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Inner Macular Hyperreflectivity Demonstrated by Optical Coherence Tomography in Niemann-Pick Disease

Danielle S. Rudich, MD1; Christine A. Curcio, PhD2; Melissa Wasserstein, MD3; Scott E. Brodie, MD, PhD1
[+] Author Affiliations
1Department of Ophthalmology, Mount Sinai Medical Center, New York, New York
2Department of Ophthalmology, University of Alabama at Birmingham
3Department of Pediatrics, Genetics, and Genomic Sciences, Mount Sinai Medical Center, New York, New York
JAMA Ophthalmol. 2013;131(9):1244-1246. doi:10.1001/jamaophthalmol.2013.2374.
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We describe spectral-domain optical coherence tomography (OCT; Heidelberg Spectralis) imaging of 3 patients with Niemann-Pick disease type B. This expands the range of previously reported anatomical correlates of the ophthalmoscopic macular halo surrounding the “cherry-red spot.”1

Article InformationCorresponding Author: Scott E. Brodie, MD, PhD, Department of Ophthalmology, Mount Sinai Medical Center, Box 1183, One Gustave L. Levy Place, New York, NY 10029 (scott.brodie@mssm.edu).

Author Contributions:Study concept and design: Brodie.

Acquisition of data: Rudich, Curcio, Brodie.

Analysis and interpretation of data: All authors.

Drafting of the manuscript: Rudich, Curcio, Brodie.

Critical revision of the manuscript for important intellectual content: All authors.

Study supervision: Brodie.

Conflict of Interest Disclosures: None reported.

Funding/Support: This work was supported in part by an unrestricted grant from Research to Prevent Blindness to the Department of Ophthalmology, Mount Sinai School of Medicine, grant EY06109 from the National Institutes of Health and unrestricted funds from Research to Prevent Blindness and the EyeSight Foundation of Alabama to the Department of Ophthalmology, University of Alabama at Birmingham, and grant 5 MO1 RR00071 from the National Center for Research Resources, National Institutes of Health to the Mount Sinai General Clinical Research Center.

Additional Contributions: The OCT images in Figure 1A were graciously provided by Martin Pearlman, MD, East Lansing, Michigan.

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Figure 1.
Fundus Photographs and Optical Coherence Tomographic Images

A, Fundus photograph shows vascular tortuosity and white macular halo with (normal) red center (“cherry-red spot”) in a 34-year-old patient with Niemann-Pick disease. Spectral-domain optical coherence tomography through the foveal center (confirmed by forward bowing of the ellipsoid line [also referred to as the inner segment–outer segment line] and the external limiting membrane) shows hyperreflective tissue in the retinal ganglion cell layer at the shoulders of the foveal depression (arrowheads), extending across the foveal floor. Hyperreflective cells are also seen between the photoreceptors and the foveal floor in the region of the central bouquet. B, Fundus photograph shows subtle clouding in the foveal region of a 5-year-old patient with Niemann-Pick disease. Optical coherence tomography demonstrates hyperreflective tissue at the retinal surface outside the foveal depression (arrow). C, Fundus photograph shows mild perifoveal clouding in a 10-year-old patient with Niemann-Pick disease. Optical coherence tomography demonstrates hyperreflective tissue (arrow) at the retinal surface, outside the fovea. D, Spectral-domain optical coherence tomographic image of a healthy 30-year-old control subject is shown for comparison with A.

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Figure 2.
Histological Analysis of the Normal Human Fovea

A, A 1-μm-thick epoxy section through the retina and choroid of an 81-year-old woman is stained with toluidine blue. Note the darker staining of the central bouquet of cone photoreceptors and Müller cells, external to the foveal floor (arrowheads). Ch indicates choroid; GCL, ganglion cell layer; HFL, Henle fiber layer; IPL, inner plexiform layer; ONL, outer nuclear layer; and RPE, retinal pigment epithelium (scale bar = 100 μm). B, Excerpt from A, with a magnified view showing isolated ganglion cells within the foveal floor (arrowheads) (toluidine blue). The ganglion cell layer consists of ganglion cells, displaced amacrine cells, and intercalated Müller cells (scale bar = 50 μm).

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