Hyporeflective Cavities Research Articles

Spectral-Domain Optical Coherence Tomography Imaging of the Typical cystoid degeneration

ABSTRACT Purpose: To describe the spectral-domain optical coherence tomography (SD-OCT) features of typical cystoid degeneration. Methods: This was a retrospective study of 11 eyes with typical cystoid degeneration (TCD). All patients had a complete ocular examination, ultra-widefield (UWF) pseudocolor fundus photography and SD-OCT with a 55° wide-field lens. We analyzed the cross-sectional structural information of SD-OCT imaging with TCD. Results: On SD-OCT, the TCD regions exhibited rolling hills patterns with irregularly elevated retinal surface, and multiple intraretinal hyporeflective cavities separated by irregular septums were seen in the neurosensory retina. Destructive changes were seen in the ellipsoid zone and the pigment epithelium. Consolidated vitreous with moderate to high reflectivity was seen over the lesion and there might be vitreoretinal adhesions and tractions. Results: SD-OCT shows exquisite structural features of the anatomy in vivo detail of the TCD. This imaging technique may deepen our structural understanding of TCD and may influence decision-making in clinical practice.

Demographic and Multimodal Imaging Features of Macular Telangiectasia Type 2: Korean Macular Telangiectasia Type 2 Study – Report No. 2

ABSTRACT Purpose: To investigate the demographic and multimodal imaging features of macular telangiectasia (MacTel) type 2 in Korea and their relationship with visual acuity and the clinical stage. Methods: A retrospective multicentre cross-sectional study was conducted in six tertiary hospitals in Korea and the study included 84 patients. Demographic data and imaging data of fundus photography, fundus autofluorescence (FAF), confocal blue-light reflectance (CBR), fluorescein angiography (FAG), and optical coherence tomography (OCT) were collected. Results: The Korean patients with MacTel type 2 were predominantly female (75%), and the mean logMAR visual acuity was 0.282 ± 0.280 at initial presentation. Most commonly presented signs were the loss of retinal transparency in fundus photographs (68.3%); increased autofluorescence in FAF (83.6%); increased blue reflectance involving the centre in CBR (68.0%); telangiectatic vessels in FAG (88.2%); and hyporeflective cavities in OCT (77.7%). The eyes diagnosed in the first half of the study period (2009–2014) showed a tendency to be diagnosed at more advanced severe stages than those diagnosed in the second half of the study period (2015–2019), using new severity scales based on FAG, FAF and OCT findings. Conclusion: The clinical features of MacTel type 2 in Korean patients assessed by newer imaging modalities suggest that Korean patients and the Caucasian-dominant population show similar presentations. This study showed that MacTel type 2 can be diagnosed in the earlier phase of the disease by using new imaging modalities and through better understanding of the disease.

Optical coherence tomographic features of macular telangiectasia type 2: Korean Macular Telangiectasia Type 2 Study\u2014Report No. 1

We analysed the imaging findings of macular telangiectasia (MacTel) type 2 in Korea using spectral domain optical coherence tomography (SD-OCT) and investigated their relationship with visual acuity and clinical stages. A retrospective multicentre cross-sectional study was conducted in six tertiary hospitals in Korea and included 129 patients. We analysed all the SD-OCT images encompassing the macular area. Hyporeflective cavities (77.7%) were the most frequently detected abnormalities in SD-OCT. Disruption of the external limiting membrane, ellipsoid zone, and interdigitation zone were found in 67 (40.4%), 87 (52.4%), and 94 eyes (56.6%), respectively. Four eyes (2.4%) had lamellar macular hole, and five eyes (3.0%) full-thickness macular hole. Neovascularisation, either subretinal or intraretinal, was found in 14 eyes (8.4%). Eyes with outer retinal hyperreflective band disruption had lower visual acuity than those without them. The presented characteristic clinical features of OCT in MacTel type 2 can not only aid in differentiating this disease from others but are also helpful for better judgement of the disease stage in daily clinical practice. Inner retinal hyporeflective cavities without outer retinal abnormalities on SD-OCT, although classified as severity scale 3, could be considered a relatively early stage in the disease process in terms of vision.

