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

2007 ◽  
Vol 91 (7) ◽  
pp. 888-890 ◽  
Author(s):  
T. Matsuo ◽  
N. Morimoto
2013 ◽  
pp. 2073 ◽  
Author(s):  
Marilita Moschos ◽  
Irini Chatziralli ◽  
George Verriopoulos ◽  
Aristeides Triglianos ◽  
Dimitrios Ladas ◽  
...  

Author(s):  
Olga Zabek ◽  
Hanna Camenzind Zuche ◽  
Ursula Müller ◽  
Hendrik P. N. Scholl ◽  
Annekatrin Rickmann ◽  
...  

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.


2019 ◽  
Vol 30 (2) ◽  
pp. NP38-NP40
Author(s):  
Francesco Romano ◽  
Giorgia Carlotta Albertini ◽  
Alessandro Arrigo ◽  
Pier Pasquale Leone ◽  
Francesco Bandello ◽  
...  

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.


2010 ◽  
Vol 41 (1) ◽  
pp. 35-39 ◽  
Author(s):  
Vlassis G. Grigoropoulos ◽  
John Emfietzoglou ◽  
Pantelis Nikolaidis ◽  
Klio Chatzistefanou ◽  
John Vergados ◽  
...  

2017 ◽  
Vol 27 (2) ◽  
pp. 201-204 ◽  
Author(s):  
Maurizio Battaglia Parodi ◽  
Pierluigi Iacono ◽  
Francesco Romano ◽  
Gianluigi Bolognesi ◽  
Francesco Fasce ◽  
...  

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.


2018 ◽  
Vol 29 (4) ◽  
pp. 444-452 ◽  
Author(s):  
Vinod Kumar ◽  
Devesh Kumawat ◽  
Ruchir Tewari ◽  
Pradeep Venkatesh

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.


2018 ◽  
Vol 96 (5) ◽  
pp. e636-e642 ◽  
Author(s):  
Seiji Takagi ◽  
Yasuhiko Hirami ◽  
Masayo Takahashi ◽  
Masashi Fujihara ◽  
Michiko Mandai ◽  
...  

Eye ◽  
2022 ◽  
Author(s):  
Daiki Sakai ◽  
Seiji Takagi ◽  
Yasuhiko Hirami ◽  
Makoto Nakamura ◽  
Yasuo Kurimoto

Abstract Objective To predict the visual prognosis of cataract surgery in patients with retinitis pigmentosa by measuring ellipsoid zone (EZ) width using spectral-domain optical coherence tomography. Methods This retrospective study included patients with retinitis pigmentosa who underwent uncomplicated cataract surgery between December 2017 and June 2020. Preoperative best-corrected visual acuity (BCVA) and the best postoperative BCVA during follow-up were collected. EZ width was measured on preoperative cross-sectional optical coherence tomography images along the horizontal/vertical meridian through the fovea. Results Thirty-eight eyes of 38 patients (22 female; mean [±standard deviation] age, 62.1 ± 11.8 years) were included. The median preoperative logarithm of the minimum angle of resolution BCVA of 0.52 (range, 0.00–3.00) significantly improved to 0.07 (range, −0.18–3.00) after surgery (P < 0.001). On preoperative spectral-domain optical coherence tomography images, the median horizontal, vertical, and average EZ widths were 783 (range, 0–9837), 761 (range, 0–10 250), and 769 (range, 0–10 043) μm, respectively. Postoperative BCVA significantly correlated with the horizontal (r = −0.784, P < 0.001), vertical (r = −0.777, P < 0.001), and average EZ widths (r = −0.777, P < 0.001). The area under the receiver operating characteristic curve for the ability of the horizontal, vertical, and average EZ widths to discriminate eyes with and without postoperative BCVA ≤ 0.3 was 0.971, 0.960, and 0.963, respectively, with best cut-off values of 513, 608, and 515 μm, respectively. Conclusions EZ width measurement can help predict the visual prognosis of cataract surgery in patients with retinitis pigmentosa. A preferable visual acuity prognosis can be expected in patients with an EZ width of approximately 600 μm.


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