Optical Coherence Tomography Angiography in Choroideremia

Jain, Nieraj; Jia, Yali; Gao, Simon S.; Zhang, Xinbo; Weleber, Richard G.; Huang, David; Pennesi, Mark E.

doi:10.1001/jamaophthalmol.2016.0874

Cited by 66 publications

(30 citation statements)

References 16 publications

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“…Regional differences in the horizontal extent of relatively preserved central retina and/or vision in CHM measured in this study and documented by others suggest intraretinal variation in disease severity and progression rates. 4,8,13,43,50,65 Of interest, a similar pattern of disease predilection for the nasal pericentral and peripapillary retina was reported in Bietti crystalline dystrophy, a disease that shares with CHM the presence of regions of scalloped central chorioretinal atrophy. 40 Further characterization of the extramacular retina at earlier disease stages in CHM, which was not addressed in our study, is also needed to better define possible regional differences in disease expression that may be exploited to shorten the length of observation intervals required to unambiguously detect progression.…”

Section: Discussionsupporting

confidence: 58%

Natural History of the Central Structural Abnormalities in Choroideremia

et al. 2017

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Purpose To describe in detail the central retinal structure of a large group of patients with Choroideremia (CHM). Design prospective, cross-sectional, descriptive study. Subjects Patients (n=97, age 6-71 years) with CHM and subjects with normal vision (n=44; ages 10-50 years) were included. Methods Subjects were examined with spectral domain optical coherence tomography (SD-OCT) and near infrared reflectance imaging. Visual acuity (VA) was measured during their encounter or obtained from recent ophthalmic examinations. Visual thresholds were measured in a subset of patients (n=24) with automated static perimetry within the central regions (±15°) examined with SD-OCT. Main outcome measures VA and visual thresholds, total, inner and outer nuclear layer (ONL) thicknesses, and the horizontal extent of the ONL and of the photoreceptor outer segment (POS) interdigitation zone. Results Earliest abnormalities in regions with normally appearing RPE were the loss of the POS and EZ zone associated with rod dysfunction. Transition zones (TZs) from relatively preserved retina to severe ONL thinning and inner retinal thickening moved centripetally with age. Most patients (88%) retained VAs better than 20/40 until their fifth decade of life. VA decline coincided with migration of the TZ near the foveal center. There were outer retinal tubulations in degenerated, non-atrophic retina in the majority (69%) of patients. In general, retinal pigment epithelium (RPE) abnormalities paralleled photoreceptor degeneration, although there were regions with detectable but abnormally thin ONL co-localizing with severe RPE depigmentation and choroidal thinning. Conclusions Abnormalities of the POS and rod dysfunction are the earliest central abnormalities observed in CHM. Foveal function is relatively preserved until late disease. TZ migration to the foveal center with foveal thinning and structural disorganization heralded central VA loss. The relationships established may help outline the eligibility criteria and outcome measures for clinical trials for CHM.

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Section: Discussionsupporting

confidence: 58%

Natural History of the Central Structural Abnormalities in Choroideremia

et al. 2017

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“…OCTA imaging of patients with choroideremia has elucidated the changes in choriocapillaris anatomy that were previously unrecognized by conventional angiography or OCT imaging. Jain et al [48]. reported the OCTA findings in 14 eyes of 7 males with choroideremia, 4 eyes of 2 female carriers and 6 eyes of 6 controls.…”

Section: Octa In Choroideremiamentioning

confidence: 99%

Optical Coherence Tomography Angiography Imaging in Inherited Retinal Diseases

Ong

Patel

Singh

2019

JCM

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Optical coherence tomography angiography (OCTA) is a novel, noninvasive imaging modality that allows depth-resolved imaging of the microvasculature in the retina and the choroid. It is a powerful research tool to study the pathobiology of retinal diseases, including inherited retinal dystrophies. In this review, we provide an overview of the evolution of OCTA technology, compare the specifications of various OCTA devices, and summarize key findings from published OCTA studies in inherited retinal dystrophies including retinitis pigmentosa, Stargardt disease, Best vitelliform macular dystrophy, and choroideremia. OCTA imaging has provided new data on characteristics of these conditions and has contributed to a deeper understanding of inherited retinal disease.

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“…8 However, Spaide et al 9 were able to obtain images of the choroidal vessels by OCTA in eyes with RPE atrophy and discussed the potential artefacts in the OCTA images. Jain et al 10 reported that the choriocapillaris in eyes with choroideremia was widely damaged in their OCTA study. In our patient, the large choroidal vessels were observed ophthalmoscopically in the area of the RPE atrophy which may indicate an atrophy of the choriocapillaris as Jain et al described.…”

Section: Discussionmentioning

confidence: 99%

Optical coherence tomography angiography and fundus autofluorescence in the eyes with choroideremia

2017

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A 65-year-old man with presumed choroideremia with preserved central vision was examined by fundus autofluorescence (FAF) and optical coherence tomography angiography (OCTA). FAF showed an isolated area of hyperautofluorescence that involved the fovea. Although the choroid capillary slab of the OCTA showed the medium and large choroidal vessels inferior to the area of retinal pigment epithelium (RPE) atrophy, the choriocapillaris was visible in a relatively wider area than the hyperautofluorescent area in the FAF images. FAF and OCTA images allowed us to detect damage of the RPE before the choriocapillaris atrophy in a case of presumed choroideremia with preserved central vision.

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Optical Coherence Tomography Angiography in Choroideremia

Cited by 66 publications

References 16 publications

Natural History of the Central Structural Abnormalities in Choroideremia

Natural History of the Central Structural Abnormalities in Choroideremia

Optical Coherence Tomography Angiography Imaging in Inherited Retinal Diseases

Optical coherence tomography angiography and fundus autofluorescence in the eyes with choroideremia

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