Features of cotton wool spots in diabetic retinopathy: a spectral-domain optical coherence tomography angiography study.

To describe the features of cotton wool spots (CWSs) in diabetic retinopathy (DR) by using spectral-domain optical coherence tomography (SD-OCT) and optical coherence tomography angiography (OCT angiography). We retrospectively studied patients with DR who presented CWSs and had been imaged by SD-OCT angiography. The retinal layer localisation and dimensions of the CWSs were assessed on SD-OCT, while the decorrelation signal generated by the CWSs and the vascular density (VD) of the superficial capillary plexus (SCP) and deep capillary plexus around the CWSs and in the macula were assessed on OCT angiography. A total of 87 eyes of 57 DR patients were included. All CWSs were located in the retinal nerve fibre layer and exhibited a decorrelation signal on OCT angiography. In 68 eyes (78.1%), hyporeflective cavities appearing as cysts in the CWS were noted, especially when were peripheral and large (P < 0.0001). The thickness averaged 211.8 ± 80.02mm and was positively influenced by recent diabetes history, glycaemic dysregulation, posterior pole localisation and a high VD of the SCP around the CWS, while the surface area averaged 0.33 ± 0.24mm2 and was influenced only by a low VD of the SCP around the CWS (P < 0.001). In 72 eyes (82.7%), the non-perfused areas were predominant in the periphery of the CWS. The features of CWSs are influenced by systemic factors, such as history and severity of diabetes, in association with local factors, such as localisation and retinal ischaemia. The peripheral localisation of ischaemia confirms the hypothesis that CWSs are sentinel lesions.

Swept-source and optical coherence tomography angiography in patients with X-linked retinoschisis

PurposeTo explore the structural features of juvenile X-linked retinoschisis (XLRS) using swept-source-optical coherence tomography (SS-OCT) and optical coherence tomography angiography (OCT-A).DesignRetrospective, observational cross-sectional study.Patients and methodsNine patients (18 eyes) diagnosed with juvenile XLRS were included. SS-OCT and OCT-A were used to evaluate the characteristics of the inner/outer retina and the choroid.ResultsSS-OCT showed that the inner nuclear layer (INL) was the most commonly affected area (16/18 eyes; 89%). No significant differences in central macular thickness (CMT) or subfield choroidal thickness (SFCT) were evidenced between eyes (CMT: 364 μm in the right eye vs 320 μm in the left eye; SFCT: 305 vs 307 μm; P=0.895). Best-corrected visual acuity (BCVA) did not correlate with CMT (rs= -0.19; P=0.445) or SFCT (rs=0.06; P=0.795). BCVA was significantly correlated with the following defects: outer plexiform layer (OPL; rs=0.50; P=0.036); external limiting membrane (ELM; rs=0.65; P=0.003); ellipsoid portion of inner segment (EPIS; rs=0.67; P=0.002); and the cone outer segment tips (COST; rs=0.69; P=0.001). Schisis at the INL revealed a spoke-like pattern in the foveal region and a reticular pattern in the parafoveal region on en-face imaging. In cases in which the schisis affected the OPL, multiple polygonal hyporeflective cavities were observed in the foveal region.ConclusionsThe hyporeflective spaces on SS-OCT were primarily located at the INL and OPL. BCVA did not correlate with CMT or SFCT; however, ELM, EPIS, and COST defects were significantly correlated with worse BCVA. There was a positive correlation between age and SFCT.

