Superior segmental optic nerve hypoplasia: A review.
Jo-Hsuan Wu1, Chao-Wen Lin2, Chun-Hsiu Liu3
1Shiley Eye Institute and Viterbi Family Department of Ophthalmology, University of California, San Diego, CA, USA.
Survey of Ophthalmology
|February 21, 2022
Summary
Superior segmental optic nerve hypoplasia (SSONH) is a rare congenital condition affecting the optic disc. Early recognition is crucial to differentiate it from glaucoma and prevent misdiagnosis.
Area of Science:
- Ophthalmology
- Developmental Biology
- Medical Diagnostics
Background:
- Superior segmental optic nerve hypoplasia (SSONH) is a congenital optic disc abnormality.
- Its prevalence is likely underestimated due to diagnostic challenges.
- Maternal diabetes is a known risk factor for SSONH.
Purpose of the Study:
- To highlight SSONH as a differential diagnosis for glaucoma.
- To describe characteristic clinical signs and diagnostic tools for SSONH.
- To emphasize the importance of differentiating SSONH from glaucoma.
Main Methods:
- Review of clinical characteristics and diagnostic findings of SSONH.
- Comparison of SSONH with glaucoma based on characteristic signs.
- Utilizing optical coherence tomography (OCT) for differentiation.
Main Results:
- SSONH presents with variable signs including superior optic disc abnormalities.
- OCT can distinguish SSONH from glaucoma by identifying superonasal vs. inferotemporal RNFL thinning.
- SSONH has a favorable, non-progressive prognosis.
Conclusions:
- Ophthalmologists must recognize SSONH to avoid misdiagnosis and unnecessary glaucoma treatment.
- Differentiating SSONH from glaucoma is essential for appropriate patient management.
- Understanding SSONH pathophysiology and diagnostic criteria improves patient outcomes.
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