Spectral-domain optical coherence tomography of early onset large colloid drusen.

PURPOSE: Large colloid drusen (LCD) are a subgroup of early onset drusen recently reported. The aim of this study was to describe morphologic features in patients affected with LCD using high-resolution spectral-domain optical coherence tomography (SD-OCT). METHODS: A complete ophthalmologic examination including SD-OCT was performed in 22 eyes of 11 consecutive patients. RESULTS: Mean age was 35 years old at diagnosis, and mean visual acuity was 20/25. In all eyes, SD-OCT showed multiple dome-shaped retinal pigment epithelium detachments corresponding to the drusen. Mean height of drusen was 199 µm (range, 108-316 µm) and mean width was 419 µm (range, 190-681 µm). Indocyanine green angiography hypofluorescent LCD were larger and more reflective on SD-OCT than indocyanine green angiography hyperfluorescent LCD. All drusen appeared convex with medium and homogeneous internal reflectivity. Retina overlying LCD appeared wavy and overall thinned. Prominent diffuse hyperreflective haze in the Henle fiber layer over drusen was noted in 73% of the eyes. No geographic atrophy or choroidal neovascularization was observed. CONCLUSION: The peculiar SD-OCT features reported in the current series give insight on the ultrastructure of LCD. Further studies with follow-up evaluation and possibly histologic evaluation are needed to clarify the clinical significance of LCD.

Multimodal morphological and functional characterization of Malattia Leventinese.

BACKGROUND: To analyze the morphological and functional characteristics of malattia leventinese. METHODS: This was a chart review of patients with Malattia Leventinese. All patients underwent a complete ophthalmologic examination, including best-corrected visual acuity (BCVA), fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), and optical coherence tomography (OCT). Microperimetry and Preferential Hyperacuity Perimeter (PHP) were performed in a subset of patients. RESULTS: Twelve eyes of six patients were included. BCVA ranged from 20/25 to 20/200. The largest drusen were round, not radially distributed, localized in the perimacular area and around the optic disc. The smallest drusen were not round, radially distributed, mostly localized temporally to the macula. FAF revealed an intense autofluorescence of large drusen. On both FA and ICGA, large round drusen turned to hyperfluorescent in the late phase, while small radial drusen progressively decreased their fluorescence. OCT showed the large round drusen as focal or diffuse deposition of hyperreflective material between the RPE and Bruch membrane within the macula, determining focal dome-shaped or diffuse RPE elevation respectively, and the small radial drusen, which ranged from irregular slight thickening of the RPE/Bruch membrane complex to sawtooth RPE elevation. In three patients (six eyes) that underwent microperimetry and PHP, there was a good correspondence between macular sensitivity and PHP score. Functional impairment correlated topographically to sub-RPE deposition of drusenoid material. CONCLUSIONS: In this series, large round drusen of Malattia Leventinese appeared similar to drusen in age-related macular degeneration, while small radial drusen of Malattia Leventinese shared similarities with early-onset cuticular drusen.

Macular edema in central retinal vein occlusion: correlation between optical coherence tomography, angiography and visual acuity.

To analyze the characteristics and the course of macular edema secondary to central retinal vein occlusion (CRVO) using optical coherence tomography (OCT) and to determine correlations between clinical, tomographic and angiographic data, in particular including retinal ischemia. In this retrospective study, 53 consecutive patients with CRVO were included. At each follow-up visit, patients underwent complete ophthalmological examination, including best-corrected visual acuity (BCVA) and OCT. Fluorescein angiography was performed at baseline and on demand during follow-up. 243 OCTs were analyzed. Mean age was 61 years and mean follow-up 13 months. The first structural change, observed very early after the onset of the occlusion, was a diffuse increase at the level of the outer nuclear layer without change at the level of the inner retina. This early change seemed characteristic of retinal vein occlusion. Cystoid spaces were subsequently observed in all retinal layers and were combined with serous retinal detachment in 51 %. During the first 6 months, central retinal thickness was higher in ischemic CRVO (mean, 691 µm) than in non-ischemic CRVO (mean, 440 µm, p < 0.01). In eyes with foveal thickness (central retinal thickness without subretinal fluid) of 700 µm or greater, peripheral ischemia was present in 69 % of eyes, final BCVA was 20/200 or less in 75 % and never reached 20/40 during follow-up. The integrity of the junction of the photoreceptors' inner and outer segments was correlated with a better prognosis (p < 0.05). Foveal thickness was inversely correlated to BCVA at each visit and could have a prognostic value. OCT examination in CRVO revealed useful data for the diagnosis of CRVO and its prognosis. The largest macular edemas seemed to be the hallmark of ischemic CRVO.

Insights into pathology of cuticular drusen from integrated confocal scanning laser ophthalmoscopy imaging and corresponding spectral domain optical coherence tomography.

