Correlation of Outer Retinal Degeneration and Choriocapillaris Loss in Stargardt Disease Using En Face Optical Coherence Tomography and Optical Coherence Tomography Angiography

Talal Alabduljalil, Rachel C. Patel, Abdullah A. Alqahtani, Simon S. Gao, Michael J. Gale, Miao Zhang, Jia Yali, David Huang, Pei-Wen Chiang, Rui Chen, Jun Wang, Richard Weleber, Mark Pennesi, Paul Yang

Research output: Contribution to journalArticle

Abstract

Purpose: This study measured and correlated degeneration of the junction between the inner and outer segments (IS/OS), the retinal pigment epithelium (RPE), and the choriocapillaris (CC) in Stargardt disease (STGD). Design: Prospective cross-sectional study. Methods: This study was conducted at the Casey Eye Institute. A total of 23 patients with STGD were enrolled and underwent optical coherence tomography angiography (OCTA). Scans were centered on the fovea. OCT slab projections and en face boundary maps were used to create masks to measure total IS/OS loss or RPE atrophy as well as regions of isolated IS/OS loss, isolated RPE atrophy, and matched IS/OS and RPE degeneration or intact IS/OS junction and RPE. CC vascular density (CCVD) was quantified from the CC angiogram. Outcomes included the area of loss, and the CCVD of degeneration in different areas was quantified and correlated. Results: The total area of IS/OS loss was strongly correlated with the total area of RPE atrophy (r = 0.96; P < 0.0001) by a 1.6:1 ratio (r 2 = 0.90). CCVD within regions of matched degeneration (85.6% ± 2.7%; P < 0.0001), isolated IS/OS junction loss (93.6% ± 1.0%; P = 0.0011), and isolated RPE atrophy (94.1% ± 1.1%; P = 0.0065) were all significantly lower than normal (99.0% ± 0.17%). There was a trend for CCVD within intact areas (97.6% ± 0.38%) to decline as the area diminished (r = 0.68). Conclusions: Photoreceptor and RPE degeneration exhibited a strong relationship wherein the IS/OS loss was 1.6-fold greater than that of RPE atrophy, supporting the theory that photoreceptor degeneration precedes RPE in STGD. Both the photoreceptors and the RPE degeneration contributed synergistically to CCVD attenuation, but extralesional CCVD also tended to be abnormal. The findings and techniques in this study may be of utility in developing endpoints for clinical trials.

Original languageEnglish (US)
Pages (from-to)79-90
Number of pages12
JournalAmerican journal of ophthalmology
Volume202
DOIs
StatePublished - Jun 1 2019

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Retinal Degeneration
Retinal Pigment Epithelium
Optical Coherence Tomography
Angiography
Blood Vessels
Atrophy
Stargardt disease 1
Masks
Cross-Sectional Studies
Clinical Trials

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Correlation of Outer Retinal Degeneration and Choriocapillaris Loss in Stargardt Disease Using En Face Optical Coherence Tomography and Optical Coherence Tomography Angiography. / Alabduljalil, Talal; Patel, Rachel C.; Alqahtani, Abdullah A.; Gao, Simon S.; Gale, Michael J.; Zhang, Miao; Yali, Jia; Huang, David; Chiang, Pei-Wen; Chen, Rui; Wang, Jun; Weleber, Richard; Pennesi, Mark; Yang, Paul.

In: American journal of ophthalmology, Vol. 202, 01.06.2019, p. 79-90.

Research output: Contribution to journalArticle

Alabduljalil, Talal ; Patel, Rachel C. ; Alqahtani, Abdullah A. ; Gao, Simon S. ; Gale, Michael J. ; Zhang, Miao ; Yali, Jia ; Huang, David ; Chiang, Pei-Wen ; Chen, Rui ; Wang, Jun ; Weleber, Richard ; Pennesi, Mark ; Yang, Paul. / Correlation of Outer Retinal Degeneration and Choriocapillaris Loss in Stargardt Disease Using En Face Optical Coherence Tomography and Optical Coherence Tomography Angiography. In: American journal of ophthalmology. 2019 ; Vol. 202. pp. 79-90.
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abstract = "Purpose: This study measured and correlated degeneration of the junction between the inner and outer segments (IS/OS), the retinal pigment epithelium (RPE), and the choriocapillaris (CC) in Stargardt disease (STGD). Design: Prospective cross-sectional study. Methods: This study was conducted at the Casey Eye Institute. A total of 23 patients with STGD were enrolled and underwent optical coherence tomography angiography (OCTA). Scans were centered on the fovea. OCT slab projections and en face boundary maps were used to create masks to measure total IS/OS loss or RPE atrophy as well as regions of isolated IS/OS loss, isolated RPE atrophy, and matched IS/OS and RPE degeneration or intact IS/OS junction and RPE. CC vascular density (CCVD) was quantified from the CC angiogram. Outcomes included the area of loss, and the CCVD of degeneration in different areas was quantified and correlated. Results: The total area of IS/OS loss was strongly correlated with the total area of RPE atrophy (r = 0.96; P < 0.0001) by a 1.6:1 ratio (r 2 = 0.90). CCVD within regions of matched degeneration (85.6{\%} ± 2.7{\%}; P < 0.0001), isolated IS/OS junction loss (93.6{\%} ± 1.0{\%}; P = 0.0011), and isolated RPE atrophy (94.1{\%} ± 1.1{\%}; P = 0.0065) were all significantly lower than normal (99.0{\%} ± 0.17{\%}). There was a trend for CCVD within intact areas (97.6{\%} ± 0.38{\%}) to decline as the area diminished (r = 0.68). Conclusions: Photoreceptor and RPE degeneration exhibited a strong relationship wherein the IS/OS loss was 1.6-fold greater than that of RPE atrophy, supporting the theory that photoreceptor degeneration precedes RPE in STGD. Both the photoreceptors and the RPE degeneration contributed synergistically to CCVD attenuation, but extralesional CCVD also tended to be abnormal. The findings and techniques in this study may be of utility in developing endpoints for clinical trials.",
author = "Talal Alabduljalil and Patel, {Rachel C.} and Alqahtani, {Abdullah A.} and Gao, {Simon S.} and Gale, {Michael J.} and Miao Zhang and Jia Yali and David Huang and Pei-Wen Chiang and Rui Chen and Jun Wang and Richard Weleber and Mark Pennesi and Paul Yang",
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T1 - Correlation of Outer Retinal Degeneration and Choriocapillaris Loss in Stargardt Disease Using En Face Optical Coherence Tomography and Optical Coherence Tomography Angiography

