Nonglaucomatous Cupping: Fundus Photography and Spectral Domain Optical Coherence Tomography Imaging Features

Nonglaucomatous cupping is commonly encountered in neuro-ophthalmic practice. However, the progression of clinical and imaging findings over time has not been well described. We present serial fundus photographs and spectral domain optical coherence tomography from a pediatric patient with neuromyelitis optic spectrum disorder, which demonstrated progression of both cupping and optic atrophy in the setting of normal intraocular pressure.

Photo Essay Section Editor: Timothy J. McCulley, MD Nonglaucomatous Cupping: Fundus Photography and Spectral Domain Optical Coherence Tomography Imaging Features Inas F. Aboobakar, MD, Pradeep Mettu, MD, Mays A. El-Dairi, MD FIG. 1. Serial fundus photographs and spectral domain optical coherence tomography images document progressive optic disc cupping and pallor in a patient with neuromyelitis optica spectrum disorder. Abstract: Nonglaucomatous cupping is commonly encountered in neuro-ophthalmic practice. However, the progression of clinical and imaging findings over time has not been well described. We present serial fundus photographs and spectral domain optical coherence tomography from a pediatric patient with neuromyelitis optic spectrum disorder, Department of Ophthalmology (IFA, PM, MAE-D), Duke University Medical Center, Durham, North Carolina. The authors report no conflicts of interest. M. A. El-Dairi is a consultant for Prana Pharmaceuticals. Address correspondence to Mays A. El-Dairi, MD, Duke University Eye Center, 2351 Erwin Road, Box 3802, Durham, NC 27710; E-mail: [e-mail] 402 which demonstrated progression of both cupping and optic atrophy in the setting of normal intraocular pressure. Journal of Neuro-Ophthalmology 2016;36:402-403 doi: 10.1097/WNO.0000000000000436 © 2016 by North American Neuro-Ophthalmology Society A 12-year-old African American girl experienced acute bilateral vision loss. Visual acuity was hand motion in each eye. Her optic nerves were normal in appearance with normal retinal nerve fiber layer (RNFL) thickness on spectral domain optical coherence tomography (Fig. 1). Intraocular pressures were 21 mm Hg, right eye, and Aboobakar et al: J Neuro-Ophthalmol 2016; 36: 402-403 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay 19 mm Hg, left eye. Magnetic resonance imaging showed bilaterally enhancing optic nerves and clinically silent lesions in the cervical and thoracic spinal cord. Aquaporin4-IgG testing was positive. She responded well to intravenous steroid treatment. On follow-up 9 months later, vision recovered to 20/20 in each eye, with normal visual fields and color vision. Intraocular pressure was 17 mm Hg in each eye. There was temporal optic disc pallor in each eye and diffuse RNFL thinning bilaterally (Fig. 1). Fourteen months after onset of symptoms, the patient experienced a drop in vision in her right eye. Visual acuity was 20/200, right eye, and 20/25, left eye. Color vision was reduced in the right eye, but normal in the left eye. Intraocular pressures were 12 mm Hg, right eye, and 11 mm Hg, left eye. Perimetry revealed superior visual field loss in the right eye. Progression of optic nerve cupping and slight progression of the diffuse RNFL thinning were noted bilaterally (Fig. 1). The patient was treated with intravenous steroids. Twenty-nine months after initially noting decreased visual acuity, vision was 20/30, right eye and 20/20, left eye. Color vision was intact and intraocular pressures were 13 mm Hg, right eye and 14 mm Hg, left eye. Despite demonstrating clinical recovery from optic neuritis, increased cupping and pallor were visible bilaterally (right eye . left eye) with increased RNFL thinning in the right eye (Fig. 1). Previous studies have examined several features that differentiate glaucomatous from nonglaucomatous optic disc cupping. Although previous reports have documented cupping with optic atrophy from optic neuritis and