Teaching NeuroImages: Superior segmental optic nerve hypoplasia confirmed by optical coherence tomography
Citation Manager Formats
Make Comment
See Comments

Article Figures & Data
Figures
Figure 1 Humphrey visual field of the patient’s right and left eye
(A) Visual fields for the right eye (top) and left eye (bottom) revealed inferior arcuate defects consistent with superior segmental optic nerve hypoplasia. (B) Optic nerve photographs of the right eye (top) and left eye (bottom) showed evidence of superior segmental optic nerve hypoplasia.
Figure 2 Optic disc of the patient’s right and left eye
Optical coherence tomography of the patient’s retinal nerve fiber layer of the right eye (A) and left eye (B) revealed superior thinning compared to normal distribution. Right and left eye comparison (C) demonstrated more superior nerve fiber layer thinning in the right eye compared to the left eye.
- Geetha Athappilly, MD and
- Victoria S. Pelak, MD
- From the Departments of Ophthalmology (G.A., V.S.P.) and Neurology (V.S.P.), University of Colorado Denver School of Medicine; and The Denver Veterans Affairs Medical Center (V.S.P.), Denver, CO.
- Address correspondence and reprint requests to Dr. Victoria S. Pelak, 12631 E. 17th Avenue, P.O. Box 6511, Mail Stop B185, Aurora, CO 80045 Victoria.Pelak{at}UCHSC.edu
A 36-year-old woman without complaints was referred for abnormal visual fields (figure 1A). She had 20/20 vision in both eyes and a right afferent pupillary defect. Fundus examination suggested superior segmental optic nerve hypoplasia (SSONH), a congenital optic nerve disorder (figure 1B). Optical coherence tomography (OCT) of the optic nerve, which measures nerve fiber layer thickness using interferometric techniques,1 showed decreased thick-ness of the superior segment of both nerves (figure 2). In this case, OCT provided a noninvasive and reliable method for confirmation of SSONH suspected by inferior arcuate visual field defects and optic nerve appearance.2
Figure 1 Humphrey visual field of the patient’s right and left eye
(A) Visual fields for the right eye (top) and left eye (bottom) revealed inferior arcuate defects consistent with superior segmental optic nerve hypoplasia. (B) Optic nerve photographs of the right eye (top) and left eye (bottom) showed evidence of superior segmental optic nerve hypoplasia.
Figure 2 Optic disc of the patient’s right and left eye
Optical coherence tomography of the patient’s retinal nerve fiber layer of the right eye (A) and left eye (B) revealed superior thinning compared to normal distribution. Right and left eye comparison (C) demonstrated more superior nerve fiber layer thinning in the right eye compared to the left eye.
Footnotes
-
Disclosure: The authors report no disclosures.
REFERENCES
- ↵
Kahook M, Noecker RJ, Wollstein G, Schuman JS. Optic nerve head and nerve fiber layer imaging. In: Albert DM, Jakobiac FA, eds. Principles and Practice of Ophthalmology. Philadelphia, PA: WB Saunders;
- ↵
Unoki K, Ohba N, Hoyt WF. Optical coherence tomography of superior segmental optic hypoplasia. Br J Ophthalmol 2002;86:910–914.
Letters: Rapid online correspondence
REQUIREMENTS
If you are uploading a letter concerning an article:
You must have updated your disclosures within six months: http://submit.neurology.org
Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.
If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.
Submission specifications:
- Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
- Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
- Submit only on articles published within 6 months of issue date.
- Do not be redundant. Read any comments already posted on the article prior to submission.
- Submitted comments are subject to editing and editor review prior to posting.
You May Also be Interested in
Hemiplegic Migraine Associated With PRRT2 Variations A Clinical and Genetic Study
Dr. Robert Shapiro and Dr. Amynah Pradhan
Related Articles
- No related articles found.