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Purpose: To detect, describe, and classify the morphologic characteristics of astrocytic hamartomas in tuberous sclerosis complex, using both spectral-domain optical coherence tomography (OCT) and infrared images., Methods: Ten subjects (20 eyes) with tuberous sclerosis complex underwent a complete ophthalmologic examination and multimodality imaging with spectral-domain OCT and infrared images. The imaging protocol included a 30°scan angle of the posterior pole and of the four quadrants. Line scans, detail, raster, and posterior pole patterns were used. The identified astrocytic hamartomas were described and characterized qualitatively and quantitatively., Results: Forty-four hamartomas were detected in 8 patients. In five cases, lesions were bilateral. Thirty of these hamartomas had not been revealed by previous ophthalmoscopy. Through multimodality imaging, it was possible to define multiple lesions with characteristic optical reflective qualities. All the 44 hamartomas were measured and morphologically characterized in terms of the type of tumor, retinal and/or vitreous involvement, calcifications, and posterior optical shadowing., Conclusion: The combined imaging with spectral-domain OCT and infrared images improves the detection of hamartomas if compared with the spectral-domain OCT technique alone. Moreover, a new subtype of hamartoma is proposed to complete a previous classification based on OCT.

Glaucoma is a major global cause of blindness, but the molecular mechanisms responsible for the neurodegenerative damage are not clear. Undoubtedly, the high intraocular pressure (IOP) and the secondary ischemic and mechanical damage of the optic nerve have a crucial role in retinal ganglion cell (RGC) death. Several studies specifically analyzed the events that lead to nerve fiber layer thinning, showing the importance of both intra- and extracellular factors. In parallel, many neuroprotective substances have been tested for their efficacy and safety in hindering the negative effects that lead to RGC death. New formulations of these compounds, also suitable for chronic oral administration, are likely to be used in clinical practice in the future along with conventional therapies, in order to control the progression of the visual impairment due to primary open-angle glaucoma (POAG). This review illustrates some of these old and new promising agents for the adjuvant treatment of POAG, with particular emphasis on forskolin and melatonin.

We report on the short-term test-retest baseline variability in macular function tests in ZEASTRESS-Pilot participants ( n = 18), on their cross-sectional correlation with macular pigment optical density (MPOD), and on the effects of four months (FUV4) of 20 mg/day zeaxanthin (ZX), followed by a four-month washout (FUV8; n = 24, age 50-81 years old). Outcomes included: MPOD at 0.5 and 2.0 deg eccentricity (MPOD-0.5 and -2.0); contrast sensitivity (CS); pattern-reversal electroretinogram (PERG) amplitude; dark-adapted 650 nm foveal cone sensitivity (DA650-FCS); and 500 mn parafoveal rod sensitivity (DA500-PFRS). All measures of macular function showed close test-retest correlation (Pearson's r range: 0.744-0.946) and low coefficients of variation (CV range: 1.13%-4.00%). MPOD correlated in a complex fashion with macular function. Following supplementation, MPOD-0.5 and MPOD-2.0 increased at both FUV4 and FUV8 ( p ≤ 0.0001 for all measures). Continued, delayed MPOD increase and a small, but significant ( p = 0.012), CS increase was seen at FUV8 only in females. PERGs increased significantly at FUV4 ( p = 0.0006), followed by a partial decline at FUV8. In conclusion, following ZX supplementation, MPOD increased significantly. There was no effect on DA-650 FCS or DA-500 PFRS. Both CS and PERG amplitudes increased following supplementation, but the effect varied between males and females. Additional studies appear warranted to confirm and characterize further these inter-gender differences.

