A 46-year-old woman with bilateral white retinal vessels

Lipemia retinalis is a very rare ocular manifestation of severe hypertriglyceridemia. It is usually symptomatic and regresses after normalization of triglycerides levels. Early recognition is important to prevent ocular and life-threatening complications. This study aimed to report a case of marked lipemia retinalis secondary to type V hypertriglyceridemia assessed with swept-source optical coherence tomography (OCT) and OCT angiography (OCT-A), with follow-up after dietary lipid restriction. Observational case report of lipemia retinal findings on color fundus photography, swept-source OCT and OCT-A, initially and after triglycerides lowering. A 32-year-old pregnant patient with gestational diabetes and a history of hypertriglyceridemia was referred for diabetic retinopathy screening. Fundus examination revealed bilateral milky-white discoloration of retinal vessels with a "salmon-colored" retina. Swept-source OCT and OCT-A revealed extremely hyperreflective and dilated retinal vessels and multiple high-flow retinal hyperreflective dots, corresponding to dilated retinal capillaries. Choroidal vessels were enlarged and engorged, and choriocapillaris layer appeared thickened and hyperreflective with dilated and tortuous capillaries. Serum triglycerides were very high (70.02 mmol/L). After 21 days of very-low-fat diet, it was lowered to 15 mmol/L. We noted a normalization of the clinical, structural, and vascular findings. However, peripheral retinal vessels remained hyperreflective, despite their clinical normalization. Swept-source OCT and OCT-A were beneficial in assessing lipemia retinalis noninvasively and monitoring choroidal and retinal vascular changes. Lipemia retinalis signs regressed initially in the posterior pole, choroidal anomalies were first to resolve, and clinical normalization preceded tomographic resolution.

We evaluated the structural and functional features of combined hamartoma of the retina and retinal pigment epithelium using multimodal imaging technologies, including the new technique of optical coherence tomography (OCT) angiography, which has previously not been used to examine this rare condition. Nine eyes of nine Caucasian patients (median age at presentation: 8 years; range: 1−36 years) were evaluated in the Eye Clinic of the University of Naples ‘Federico II’ between September 2014 and February 2015. Patients underwent complete ophthalmic examination, bulbar echography, enhanced depth imaging optical coherence tomography (EDI-OCT), wide-field en-face OCT, multicolour imaging, fluorescein and indocyanine angiography and OCT angiography. The mean best-corrected visual acuity was 20/40 ± 4.2 Snellen. At standardized A-scan echography, the mean tumour basal dimension was 3 mm. All tumours were in the temporal quadrant, and seven affected the left eye. The OCT findings revealed a mean foveal retinal thickness of 522 ± 248 (mean ± standard deviation) μm in affected eyes versus 226 ± 22 μm in unaffected eyes. The mean retinal thickness through the tumour epicentre in affected and unaffected eyes was 809 ± 269 μm and 361 ± 44 μm, respectively. The mean tumour thickness was 752 μm. On EDI-OCT, the mean choroidal thickness below the tumour epicentre was significantly less in affected eyes than the mean choroidal thickness in unaffected eyes (251 ± 50 μm versus 388 ± 38 μm). Both intraretinal and subretinal fluid was present in two affected eyes, intraretinal fluid alone in three affected eyes, retinal striae overlying the tumour in two eyes and retinal schisis in two eyes. Based on multicolour images, hamartomas were classified green or grey, indicating more or less abundant tissue, respectively. En-face OCT revealed an epiretinal membrane and vitreoretinal traction in all patients. Fluorescein and indocyanine angiography in the early phase showed such vascular irregularities as dilation or vascular tortuosity which were not visible in the later phase due to leakage. However, at OCT angiography, vascular irregularities were clearly seen in the tumoral area. Moreover, OCT angiography showed that the main superficial retinal vessels lose most of their collateral branches and present many loops; capillaries were rare and there were anomalies in vessel size. At deep plexus level, changes in vessel size and morphology were evident and the capillary fans were irregular. No functional or anatomic changes versus baseline were identified at the 24-month follow-up observation, except in a 21-year-old man in whom visual acuity decreased. As shown in Fig. 1, multicolour imaging at follow-up in this patient revealed retinal haemorrhage and increased retinal traction. A-scan echography and OCT showed an increase in the size of the tumour base, retinal thickness and retinal traction, which can cause retinal haemorrhage. Optical coherence tomography angiography revealed vessel stretching and a slight reduction in anastomosis. In this patient, the hamartoma slowly increased in size thereby causing shrinkage of the internal membrane of the tumour, which in turn leads to deformation of the retinal vessels as they are attracted towards the centre of the neoplasm (Mele et al. 1984). Surgery in the management of combined hamartoma remains controversial (Bruè et al. 2013). In fact, the surface glial membrane causing the retinal distortion is often an integral part of the tumour, which can mean that is difficult or impossible to strip the membrane and that there is little chance of central vision (Chhablani et al. 2015). In conclusion, our results indicate that the new OCT angiography technique is a non-invasive, reliable method with which to evaluate vascular irregularities in the tumour area without the injection of any dye. Consequently, OCT angiography is an ideal procedure with which to evaluate this condition in young patients.

