Teaching NeuroImages: Superior segmental optic nerve hypoplasia confirmed by optical coherence tomography
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 abnormalvisual fields (figure 1A). She had 20/20 vision in both eyesand a right afferent pupillary defect. Fundus examination suggestedsuperior segmental optic nerve hypoplasia (SSONH), a congenitaloptic nerve disorder (figure 1B). Optical coherence tomography(OCT) of the optic nerve, which measures nerve fiber layer thicknessusing interferometric techniques,1 showed decreased thick-nessof the superior segment of both nerves (figure 2). In this case,OCT provided a noninvasive and reliable method for confirmationof SSONH suspected by inferior arcuate visual field defectsand 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.
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.[Abstract/Free Full Text]
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