Purpose: The aim of this article is to describe a novel surgical technique for sutureless scleral fixation of an intraocular lens using the newly developed FIL SSF Carlevale IOL (Soleko, Italy). Methods: Four eyes of four patients with poor capsular support were recruited to our study, three resulting from intraocular lens subluxation and one case resulting from traumatic cataract. A novel sutureless sclera-fixated intraocular lens was implanted into the posterior chamber of each eye with sclerocorneal plugs fixating the lens to the wall of the eye.Results: Mean age of patients was 52±16 years, ranging from 35 to 70 years. Mean follow-up was 6.50±1.29 months (range: 5–7 months). Mean preoperative best-corrected visual acuity was 0.50±0.33 logMAR (range: 1–0.3 logMAR). Postoperative best-corrected visual acuity improved to 0.08±0.08 logMAR (range: 0.2–0 logMAR). There was no significant change in the mean intraocular pressure and there were no postoperative complications, such as iatrogenic distortion or breakage of the intraocular lens haptic, intraocular lens decentration, endophthalmitis, or retinal detachment. Discussion: To the best of our knowledge, this is the first report of outcomes using the novel sutureless sclera-fixated FIL SSF Carlevale IOL. This new surgical technique offers a simplified and effective approach for sutureless scleral intraocular lens fixation with good refractive outcomes.

Central serous chorioretinopathy (CSC) is a complex and not entirely understood retinal disease. The aim of our research was to describe a novel optical coherence tomography (OCT) finding named “choroidal rift”, which may be identified in the choroid of eyes with CSC. We collected data from 357 patients (488 eyes) with CSC who had structural OCT and OCT angiography (OCTA) scans obtained. Choroidal rifts were identified as polygonal (and not round-shaped) hyporeflective lesions without hyperreflective margins. Choroidal rifts had to be characterized by a size superior to that of the largest choroidal vessel. Finally, hyporeflective lesions were graded as choroidal rifts only if these lesions had a main development perpendicular to the retinal pigment epithelium. OCT analysis allowed the identification of choroidal rifts in ten eyes from nine patients, all with chronic CSC, with an estimated prevalence rate of 2.1%. In three out of ten cases with choroidal rifts, these lesions spanned all the choroidal layers. In the remaining cases, choroidal rifts only partially spanned the choroidal thickness. In OCTA, choroidal rifts were characterized by the absence of flow. Combining structural OCT and OCTA information, we hypothesized that choroidal rifts may represent interruptions of the choroidal stroma in correspondence of fragile regions (in between expanded larger-sized choroidal vessels). Choroidal rift represents a novel OCT feature, which may characterize eyes with chronic CSC and may have a role in the development of irreversible chorio-retinal changes.

Purpose: The aim of this article is to describe a novel surgical technique for sutureless scleral fixation of an intraocular lens using the newly developed FIL SSF Carlevale IOL (Soleko, Italy). Methods: Four eyes of four patients with poor capsular support were recruited to our study, three resulting from intraocular lens subluxation and one case resulting from traumatic cataract. A novel sutureless sclera-fixated intraocular lens was implanted into the posterior chamber of each eye with sclerocorneal plugs fixating the lens to the wall of the eye. Results: Mean age of patients was 52 +/- 16 years, ranging from 35 to 70 years. Mean follow-up was 6.50 +/- 1.29 months (range: 5-7 months). Mean preoperative best-corrected visual acuity was 0.50 +/- 0.33 logMAR (range: 1-0.3 logMAR). Postoperative best-corrected visual acuity improved to 0.08 +/- 0.08 logMAR (range: 0.2-0 logMAR). There was no significant change in the mean intraocular pressure and there were no postoperative complications, such as iatrogenic distortion or breakage of the intraocular lens haptic, intraocular lens decentration, endophthalmitis, or retinal detachment. Discussion: To the best of our knowledge, this is the first report of outcomes using the novel sutureless sclera-fixated FIL SSF Carlevale IOL. This new surgical technique offers a simplified and effective approach for sutureless scleral intraocular lens fixation with good refractive outcomes.