Choroidal osteoma (CO), also known as osseous choristoma, is a rare benign osseous tumour of the choroid. It is diagnosed based on the presence of a yellowish-white to orange lesion deep in the retinal pigment epithelium (RPE) with well-defined margins and bone density in ultrasonography (Pellegrini et al. 2014). Patients are usually asymptomatic and the lesion is found incidentally. Choroidal neovascularization (CNV) is the most frequent complication of CO and leads to severe visual impairment. Anti-vascular endothelial growth factor (Anti-VEGF) therapy has been recommended for CNV-related CO (Song & Roh 2009). Here, we report a case of extrafoveal CO associated with CNV. A 75-year-old woman, incidentally diagnosed with CO at the age of 68 years, presented with a two-month history of metamorphopsia in the left eye. Best-corrected visual acuity (BCVA) was 0 logMar in the right eye and 0.7 logMar in the left eye. Anterior segment findings were unremarkable in both eyes. A fundus examination of the right eye was unremarkable, whereas, in the left eye, it revealed a yellowish-white area with small haemorrhages in the superior temporal retina that suggested an extrafoveal CO associated with CNV (Fig. 1A,B). Baseline evaluation of the right eye. (A, B) Colour fundus photography and multicolour image showing the choroidal osteoma with subretinal haemorrhage. The black circle underlines macular haemorrhage spots. (C−E) Optical coherence tomography (OCT) angiography, fluorescein angiography and indocyanine green angiography revealed late-stage choroidal neovascularization within the tumour. The red circle underlines macular haemorrhage spots (F, G) A and B scan echography showed a slightly elevated choroidal mass with high reflectivity and acoustic shadowing. (H) Enhanced depth imaging optical coherence tomography (EDI-OCT) showed a hyper-reflective mass in the subretinal space associated with a neurosensory detachment and detected a sponge-like structure of the choroid. (I, J) Fluorescein and indocyanine angiography of the superotemporal region of the contralateral normal eye. (K) Optical coherence tomography (OCT) scan of the macular region without any pathological feature. (L−P) Colour fundus photography, multicolour image, fluorescein angiography and indocyanine green angiography and OCT angiography showing regression of the choroidal neovascularization after four months of treatment. (Q) Enhanced depth imaging optical coherence tomography (EDI-OCT) revealed resolution of the subretinal fluid. [Correction added on 17 June 2016 after first online publication: Figure 1 has been replaced to correct the labelling of Fig. 1E and 1O]. Optical coherence tomography (OCT) angiography (angiovue software RTVue; Optovue Inc., Fremont, CA, USA) showed a hyporeflective area surrounded by a hyper-reflective-edged ring in direct relation to the osteoma. It also showed a dense irregular vascular network within the tumour in the outer retinal layer and choroid capillary layers. Moreover, the CNV detected in the choroidal layer had fine glomerular-like hyper-reflection (Fig. 1C). Visualization of vessels is better with non-invasive OCT angiography than with fluorescein angiography (FA) and Indocyanine green angiography (ICGA); in fact, with the latter two techniques, the leaking vessels of the CNV usually are overshadowed by the dye in the late phases (Wang et al. 2015). Fluorescein angiography (FA) showed early hyperfluorescence due to leakage surrounded by an area of blocked fluorescence, and late staining of the lesion showed well-defined borders (Fig. 1D). Indocyanine green angiography (ICGA) confirmed the presence of an active CNV secondary to CO (Fig. 1E). Standardized A and B scan echography revealed a slightly elevated choroidal mass with high reflectivity and acoustic shadowing (Fig. 1F,G). Enhanced depth imaging optical coherence tomography (EDI-OCT) performed with the Heidelberg Spectralis HRA + OCT (Heidelberg Engineering, Heidelberg, Germany) showed a hyper-reflective mass in the subretinal space associated with neurosensory detachment and elongation of the outer segments. Enhanced depth imaging optical coherence tomography (EDI-OCT) also revealed a sponge-like structure of the choroid with horizontal tubules (Fig. 1H). The findings obtained in our patient strongly support the diagnosis of OC as opposed to sclerochroidal calcification (SCC). In fact, unlike OC, SCC does not feature growth, decalcification or intralesional vessels of CNV, as recently reported by Shields et al. (2015). After obtaining informed consent, we administered three monthly intravitreal injections of ranibizumab in the left eye. Four months later, the patient's BCVA was 0.1 logMar, and a fundus examination revealed resolution of the subretinal haemorrhage (Fig. 1L,M). Fluorescein angiography (FA) and ICGA showed regression of the CNV (Fig. 1N,O) and spectral domain OCT revealed no subretinal fluid (Fig. 1Q). Optical coherence tomography (OCT) angiography demonstrated a smaller and more rarified vascular network inside a capsular formation (Fig. 1P). These findings remained unchanged during the 12-month follow-up. In summary, OCT angiography reveals unique features in the vascular changes of CNV in CO not visualized with other imaging methods. However, additional studies are needed to verify whether this technique can partially replace FA and ICGA in the diagnosis and follow-up of CNV secondary to CO.

