Denne Christian, Käsmann-Kellner Barbara, Ruprecht Klaus W
Orthoptik und Kinderophtalmologie, Universitäts-Augenklinik Homburg/Saar.
Klin Monbl Augenheilkd. 2003 Nov;220(11):767-73. doi: 10.1055/s-2003-44615.
Optic atrophy is one of the most common causes of severe visual impairment in children. So far an analysis of ocular and systemic findings comparing patients with and without optic atrophy has not performed.
Ocular and systemic findings of a total of 1042 patients (examination of all patients in May 1995 [N = 485] and May 2001 [N = 557]) of the department of paediatric ophthalmology in Homburg/Saar were retrospectively evaluated and diagnoses of patients with and without optic atrophy were compared. Optic atrophy was diagnosed ophthalmoscopically and in 1/3rd of the patients by VEP as well.
18 % of all patients (N = 188; 87.2 % [N = 164] were children) had optic atrophy. Nearly half of these children were prematurely born (46.7 %). 53.2 % of patients with optic atrophy (N = 88) showed nystagmus (without atrophy: 10.7 %), especially sensory defect nystagmus. Median of visual acuity level was 0.2 with optic atrophy and 0.8 without. Albinism and buphthalmia were common findings. 69.5 % of all patients with optic atrophy suffered from systemic diseases (without atrophy: 25.2 %), especially mental retardation, neurologic findings and oculocutaneous albinism. In 10.4 % more than two systemic diagnoses could be found. 55.3 % of all the patients with optic atrophy were disabled, 31.9 % multiply disabled.
Sequelae of prematurity, peripartal asphyxia and congenital brain damages are the main findings in patients with optic nerve atrophy. Such children are worst-case patients of any paediatric ophthalmology department with a high prevalence of severe visual impairment, mental retardation and multiple disability. Treatment in a specialised center is therefore necessary for an efficient therapy. In addition, the statistical survey shows that in children who survived the critical phases of prematurity secondary damages can lead to persistent impairment and to alterations of visual and general development.
视神经萎缩是儿童严重视力损害的最常见原因之一。迄今为止，尚未对有无视神经萎缩的患者的眼部和全身检查结果进行分析。
对洪堡/萨尔儿科眼科的总共1042例患者（1995年5月检查了所有患者 [N = 485]，2001年5月检查了所有患者 [N = 557]）的眼部和全身检查结果进行回顾性评估，并比较有无视神经萎缩患者的诊断情况。通过检眼镜诊断视神经萎缩，三分之一的患者还通过视觉诱发电位进行诊断。
所有患者中有18%（N = 188；87.2% [N = 164] 为儿童）患有视神经萎缩。这些儿童中近一半为早产儿（46.7%）。53.2% 的视神经萎缩患者（N = 88）出现眼球震颤（无萎缩者为10.7%），尤其是感觉缺陷性眼球震颤。视神经萎缩患者的视力中位数为0.2，无萎缩者为0.8。白化病和牛眼是常见表现。所有视神经萎缩患者中有69.5% 患有全身性疾病（无萎缩者为25.2%），尤其是智力发育迟缓、神经系统表现和眼皮肤白化病。10.4% 的患者可发现两种以上的全身性诊断。所有视神经萎缩患者中有55.3% 残疾，31.9% 为多重残疾。
早产、围产期窒息和先天性脑损伤的后遗症是视神经萎缩患者的主要表现。这类儿童是任何儿科眼科最严重的患者，严重视力损害、智力发育迟缓和多重残疾的患病率很高。因此，为了进行有效治疗，有必要在专门的中心进行治疗。此外，统计调查表明，在早产危险期存活下来的儿童中，继发性损害可导致持续性损害以及视力和总体发育的改变。
