Optical coherence tomography angiography in choroideremia: Correlating choriocapillaris loss with overlying degeneration

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Abstract

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

Original languageEnglish (US)
Pages (from-to)697-702
Number of pages6
JournalJAMA Ophthalmology
Volume134
Issue number6
DOIs
StatePublished - Jun 1 2016

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Choroideremia
Optical Coherence Tomography
Angiography
Multimodal Imaging
Electroretinography
Carrier State
Vertebrate Photoreceptor Cells
Retinal Pigment Epithelium

ASJC Scopus subject areas

  • Ophthalmology

Cite this

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title = "Optical coherence tomography angiography in choroideremia: Correlating choriocapillaris loss with overlying degeneration",
abstract = "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",
author = "Nieraj Jain and Jia Yali and Gao, {Simon S.} and Xinbo Zhang and Richard Weleber and David Huang and Mark Pennesi",
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T1 - Optical coherence tomography angiography in choroideremia

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AU - Jain, Nieraj

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AU - Gao, Simon S.

AU - Zhang, Xinbo

AU - Weleber, Richard

AU - Huang, David

AU - Pennesi, Mark

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N2 - 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

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