To evaluate the efficacy of optical coherence tomography (OCT) angiography versus fluorescein angiography (FA) in terms of retinal vessel imaging in ischaemic diabetic maculopathy defined according to the Early Treatment Diabetic Retinopathy Study (ETDRS) classification. Twenty patients (31 eyes) with ischaemic diabetic maculopathy and 17 control subjects (27 eyes) were enrolled in this prospective study. Patients and control subjects underwent complete ophthalmic examination, including best-corrected visual acuity (BCVA), intraocular pressure, FA, Fourier domain optical coherence tomography (FD-OCT) and OCT angiography. Fluorescein angiograms and OCT angiography images were graded according to the foveal avascular zone (FAZ) of the ETDRS group. Ganglion cell complex (GCC) thickness was evaluated with FD-OCT. Optical coherence tomography (OCT) angiography images closely correlated with FA in terms of FAZ parameters. The correlation was strongest with OCT angiography deep imaging. The average GCC thickness was smaller in patients than in controls. Neither GCC parameters nor FAZ was correlated to BCVA. Given the correlation between FA and OCT angiography in terms of FAZ parameters, the newer method can be considered a valid, reliable and easy-to-perform method with which to evaluate ischaemic diabetic maculopathy without contrast injection, and thus to visualize and quantify non-perfusion areas without risks of anaphylactic reactions.

