Federico Corvi

Optical coherence tomography angiography of myopic choroidal neovascularisation

Background/aims To describe the morphological features of choroidal neovascularisation (CNV) and to report the ability of optical coherence tomography angiography (OCT-A) to detect the presence of myopic CNV by means of this new technique. Methods Myopic CNV cases were individuated from a pool of patients with pathological myopia consecutively presenting between October 2015 and March 2016. OCT-A images were assessed for classification of morphological features, and to estimate sensitivity and specificity. Results Thirty-six eyes of 28 consecutive patients with myopic CNV were included. In 4 out of 36 eyes it was not possible to classify the CNV ‘shape’, ‘core’, ‘margin’ and ‘appearance’ because the vascular network was not clearly visualised due to the poor quality of the examination. CNV shape on OCT-A was rated as circular in 9 eyes and irregular in 23 eyes. CNV core was visible in 11 eyes. CNV margin was considered as well defined in 16 eyes and poorly defined in 16 eyes. CNV appearance showed an ‘interlacing’ aspect in 16 eyes and a ‘tangled’ aspect in the other 16 eyes. A total of 11 CNVs were defined as active, 9 of which (81.8%) were interlacing, while a total of 21 were inactive, 14 of which (66.7%) were tangled. OCT-A sensitivity turned out to be 90.48% and specificity was 93.75%. Conclusions We describe the OCT-A features of myopic CNV secondary to pathological myopia and demonstrate its high sensitivity and specificity for neovascular detection. Qualitative evaluation of OCT-A characteristics may allow one to recognise different patterns, possibly corresponding to different degrees of neovascular activity.

Optical coherence tomography angiography in adult-onset foveomacular vitelliform dystrophy

Purpose To describe structural features of eyes with adult-onset foveomacular vitelliform dystrophy (AFVD) on optical coherence tomography angiography (OCT-A) and to evaluate the ability to detect the presence of choroidal neovascularisation (CNV). Methods Consecutive patients presenting at the University Eye Clinic of Creteil with diagnosis of AFVD were included. All patients underwent a complete ophthalmological examination, including fundus autofluorescence, fluorescein angiography, indocyanine green angiography, spectral domain optical coherence tomography and OCT-A by Optovue RTVue XR Avanti. Results Twenty-two eyes of 18 consecutive patients (8 women and 10 men; 68±12.8 years) were included. On OCT-A the presence of subretinal material leads to displacement of blood vessels at both the superficial and deep capillary plexuses of the retina. In one case, these vascular abnormalities were associated with long filamentous vessels running thorough the foveal avascular area. In all cases, a rarefaction of the choriocapillaris was also observed. In two eyes OCT-A distinctly disclosed the presence of a CNV secondary to AFVD while conventional imaging did not show clearly the neovascularisation due to masking effect of the subretinal vitelliform material. Conclusions In patients with AFVD, OCT-A showed vascular network rarefaction with less blood vessels at the superficial and deep capillary plexuses, and the choriocapillaris layer. These vascular abnormalities may play a role in the pathogenesis or simply represent a consequence of material accumulation and reabsorption in AFVD. In two cases, the conventional imaging did not show clearly the neovascularisation due to masking effect of the subretinal vitelliform material, while OCT-A showed distinctly the CNV.

Choroidal structure in eyes with drusen and reticular pseudodrusen determined by binarisation of optical coherence tomographic images

Purpose To compare luminal and stromal area of the choroid in eyes with drusen and reticular pseudodrusen (RPD) and to investigate their changes over 24 months. Methods In eyes with drusen and RPD and control subjects, total choroidal, luminal and stromal area were measured on optical coherence tomography B-scans converted to binary images, at baseline and after 24 months. Results Eighteen eyes of 18 subjects for each group were included. In drusen and RPD, we found reduction of mean total choroidal (p=0.0005 and p<0.0001, respectively), luminal (p=0.003 and p<0.0001, respectively) and stromal area (p=0.007 and p=0.0002, respectively) from baseline to month 24; no change of ratio between luminal–stromal and the choroidal area was recorded. Mean luminal, stromal and total choroidal areas were reduced in RPD, as compared with drusen and controls at both baseline and month 24 (p<0.05 for all). In RPD, the stromal area was more represented, as we found lower mean ratio of luminal and total choroidal area compared with drusen and control at both baseline and month 24 (p<0.05 for all). Conclusions Mean total choroidal, luminal and stromal area decreased over 24 months similarly in eyes with drusen and RPD. Mean total choroidal, luminal and stromal area were more reduced in eyes with RPD, as compared with eyes with drusen and controls; however, stromal area was more represented in eyes with RPD suggesting a possible role of choroidal vascular depletion and fibrotic replacement in the pathogenesis and disease progression.

DYNAMIC AND STATIC RETINAL VESSEL ANALYSES IN PATIENTS WITH MACULAR EDEMA SECONDARY TO RETINAL VEIN OCCLUSION.