PURPOSE:Staphylococcus aureus bacteriemia (SAB) as critical condition for the life and occasionally involves the eyes. The aim of this report is to describe the ocular involvement with multimodal imaging.OBSERVATIONS:A patient admitted for evaluation of acute onset of confusion, disorientation, and generalized malaise and found to have methicillin-resistant staphylococcus aureus (MRSA)-associated endocarditis and multifocal brain abscesses was evaluated by the ophthalmology service. The patient's visual acuity was 20/20 OU without relative afferent pupillary defect and normal intraocular pressures. Bedside anterior segment examination was normal. Posterior segment examination revealed intraretinal hemorrhages and Roth spots in the posterior pole of the right eye, and two deep well-defined focal white chorioretinal infiltrates and a hemorrhagic pigment epithelium detachment in the temporal quadrant of the left eye. Multimodal imaging was utilized to document these findings and ensure adequate antibiotic therapy.CONCLUSION:SAB has the potential for poor visual outcomes as well as significant morbidity and mortality. Multimodal imaging of SAB-related chorioretinitis allows for accurate diagnosis as well as assessment of response to antimicrobial therapy.

Purpose: To evaluate choroidal structural changes in patients with multiple evanescent white dot syndrome (MEWDS) during the acute and recovery stages.Methods: Enhanced-depth imaging optical coherence tomography (EDI-OCT) scans of 16 patients with unilateral MEWDS were acquired during the acute and recovery stages in both eyes. Images were binarized with the ImageJ software to measure subfoveal choroidal thickness (CT), total choroid area, luminal area and choroidal vascularity index (CVI).Results: In the acute stage, subfoveal CT, total choroidal area and CVI were significantly higher in eyes with MEWDS compared to fellow eyes (371.2 101.8 vs 317.1 +/- 90.3 mu m, p = .001; 2.826 +/- 0.686 vs 2.524 +/- 0.674 mm(2), p = .014; 69.49 +/- 3.51 vs 68.27 +/- 3.41%, p = .044, respectively). In the recovery stage, subfoveal CT, total choroidal area and CVI in eyes with MEWDS significantly decreased to respectively 333.4 +/- 90.5 mu m, p = .007, 2.592 +/- 0.570 p = .002, and 67.31 +/- 2.74%, p = .014.Conclusions: Choroidal thickness and vascularity are significantly increased during the acute stage of MEWDS.

PURPOSE:The aim of this study is to describe the clinical presentation of vortex vein varices with multimodal imaging.METHODS:The authors carried out a retrospective case series of eight patients (7 female, 1 male) with an average age of 60.2 years (min 8, max 84, median 68.5) presenting with vortex vein varices. All patients were evaluated at the Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy and at Luigi Sacco Hospital, University of Milan, Milan, Italy. Patients underwent complete ophthalmologic examinations, including best corrected visual acuity, intraocular pressure, anterior segment, and fundus examination. Imaging studies, including fundus color photography, near-infrared reflectance imaging, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, and spectral-domain enhanced depth imaging optical coherence tomography were also performed. Ultra-widefield fluorescein angiography and ultra-widefield indocyanine angiography using the Heidelberg Retina Angiograph and the Staurenghi 230 SLO Retina Lens were used to demonstrate the disappearance of all retinal lesions when pressure was applied to the globe.RESULTS:All eight cases initially presented to the emergency room. One patient presented secondary to trauma, two patients presented for suspected hemangioma, whereas the other five were referred to the authors' hospitals for suspected retinal lesions. On examination, retinal abnormalities were identified in all 8 patients, with 7 (87.5%) oculus dexter and 1 (12.5%) oculus sinister, and with 1 (12.5%) inferotemporally, 3 (37.5%) superonasally, 3 (37.5%) inferonasally, and 1 (12.5%) inferiorly. Fundus color photography showed an elevated lesion in seven patients and a nonelevated red lesion in one patient. In all patients, near-infrared reflectance imaging showed a hyporeflective lesion in the periphery of the retina. Fundus autofluorescence identified round hypofluorescent rings surrounding weakly hyperfluorescent lesions in all patients. On fluorescein angiography, all lesions were initially hyperfluorescent with a hypofluorescent ring, with the lesion becoming hyperfluorescent after injection of dye. Indocyanine green angiography demonstrated dilation of the vortex vein ampullae in all patients. Spectral-domain enhanced depth imaging optical coherence tomography demonstrated dilated choroidal vessels and a hyporeflective cavity without subretinal fluid in all patients. Ultra-widefield fluorescein angiography and ultra-widefield indocyanine angiography demonstrated disappearance of all retinal lesions when pressure was applied to the globe. Findings are consistent with the diagnosis of vortex vein varix in all eight patients, with six patients (75%) exhibiting a single varix and two patients (25%) exhibiting a double varix.CONCLUSION:The diagnosis of vortex vein varices can be confirmed through clinical examination through the use of digital pressure to the globe during ophthalmoscopic examination. Adjunctive multimodal imaging (fundus color photography, near-infrared reflectance imaging, fundus autofluorescence, fluorescein angiography, indocyanine angiography, and spectral-domain enhanced depth imaging optical coherence tomography) was useful in the diagnosis of vortex vein varices in the authors' clinical cases. However, in more challenging clinical cases, the authors' novel use of the ultra-widefield contact lens for application of ocular pressure with a resulting resolution of the varix proved to be a useful and easy diagnostic imaging method for confirming the presence of vortex vein varices.

