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중격 시신경 형성 장애 플러스

Septo-optic Dysplasia Plus

검안 및 콘택트렌즈학회지 2019년 18권 3호 p.100 ~ 104
오주현 ( Oh Ju-Heun ) - 가톨릭대학교 의과대학 여의도성모병원 안과 및 시과학교실

박미라 ( Park Mi-Ra ) - 가톨릭대학교 의과대학 여의도성모병원 안과 및 시과학교실


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.


Optic nerve hypoplasia; Septo-optic dysplasia; Schizencephaly
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