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A case of sept optic dysplasia/De Morsier syndrome diagnosed from unconscious homonymous hemianopsia Kosuke Ozawa 1 , Yoshihiro Nakagawa 1 , Takahiro Suzuki 1 1Department of Ophthalmology, Tokai University Hospital pp.1043-1048
Published Date 2025/8/15
DOI https://doi.org/10.11477/mf.037055790790081043
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Abstract Purpose:Septo-optic dysplasia(SOD)/De Morsier syndrome is a rare congenital disorder associated with ocular symptoms, hypopituitarism, and abnormalities in the median mesencephalic structures. This report presents a case of SOD diagnosed based on the presence of an unrecognized homonymous hemianopsia.

Case:A 38-year-old healthy woman with no developmental delay or mental retardation was referred to our hospital for further examination of homonymous hemianopia. This condition was discovered during a pre-operative examination conducted before intraocular contact lens insertion surgery at another hospital. No subjective symptom of visual field defects or difficulty in daily life was reported;therefore, a congenital disease was suspected. The corrected visual acuity was 1.2 in both eyes. Goldmann visual field test revealed clear homonymous hemianopsia. Optical coherence tomography revealed homonymous focal thinning of the ganglion cell layer in the macula, consistent with the diagnosis of hemianopsia. Endocrine examination revealed decreased thyroid stimulating hormone levels.

 Magnetic resonance(MR) imaging of the head revealed no occupying lesions or cerebral infarcts;however, hypoplasia of the optic chiasm and right calcarine sulcus were observed. MR tractography revealed a difference between the right and left hemianopia in terms of the density of fibers in the occipitothalamic region, suggesting the presence of SOD.

Conclusion:Herein, SOD was detected based on the presence of congenital homonymous hemianopsia. SOD is often diagnosed during childhood;however, the severity of the disease varies. This case was considered mild. The possibility of congenital disease must be considered in patients with hemianopsia without subjective symptoms;SOD must be considered as a differential diagnosis for further investigation.


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電子版ISSN 1882-1308 印刷版ISSN 0370-5579 医学書院

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