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Optical Coherence Tomography in Optic Nerve Hypoplasia: Correlation With Optic Disc Diameter, Nerve Fiber Layer Thickness, and Visual Function

Kelly, John P., PhD; Baran, Francine, MD; Phillips, James O., PhD; Weiss, Avery H., MD

doi: 10.1097/WNO.0000000000000596
Original Contribution

Background: The correlation between optic disc diameters (DDs) with average retinal nerve fiber layer thickness (RNFLT) and visual function in children with optic nerve hypoplasia (ONH) having nystagmus is unknown.

Methods: Data were obtained from a retrospective review of 28 children (mean age: 9.4 years; ±5.1). Optic DD was defined as the maximal horizontal opening of Bruch membrane with spectral optical coherence tomography combined with a confocal laser ophthalmoscope. Average RNFLT was obtained from circumpapillary b-scans. RNFLT was also remeasured at eccentricities that were proportionate with DD to rule out potential sampling artifacts. Visual function was assessed by visual acuity at last follow-up and by visual evoked potentials (VEP) in 11 patients. The eye with the larger DD, which had better visual acuity, was analyzed to exclude potential effects of amblyopia.

Results: DD was correlated with average RNFLT (r2 = 0.61), visual acuity (r2 = 0.32), and VEPs (r2 = 0.66). The relationship between RNFLT and DD was as follows: average RNFLT (μm) = 0.074 * DD (μm) − 18.8. RNFLT also correlated with the ratio of horizontal optic DD to macula-disc-margin distance (DD:DM; r2 = 0.59). RNFLT measured at eccentricities proportionate with DD showed progressive decrease in thickness only for DDs <1,100 μm. All patients with DD <1,000 μm had subnormal visual acuity, whereas those with DD <1,200 μm had subnormal VEPs.

Conclusions: DD correlates with average RNFLT and with visual function in children with ONH. Using OCT imaging, DD can be obtained in children with nystagmus and provides objective information.

Roger H. Johnson Vision Lab (JPK, FB, JOP, AHW), Division of Ophthalmology, Seattle Children's Hospital, Seattle, Washington; Department of Ophthalmology (JPK, FB, AHW), University of Washington Medical Center, Seattle, Washington; and Department of Otolaryngology (JOP), University of Washington School of Medicine, Seattle, Washington.

Address correspondence to John P. Kelly, PhD, Division of Ophthalmology, OA.5.342, Seattle Children's Hospital, 4800 Sand Point Way NE, Seattle, WA 98105; E-mail:

Supported by an unrestricted grant from the Peter LeHaye, Barbara Anderson, and William O. Rogers Endowment Funds.

None of the authors have any proprietary or financial interest in this manuscript, its software or devices stated wherein, or the funding agencies.

© 2018 by North American Neuro-Ophthalmology Society