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Septo-optic Dysplasia Plus
Ann Optom Contact Lens 2019;18:100-104
Published online September 25, 2019
© 2019 The Korean Optometry & Contact Lens Study Society

Ju Heun Oh, MD, Mi Ra Park, MD, PhD

Department of Ophthalmology and Visual Science, Yeouido St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
Correspondence to: Mi Ra Park, MD, PhD
Department of Ophthalmology and Visual Science, Yeouido St. Mary's Hospital, #10 63-ro, Yeoundeungpo-gu, Seoul 07345, Korea
Tel: 82-2-3779-1240, Fax: 82-2-761-6869
Received July 18, 2019; Revised July 25, 2019; Accepted July 29, 2019.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Purpose: We report a case of septo-optic dysplasia plus diagnosed by brain magnetic resonance imaging (MRI) in patient with exotropia, low visual acuity, visual field defect, nystagmus and optic nerve hypoplasia.
Case summary: A 26-year-old male patient visited to clinic for left eye exotropia. At initial visit, the best corrected visual acuity was 0.25 in the right eye and 0.16 in the left eye. Prism cover test showed 30 prism diopters exotropia at primary gaze, and nystagmus was observed. Dilated fundus examination revealed optic nerve hypoplasia in both eyes, and the visual field test showed temporal hemianopsia in the left eye, superotemporal visual defect in the right eye. MRI showed an open lip schizencephaly in frontal lobe, fusion of the frontal horns of the lateral ventricles and wide communication of this fused segment with the third ventricle, hypoplastic optic chiasm and optic nerves, absent septum pellucidum, and he was diagnosed as septo-optic dysplasia plus by brain MRI.
Conclusions: Septo-optic dysplasia plus is a condition characterized by optic nerve hypoplasia, absence of septum pellucidum, and schizencephaly. We experienced septo-optic dysplasia plus and more extensive ocular findings using fundus photo, visual field.
Keywords : Optic nerve hypoplasia; Septo-optic dysplasia; Schizencephaly

September 2019, 18 (3)