BACKGROUND: To analyze the integrated confocal scanning laser ophthalmoscopy (cSLO) fundus and angiographic imaging and corresponding spectral domain optical coherence tomography (SD-OCT) features of cuticular drusen. METHODS: Twenty-one consecutive patients with cuticular drusen were submitted to cSLO fundus and angiographic imaging [infrared reflectance (IR), fundus autofluorescence (FAF), near-infrared autofluorescence (NIA), fluorescein angiography (FA), and indocyanine green angiography (ICGA)) and "eye-tracked" SD-OCT. RESULTS: A total of 42 eyes were included for analysis. BCVA ranged from 20/20 to 20/400. In 5/42 eyes, cSLO imaging and corresponding SD-OCT showed coincident vitelliform macular detachment, and in 9/42 eyes showed coincident geographic atrophy (GA). The "typical" cuticular drusen, intensely staining on early FA phase ("stars-in-the-sky" appearance in the fundus), appeared as "sawtooth" retinal pigment epithelium (RPE) elevation on SD-OCT. Some "atypical" cuticular drusen appeared, on early FA and ICGA frames, as hyper-fluoresecent lesions surrounded by faint hypo-fluoresecent halos. These lesions, which became intensely hyper-fluorescent in the late FA and ICGA phases, appeared, on SD-OCT, as small, confluent "dome-shaped" RPE elevations. Interestingly, some less intensely staining cuticular drusen (FA and ICGA) appeared as irregular slight thickening of RPE/Bruch's membrane complex on SD-OCT scans. CONCLUSION: Integrated imaging makes it possible to highlight different features within cuticular drusen-containing regions, and gives insights into pathology. We suggest that "typical" cuticular drusen may represent a continuous layer of early basal laminar deposit (BLamD) associated with membranous debris accumulation. As early BLamD thicken, the lesions become richer in solid lipid particles, and "atypical" cuticular drusen may develop.

Angiography features of early onset drusen.

AIMS: Drusen are rarely observed in patients < 50 years of age. Two types of early onset drusen (EOD) are commonly described: basal laminar drusen (BLD) and drusen associated with Malattia Leventinese (ML). Our purpose was to classify the phenotype of EOD on the basis of fundus examination, and fluorescein angiography (FA) and indocyanine green angiography (ICGA) features. METHODS: We performed a prospective study including 48 consecutive EOD patients. All of them had a complete ophthalmologic examination including FA and ICGA. RESULTS: BLD (67%) were extremely hyperfluorescent on FA and ICGA. ML (10%) was characterised by a combination of small radial and large round drusen with differences in staining in both FA and ICGA. We evidenced a third type of EOD (23%) harbouring an aspect of large colloid drusen (LCD), mildly hyperfluorescent in the early phases of FA, with a progressive staining in late phases. In intermediate and late phases of ICGA, LCD presented as hypofluorescent dot surrounded by a hyperfluorescent halo bordered by a thin hypofluorescent ring. CONCLUSION: Three types of EOD are distinguished by their FA and ICGA features. We report a new kind of juvenile drusen, distinct from BLD and ML, named LCD, associated with a good vision and absence of complications.

Intravitreal ranibizumab for choroidal neovascularization secondary to choroidal nevus.

PURPOSE: To describe a patient treated with ranibizumab for juxtafoveal choroidal neovascularization secondary to choroidal nevus. METHODS: A 71-year-old man presented with juxtafoveal choroidal neovascularization secondary to choroidal nevus. Choroidal neovascularization was initially treated with a single intravitreal injection of ranibizumab. RESULTS: The treatment was successful and best-corrected visual acuity improved from 20/50 to 20/25 and remained stable during 7 months. A recurrence was observed 9 months after initial treatment. CONCLUSION: Ranibizumab seems to be an effective treatment for choroidal neovascularization secondary to choroidal nevus. However, late recurrence may occur.

Indocyanine green angiography features of central areolar choroidal dystrophy.

PURPOSE: Central areolar choroidal dystrophy is an inherited autosomal dominant macular disease characterized by a central atrophy of the retinal pigment epithelium and choriocapillaris. Our purpose was to describe fluorescein angiography and confocal indocyanine green angiography features of central areolar choroidal dystrophy. METHODS: We performed a complete ophthalmologic examination including best corrected visual acuity, fundus examination, color fundus photographs, red free frames, fluorescein angiography, infrared, confocal indocyanine green, and electroretinography in a cohort of patients with a family history of central areolar choroidal dystrophy. RESULTS: Eleven patients (22 eyes) affected were prospectively included. Indocyanine green differentiated two distinct phenotypes. In 9/11 patients, atrophy area was hyperfluorescent or normofluorescent. In the two other patients, the lesion was hypofluorescent from early to late phases and pinpoints were observed on the late phases. CONCLUSION: In our small series, indocyanine green angiography distinguished two phenotypes of central areolar choroidal dystrophy, correlated with fluorescein angiography features.