PURPOSE:To present multimodal imaging of multifocal chorioretinitis secondary to endogenous candida infection in a young adult.METHODS:A 49-year-old woman who presented for evaluation of bilateral endogenous candida chorioretinitis underwent complete ophthalmic examination, in addition to fundus photography (FP), enhanced depth imaging optical coherence tomography, fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA) and optical coherence tomography angiography (OCTA).RESULTS:Multimodal imaging of both eyes of the patient affected by endogenous candida chorioretinitis was performed. FP showed multiple white chorioretinal lesions at the posterior pole, FAF showed dark dot at the posterior pole surrounded by hyperautofluorescence area, FA showed early hyperfluorescence round perifoveal lesion at the posterior pole and small hyperfluorescence dots under the inferior retinal vessels. Early ICGA showed hypofluorescence dots at the posterior pole. Late ICGA showed dark hypofluorescence dots at the posterior pole surrounded by faint hyperautofluorescent ring. OCTA showed dark areas corresponded to hypoperfusion areas seen with early ICGA.CONCLUSION:We reported multimodal imaging of an unusual occurrence of multifocal chorioretinitis due to immunosuppression. These findings suggested that the infection resulted from choroidal infiltration via the short posterior ciliary arteries with resultant breakthrough into the retina, rather than via the central retinal artery. By comparing findings on OCTA with data obtained from traditional systems, we are gaining essential information on the pathogenesis of endogenous candida chorioretinitis.

PurposeTo compare the capability of indocyanine green angiography (ICGA) and optical coherence tomography angiography (OCTA) in detecting choroidal neovascularization (CNV).MethodsIn this prospective study, patients with CNV detected with fluorescein angiography (FA) underwent ICGA and OCTA, spectral domain OCT (SD-OCT), and infrared or fundus color photographs. CNV lesions were outlined on ICGA and OCTA images, and the composition and size of the CNV was documented.ResultsOne hundred eighty-two eyes were included. With ICGA, well-defined lesions were observed in 37.9%, partly defined in 44.5%, and undefined in 17% of eyes. On OCTA, well-defined, partly defined, and undefined vessels were observed in 53.8%, 27.5%, and 18.7% of eyes, respectively. There was a good correlation between CNV size measured with the two instruments (r = 0.84). However, OCTA underestimated CNV area by about 4.5% (slope coefficient with linear regression: 0.55, 95% confidence interval [CI]: 0.46 to 0.65; intercept: 0.27, 95% CI: -0.2 to 0.56). On ICGA, CNV composition was capillary in 28%, mature in 14.3%, and mixed (capillary and major neovascular complex) in 57.7% of eyes. Similarly, OCTA revealed capillary, mature, and mixed CNV in 28.9%, 15.9%, and 55.5% of eyes, respectively.ConclusionsOCTA provides the clinician the ability to perform precise structural and vascular assessment of CNV noninvasively. Our study is, to our knowledge, the largest OCTA analysis to date of CNV secondary to neovascular AMD analyzed simultaneously by ICGA and OCTA.

Veronese, Chiara MD; Maiolo, Chiara MD; Mora, Livia D. MD; Morara, Mariachiara MD; Armstrong, Grayson W. MD, MPH; Ciardella, Antonio P. MDEditor(s): Toth, Cynthia A.; Thomas, Akshay S.; Zhang, Wenlan Author Information

