Engstrom R E, Holland G N, Margolis T P, Muccioli C, Lindley J I, Belfort R, Holland S P, Johnston W H, Wolitz R A, Kreiger A E
UCLA Ocular Inflammatory Disease Center.
Ophthalmology. 1994 Sep;101(9):1488-502. doi: 10.1016/s0161-6420(94)31142-0.
The progressive outer retinal necrosis syndrome is a recently recognized variant of necrotizing herpetic retinopathy. This report characterizes more fully its clinical features and course.
Using standardized clinical criteria, patients with progressive outer retinal necrosis syndrome from four institutions were identified. Patient records were reviewed retrospectively for the following data: medical and demographic characteristics, presenting symptoms, physical findings, course, responses to treatment, and outcomes.
Thirty-eight patients (65 involved eyes) were studied. All had acquired immune deficiency syndrome. A known history of cutaneous zoster was documented in 22 (67%) of 33 patients. Median CD4 lymphocyte count was 21/mm3 (range, 0-130/mm3). Median follow-up was 12 weeks. The most common presenting symptom was unilateral decreased vision (35 of 65 eyes, 54%); median visual acuity at presentation was 20/30 (range, 20/20 to no light perception [NLP]). Anterior chamber and vitreous inflammatory reactions were absent or minimal in all patients. Typical retinal lesions were multifocal, deep opacities scattered throughout the periphery, although macular lesions also were present in 21 eyes (32%) at diagnosis. Lesions progressed rapidly to confluence. Initial intravenous antiviral therapy appeared to reduce disease activity in 17 (53%) of 32 eyes, but treatment did not alter final visual outcome. Visual acuity was NLP in 42 (67%) of 63 eyes within 4 weeks after diagnosis. Retinal detachment occurred in 43 (70%) of 61 eyes, including 13 (93%) of 14 eyes that received prophylactic laser retinopexy.
The progressive outer retinal necrosis syndrome is characterized by features that distinguish it from cytomegalovirus retinopathy, acute retinal necrosis syndrome, and other necrotizing herpetic retinopathies. Visual prognosis is poor with current therapies.
进行性外层视网膜坏死综合征是一种最近才被认识的坏死性疱疹性视网膜病变的变异型。本报告更全面地描述了其临床特征和病程。
采用标准化临床标准，确定了来自四个机构的进行性外层视网膜坏死综合征患者。回顾性查阅患者记录以获取以下数据：医学和人口统计学特征、首发症状、体格检查结果、病程、治疗反应及结局。
对38例患者（65只受累眼）进行了研究。所有患者均患有获得性免疫缺陷综合征。33例患者中有22例（67%）有带状疱疹病史记录。CD4淋巴细胞计数中位数为21/mm³（范围为0 - 130/mm³）。中位随访时间为12周。最常见的首发症状是单眼视力下降（65只眼中的35只，54%）；首发时的中位视力为20/30（范围为20/20至无光感[NLP]）。所有患者前房和玻璃体炎症反应均不存在或很轻微。典型的视网膜病变为多灶性，深部混浊散在分布于整个周边部，不过诊断时21只眼（32%）也有黄斑病变。病变迅速进展至融合。初始静脉抗病毒治疗似乎使32只眼中的17只（53%）疾病活动度降低，但治疗并未改变最终视力结局。诊断后4周内，63只眼中的42只（67%）视力为无光感。61只眼中有43只（70%）发生视网膜脱离，其中接受预防性激光视网膜光凝的14只眼中有13只（93%）发生视网膜脱离。
进行性外层视网膜坏死综合征具有一些特征，使其有别于巨细胞病毒性视网膜病变、急性视网膜坏死综合征及其他坏死性疱疹性视网膜病变。目前的治疗方法视力预后较差。