Abnormal choroidal blood flow is considered important in the pathogenesis of chronic central serous chorioretinopathy (CSC). Optical coherence tomography (OCT) angiography can image ocular blood cell flow and could thus provide novel insights in disease mechanisms of CSC. We evaluated depth-resolved flow in chronic CSC by OCT angiography compared to fluorescein angiography (FA) and indocyanine green angiography (ICGA). Eighteen eyes with chronic CSC, and six healthy controls, were included. Two human observers annotated areas of staining, hypofluorescence, and hotspots on FA and ICGA, and areas of abnormal flow on OCT angiography. Interobserver agreement in annotating OCT angiography and FA/ICGA was measured by Jaccard indices (JIs). We assessed colocation of flow abnormalities and subretinal fluid visible on OCT, and the distance between hotspots on ICGA from flow abnormalities. Abnormal areas were most frequently annotated in late-phase ICGA and choriocapillary OCT angiography, with moderately high (median JI, 0.74) and moderate (median JI, 0.52) interobserver agreement, respectively. Abnormalities on late-phase ICGA and FA colocated with those on OCT angiography. Aberrant choriocapillary OCT angiography presented as foci of reduced flow surrounded by hyperperfused areas. Hotspots on ICGA were located near hypoperfused spots on OCT angiography (mean distance, 168 μm). Areas with current or former subretinal fluid were colocated with flow abnormalities. On OCT angiography, chronic CSC showed irregular choriocapillary flow patterns, corresponding to ICGA abnormalities. These results suggest focal choriocapillary ischemia with surrounding hyperperfusion that may lead to subretinal fluid leakage.

PurposeTo propose a noninvasive way of classifying multimodal imaging of retinal microaneurysms (MA) secondary to diabetic retinopathy (DR).MethodsThe research was designed as a cross-sectional, observational study of patients affected by DR. Multimodal imaging included confocal MultiColor imaging, optical coherence tomography (OCT) and OCT angiography (OCTA). MA green- and infrared-reflectance components were assessed by confocal MultiColor imaging, reflectivity properties by OCT, and MA perfusion features by OCTA. In addition, we included high-resolution (HR) and high-speed (HS) OCTA scans to assess HR–HS agreement in detecting retinal MA and to highlight different perfusion features detected by both OCTA acquisitions.ResultsWe analyzed 216 retinal MAs, divided into green (46; 21%), red (58; 27%) and mixed types (112; 52%). Green MAs were mainly hyper-reflective on OCT, with no or poor filling on OCTA. Red MAs were characterized by an isoreflective signal on OCT and full filling on OCTA. Mixed MAs showed a hyper-reflective border and a hyporeflective core on OCT and partial filling on OCTA. No differences in red MA HR/HS size discrepancy and reflectivity were found, whereas these progressively increased as the MA MultiColor signal changed from infrared to green. MA types significantly correlated with visual acuity, DR duration, and DR severity.ConclusionsRetinal MA can be classified reliably by means of a fully noninvasive multimodal imaging-based assessment. MA types are matched with visual acuity, DR duration and DR severity. Both HR and HS OCTA are highly effective in detecting MA, although HR OCTA is to be preferred in the presence of fibrotic evolution.Translational RelevanceThis study outlines a proposed novel MA classification based on noninvasive multimodal imaging. The findings presented in this paper endorse the clinical relevance of this approach, highlighting how this classification is associated with both DR duration and severity.

Recent decade has seen a shift in the causes of childhood blinding diseases from anterior segment to retinal disease in both developed and developing countries. The common retinal disorders are retinopathy of prematurity and vitreoretinal infections in neonates, congenital anomalies in infants, and vascular retinopathies including type 1 diabetes, tumors, and inherited retinal diseases in children (up to 12 years). Retinal imaging helps in diagnosis, management, follow up and prognostication in all these disorders. These imaging modalities include fundus photography, fluorescein angiography, ultrasonography, retinal vascular and structural studies, and electrodiagnosis. Over the decades there has been tremendous advances both in design (compact, multifunctional, tele-consult capable) and technology (wide- and ultra-wide field and noninvasive retinal angiography). These new advances have application in most of the pediatric retinal diseases though at most times the designs of new devices have remained confined to use in adults. Poor patient cooperation and insufficient attention span in children demand careful crafting of the devices. The newer attempts of hand-held retinal diagnostic devices are welcome additions in this direction. While much has been done, there is still much to do in the coming years. One of the compelling and immediate needs is the pediatric version of optical coherence tomography angiography. These needs and demands would increase many folds in future. A sound policy could be the simultaneous development of adult and pediatric version of all ophthalmic diagnostic devices, coupled with capacity building of trained medical personnel.

