Selected Ophthalmological Features in Children with Septo-Optic Dysplasia and Optic Nerve Hypoplasia

Michael S Salman; Shakhawat Hossain; Elizabeth Carson; Chelsea A Ruth; Ian H Clark

doi:10.1080/01658107.2022.2077967

Selected Ophthalmological Features in Children with Septo-Optic Dysplasia and Optic Nerve Hypoplasia

Neuroophthalmology. 2022 Jul 7;46(6):367-374. doi: 10.1080/01658107.2022.2077967. eCollection 2022.

Authors

Michael S Salman¹, Shakhawat Hossain², Elizabeth Carson³, Chelsea A Ruth⁴, Ian H Clark⁵

Affiliations

¹ Section of Pediatric Neurology, Winnipeg Children's Hospital and Department of Pediatrics and Child Health, Max Rady College of Medicine, Rady Faculty of Health Sciences, University of Manitoba, Winnipeg, Manitoba, Canada.
² Department of Mathematics and Statistics, University of Winnipeg, Winnipeg, Manitoba, Canada.
³ Pediatric Ophthalmology, Children's Hospital, Health Science Centre, Winnipeg, Manitoba, Canada.
⁴ Section of Neonatology, Department of Paediatrics and Child Health, Max Rady College of Medicine, Rady Faculty of Health Sciences, University of Manitoba, Winnipeg, Manitoba, Canada.
⁵ Section of Pediatric Ophthalmology, Department of Ophthalmology, Max Rady College of Medicine, Rady Faculty of Health Sciences, University of Manitoba, Winnipeg, Manitoba, Canada.

Abstract

Septo-optic dysplasia (SOD) and optic nerve hypoplasia (ONH) cause congenital visual impairment. Their aetiology is mostly unknown. Our aim was to investigate the prevalence of selected ophthalmological features in patients with these disorders. A chart review was performed on patients with SOD/ONH. Ophthalmological data were extracted. There were 102 patients (56 males). The median age at the end of the study was 12.7 years. Best-corrected visual acuity ranged from normal to no light perception. Bilateral ONH was more common than unilateral ONH. Strabismus (85%) and to a lesser extent nystagmus (52%) were both very common in our cohort. Patients with esotropia had worse visual acuity than those who had exotropia. The presence of nystagmus was more likely in cases with bilateral ONH. Therefore, patients with SOD/ONH may have normal visual acuity. Many have strabismus, which may cause amblyopia thereby further decreasing visual acuity. Nystagmus occurs commonly and its presence typically indicates bilateral ONH.

Keywords: Vision; nystagmus; optic nerve hypoplasia; paediatrics; septo-optic dysplasia; strabismus.

Grants and funding

The authors reported there is no funding associated with the work featured in this article.