Research progress in the pathogenesis and imaging of peripapillary intrachoroidal cavitation

Peripapillary intrachoroidal cavitation (PICC) is a common pathological change observed in high myopia. The exact pathogenesis of PICC is still unclear. Expansion and mechanical stretching of the peripapillary sclera, breakage and defect in the retina near the border of the myopic conus and communication between intrachoroidal cavity and the vitreous space may be important segments during the development of PICC. Color fundus photography shows a localized and well-circumscribed peripapillary lesion with yellow- orange colour, often accompanied by fundus changes, such as myopic conus excavation, optic disc tilting and inferotemporal retinal vein bending at the transition from the PICC to the myopic conus. However, the PICC lesion is not easy to be recognized in the fundus photography. Fluorescein angiography shows early hypofluorescence and later progressively staining in the lesion. Indocyanine green angiography shows hypofluorescence throughout the examination. Optical coherence tomography (OCT) is vital in diagnosing PICC. Hyporeflective cavities inside the choroid, sometimes communicating with the vitreous chamber, can be observed in OCT images. OCT angiography indicates lower vessel density or even absence of choriocapillary network inside or around PICC lesions. Key words: Choroid diseases/diagnosis; Myopia, degenerative/complications; Fluorescein angiography; Tomography, optical coherence; Review

Choroidal Caverns: A Novel Optical Coherence Tomography Finding in Geographic Atrophy.

PurposeTo describe and interpret “choroidal caverns,” an unreported optical coherence tomography (OCT) finding in the choroid of patients with geographic atrophy (GA) secondary to atrophic AMD.MethodsRetrospective analysis of patients with GA. Main outcomes measures included estimation of the prevalence of choroidal caverns, their localization and relation with retinal-choroidal structures by reviewing medical records and multimodal imaging.ResultsOne hundred twenty consecutive patients (mean age 80.5 ± 8.61 years) were included. Among the 201 eyes with GA, 17 eyes of 15 patients presented choroidal caverns on OCT B-scan in GA areas (a total of 43 choroidal caverns, mean 2.5/eye, variably localized in the Sattler and Haller layers, with relative preservation of the choriocapillaris). This accounts for 12.5% estimated prevalence (6.5–18.5, 95% confidence interval [CI]) of choroidal caverns in GA areas of atrophic AMD patients. Choroidal caverns appeared on OCT (both B-scan and en face) as gaping hyporeflective cavities in the choroid, typically empty, angular, without hyperreflective borders, often with punctate/linear hyperreflectivities internally. Indocyanine angiography and OCT-Angiography confirmed that the areas occupied by these cavities do not represent perfused choroidal blood vessels.ConclusionsChoroidal caverns represent a relatively infrequent peculiar finding in GA areas of atrophic AMD eyes. They appear as gaping angular hyporeflective cavities in areas devoid of choroidal vessels, often with punctate/linear hyperreflectivities internally. Choroidal caverns may possibly arise from nonperfused ghost vessels and persistence of stromal pillars where the vessels were originally situated.

Morphological characteristics in macular telangiectasia type 2

Macular telangiectasia type 2 is characterized by atrophic alterations of the central retina which is accompanied by a defined vascular phenotype. The disease manifests within an oval central retinal area the size of approximately two disc diameters, with a topographic predisposition temporal to the foveal center. Funduscopy reveals reduced retinal transparency, crystalline deposits, mildly ectatic capillaries, thickened venules and retinal pigment plaques. Secondary neovascularization and macular holes may occur during the disease course. Fluorescein angiography usually shows a diffuse leakage and often ectatic capillaries. On optical coherence tomography (OCT) examination, hyporeflective cavities and focal atrophy of the photoreceptor layer represent a frequent finding. A characteristic sign is an increased (para) central signal on fundus autofluorescence imaging due to a reduced density of macular pigment.

