[Prevalence of optic atrophy and associated ocular and systemic diseases in a department of paediatric ophthalmology].

Background: 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.

Patients And Methods: 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.

Results: 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.

Conclusions: 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.