏向于疾病程度较轻或较重的患者。