other nonglaucomatous pathologies (1,2), some have argued that the cupping observed in these cases is a physiologically cupped nerve that has developed pallor. In addition, progressive cupping in optic neuritis has been reported previously (3,4). It has been proposed this may be due to resolution of mild RNFL swelling seen in the acute phase of the optic neuritis (5). Our patient demonstrated progression of optic disc cupping with no evidence of optic disc edema. The clinical and imaging findings in our patient are consistent with several characteristics of nonglaucomatous cupping previously described, including 1) diffuse cupping, in contrast to the focal notching seen in glaucoma (6); 2) significant neuroretinal rim pallor; 3) a diffuse pattern of RNFL loss, including temporal and nasal thinning (1,7); 4) absence of vascular changes seen in glaucoma, such as bayoneting of vessels; 5) normal intraocular pressures; and 6) decreased visual acuity. Armaly (8) first introduced the concept of cup/disc ratio to provide a standardized method for assessing optic disc Aboobakar et al: J Neuro-Ophthalmol 2016; 36: 402-403 appearance. According to the Armaly scale, an enlarged cup/disc ratio due to RNFL loss is characteristic of glaucomatous damage. In contrast, Jonas et al (9) argued that changes in neuroretinal rim area, which lead to a decreased horizontal to vertical cup/disc ratio, is more specific for glaucomatous damage. Our patient demonstrated a diffuse pattern of RNFL thinning, in contrast to the classic superotemporal and inferotemporal thinning characteristic of glaucoma (10). Our case would be consistent with glaucomatous cupping based on the Armaly scale but not based on the Jonas scale. Additional prospective studies are necessary to identify specific imaging or other biomarkers that improve discrimination of glaucomatous from nonglaucomatous optic disc cupping. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: I. F. Aboobakar; P. Mettu; M. A. El-Dairi; b. Acquisition of data: I. F. Aboobakar; P. Mettu; M. A. El-Dairi; c. Analysis and interpretation of data: I. F. Aboobakar; P. Mettu; M. A. El-Dairi. Category 2: a. Drafting the manuscript: I. F. Aboobakar; P. Mettu; M. A. El-Dairi; b. Revising it for intellectual content: I. F. Aboobakar; P. Mettu; M. A. El-Dairi. Category 3: a. Final approval of the completed manuscript: I. F. Aboobakar; P. Mettu; M. A. El-Dairi. REFERENCES 1. Gupta PK, Asrani S, Freedman SF, El-Dairi M, Bhatti MT. Differentiating glaucomatous from non-glaucomatous optic nerve cupping by optical coherence tomography. Open Neurol J. 2011;5:1-7. 2. Rebolleda G, Noval S, Contreras I, Amalich-Montiel F, GarciaPerez JL, Munoz-Negrete FJ. Optic disc cupping after optic neuritis evaluated with optic coherence tomography. Eye (Lond). 2009;23:890-894. 3. Wan MJ, Adebima O, Benson LA, Gorman MP, Heidary G. Visual outcomes in pediatric optic neuritis. Am J Ophthalmol. 2014;158:503-507. 4. Balcer LJ. Clinical practice. Optic neuritis. N Engl J Med. 2006;354:1273-1280. 5. Kupersmith MJ, Mandel G, Anderson S, Meltzer DE, Kardon R. Baseline, one and three month changes in the peripapillary retinal nerve fiber layer in acute optic neuritis: relation to baseline vision and MRI. J Neurol Sci. 2011;308:117-123. 6. Zhang YX, Huang HB, Wei SH. Clinical characteristics of nonglaucomatous optic disc cupping. Exp Ther Med. 2014;7:995-999. 7. Pasol J. Neuro-ophthalmic disease and optical coherence tomography: glaucoma look-alikes. Curr Opin Ophthalmol. 2011;22:124-132. 8. Armaly MF. Genetic determination of cup/disc ratio of the optic nerve. Arch Ophthalmol. 1967;78:35-43. 9. Jonas JB, Gusek GC, Naumann GO. Optic disc morphometry in chronic primary open-angle glaucoma. I. Morphometric intrapapillary characteristics. Graefes Arch Clin Exp Ophthalmol. 1988;226:522-530. 10. Jonas JB, Fernandez MC, Sturmer J. Pattern of glaucomatous neuroretinal rim loss. Ophthalmology. 1993;100:63-68. 403 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.