TY - JOUR
T1 - Optical coherence tomography angiography of choroidal neovascularization in four inherited retinal dystrophies
AU - Patel, Rachel C.
AU - Gao, Simon S.
AU - Zhang, Miao
AU - Alabduljalil, Talal
AU - Al-Qahtani, Abdullah
AU - Weleber, Richard G.
AU - Yang, Paul
AU - Jia, Yali
AU - Huang, David
AU - Pennesi, Mark E.
N1 - Funding Information:
Supported by the Choroideremia Research Foundation; NIH grants R01 EY023285, R01 EY024544, DP3 DK104397, P30 EY010572, 5 K08 EY021186-05; Foundation Fighting Blindness grants C-CL-0711-0534-OHSU01, CD-NMT-0714-0648-OHSU, CD-NMT-0914-0659-OHSU; a Research to Prevent Blindness Career Development Award, and an unrestricted grant from Research to Prevent Blindness
PY - 2016/12/1
Y1 - 2016/12/1
N2 - Purpose: To demonstrate the clinical utility of optical coherence tomography (OCT) angiography (OCT-A) in inherited retinal dystrophies complicated by choroidal neovascularization (CNV). Methods: Optical coherence tomography angiography and structural OCT were performed using a 70-kHz spectral domain OCT system using the split-spectrum amplitude-decorrelation angiography algorithm. Semiautomated image processing software was used to segment and measure the CNV. Results: Four participants were enrolled to study the following inherited retinal dystrophies complicated by CNV: choroideremia, EFEMP1-related retinopathy, Best vitelliform dystrophy, and adult-onset vitelliform dystrophy. Interpretation of fluorescein angiography was difficult because of abnormal retinal architecture but suggested the presence of CNV. Structural OCT revealed subretinal or subretinal pigment epithelium fibrovascular tissue, within which flow signal was observed on OCT-A. The CNV morphology varied from dense capillary networks in active lesions to asymptomatic large caliber loops. Baseline CNV vessel areas ranged from 0.07 mm 2 to 0.98 mm 2. After treatment with intravitreal bevacizumab, the CNV in choroideremia decreased in the vessel area then rebounded, whereas the one in EFEMP1-related retinopathy remained largely unchanged. Conclusion: Optical coherence tomography angiography enables morphologic characterization and quantification of CNV in patients with retinal dystrophies despite distorted retinal architecture, can assess response to treatment, and may facilitate differentiation between active and regressed lesions.
AB - Purpose: To demonstrate the clinical utility of optical coherence tomography (OCT) angiography (OCT-A) in inherited retinal dystrophies complicated by choroidal neovascularization (CNV). Methods: Optical coherence tomography angiography and structural OCT were performed using a 70-kHz spectral domain OCT system using the split-spectrum amplitude-decorrelation angiography algorithm. Semiautomated image processing software was used to segment and measure the CNV. Results: Four participants were enrolled to study the following inherited retinal dystrophies complicated by CNV: choroideremia, EFEMP1-related retinopathy, Best vitelliform dystrophy, and adult-onset vitelliform dystrophy. Interpretation of fluorescein angiography was difficult because of abnormal retinal architecture but suggested the presence of CNV. Structural OCT revealed subretinal or subretinal pigment epithelium fibrovascular tissue, within which flow signal was observed on OCT-A. The CNV morphology varied from dense capillary networks in active lesions to asymptomatic large caliber loops. Baseline CNV vessel areas ranged from 0.07 mm 2 to 0.98 mm 2. After treatment with intravitreal bevacizumab, the CNV in choroideremia decreased in the vessel area then rebounded, whereas the one in EFEMP1-related retinopathy remained largely unchanged. Conclusion: Optical coherence tomography angiography enables morphologic characterization and quantification of CNV in patients with retinal dystrophies despite distorted retinal architecture, can assess response to treatment, and may facilitate differentiation between active and regressed lesions.
KW - EFEMP1 -related maculopathy
KW - OCT angiography
KW - adult-onset vitelliform dystrophy
KW - best vitelliform dystrophy
KW - choroidal neovascularization
KW - choroideremia
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U2 - 10.1097/IAE.0000000000001159
DO - 10.1097/IAE.0000000000001159
M3 - Article
C2 - 27336230
AN - SCOPUS:84976319240
SN - 0275-004X
VL - 36
SP - 2339
EP - 2347
JO - Retina
JF - Retina
IS - 12
ER -