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The article presents a case study of a 12-year-old girl with an 8-month history of central retinal vein occlusion (CRVO) in the left eye. She was examined with her intraocular pressure 12 mmHg in both eyes. Normal findings for the right eye and evidence of chronic CRVO in the left eye were revealed in the Fundus examination through an association with disseminated laser scars and central macular fibrosis. The presence of an arteriovenous anastomosis in central retinal vein is discussed.

Purpose: To investigate changes to the vitreoretinal interface in uveitis with multimodal imaging including adaptive optics., Methods: Four eyes (four patients) affected by fovea-attached (subtype 1A) or fovea-sparing epiretinal membranes (ERMs) on spectral-domain optical coherence tomography or visible internal limiting membrane (ILM) on infrared scanning laser ophthalmoscope (SLO) fundus imaging were recruited in this pilot study. The microstructure of the vitreoretinal interface was imaged using flood-illumination adaptive optics (FIAO), and the images were compared with the cross-sectional spectral-domain optical coherence tomography data., Results: Adaptive optics images revealed multiple abnormalities of the vitreoretinal interface, such as deep linear striae in ERM, and hyperreflective microstructures at the location of ERMs and ILMs. The cone mosaic was imaged by FIAO and was found altered in the four eyes with ERMs or visible ILM. The same four eyes presented alteration of photopic 30 Hz flicker that was reduced in amplitude indicating cone inner retinal layer dysfunction., Conclusion: FIAO imaging can identify specific patterns associated with ERMs and ILMs. Correlating FIAO imaging of the vitreomacular interface with the structural alterations seen in FIAO at the level of the outer retinal structures can help understand the cause of significant macular dysfunction associated with ERM.

Purpose: Macular edema is a common retinal disease which may leave important anatomical and functional sequelaes. Directional fundus imaging consists of comparing on- and off-axis images to reveal angle-dependent reflectance properties of fundus structures, which may be related to misaligned photoreceptors. Here, we analyzed directional optical coherence tomography (OCT) and flood-illumination adaptive optics ophthalmoscopy images to detect evidence of misaligned photoreceptors following macular edema., Methods: Transversal, observational study. Nine patients having recovered a normal macular profile after macular edema due to retinal vein occlusion were included. For each patient, a reference OCT scan (i.e., with the incident beam normal to the fovea) was acquired, and off-axis scans were then acquired by laterally shifting the entry pupil. In addition, in four of these eyes, directional adaptive optics ophthalmoscopy documented the directional variations of cone metrics., Results: Included patients comprised two women and seven men (age range, 19-76 years). Reference OCT scans showed patchy attenuation of the cone outer segment tips and to a lesser extent of the inner segment/outer segment lines in all, but two eyes; these. Increased intensity of the cone outer segment tips and inner segment/outer segment lines could be observed on off-axis scans. Accordingly, fusion images showed 66% average reduction of the length of cone outer segment tips attenuation. In two cases, although reference scans showed continuity of outer bands, focal attenuation was evidenced in off-axis images. Directional adaptive optics ophthalmoscopy imaging showed a strong directional variability of cone counts in these areas, ranging from near absence to roughly two-third of reference values. In each case, directional variations of cone counts paralleled those of the reflectance of outer bands., Conclusion: After macular edema, focal attenuations of the inner segment/outer segment and of the cone outer segment tips lines may be present on OCT. These areas may show a strong directional variability by both OCT and adaptive optics ophthalmoscopy, suggesting that misaligned photoreceptor outer segments contribute to such features. The evaluation of outer retinal damage following macular edema should therefore take into account the optical Stiles-Crawford effect to disambiguate missing from misaligned cones.

Purpose: To report a novel finding in patients with Fabry disease, that is, the observation by adaptive optics ophthalmoscopy of intracellular lipidic deposits in retinal vessels., Methods: Observational two-center case series. Eighteen patients with genetically proven Fabry disease underwent flood-illumination adaptive optics ophthalmoscopy imaging (rtx1; Imagine Eyes, Orsay, France) of retinal vessels., Results: Fourteen patients (78% of all patients; 7 of the 10 women and 7 of the 8 men) showed paravascular punctuate or linear opacities in both eyes. In the least-affected patients, these were seen only in the wall of precapillary arterioles as discrete spots of 5 µm to 10 µm large, whereas in those more severely affected, capillaries and first-order vessels were also involved with diffuse opacification of the wall. These deposits sometime showed a striated pattern, suggesting colocalization with vascular smooth muscle cells., Conclusion: Adaptive optics ophthalmoscopy of retinal vessels may be of interest for patients with Fabry disease, providing noninvasive, gradable evaluation of microvascular involvement.

