Visual acuity and perimacular retinal layers detected by optical coherence tomography in patients with retinitis pigmentosa

Abstract Purpose Transcorneal electrical stimulation (TES) is a novel treatment approach for patients with retinitis pigmentosa (RP). The aim of our study was to observe changes in optical coherence tomography angiography (OCTA) that would be attributed to TES treatment. Methods A total of 73 eyes were included: 43 eyes of 22 subjects (11 ♀, 11 ♂) suffering from RP were examined at baseline (BL), after first stimulation (TS), 1 week (1W), and 6 months (6M) after treatment initiation and were compared with 30 control eyes of 15 subjects (8 ♀, 7 ♂). TES was performed simultaneously on both eyes for 30 min weekly. OCTA scans of 9 × 15 mm were recorded with a PLEX Elite 9000 swept-source OCTA device (Carl Zeiss Meditec AG, Jena). Vascular density metrics such as perfusion density (PD) and vessel density (VD) were calculated automatically for the macular area by using standardised extended early treatment diabetic retinopathy study (ETDRS) grids centred around the fovea. In addition, the capillary perfusion density (CPD) and the capillary flux index (CFI) of the peripapillary nerve fibre layer microvasculature in all four quadrants of an annulus centred at the optic disc were measured. All parameters were determined over all retinal layers and separately for the superficial (SCP) and deep capillary plexus (DCP). ANOVA-based linear mixed-effects models were calculated with SPSS®. Results Throughout the course of TES treatment, the macular VD and PD of all retinal layers in all subsections showed a slight decrement without reaching statistical significance, also when analysed separately in the SCP and DCP (p > 0.08). In analogy, the average CPD and CFI also presented with a slight decrement (p > 0.20). However, when compared with controls, most OCTA parameters showed a significant decrement (p < 0.05). When analysed systematically in all subsections of the extended ETDRS grid, the temporal macular subsections within the outer ring (radius 1.5–3 mm) and also of the peripheral C1, C2, and C3 rings (radius 3–7.5 mm) showed lower VD and PD values when compared with the other subsections (p < 0.05). Conclusion Vascular density metrics in the macular region and the peripapillary microvasculature appear to remain unaffected by continuous TES treatment within a period of 6 months.

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Usher syndrome in a patient with Ellis–van Creveld syndrome

European Journal of Ophthalmology ◽

10.1177/1120672119841778 ◽

2019 ◽

Vol 30 (2) ◽

pp. NP38-NP40

Author(s):

Francesco Romano ◽

Giorgia Carlotta Albertini ◽

Alessandro Arrigo ◽

Pier Pasquale Leone ◽

Francesco Bandello ◽

...

Keyword(s):

Optical Coherence Tomography ◽

Visual Acuity ◽

Retinitis Pigmentosa ◽

Usher Syndrome ◽

Fundus Photography ◽

Optical Coherence ◽

Genetic Tests ◽

Ellis Van Creveld Syndrome ◽

Atypical Presentations

Purpose: To describe a case of Ellis–van Creveld syndrome with concomitant Usher syndrome. Methods: A 24-year-old lady with a diagnosis of Ellis–van Creveld syndrome came to our attention in 2015 complaining of nyctalopia. She underwent yearly ophthalmologic examinations, including visual acuity, dilated fundoscopy, optical coherence tomography and colour fundus photography. Results: On the day of her first examination, her visual acuity was 20/20, whereas fundus examination revealed diffuse peripheral retinal atrophy with pigmented bone spicules, waxy pallor of the disc and macular sparing in both eyes, compatible with retinitis pigmentosa. Due to the severe retinitis pigmentosa phenotype for the age and the concomitant neurosensory hearing loss, ancillary electrophysiological and genetic tests were requested. At the end of follow-up, visual function remained stable, with electroretinogram tests confirming the peripheral dysfunction. Interestingly, next generation sequencing test revealed a mutation in USH2A gene, suggestive of an overlapping Usher syndrome. On optical coherence tomography angiography, all plexuses appeared altered, with some degree of impairment also in the choriocapillaris of the spared macula. Conclusion: Our report emphasizes the advantage of new genetic tests to investigate atypical presentations of known retinal disorders found in syndromic settings. In addition, we speculate that the underlying ciliopathy might possibly aggravate the phenotype of this case of Usher syndrome.