Purpose: To evaluate the effects of a food supplement containing forskolin, homotaurine, carnosine, folic acid, vitamins B1, B2, B6, and magnesium in patients with primary open angle glaucoma (POAG) already in treatment and compensated by intraocular pressure (IOP)-lowering drugs, during a period of 12 months., Methods: Twenty-two patients (44 eyes) with POAG, with their IOP compensated by topical drugs, were enrolled and randomly assigned to the food supplement or control treatment group. The additional food supplement treatment consisted of 2 tablets per day (1 in the morning, 1 in the evening) given for 1 year of a balanced association of homotaurine, Coleus forskohlii root extract, L-carnosine, folic acid, vitamins B1, B2, B6, and magnesium. Pattern Electroretinogram (PERG) amplitude, foveal sensitivity obtained with the visual field analyzer frequency doubling technology, and IOP were detected at enrollment (T0), 3 months (T1), 6 months (T2), 9 months (T3), and 12 months (T4)., Results: We observed in treated patients a significant further decrease of IOP and an improvement of PERG amplitude at 6, 9, and 12 months, and foveal sensitivity at 12 months. All values remained substantially stable in control patients., Conclusions: The results of the present pilot study indicate that the components of the food supplement reach the eye in a detectable manner, as evidenced by the effects on the IOP. Moreover, they suggest a short-term neuroactive effect, as indicated by the improvement of PERG amplitude and foveal sensitivity in treated, but not in control patients.

Purpose. To study the geographical distribution of corneal temperature (CT) and its influence on the intraocular pressure (IOP) of healthy human volunteers. Materials and Methods. Fifteen subjects (7 M, 8 F), 33.8 ± 17.4 years old, were enrolled in this pilot, cross-sectional study. Measurements of CT were taken after one hour with closed eyelids (CET) or closed eyelids with a cooling mask (cm-CET) and compared to baseline. Results. If compared to baseline, after CET, average CT significantly increased by 0.56°C in the RE and by 0.48°C in the LE (p < 0.001) and IOP concomitantly significantly increased by 1.13 mmHg and 1.46 mmHg, respectively, in each eye (p < 0.001). After cm-CET, average CT significantly decreased by 0.11°C and 0.20°C, respectively, in the RE and LE (RE p = 0.04; LE p = 0.024), followed by a significant IOP decrease of 2.19 mmHg and 1.54 mmHg, respectively, in each eye (RE p < 0.001; LE p = 0.0019). Conclusion. Significant variations of CT occurred after CET and cm-CET and were directly correlated with significant differences of IOP. It can be speculated that both oxidative stress and sympathetic nerve fiber stimulation by temperature oscillations may affect the regulation of AH vortex flow and turnover, thus influencing IOP values.

Purpose: To present a case of Weber-Christian disease with symptomatic ocular involvment. Weber-Christian disease is a relapsing febrile nodular nonsuppurative panniculitis. It is characterized by malaise and fever accompanied by subcutaneous inflammatory nodules on the trunk and extremities. It can affect several organs, but ocular signs have been infrequently described in literature., Methods: A 20-year-old woman with Weber-Christian disease presented with severe bilateral ocular inflammation. A complete ophthalmologic examination was performed., Results: Visual acuity was 20/100 in both eyes and slit-lamp examination showed bilateral iridocyclitis. Bilateral cortico-nuclear cataract did not allow funduscopy and she underwent cataract extraction. Retinal vasculitis was detected., Conclusions: Patients with Weber-Christian disease can develop severe ocular inflammation. A complete ophthalmolgic examination should be done in these patients, especially when ocular involvement is the main sign of the disease. A further understanding of the severity of ocular inflammation proved fundamental in the management of the disease.

Purpose: The aim of our study was to non-invasively investigate central nervous system axonal integrity in patients with tuberous sclerosis complex (TSC). Diffuse microstructural white matter abnormalities reflecting axonal disorganization, reduced/altered myelination, or gliosis have been described in individuals with TSC. Optical coherence tomography (OCT) is a fast, easy-to-perform, non-invasive, and cost-efficient method to assess retinal morphology in vivo and to measure the thickness of the retinal nerve fiber layer (RNFL)., Methods: In order to assess central nervous system axonal integrity, eight subjects with TSC have been investigated by OCT to evaluate RNFL and they have been compared with matched healthy controls., Results: When comparing mean overall RNFL thicknesses of the TSC group with those of the control group, the TSC group presented with significantly lower RNFL values, compared to the control group, in the temporal quadrant (62.5 ± 6.9 vs. 76.9 ± 5.4; t = 14.438; p < 0.0001)., Conclusions: Since a reduced RNFL thickness might be seen as an indicator of chronic axonal degeneration or lack of appropriate neuronal development, our results support the presence of axonal alterations in TSC and also that white matter disorganization could be much more diffuse than originally thought. Since axonal alterations directly derive from mammalian target of rapamycin (mTOR) overactivation, which occurs early during fetus development, the RNFL thinning we observed could represent one of the facets of such early neurodevelopmental abnormalities.

