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Brief Report |

Optical Coherence Tomography Angiography in Choroideremia Correlating Choriocapillaris Loss With Overlying Degeneration

Nieraj Jain, MD1,2; Yali Jia, PhD2; Simon S. Gao, PhD2; Xinbo Zhang, PhD3; Richard G. Weleber, MD2; David Huang, MD, PhD2; Mark E. Pennesi, MD2
[+] Author Affiliations
1Department of Ophthalmology, Emory University, Atlanta, Georgia
2Casey Eye Institute, Oregon Health & Science University, Portland
3Division of Biostatistics, Department of Public Health and Preventive Medicine, Oregon Health & Science University, Portland
JAMA Ophthalmol. 2016;134(6):697-702. doi:10.1001/jamaophthalmol.2016.0874.
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Importance  Novel therapies for choroideremia, an X-linked recessive chorioretinal degeneration, demand a better understanding of the primary site(s) of cellular degeneration. Optical coherence tomography angiography allows for choriocapillaris (CC) imaging. We compared the extent of structural alterations of the CC, retinal pigment epithelium, and photoreceptors with multimodal imaging.

Observations  In a clinical case series conducted from September 15, 2014, through February 5, 2015, 14 eyes of 7 male patients with choroideremia (median age, 34 years [interquartile range, 15-46 years]; age range, 13-48 years), 4 eyes of 2 women with choroideremia carrier state (both in mid-50s), and 6 eyes of 6 controls (median age, 42.5 years [interquartile range, 33-55 years]; age range, 24-55 years) underwent multimodal imaging with optical coherence tomography angiography and electroretinography. The mean (SD) macular CC density was 82.9% (13.4%) in patients with choroideremia, 93.0% (3.8%) in female carriers, and 98.2% (1.3%) in controls. The mean (SD) CC density in affected eyes was higher in regions with preserved (92.6% [5.8%]) vs absent (75.9% [12.6%]) ellipsoid zone (mean difference, 16.7%; 95% CI, 12.1% to 21.3%; P < .001). Seventeen of 18 eyes of the patients and carriers had outer retinal tubulations forming pseudopod-like extensions from islands of preserved ellipsoid zone. Outer retinal tubulations were associated with absence of underlying retinal pigment epithelium and were longer (r = −0.62; 95% CI, −0.84 to −0.19; P < .001) and more numerous (r = −0.71; 95% CI, −0.91 to −0.27; P < .001) in more severely affected eyes.

Conclusions and Relevance  These findings suggest that regional changes in CC density correlate with photoreceptor structural alterations in choroideremia. Although closely coupled, the results suggest that retinal pigment epithelium loss is more extensive than photoreceptor loss.

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Figure 1.
Multimodal Imaging in a Man in His Mid-30s With Choroideremia

A, A 6-mm optical coherence tomography (OCT) B-scan with color overlay of flow signal (purple indicates retinal flow; red, choroidal flow). There is an abrupt transition zone between intact and atrophic retinal pigment epithelium and outer retina. An outer retinal tubulation (arrowhead), captured at an oblique angle, is present lateral to the region of intact retinal pigment epithelium. A prominent inner retinal vessel (circle) projects a dynamic shadow on deeper layers. Larger choroidal vessels (rectangle) abut the Bruch membrane in areas of choriocapillaris (CC) atrophy. B, En face OCT image of the segmented outer retina capturing the ellipsoid zone reflectivity, demonstrating a central island of relatively preserved ellipsoid zone with pseudopodial extensions. An oblique B-scan section through a pseudopod (arrowhead) corresponds to the outer retinal tubulation in panel A. Pseudopod length was measured as indicated with the blue line. B-F, Green line indicates the y-position of the B-scan in panel A. C, Choroidal angiogram demonstrating a central area of relatively intact CC with exposure of larger choroidal vessels in areas of CC atrophy (rectangle indicates choroidal vessels indicated by corresponding rectangle in panel A). D, Choriocapillaris angiogram obtained by segmentation of the decorrelation signal at the level of the CC, demonstrating that the greatest vessel density is associated with regions of intact outer retina. This image contains projection artifact from flow in large retinal vessels. D-F, Circle indicates the large retinal vessel from corresponding circle in panel A. E, Binary mask of retinal flow. Black pixels within this mask, consisting of 9.1% of the total number of pixels, are excluded in CC density calculations. F, Same image as in panel D with retinal vessels shown in purple for clarity. Large choroidal vessels (arrowheads) remain apparent in this CC angiogram owing to extensive CC atrophy. G, Mapping of CC density, showing regions of low CC density. Pixels containing retinal flow projection artifact are treated as empty pixels for density map generation.

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Figure 2.
Range of Choriocapillaris (CC) and Retinal Pigment Epithelium Alterations in Patients and Carriers in Coregistered Images

A, En face optical coherence tomography (OCT) of ellipsoid zone (EZ) reflectivity demonstrates progressive EZ loss. B, Fundus autofluorescence imaging demonstrates relative preservation of retinal pigment epithelium autofluorescence corresponding to regions of intact EZ. Retinal pigment epithelium loss is more extensive than EZ loss in nearly all eyes. C, Choroidal angiogram demonstrates increasing degrees of CC atrophy with exposure of underlying choroidal vessels. D, Segmented CC angiogram demonstrates that CC density is subnormal in affected eyes throughout the imaged field but is worse underlying regions of EZ and retinal pigment epithelium loss. Projection artifact from large inner retinal vessels is indicated in purple. E, Mapping of CC density highlights areas of CC loss.

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