Clinical Characteristics of the Congenital Optic Nerve Abnormalities.

Kim, Seok Hwan; Choi, Mi Young; Yu, Young Suk

Original Article

Journal of the Korean Ophthalmological Society 2003;44(5):1131-1138.
Published online May 1, 2003.

Clinical Characteristics of the Congenital Optic Nerve Abnormalities.

Seok Hwan Kim, Mi Young Choi, Young Suk Yu

¹Department of Ophthalmology, Seoul National University College of Medicine, Korea. ysyu@snu.ac.kr
²Department of Ophthalmology, Chungbuk National University College of Medicine, Chungbuk National University Medical Research Institute, Korea.
³Seoul Artificial Eye Center, Seoul National University Hospital Clinical Research Institute, Korea.

선천시신경이상의 임상 양상

김석환 ( Seok Hwan Kim ) , 최미영 ( Mi Young Choi ) , 유영석 ( Young Suk Yu )

Abstract

PURPOSE
To identify clinical characteristics, associated ocular and systemic abnormalities, and the visual prognosis of congenital optic nerve abnormalities. METHODS: The medical records of the patients who were diagnosed to have congenital optic nerve abnormalities between 1987 and 2001 were retrospectively reviewed. RESULTS: Of the 152 patients (M: F=92: 60, 216 eyes), 42% had bilateral abnormalities. Mean follow up period was 33 months. Percentages of the congenital optic nerve abnormalities were as follows; optic nerve hypoplasia 42%, optic disc coloboma 34%, peripapillary staphyloma 9%, morning glory disc anomaly 8%, myelinated nerve fibers 5%, and optic disc aplasia 2%. Brain MRI was done in 56 patients and abnormalities were found in 64%. Associated ocular abnormalities were found in 36% of total patients and associated systemic abnormalities were found in 26%. Refractive error, especially myopia was common. Occlusion therapy was done in 57 patients and the visual acuity of 4 patients improved after follow-up of 20 months. CONCLUSIONS: Optic nerve hypoplasia was the most common congenital optic nerve abnormality. Attention must be paid to detect the associated ocular and systemic abnormalities. Visual prognosis was generally poor, but the best-corrected visual acuity must be achieved with the correction of refractive error and occlusion therapy.

Key Words: Congenital optic nerve abnormalities;Occlusion therapy;Optic nerve hypoplasia