Purpose. To determine the agreement between Moorfields Regression Analysis (MRA), Glaucoma Probability Score (GPS) of Heidelberg retinal tomograph (HRT III), and peripapillary nerve fibers thickness by iVue Optical Coherence Tomography (OCT). Methods. 72 eyes with ocular hypertension or primary open angle glaucoma (POAG) were included in the study: 54 eyes had normal visual fields (VF) and 18 had VF damage. All subjects performed achromatic 30° VF by Octopus Program G1X dynamic strategy and were imaged with HRT III and iVue OCT. Sectorial and global MRA, GPS, and OCT parameters were used for the analysis. Kappa statistic was used to assess the agreement between methods. Results. A significant agreement between iVue OCT and GPS for the inferotemporal quadrant (κ: 0.555) was found in patients with abnormal VF. A good overall agreement between GPS and MRA was found in all the eyes tested (κ: 0.511). A good agreement between iVue OCT and MRA was shown in the superonasal (κ: 0.656) and nasal (κ: 0.627) quadrants followed by the superotemporal (κ: 0.602) and inferotemporal (κ: 0.586) sectors in all the studied eyes. Conclusion. The highest percentages of agreement were found per quadrant of the MRA and the iVue OCT confirming that in glaucoma damage starts from the temporal hemiretina.

Recent years have seen an increase in the use of retinal nerve fiber layer (RNFL) evaluation as an easy-to-use, reproducible, proxy-measure of brain structural abnormalities. Here, we evaluated RNFL thickness in a group of subjects with high functioning autism (HFA) or with Asperger Syndrome (AS) to its potential as a tool to study autism pathophysiology. All subjects underwent high-resolution spectral domain optical coherence tomography to evaluate RNFL thickness. HFA subjects presented with reduced global RNFL thickness compared both to AS subjects and controls. AS subjects showed a reduced nasal quadrant RNFL thickness compared to controls. Verbal-IQ/performance-IQ discrepancy correlated with RNFL thickness. Our data suggest that RNFL evaluation could help in the development of biological markers of autism pathophysiology.

Purpose: The purpose of this study is to report the first case, to our knowledge, of intracoital subfoveal hemorrhage occurring 1 hour after tadalafil premedication in a patient with previously undiagnosed retinal angiomatous proliferation and erectile dysfunction., Methods: This is an interventional case report. The patient underwent Snellen vision acuity measurement, anterior and posterior segment biomicroscopy, and indirect ophthalmoscopy. Optical coherence tomography, fluorescein angiography, and indocyanine green angiography were also performed as needed. Recombinant tissue plasminogen activator 100 μg/0.1 mL and 0.3 mL pure SF6 gas were injected in the vitreous on presentation. Four days later, the patient received 1.25 mg/0.1 mL of intravitreal bevacizumab., Results: One day after recombinant tissue plasminogen activator and gas injection, visual acuity rose to 20/60-2. A week later, visual acuity was unchanged, and the subretinal hemorrhage displaced from the foveola. Optical coherence tomography showed a marked regression of submacular thickening., Conclusion: Despite strict time correlation, the submacular hemorrhage cannot be unequivocally attributed to tadalafil assumption because many other risk factors are to be considered: the Valsalva maneuver above all. Nonetheless, a suspicion should be raised, at least as a cofactor, given the drug mechanism of action and the report of vascular abnormalities bleeding in the literature, including cerebral arterovenous malformation and epistaxis. Caution should be exercised in the prescription of phosphodiesterase type 5 inhibitors to patients with known vascular abnormalities including retinal and choroidal abnormalities: retinal angiomatous proliferation, classic and occult neovascularization of any origin, and also, possibly, hemangioma.