Purpose Kyrieleis retinal periarteritis reflects the severe intraocular inflammation experienced by the eye. Its aetiology has not been well established, since only nine cases have been reported and there is no pathological study available in the literature. We determine the pathogenesis of Kyrieleis periarteritis based on interpretation of multimodal imaging findings.Methods Charts of patients with Kyrieleis arteritis seen between 2006 and 2014 were retrieved from eight uveitis referral centres throughout the world. Follow-up ranged from 5 to 12months.Results Twenty-five eyes with Kyrieleis arteritis from 25 patients were included in the study. Nineteen patients (72%) were male and six (28%) were female. Twenty-three patients were diagnosed with toxoplasmosis retinochoroiditis and two patients had cytomegalovirus retinitis. Fluorescein angiography, fundus autofluorescence and indocyanine green angiography were performed on 25/25 (100%) eyes. In eight eyes (32%), baseline spectral domain optical coherence tomography (SD-OCT) scans were performed along the segmental Kyrieleis arteritis. Fluorescein angiography showed early hypofluorescence and intermediate hyperfluorescence associated with the areas of focal arteritis, whereas indocyanine green angiography of these accumulations showed early hypofluorescence and late hyperfluorescence. Fundus autofluorescence revealed an increased autofluorescence of the vessels corresponding to the Kyrieleis plaques, while SD-OCT scans along the segmental Kyrieleis arteritis showed hyperreflectivity of the vessel wall.Conclusions These imaging modalities provide in vivo, quasi-histologic images demonstrating that Kyrieleis plaques are characterised by an inflammatory involvement within the vessels' endothelium.

Purpose The purpose of our study was to describe the feature of acute non-arteritic or arteritic anterior ischemic optic neuropathy (NA-AION and A-AION) using optical coherence tomography angiography (OCT-A) and to compare it with fluorescein angiography (FA) and indocyanine green angiography (ICGA). Methods In this retrospective, observational case-control study four NA-AION patients and one A-AION patient were examined by FA, ICGA and OCT-A within 2 weeks from disease presentation. The characteristics of the images were analyzed. Optic nerve head (ONH) and radial peripapillary capillaries (RPC) vessel densities (VDs) were compared between NA-AION and controls. Results In two of four NA-AION cases and in the A-AION patient, OCT-A clearly identified the boundary of the ischemic area at the level of the optic nerve head, which was comparable to optic disc filling defects detected by FA. In the other two NA-AION cases, a generalized leakage from the disc was visible with FA, yet OCT-A still demonstrated sectorial peripapillary capillary network reduction. Both ONH and RPC VDs were reduced in NA-AION patients, when compared to controls. Conclusions OCT-A was able to identify microvascular defects and VD reduction in cases of acute optic disc edema due to NA-AION and A-AION. OCT-A provides additional information in ischemic conditions of the optic nerve head.

To describe the OCT angiography characteristics of flat irregular pigment epithelial detachments (PEDs), an overlapping tomographic feature of various macular disorders.

PurposeTo present a single case of bilateral multiple evanescent white dot syndrome (MEWDS).MethodsA single case with three months of follow-up using imaging studies including fundus color photography (FP), fluorescein angiography (FA), indocyanine green angiography (ICGA), fundus autofluorescence (FAF), spectral-domain optical coherence tomography (SD-OCT), en face SD-OCT and optical coherence tomography angiography (OCTA) is presented.ResultsThe patient presented with bilateral MEWDS, ultimately with complete resolution of symptoms. FP revealed foveal granularity and white punctate deep retinal spots, FA found early wreath-like hyperfluorescence, while ICGA showed hypofluorescent dots and spots in the early and late stages. FAF showed areas of hyperautofluorescence. SD-OCT revealed disruption of the ellipsoid zone (EZ) and accumulation of hyperreflective material of variable size and shape. En face SD-OCT demonstrated hyporeflective areas corresponding to areas of EZ disruption as well as hyperreflective dots in the outer nuclear layer. OCTA showed areas of photoreceptor slab black-out corresponding to areas of EZ disruption and light areas of flow void or flow disturbance in the choriocapillaris slab.ConclusionsThis case represents an unusual case of bilateral MEWDS with complete resolution within three months.

Veronese, Chiara MD; Maiolo, Chiara MD; Morara, Mariachiara MD; Armstrong, Grayson W. MD; Ciardella, Antonio P. MD Author Information

PURPOSE:To report two cases of primary vitreoretinal lymphoma (PVRL), which presented as intermediate and posterior uveitis.METHODS:Combined clinical assessment, multimodal imaging with spectral-domain optical coherence tomography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, brain magnetic resonance imaging and vitreous and retinal biopsy. Case 1 was a 48-year-old woman who complained of visual loss in her right eye secondary to a diffuse vitreous opacification and multiple chorioretinal lesions. Case 2, a 74-year-old man, presented with low vision in his right eye due to a wide chorioretinal lesion at the posterior pole, vitreous opacification and posterior uveitis in both eyes.RESULTS:Diffuse large B cell lymphoma was histologically diagnosed in the cerebellum in the first case and in chorioretinal tissue in the second patient. Atypical lymphoid cells were detected and allowed to make a diagnosis of primary central nervous system lymphoma in case 1 and PVRL in case 2.CONCLUSION:PVRL often masquerades ad intermediate or posterior uveitis. The management of the patients needed a team of pathologists, haematologists and ophthalmologists to achieve the correct diagnosis and choose the more appropriate therapy. Some peculiar characteristics on multimodal imaging, even in atypical cases of PVRL, should raise suspicious for PVRL and lead to a diagnostic vitrectomy and/or retinal biopsy.