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Grading severity in retinitis pigmentosa using clinical assessment, visual acuity, perimetry and optical coherence tomography

International Ophthalmology ◽

10.1007/s10792-012-9678-2 ◽

2012 ◽

Vol 33 (3) ◽

pp. 237-244 ◽

Cited By ~ 4

Author(s):

Henry B. Smith ◽

Aman Chandra ◽

Hadi Zambarakji

Keyword(s):

Optical Coherence Tomography ◽

Visual Acuity ◽

Retinitis Pigmentosa ◽

Clinical Assessment ◽

Optical Coherence

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Optical Coherence Tomography Findings in Patients with Retinitis Pigmentosa and Low Visual Acuity

Ophthalmic Surgery Lasers and Imaging ◽

10.3928/15428877-20091230-07 ◽

2010 ◽

Vol 41 (1) ◽

pp. 35-39 ◽

Cited By ~ 10

Author(s):

Vlassis G. Grigoropoulos ◽

John Emfietzoglou ◽

Pantelis Nikolaidis ◽

Klio Chatzistefanou ◽

John Vergados ◽

...

Keyword(s):

Optical Coherence Tomography ◽

Visual Acuity ◽

Retinitis Pigmentosa ◽

Optical Coherence

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Optical Coherence Tomography in Best Vitelliform Macular Dystrophy

European Journal of Ophthalmology ◽

10.5301/ejo.5000878 ◽

2017 ◽

Vol 27 (2) ◽

pp. 201-204 ◽

Cited By ~ 8

Author(s):

Maurizio Battaglia Parodi ◽

Pierluigi Iacono ◽

Francesco Romano ◽

Gianluigi Bolognesi ◽

Francesco Fasce ◽

...

Keyword(s):

Optical Coherence Tomography ◽

Visual Acuity ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Macular Dystrophy ◽

Optical Coherence ◽

Vitelliform Macular Dystrophy ◽

Retinal Layers ◽

Hyperreflective Foci ◽

Sd Oct

Purpose To analyze spectral-domain optical coherence tomography (SD-OCT)-specific findings in the different stages of vitelliform macular dystrophy (VMD). Methods Thirty-seven patients were prospectively recruited. All the patients underwent a complete ophthalmologic examination, including best-corrected visual acuity (BCVA), biomicroscopy, and SD-OCT. The examined findings were vitelliform material, neurosensory detachment, intraretinal hyperreflective foci, and the status of external limiting membrane, ellipsoid zone, and retinal pigment epithelium. The primary outcome was the stratification of SD-OCT findings in each VMD stage. Secondary outcomes included the description of different characteristics related to intraretinal hyperreflective foci. Results Outer retinal layers were preserved almost exclusively in stage 1 (range 70%-100%), whereas their disruption and absence were typical of stages 2 to 4 (83%-100%) and stage 5 (67%-83%), respectively. Vitelliform material was found always in stages 2 and 3, 89% of stage 4, and rarely in stage 5 (33%). Neurosensory detachment was to some extent representative of stages 3 and 4 (80% and 72%, respectively) when compared with the other stages (p<0.001). Hyperreflective foci (16% of all eyes) demonstrated a progressive increase across stages 2 to 4, with slightly reduced figure in stage 5. These foci were located in the outer nuclear and plexiform layers, showed different sizes, and were not associated with a visual acuity reduction (p = 0.64). Conclusions A progressive deterioration of the outer retinal layers was noticeable in more advanced stages of VMD. The reduction of vitelliform material from stage 3 to 4 was paralleled by an increased evidence of neurosensory detachment. Although showing different size and location, hyperreflective foci did not correlate with worse BCVA.

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Ultra-wide field imaging of pigmented para-venous retino-choroidal atrophy

European Journal of Ophthalmology ◽

10.1177/1120672118795056 ◽

2018 ◽

Vol 29 (4) ◽

pp. 444-452 ◽

Cited By ~ 1

Author(s):

Vinod Kumar ◽

Devesh Kumawat ◽

Ruchir Tewari ◽

Pradeep Venkatesh

Keyword(s):