To evaluate and classify image artefacts in optical coherence tomography (OCT) angiography (OCTA) of the choroid in a group of patients with macular diseases. Retrospective observational study. Five patients with age-related macular degeneration, three with central serous retinopathy, one with polypoidal choroidal vasculopathy and one with multiple evanescent white dot syndrome. OCTA and OCT reflectivity (OCTR) maps were reviewed along with their fluorescein angiography and indocyanine green angiography. Sixty OCTA images (20 outer retina, 20 Sattler and 20 Haller layers) were graded for image artefacts by two examiners independently. OCTA artefacts and their correlation with OCTR maps, angiography and OCT B-scans. Artefacts (frequency) were classified into (i) motion (70-100%), (ii) fringe washout (100%), (iii) decorrelation projection (0-20%), (iv) masking and unmasking (50-65%) and (v) stromal decorrelation signal (100%). Motion artefact in OCTA is characterized by horizontal dark lines or bands not apparent on OCTR map. Fringe washout creates signal void within choroidal vessels because of fast blood flow. Decorrelation projection from retinal vasculature and choroidal new vessels above the Bruch's membrane are seen within the choroidal OCTA image. Masking and unmasking artefacts occur in regions of pigment epithelial detachment and atrophy. Decorrelation signals can also be seen in the choroidal stroma. Our classification system of artefact in choroidal OCTA establishes a common terminology for clinical interpretation. This is important in enhancing our understanding of the principles of OCTA acquisition, and it also serves as a bench mark for reading centres.

Spectral-Domain Optical Coherence Tomography Angiography of Choroidal Neovascularization

To describe the multimodal imaging, including en face optical coherence tomography (OCT) and OCT angiography, findings of a case of macular microcysts associated with neuromyelitis optica. Findings on clinical examination, color fundus photography, fluorescein angiography, fundus autofluorescence, visual fields, and OCT including en face OCT and OCT angiography are presented. A 12-year-old African American boy presented with bilateral optic atrophy from neuromyelitis optica. Clinical examination was notable for bilateral optic nerve head pallor. Visual fields of both eyes showed generalized depression. Fluorescein angiography and fundus autofluorescence were unremarkable. Spectral domain OCT B-scan images showed characteristic paracentral, hyporeflective, microcystic lesions in the inner nuclear layer of both eyes, and en face OCT images demonstrated a corresponding pattern of large paracentral cysts radiating peripherally into smaller diffuse cysts. Optical coherence tomographic angiography of the superficial and deep retinal capillary plexuses was unremarkable. Macular microcysts have been associated with various forms of optic atrophy, including neuromyelitis optica. Spectral domain and en face OCT imaging of the microcysts demonstrated a very characteristic pattern. Normal fluorescein and OCT angiography suggest that nonvascular causes, such as Müller cell degeneration, might contribute to the etiologic mechanism.