Purpose: To investigate the vasomotor responses and diameter of retinal vessels in patients with macular edema secondary to retinal vein occlusion (RVO). Methods: A total of 18 eyes of 18 RVO patients were consecutively included and compared with age- and sex-matched controls. Participants underwent an examination with optical coherence tomography and dynamic and static retinal vessel analyses using the Dynamic Vessel Analyzer. Results: Dynamic vessel analysis in RVO showed mean maximum venous and arterial dilation of 2.22 ± 1.23% and 1.87 ± 1.41%, respectively, as compared with 5.05 ± 2.75% (P = 0.001) and 3.95 ± 1.44% (P = 0.0004), respectively, in controls. Static retinal vessel analysis in RVO revealed a mean arteriovenous ratio (AVR) of 0.74 ± 0.09 versus 0.90 ± 0.04 (P < 0.0001) in controls. Mean AVRs of occluded and nonoccluded quadrants in RVO were 0.71 ± 0.13 and 0.85 ± 0.19, respectively; in the corresponding quadrants of controls, mean AVRs were 0.90 ± 0.19 (P < 0.0001) and 0.86 ± 0.17 (P = 0.89), respectively. In branch RVO patients, mean AVRs of occluded versus nonoccluded quadrants were 0.70 ± 0.06 and 0.90 ± 0.22 (P = 0.002), respectively. Conclusion: In patients with macular edema secondary to RVO, dynamic vessel analysis showed an impairment of both venous and arterial motility and/or reactivity and static vessel analysis showed a reduced AVR indicating a general enlargement of the retinal venous network. Moreover, in branch RVO patients, static analysis demonstrated that retinal vessels could actually be uninvolved by the occlusive process in areas spared by disease.

Lacquer Cracks and Perforating Scleral Vessels in Pathologic Myopia: A Possible Causal Relationship

PURPOSE To describe a possible causal association between the position of perforating scleral vessels and the position of lacquer cracks in eyes with pathologic myopia. DESIGN Retrospective case series. METHODS Medical records and multimodal imaging results, including confocal scanning laser ophthalmoscopy and spectral-domain optical coherence tomography, were reviewed from patients with lacquer cracks secondary to pathologic myopia who presented between 2010 and 2014 to 2 institutions. Main outcome measures were the prevalence of perforating scleral vessels at the site of the lacquer crack, the position of the lacquer crack within the macula, and the relationships between perforating scleral vessels and retinal-choroidal structures. RESULTS A total of 35 eyes of 30 patients with lacquer cracks were included. The average number of lacquer cracks was 1.2 ± 0.5/eye and in 37 out of 45 lacquer cracks (82%) retrobulbar vessels were found to perforate the sclera at the site of the lacquer crack. Lacquer cracks were more prevalent in the central macula (51%) than in the nasal (19%), temporal (14%), inferior (11%), and superior macula (5%) (P = .001). Transverse en face images through the area of lacquer cracks were available for 8 cases and clearly depicted the perforating vessels course through the sclera and its termination in the choroid, directly beneath the lacquer cracks. CONCLUSIONS Perforating scleral vessels are often present beneath the site at which lacquer cracks form in pathologic myopia. We hypothesize that scleral expansion at the location of these perforating vessels may play a role in the formation of lacquer cracks.

Pilot evaluation of short-term changes in macular pigment and retinal sensitivity in different phenotypes of early age-related macular degeneration after carotenoid supplementation

Purpose To investigate the response of carotenoid supplementation in different phenotypes of early age-related macular degeneration (AMD) by measuring macular pigment optical density (MPOD) and retinal sensitivity. Methods Consecutive patients with only medium/large drusen and only reticular pseudodrusen (RPD) and age-matched and sex-matched controls were enrolled. At baseline, participants underwent a complete ophthalmological examination including measurement of best-corrected visual acuity (BCVA), MPOD and retinal sensitivity. Patients were put on vitamin supplementation (lutein 10 mg/day, zeaxanthin 2 mg/day) and 3 months later underwent a repeated ophthalmological examination. Results Twenty patients with medium/large drusen, 19 with RPD and 15 control subjects were included. At baseline, in controls, mean MPOD and BCVA were significantly higher compared with RPD (p=0.001 and p=0.01) but similar to medium/large drusen (p=0.9 and p=0.4). Mean retinal sensitivity was significantly higher in controls compared with RPD and medium/large drusen (for all p<0.0001). After 3 months of carotenoid supplementation the mean MPOD significantly increased in RPD (p=0.002), thus showing no more difference compared with controls (p=0.3); no significant changes were found in mean retinal sensitivity and BCVA (p=0.3 and p=0.7). Medium/large drusen did not show significant changes on MPOD, retinal sensitivity and BCVA (p=0.5, p=0.7 and p=0.7, respectively). Conclusions Patients with early AMD, especially RPD phenotype, show lower macular sensitivity and MPOD than controls. After supplementation, MPOD significantly increased in RPD. These results suggest different pathophysiology for RPD as compared with medium/large drusen and may open new ways to identifying further therapeutic targets in this phenotype of early AMD.