AU - Alabduljalil, Talal

AU - Patel, Rachel C.

AU - Alqahtani, Abdullah A.

AU - Gao, Simon S.

AU - Gale, Michael J.

AU - Zhang, Miao

AU - Yali, Jia

AU - Huang, David

AU - Chiang, Pei-Wen

AU - Chen, Rui

AU - Wang, Jun

AU - Weleber, Richard

AU - Pennesi, Mark

AU - Yang, Paul

PY - 2019/6/1

Y1 - 2019/6/1

N2 - Purpose: This study measured and correlated degeneration of the junction between the inner and outer segments (IS/OS), the retinal pigment epithelium (RPE), and the choriocapillaris (CC) in Stargardt disease (STGD). Design: Prospective cross-sectional study. Methods: This study was conducted at the Casey Eye Institute. A total of 23 patients with STGD were enrolled and underwent optical coherence tomography angiography (OCTA). Scans were centered on the fovea. OCT slab projections and en face boundary maps were used to create masks to measure total IS/OS loss or RPE atrophy as well as regions of isolated IS/OS loss, isolated RPE atrophy, and matched IS/OS and RPE degeneration or intact IS/OS junction and RPE. CC vascular density (CCVD) was quantified from the CC angiogram. Outcomes included the area of loss, and the CCVD of degeneration in different areas was quantified and correlated. Results: The total area of IS/OS loss was strongly correlated with the total area of RPE atrophy (r = 0.96; P < 0.0001) by a 1.6:1 ratio (r 2 = 0.90). CCVD within regions of matched degeneration (85.6% ± 2.7%; P < 0.0001), isolated IS/OS junction loss (93.6% ± 1.0%; P = 0.0011), and isolated RPE atrophy (94.1% ± 1.1%; P = 0.0065) were all significantly lower than normal (99.0% ± 0.17%). There was a trend for CCVD within intact areas (97.6% ± 0.38%) to decline as the area diminished (r = 0.68). Conclusions: Photoreceptor and RPE degeneration exhibited a strong relationship wherein the IS/OS loss was 1.6-fold greater than that of RPE atrophy, supporting the theory that photoreceptor degeneration precedes RPE in STGD. Both the photoreceptors and the RPE degeneration contributed synergistically to CCVD attenuation, but extralesional CCVD also tended to be abnormal. The findings and techniques in this study may be of utility in developing endpoints for clinical trials.

AB - Purpose: This study measured and correlated degeneration of the junction between the inner and outer segments (IS/OS), the retinal pigment epithelium (RPE), and the choriocapillaris (CC) in Stargardt disease (STGD). Design: Prospective cross-sectional study. Methods: This study was conducted at the Casey Eye Institute. A total of 23 patients with STGD were enrolled and underwent optical coherence tomography angiography (OCTA). Scans were centered on the fovea. OCT slab projections and en face boundary maps were used to create masks to measure total IS/OS loss or RPE atrophy as well as regions of isolated IS/OS loss, isolated RPE atrophy, and matched IS/OS and RPE degeneration or intact IS/OS junction and RPE. CC vascular density (CCVD) was quantified from the CC angiogram. Outcomes included the area of loss, and the CCVD of degeneration in different areas was quantified and correlated. Results: The total area of IS/OS loss was strongly correlated with the total area of RPE atrophy (r = 0.96; P < 0.0001) by a 1.6:1 ratio (r 2 = 0.90). CCVD within regions of matched degeneration (85.6% ± 2.7%; P < 0.0001), isolated IS/OS junction loss (93.6% ± 1.0%; P = 0.0011), and isolated RPE atrophy (94.1% ± 1.1%; P = 0.0065) were all significantly lower than normal (99.0% ± 0.17%). There was a trend for CCVD within intact areas (97.6% ± 0.38%) to decline as the area diminished (r = 0.68). Conclusions: Photoreceptor and RPE degeneration exhibited a strong relationship wherein the IS/OS loss was 1.6-fold greater than that of RPE atrophy, supporting the theory that photoreceptor degeneration precedes RPE in STGD. Both the photoreceptors and the RPE degeneration contributed synergistically to CCVD attenuation, but extralesional CCVD also tended to be abnormal. The findings and techniques in this study may be of utility in developing endpoints for clinical trials.

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