Objective To compare the consistency and difference of optical coherence tomography angiography (OCTA) and traditional multimodal fundus imaging in the diagnosis and activity evaluation of choroidal neovascularization (CNV) in exudative age-related macular degeneration (AMD). Methods A total of 112 exudative AMD patients (130 eyes) were included in this retrospective study, 62 were men (71 eyes) and 50 were women (59 eyes). The mean age was (68.250±9.789) years (range 50–91 years). All patients were underwent traditional multimodal fundus imaging including fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA) and spectral domain optical coherence tomography (OCT); OCTA was performed at the same time. The CNV type was divided into active and non-active according to the results of traditional multimodal fundus imaging. The vascular pattern was divided into active and non-active according to the results of OCTA. Using traditional multimodal fundus imaging as the standard, the sensitivity and specialty of OCTA was evaluated. Results In 130 eyes, CNV was visualized on traditional multimodal fundus imaging in 109 eyes (83.8%); CNV was visualized on OCTA in 103 eyes (79.2%), which including 7 eyes of false negative and 1 eye of false positive. The sensitivity of OCTA for CNV diagnosis was 93.6%, with specificity of 95.2%. The CNV detection rate between two methods had no significant difference (Youden index=0.89, Kappa value=0.796, P=0.07). In 109 eyes diagnosed with CNV by traditional multimodal fundus imaging, 73 eyes (67.0%) were active CNV and 36 eyes (33.0%) were non-active CNV; the vascular pattern was active in 60 eyes (55.0%) and non-active in 49 eyes (45.0%). The sensitivity of OCTA for the detection of active CNV was 82.2%, with specificity of 100.0%. The active CNV detection rate between two methods had no significant difference (Youden index=0.82, Kappa value=0.753, P=0.00). Conclusion In the diagnosis and activity evaluation of CNV in exudative AMD, there is remarkable consistency between OCTA and traditional multimodal fundus imaging. Key words: Choroidal neovascularization /diagnosis; Tomography, optical coherence; Fluorescein angiography; Indocyanine green/diagnostic use

Enhanced resolution and speckle-free three-dimensional printing of macular optical coherence tomography angiography.

Microvascular Abnormalities on Optical Coherence Tomography Angiography in Macular Edema Associated With Branch Retinal Vein Occlusion

Glaucoma Progression Detection Time Using OCT and OCTA in African and European Descendants.

To assess the ability of optical coherence tomography (OCT) alone and in combination with OCT angiography (OCTA) to differentiate polypoidal choroidal vasculopathy (PCV) from neovascular age-related macular degeneration, as compared to fluorescein angiography and indocyanine green angiography. This is a cross-sectional study. All participants had a standardized history, clinical examination including measurement of best-corrected visual acuity, slit-lamp biomicroscopy, and indirect fundus examination, and underwent standardized imaging (color photography, fluorescein and indocyanine green angiography, OCT, and OCTA) after predefined protocols. We used a 2-step approach (Step 1: spectral domain OCT; Step 2: addition of OCTA) combining structural OCT and OCTA to differentiate 50 treatment-naive eyes with PCV, choroidal neovascularization, and retinal angiomatous proliferation and compared with the diagnosis based on fluorescein angiography and indocyanine green angiography. Spectral domain OCT signs used to diagnose PCV included presence of two out of three of any retinal pigment epithelium detachment (pigment epithelial detachment/double-layer sign), notched or narrow-peaked pigment epithelial detachment, or round subretinal pigment epithelium structure. Optical coherence tomography angiography signs used to diagnose PCV included presence of a localized subretinal pigment epithelium hyperflow signal in the cross-sectional OCTA and/or presence of a focal hyperflow sign in en face OCTA based on outer retina slab. Based on fluorescein angiography and indocyanine green angiography, the diagnosis was choroidal neovascularization in 24 eyes, PCV in 23 eyes, and retinal angiomatous proliferation in 3 eyes. Based on spectral domain OCT signs, PCV was diagnosed in 19/23 (82.6%) eyes; however, specificity of OCT was only 51.9%. Cross-sectional OCTA showed a diffuse hyperflow signal in all 24 (100.0%) eyes with choroidal neovascularization, whereas a localized subretinal pigment epithelium hyperflow signal was detected in 19/23 (82.6%) eyes with PCV. En face OCTA only detected a nodular hyperflow signal in 10/23 eyes (43.5%) with PCV. Combination of OCT and OCTA achieved 82.6% sensitivity and 100.0% specificity for differentiating PCV from choroidal neovascularization/retinal angiomatous proliferation. Cross-sectional OCTA is more sensitive than en face OCTA in detecting flow signal in polyps. Combination of structural OCT and OCTA can be used to screen for PCV with a high level of sensitivity and specificity.