Purpose: To determine whether cone photoreceptors are impaired early in macular telangiectasia type 2 (MacTel 2) progression., Methods: Eight patients with MacTel 2 lacking intraretinal cavitation underwent multimodal retinal imaging including adaptive optics. Cone packing metrics were determined in 5 sampling windows at different eccentricities from the fovea. Results were compared with a previously established normative database., Results: In MacTel 2 eyes, cone density was significantly lower than normal at all eccentricities (P < 0.0001). Mean cone spacing and mean percentage of hexagonally organized cone photoreceptors were respectively significantly larger and lower than normal at all eccentricities (P = 0.0488 and P < 0.0001). In MacTel 2 patients, adaptive optics showed an irregular patchy disturbance of the cone mosaic corresponding to some fragmentation of the interdigitation zone on optical coherence tomography. The ellipsoid zone remained intact in the studied area., Conclusion: Adaptive optics showed that the macular cone density was lower than normal even outside the telangiectasia in MacTel 2 lacking intraretinal cavitation, although the ellipsoid zone remained intact on optical coherence tomography. These findings do not indicate that the cone density loss is causative of the disease as it might be secondary to Müller cell or rod loss in this area. However, cone density assessment could become a useful parameter to monitor disease progression.

Purpose: To describe the macular deep capillary plexus (DCP) in normal eyes using optical coherence tomography angiography., Methods: Retrospective study including 41 consecutive normal eyes imaged using optical coherence tomography angiography (RTVue XR Avanti; Optovue Inc). Default autosegmentation of the superficial capillary plexus (SCP) and DCP, and manual adjustments of "deep settings" were used to analyze the organization of the normal macular microvascularization and to investigate in vivo the connection between these capillary networks., Results: Mean age was 31 years (range, 22-55 years). The SCP and DCP had 2 different organizations, but the plexus autosegmentation was imperfect: In 68% of cases, the image of the SCP variably superimposed on the DCP, interfering with its analysis. The SCP was composed on average of 7 pairs of arterioles and venules obvious on each 3-mm × 3-mm optical coherence tomography angiography scanning area. The DCP was composed of a capillary vortex arrangement, whose centers were aligned along the course of the macular superficial venules., Conclusion: The SCP and DCP had two different topographic organizations. The pattern of the capillary units converging into capillary vortexes highly suggests that they drain into the superficial venules. The different structural properties of the SCP and DCP could explain the differences in flow resistance and perfusion.

Background: In healthy fundi, glistening whitish dots (so-called Gunn's dots) can often be seen, especially in young subjects. They are commonly attributed to the reflectance of Müller cell's footplates. However, despite their potential interest as biomarkers of retinal diseases, Gunn's dots have received little attention in the scientific literature., Methods: Scanning laser ophthalmoscope reflectance imaging and adaptive optics infrared flood imaging were performed in 18 healthy subjects (age range, 18-58 years) to analyze the localization, density, and shape of Gunn's dots., Results: Gunn's dots were more easily observed in the midperipheral retina along temporal vessels, although in two subjects, they could be detected in the macula. The reflectance of Gunn's dots showed a strong directional variability, which paralleled that of the inner limiting membrane. The mean (±SD) diameter of Gunn's dots was 13.3 µm (±3.5). Their density peaked at ∼120 per square millimeter and decreased with age to become barely detectable after 50 years., Conclusion: Gunn's dots are highly anisotropic structures close to the inner limiting membrane. Their density, size, and age-related decline are closer to the characteristics of hyalocytes than those of Müller cells. Further studies are necessary to progress in the determination of their origin and interest as biomarkers of retinal diseases.

Purpose: To describe small hyperreflective areas using spectral-domain optical coherence tomography (SD-OCT) imaging in eyes that have had silicone oil tamponade., Methods: Retrospective case series of 11 eyes of 11 patients. The authors retrospectively identified patients who underwent vitrectomy and silicone oil tamponade secondary to a rhegmatogenous retinal detachment (nine patients), panuveitis with retinal necrosis (one patient), or recurrent full-thickness macular hole surgery (one patient) who had manifestations of silicone oil emulsion on SD-OCT imaging. Patients were monitored during the postoperative period by clinical examination and using SD-OCT. A model eye in which emulsified silicone oil had been injected in the anterior chamber was used to obtain anterior segment SD-OCT images for comparison., Results: The mean age of our patients was 50 years (range, 39-76 years). In eight eyes, the SD-OCT examination was carried out after silicone oil removal, and in three eyes, the SD-OCT examination was carried out with the oil in situ. Of the nine eyes treated for rhegmatogenous retinal detachment, five had a relieving retinectomy for advanced anterior proliferative vitreoretinopathy or for traumatic retinal incarceration (one eye). The eye treated for full-thickness macular hole had a vitrectomy, internal limiting membrane peel, and silicone oil injection for recurrent macular hole. Ten eyes showed hyperreflective, spherical, tiny droplets using SD-OCT imaging. These were thought to represent silicone oil droplets intraretinally or underneath epiretinal membranes, and one eye showed hyperreflective areas subretinally (retina detached). One additional patient was found to have tiny intravitreal silicone oil droplets after silicone oil removal. Similarly, the silicone oil appeared as multiple hyperreflective spherical droplets as detected by SD-OCT. Anterior segment studies of silicone oil emulsification in the experimental model revealed a similar appearance to that seen with in vivo SD-OCT imaging., Conclusion: The authors have found small hyperreflective areas intraretinally, subretinally, and underneath epiretinal membranes on SD-OCT in eyes that have had silicone oil tamponade for a variety of indications. The authors have seen a similar appearance when silicone oil emulsification is examined in vivo. The authors conclude that the hyperreflective areas are likely (but not certain) to be very small bubbles of emulsified silicone. Further studies are required to determine the incidence, clinicopathologic, and functional significance of probable silicone oil emulsification and deposition within the retinal layers.