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Show At the age of 7 years, left hand weakness developed due to nerve sheath tumors affecting the roots of C8 and T1. Genetic testing confirmed the diagnosis of NF2. We agree with Barrett et al that there is no clear explanation for the recurrent third nerve palsy, but that it can be a presenting symptom of NF2 in children. Manish Prasad, MBBS, MD, MRCPCH Dewsbury District Hospital, Dewsbury, United Kingdom Jessy Choi, MBChB, FRCOpth Department of Paediatric Opthalmology Peter Baxter, MBBS, MRCPCH Department of Paediatric Neurology, Sheffield Children's Hospital, Sheffield, United Kingdom The authors report no conflicts of interest. REFERENCE 1. Barrett VJM, Tan MH, Elston JS. Recurrent third nerve palsy as the presenting feature of neurofibromatosis type 2. J Neuroophthalmol. 2012;32:329-331. Utilizing Optical Coherence Tomography in Diagnosing a Unique Presentation of Chiasmal Hypoplasia Variant of Septo-Optic Dysplasia We evaluated a patient whose findings relate to 2 recent reviews in the Journal, that of Borchert (1) on optic nerve hypoplasia and Fraser et al (2) dealing with nonglau-comatous optic disc cupping. A 13-year-old adopted boy was referred for abnormal optic nerve appearance. On examination, the visual acuity was 20/20 in each eye and intraocular pressure was 13 mm Hg, right eye, and 14 mm Hg, left eye. Funduscopy revealed bilateral excavated and pale optic nerves (Fig. 1). Retinal nerve fiber layer (RNFL) analysis by spectral-domain optical coherence tomography showed a pattern consistent with bow-tie atrophy in each eye (Fig. 2). Automated visual field testing revealed bitemporal hemianopia. Magnetic resonance imaging (MRI) of the brain was remarkable for absence of septum pellucidum and chiasmal atrophy (Fig. 3). Based on these findings, the diagnosis of chiasmal hypoplasia-variant septo-optic dysplasia (SOD) was made. Our patient presented with optic nerve cupping, raising suspicion for juvenile open-angle glaucoma. However, the RNFL analysis revealed thinning of nasal and temporal quadrants, unlike glaucomatous damage, which typically involves thinning of the superior and inferior quadrants. The relatively preserved superior and inferior RNFL bundles on optical coherence tomo-graphic analysis correlated well with the findings of bitemporal hemianopia and the MRI findings of chias-mal hypoplasia. To our knowledge, this is the first description of RNFL analysis in the chiasmal hypoplasia variant of SOD. This FIG. 1. Optic discs show thinning of the nasal and temporal rims, consistent with bow-tie optic atrophy. Letters to the Editor: J Neuro-Ophthalmol 2014; 34: 95-104 103 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. analysis was instrumental in differentiating nonglaucoma-tous from glaucomatous optic nerve cupping and estab-lished correlation between structure and function. Aliza E. Epstein, BA Miller School of Medicine, University of Miami, Miami, Florida aeepstein@med.miami.edu Kara M. Cavuoto, MD Ta C. Chang, MD Department of Ophthalmology, Bascom Palmer Eye Institute, Miller School of Medicine, University of Miami, Miami, Florida The authors report no conflicts of interest. REFERENCES 1. Borchert M. Reappraisal of the optic nerve hypoplasia syndrome. J Neuroophthalmol. 2012;32:58-67. 2. Fraser CL, White AJ, Plant GT, Martin KR. Optic nerve cupping and the neuro- ophthalmologist. J Neuroophthalmol. 2013;33:377-389. FIG. 2. Spectral-domain optical coherence tomography confirms thinning of the temporal and nasal retinal nerve fiber layer. I, inferior; N, nasal; S, superior; T, temporal. FIG. 3. T2 coronal magnetic resonance imaging reveals atro-phy of optic chiasm (arrow) and absence of septum pellucidum. 104 Letters to the Editor: J Neuro-Ophthalmol 2014; 34: 95-104 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. |