Purpose: To report two cases of pediatric choroidal neovascularization (CNV) and the associated neovascular and retinal findings identified on Optical Coherence Tomography Angiography (OCTA) imaging. Methods: A 14-year-old boy with handheld laser-induced maculopathy-related CNV and a 13-year-old boy with idiopathic CNV were evaluated with visual acuity testing, slit-lamp exam, fundus photography, fluorescein angiography, indocyanine green angiography, spectral domain optical coherence tomography, and OCTA. Results: Macular CNV were identified in both pediatric patients using OCTA imaging. The first case demonstrated a classic pediatric type II CNV with a “tree-like” pattern and a single vessel in-growth site, while the second case demonstrated a type I CNV with a “glomerular” pattern. Conclusion: Distinct choroidal neovascular patterns were visualized in these two cases of pediatric CNV when compared to adult subtypes. OCTA is a noninvasive imaging modality capable of evaluating and characterizing pediatric CNV and their associated vascular patterns.

To propose a classification of retinal astrocytic hamartoma based on spectral domain optical coherence tomography and correlate each class with systemic manifestations of tuberous sclerosis complex.Retrospective chart review conducted at four international referral medical retina centers. There were 43 consecutive patients with an established diagnosis of tuberous sclerosis complex based on presence of at least 2 major or 1 major and 2 minor features of the diagnostic criteria. Clinical and spectral domain optical coherence tomography features regarding retinal astrocytic hamartoma were documented.The mean patient age at presentation was 16.2 years. The retinal astrocytic hamartoma was classified as Type I (n = 41), Type II (n = 25), Type III (n = 20), or Type IV (n = 12). Patients with Type II showed greater number of cutaneous fibrous plaques (odds ratio = 64.8; 92% confidence interval: 64.2-65; P < 0.001); those with Type III displayed higher incidence of subependymal giant-cell astrocytomas (odds ratio = 43.2; 95% confidence interval: 43.0-43.3; P < 0.001); and those with Type IV showed higher incidence of pulmonary lymphangiomyomatosis (odds ratio = 126; 95% confidence interval: 122-128; P < 0.001).Retinal astrocytic hamartoma can be classified into four morphologic groups, based on spectral domain optical coherence tomography. There are important systemic tuberous sclerosis complex correlations with each class.

Purpose Cross-characterization of retinal astrocytomas (RA) using ocular diagnostic imaging techniques. Methods Fundus photography, fluorescein angiography, optical coherence tomography, fundus autofluorescence images, and ultrasonography were used to define characteristics of RA. Results Thirteen patients had sporadic RA and 12 had tuberous sclerosis. Of the latter, 1 patient had multiple (n = 14), bilateral tumors and was excluded so as not to skew the analysis. Of those patients with a solitary RA, 63% were juxtapapillary. All were white to yellow. A mulberry-shaped surface was observed in 33%. Fluorescein angiography revealed intrinsic tumor vessels and late diffuse tumor staining in 42%. Optical coherence tomography revealed cells spreading from the tumor surface into the vitreous (75%), intraretinal fluid (25%), and multiple, ovoid hypolucencies within the tumor stroma (54%). Fundus autofluorescent imaging revealed that hypolucencies were hyperautofluorescent and solid tumor stroma appeared hypoautofluorescent. B-scan imaging revealed moderate to high internal tumor reflectivity (100%) with orbital shadowing (46%). Optical coherence tomography revealed a mean tumor thickness of 0.8 mm. Ultrasound imaging showed a mean thickness of 1.4 mm (range 1.0–1.7). Of the 21 patients subsequently followed in our centers, no tumor growth was noted (mean 54 months, range 1–96). Conclusions This study reveals unique characteristics of RA and suggests that RA were most commonly amelanotic, moderate to high echoic, ≤1.7 mm in thickness, near the optic disc, and nonprogressive. When observed, a mulberry-shaped surface was pathognomonic.