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; Yali Jia; David Huang; Pei Wen Chiang; Rui Chen; Jun Wang; Richard G. Weleber; Mark E. Pennesi; Paul Yang

doi:10.1016/j.ajo.2019.02.007

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, Yali Jia, David Huang, Pei Wen Chiang, Rui Chen, Jun Wang, Richard G. Weleber, Mark E. Pennesi, Paul Yang

Ophthalmology

Research output: Contribution to journal › Article › peer-review

15 Scopus citations

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 (r² = 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 language	English (US)
Pages (from-to)	79-90
Number of pages	12
Journal	American journal of ophthalmology
Volume	202
DOIs	https://doi.org/10.1016/j.ajo.2019.02.007
State	Published - Jun 2019

ASJC Scopus subject areas

Ophthalmology

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10.1016/j.ajo.2019.02.007

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Alabduljalil, T., Patel, R. C., Alqahtani, A. A., Gao, S. S., Gale, M. J., Zhang, M., Jia, Y., Huang, D., Chiang, P. W., Chen, R., Wang, J., Weleber, R. G., Pennesi, M. E., & Yang, P. (2019). Correlation of Outer Retinal Degeneration and Choriocapillaris Loss in Stargardt Disease Using En Face Optical Coherence Tomography and Optical Coherence Tomography Angiography. American journal of ophthalmology, 202, 79-90. https://doi.org/10.1016/j.ajo.2019.02.007

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; Jia, Yali ; Huang, David; Chiang, Pei Wen; Chen, Rui; Wang, Jun; Weleber, Richard G.; Pennesi, Mark E.; Yang, Paul.

In: American journal of ophthalmology, Vol. 202, 06.2019, p. 79-90.

Research output: Contribution to journal › Article › peer-review

Alabduljalil, T, Patel, RC, Alqahtani, AA, Gao, SS, Gale, MJ, Zhang, M, Jia, Y , Huang, D, Chiang, PW, Chen, R, Wang, J, Weleber, RG , Pennesi, ME & Yang, P 2019, 'Correlation of Outer Retinal Degeneration and Choriocapillaris Loss in Stargardt Disease Using En Face Optical Coherence Tomography and Optical Coherence Tomography Angiography', American journal of ophthalmology, vol. 202, pp. 79-90. https://doi.org/10.1016/j.ajo.2019.02.007

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

@article{9e7a437a09aa42ae9957d0836667db93,

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

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 (r2 = 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 Yali Jia and David Huang and Chiang, {Pei Wen} and Rui Chen and Jun Wang and Weleber, {Richard G.} and Pennesi, {Mark E.} and Paul Yang",

note = "Funding Information: Funding/support: Supported by an unrestricted grant from Research to Prevent Blindness , New York, New York, USA; a Career Development Award and a William and Mary Greve Special Scholar Award (to Y.J.); a Foundation Fighting Blindness , Columbia, Maryland, USA, center grant/ C-Cl-0711-0534-OHSU01 ; Enhanced Career Development Award/CD-NMT-0914-0659-OHSU (to M.E.P.); Career Development Award/CD-NMT-0714-0648 (to P.Y.); National Institutes of Health , Bethesda, MD, USA, Core Grants P30 Ey010572 (to C.E.I.), R01 EY027833 (to Y.J.), R01 EY024544 (to Y.J.), DP3 DK104397 (to Y.J.), K08 EY026650 (to P.Y.), and 5R24EY022023 (subaward); and Choroideremia Research Foundation , Springfield, Massachusetts (to M.E.P.). Publisher Copyright: {\textcopyright} 2019 Elsevier Inc.",

year = "2019",

month = jun,

doi = "10.1016/j.ajo.2019.02.007",

language = "English (US)",

volume = "202",

pages = "79--90",

journal = "American Journal of Ophthalmology",

issn = "0002-9394",

publisher = "Elsevier USA",

}

TY - JOUR

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 - Jia, Yali

AU - Huang, David

AU - Chiang, Pei Wen

AU - Chen, Rui

AU - Wang, Jun

AU - Weleber, Richard G.

AU - Pennesi, Mark E.

AU - Yang, Paul

N1 - Funding Information: Funding/support: Supported by an unrestricted grant from Research to Prevent Blindness , New York, New York, USA; a Career Development Award and a William and Mary Greve Special Scholar Award (to Y.J.); a Foundation Fighting Blindness , Columbia, Maryland, USA, center grant/ C-Cl-0711-0534-OHSU01 ; Enhanced Career Development Award/CD-NMT-0914-0659-OHSU (to M.E.P.); Career Development Award/CD-NMT-0714-0648 (to P.Y.); National Institutes of Health , Bethesda, MD, USA, Core Grants P30 Ey010572 (to C.E.I.), R01 EY027833 (to Y.J.), R01 EY024544 (to Y.J.), DP3 DK104397 (to Y.J.), K08 EY026650 (to P.Y.), and 5R24EY022023 (subaward); and Choroideremia Research Foundation , Springfield, Massachusetts (to M.E.P.). Publisher Copyright: © 2019 Elsevier Inc.

PY - 2019/6

Y1 - 2019/6

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 (r2 = 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 (r2 = 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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Correlation of Outer Retinal Degeneration and Choriocapillaris Loss in Stargardt Disease Using En Face Optical Coherence Tomography and Optical Coherence Tomography Angiography

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