Comparison of mathematical morphological descriptors of hyporeflective cavities in optical coherence tomography of patients with macular telangiectasia compared to patients with diabetic maculopathy

Comparison of mathematical morphological descriptors of hyporeflective cavities in optical coherence tomography of patients with macular telangiectasia compared to patients with diabetic maculopathy

Télangiectasies maculaires idiopathiques au cours d’une rectocolite hémorragique

Macular telangiectasia type 2 is a bilateral disease of unknown cause with characteristic alterations of the macular capillary network and neurosensory atrophy. Its prevalence may be underestimated and has recently been shown to be as high as 0.1% in persons 40 years and older. Biomicroscopy may show reduced retinal transparency, crystalline deposits, mildly ectatic capillaries, blunted venules, retinal pigment plaques, foveal atrophy, and neovascular complexes. Fluorescein angiography shows telangiectatic capillaries predominantly temporal to the foveola in the early phase and a diffuse hyperfluorescence in the late phase. High-resolution optical coherence tomography (OCT) may reveal disruption of the photoreceptor inner segment–outer segment border, hyporeflective cavities at the level of the inner or outer retina, and atrophy of the retina in later stages. Macular telangiectasia type 2 shows a unique depletion of the macular pigment in the central retina and recent therapeutic trials showed that such depleted areas cannot re-accumulate lutein and zeaxanthin after oral supplementation. There have been various therapeutic approaches with limited or no efficacy. Recent clinical trials with compounds that block vascular endothelial growth factor (VEGF) have established the role of VEGF in the pathophysiology of the disease, but have not shown significant efficacy, at least for the non-neovascular disease stages. Recent progress in structure–function correlation may help to develop surrogate outcome measures for future clinical trials.In this review article, we summarize the current knowledge on macular telangiectasia type 2, including the epidemiology, the genetics, the clinical findings, the staging and the differential diagnosis of the disease. Findings using retinal imaging are discussed, including fluorescein angiography, OCT, adaptive optics imaging, confocal scanning laser ophthalmoscopy, and fundus autofluorescence, as are the findings using visual function testing including visual acuity and fundus-controlled microperimetry. We provide an overview of the therapeutic approaches for both non-neovascular and neovascular disease stages and provide a perspective of future directions including animal models and potential therapeutic approaches.

Macular telangiectasia type 2

Macular telangiectasia type 2 is a bilateral disease of unknown cause with characteristic alterations of the macular capillary network and neurosensory atrophy. Its prevalence may be underestimated and has recently been shown to be as high as 0.1% in persons 40 years and older. Biomicroscopy may show reduced retinal transparency, crystalline deposits, mildly ectatic capillaries, blunted venules, retinal pigment plaques, foveal atrophy, and neovascular complexes. Fluorescein angiography shows telangiectatic capillaries predominantly temporal to the foveola in the early phase and a diffuse hyperfluorescence in the late phase. High-resolution optical coherence tomography (OCT) may reveal disruption of the photoreceptor inner segment-outer segment border, hyporeflective cavities at the level of the inner or outer retina, and atrophy of the retina in later stages. Macular telangiectasia type 2 shows a unique depletion of the macular pigment in the central retina and recent therapeutic trials showed that such depleted areas cannot re-accumulate lutein and zeaxanthin after oral supplementation. There have been various therapeutic approaches with limited or no efficacy. Recent clinical trials with compounds that block vascular endothelial growth factor (VEGF) have established the role of VEGF in the pathophysiology of the disease, but have not shown significant efficacy, at least for the non-neovascular disease stages. Recent progress in structure-function correlation may help to develop surrogate outcome measures for future clinical trials. In this review article, we summarize the current knowledge on macular telangiectasia type 2, including the epidemiology, the genetics, the clinical findings, the staging and the differential diagnosis of the disease. Findings using retinal imaging are discussed, including fluorescein angiography, OCT, adaptive optics imaging, confocal scanning laser ophthalmoscopy, and fundus autofluorescence, as are the findings using visual function testing including visual acuity and fundus-controlled microperimetry. We provide an overview of the therapeutic approaches for both non-neovascular and neovascular disease stages and provide a perspective of future directions including animal models and potential therapeutic approaches.