Spontaneous retinal pigment epithelium tear in geographic atrophy

PURPOSE To report two cases of spontaneous retinal pigment epithelial (RPE) tears occurring in two patients affected with geographic atrophy (GA) due to non-exudative age-related macular degeneration (AMD). CASE REPORT Two patients (a 79-year-old man and a 71-year-old woman) presented to our department with progressive visual loss. The man had a best-corrected visual acuity (BCVA) of 20/100 in the right eye (RE) and 20/50 in the left eye (LE); the woman had a BCVA of 20/200 in the RE and 20/160 in the LE. Upon complete ophthalmologic examination, revealing a large area of atrophy (>175 μm in diameter) along with pigmentary changes, calcified drusen and no choroidal neovascularization (CNV) in either eye, the patients were diagnosed with GA due to non-exudative AMD. Interestingly, the imaging modalities performed, including fluorescein angiography (FA), indocyanine green angiography (ICGA) and spectral-domain optical coherence tomography (SD-OCT), clearly highlighted the presence of spontaneous RPE tears in the context of non-exudative AMD, while in general, RPE tears are a well-recognized complication of exudative AMD. CONCLUSIONS To our knowledge, this is the first description of spontaneous RPE tears as a possible complication of GA due to non-exudative AMD.

Posterior polymorphous corneal dystrophy concomitant to large colloid drusen

Purpose To describe the previously unreported concomitance of 2 uncommon ocular conditions: posterior polymorphous corneal dystrophy (PPCD) and large colloid drusen (LCD). Methods A 45-year-old woman underwent a complete ophthalmologic examination with slit-lamp biomicroscopy and blue fundus autofluorescence with spectral-domain optical coherence tomography, as well as complete systemic examination and renal function investigation. Results On slit-lamp biomicroscopy, a corneal lesion located at Descemet membrane was observed in the right eye. The clinical features of deep posterior stromal-endothelial linear bands with vesicles and irregular opacities of posterior corneal surface were consistent with the diagnosis of PPCD. Fundus biomicroscopy and blue fundus autofluorescence showed LCD. Discussions We report the unusual coexistence of PPCD and LCD in a young, healthy subject. Posterior polymorphous corneal dystrophy and LCD share morphologic similarities and dysfunctions of collagen architecture in the basement membrane layer, which suggests a possible common pathogenic pathway.

Central retinal vein occlusion in a young patient following cannabis smoke inhalation.

Purpose To report a case of central retinal vein occlusion (CRVO) following cannabis smoke inhalation in a young patient. Methods An otherwise healthy 18-year-old man without risk factors for retinal vein occlusion presented with reduced visual acuity (20/200) secondary to CRVO following cannabis smoke inhalation. Central retinal vein occlusion was diagnosed on the basis of slit-lamp fundus biomicroscopy and fluorescein angiography. Results Tests for systemic causes were negative. Following one intravitreal dexamethasone implant (Ozurdex) and one ranibizumab injection (Lucentis), functional (20/20 visual acuity) and anatomic improvement was recorded. Fluorescein angiography showed a decrease in the vascular caliber and tortuosity, with no signs of retinal ischemia or edema. Conclusions We report CRVO in a young adult following cannabis smoke inhalation. Its occurrence immediately after cannabis smoking further supports a link between the use of cannabis and vascular alterations.

RETINAL PIGMENT EPITHELIUM APERTURE: A Previously Unreported Finding in the Evolution of Avascular Pigment Epithelium Detachment.

Purpose: To describe retinal pigment epithelium (RPE) aperture and to generate hypotheses about pathogenesis of this previously unreported finding in the evolution of avascular pigment epithelium detachment (PED) secondary to age-related macular degeneration. Methods: Medical records and multimodal imaging results from 10 patients with RPE apertures were reviewed between January 2009 and December 2014 by 2 institutions. Main outcome measures were analysis of RPE aperture imaging characteristics, including aperture areas and PED diameters, and their temporal course. Lesions preceding RPE aperture development were also evaluated. Results: Eleven RPE apertures were identified in 10 eyes of 10 patients (1 male, 9 females; mean age 73.1 ± 6.7 years) and included for analysis. The RPE apertures appeared as round discontinuities either at the apex or at the base of avascular PED. No rippling or retraction of the RPE was found at the sites of aperture. The RPE apertures enlarged homogeneously (mean round area of hypoautofluorescence significantly increased from 0.18 ± 0.13 to 0.93 ± 1.2; P = 0.005), and PED flattened (PED maximal height on spectral domain optical coherence significantly decreased from 445.2 ± 259 to 206.4 ± 218; P = 0.04) after a mean of 38.6 ± 16.3 months. Analysis of lesions preceding RPE apertures revealed areas of focal hyperautofluorescence at the site of development, in some cases appearing as drusenoid material connected with the base of avascular PED. Conclusion: The RPE aperture represents a previously unreported possible evolution of avascular PED, which should be distinguished by typical RPE tears. Analysis of lesions preceding RPE apertures suggests focal atrophic progression of drusenoid material in its pathogenesis.