Optical Coherence Tomography ◽

Retinitis Pigmentosa ◽

Retrospective Review ◽

Wide Field ◽

Imaging Features ◽

Optical Coherence ◽

Swept Source ◽

Retinal Layers ◽

Field Imaging ◽

Wide Field Imaging

Objective: To describe the ultra-wide field imaging features of pigmented para-venous retino-choroidal atrophy. Design: Retrospective review at a tertiary care centre. Participants: Eight eyes of five patients with pigmented para-venous retino-choroidal atrophy who presented to our retina clinic over last 2 years. Methods: Retrospective review of ultra-wide field pseudo-colour and short wave autofluorescence imaging was performed. In vivo histology of the macula and areas of retino-choroidal atrophy was studied with swept source optical coherence tomography (SS-OCT). Results: The median age was 40 years (range: 22–67 years). Best corrected visual acuity ranged from perception of light to 20/20. The para-venous retino-choroidal atrophy and pigment clumping not only involved the major arcade vessels but also extended into the peripapillary area and retinal periphery. The affected areas demonstrated hypoautofluorescence with sharp hyperautofluorescent borders. Macular atrophy, epiretinal membrane and optic disc pallor were noted in two eyes each. In all cases, the affected pigmentary area had disorganization of inner retinal layers, disruption of outer retinal layers and retinal pigment epithelium and markedly thinned out choroid on swept source optical coherence tomography. Concurrent involvement with retinitis pigmentosa in the fellow eye was noted in two patients. Conclusion: Ultra-wide field imaging of pigmented para-venous retino-choroidal atrophy sheds light onto the widespread retino-choroidal abnormalities. Concurrent disc and macular involvement may jeopardize the visual function. Pigmented para-venous retino-choroidal atrophy may be considered as a self-limited form of retinitis pigmentosa.

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Optical coherence tomography angiography in patients with retinitis pigmentosa who have normal visual acuity

Acta Ophthalmologica ◽

10.1111/aos.13680 ◽

2018 ◽

Vol 96 (5) ◽

pp. e636-e642 ◽

Cited By ~ 11

Author(s):

Seiji Takagi ◽

Yasuhiko Hirami ◽

Masayo Takahashi ◽

Masashi Fujihara ◽

Michiko Mandai ◽

...

Keyword(s):

Optical Coherence Tomography ◽

Visual Acuity ◽

Retinitis Pigmentosa ◽

Optical Coherence Tomography Angiography ◽

Optical Coherence ◽

Normal Visual Acuity

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Thickness of Receptor and Post-receptor Retinal Layers in Patients with Retinitis Pigmentosa Measured with Frequency-Domain Optical Coherence Tomography

Investigative Opthalmology & Visual Science ◽

10.1167/iovs.08-2936 ◽

2009 ◽

Vol 50 (5) ◽

pp. 2328 ◽

Cited By ~ 112

Author(s):

Donald C. Hood ◽

Christine E. Lin ◽

Margot A. Lazow ◽

Kirsten G. Locke ◽

Xian Zhang ◽

...

Keyword(s):

Optical Coherence Tomography ◽

Retinitis Pigmentosa ◽

Frequency Domain ◽

Optical Coherence ◽

Retinal Layers

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Large choroidal excavation in retinitis pigmentosa: A case report

European Journal of Ophthalmology ◽

10.1177/1120672119892438 ◽

2019 ◽

Vol 30 (5) ◽

pp. NP66-NP68 ◽

Cited By ~ 1

Author(s):

Pierluigi Iacono ◽

Maurizio Battaglia Parodi ◽

Mariacristina Parravano ◽

Monica Varano

Keyword(s):

Optical Coherence Tomography ◽

Visual Acuity ◽

Retinitis Pigmentosa ◽

Retinal Pigment ◽

Posterior Pole ◽

Spectral Domain ◽

Fundus Examination ◽

Optical Coherence ◽

Corrected Visual Acuity ◽

Best Corrected Visual Acuity

Purpose: To report the morphological and clinical features of a case of retinitis pigmentosa with large choroidal excavation. Methods: The patient underwent a complete ophthalmologic examination including best-corrected visual acuity assessment, anterior segment and dilated fundus examination and spectral-domain optical coherence tomography. Results: A 41-year-old woman affected by retinitis pigmentosa with genetic confirmation with mutation in RDH12 gene had a best-corrected visual acuity of 20/50 in both eyes. Dilated fundus examination revealed waxy pallor of the optic disc, diffuse narrowing of the retinal arterioles and a generalized retinal pigment epithelium mottling with bony spicule associated with diffuse retinal atrophy. At the posterior pole, an extended bilateral chorioretinal atrophy was evident with a partial sparing of the macular area. On spectral-domain optical coherence tomography, a bilateral large choroidal excavation could be clearly detected and it was associated with a diffuse retinal thinning at the posterior pole and a partial sparing of the fovea. Conclusion: Large choroidal excavation has been rarely reported. Although the pathogenetic mechanisms leading to the formation of large choroidal excavation are still a matter of debate, a combination of primary degenerative-inflammatory factors could be retained responsible for the large choroidal excavation development.

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