Background: Retinal detachment (RD) associated with aniridia due to globe rupture (GR) is an uncommon condition with a severe prognosis. Surgical technique must address anterior and posterior segment issues secondary to the altered compartmentalization and increased risk for corneal toxicity. The purpose of this paper is to report a series of GR patients undergoing combined pars plana vitrectomy (PPV) and artificial iris diaphragm (AID) implant for the repair of RD associated to aniridia., Methods: The authors retrospectively reviewed 12 consecutive patients operated on by a single surgeon. Surgery consisted of a standard three-port PPV with extensive bimanual dissection of vitreous base and ciliary body membrane and combined AID implant. Office visits included Snellen visual acuity (VA), intraocular pressure measurement, biomicroscopy and indirect ophthalmoscopy. AID prosthesis included aniridic IOLs, Heimann's PMMA and silicone diaphragm., Results: Mean age was 53 years and mean follow-up was 19 months. At the end of follow-up, seven patients gained more than two lines (58.3%), two lost their vision (16.6%) and three were unchanged (25%). Seven patients (58.3%) had a VA better than 20/400 and one (8%) 20/40 vision. Eight patients (66.6%) retained a clear cornea, two (16.6%) had minimal corneal oedema and two (16.6%) corneal decompensation. Implanted prosthesis included two silicone diaphragms, four PMMA diaphragms and six aniridic IOLs. After an average 1.6 operations, the retina was completely attached in six patients (50%), partially attached in four (33.3%) and detached in two (16.6%)., Conclusion: RD associated to GR carries a guarded prognosis both due to RD complexity and hypotony. The combined repair of RD and aniridia after GR offers the advantage of addressing all issues at one time allowing correct eye compartmentalization and better tamponade effect. Successful anatomical and functional results can be achieved although multiple surgeries are often needed.

Purpose: To calculate the reliability of an eye-dedicated triaging system named Rome Eye Scoring System for Urgency and Emergency (RESCUE)., Methods: There were four coding parameters: pain, redness, loss of vision, and risk of open globe. Each parameter is assigned a score, the sum of which allows color coding. There were 1000 consecutive patients divided into urgent (U) or non-urgent (NU) based upon diagnosis, need for treatment, hospitalization, and/or follow-up visit. Correlation between RESCUE triage scoring as assigned by the nurse on presentation and urgency as estimated retrospectively was calculated. Accuracy, sensitivity, and specificity have been calculated. False positives (FP) have been defined as patients assigned a RESCUE green or yellow code while retrospectively judged NU and false negatives (FN) have been defined as patients assigned a white code despite being considered U., Results: Of 1000 patients, 332 (33.2%) were classified as U and 668 (66.8%) NU. The difference in RESCUE scoring between U and NU patients was significant (p<0.001), as well as the correlation between RESCUE scoring and urgency status. Accuracy was 95% with 9.3% FP and 2.7% FN. Sensitivity was 90.7% and specificity 97.2%. Positive predictive value was 94.6%, and negative predictive value was 95.2%. All 32 hospitalized patients and 147/198 (74.2%) patients given a return appointment properly received a yellow or green code., Conclusions: RESCUE accuracy, sensitivity, and specificity yield encouraging results, confirming the system''s ability to properly spot the most urgent cases. The concept of urgency in ophthalmology can be difficult to establish; nonetheless, an eye-dedicated triage can help in properly prioritizing urgent patients.

Purpose: Ophthalmic emergency (OE) triage is essential for prompt recognition of urgent cases. To date, no formal eye-dedicated triaging system has been widely accepted. The purpose of the present study is to propose a fast, accurate, and reproducible coding scale called the Rome Eye System for Scoring Urgency and Emergency (Rescue)., Methods: Phase 1 of the study is a retrospective analysis of electronic medical records (EMR); phase 2 is a prospective consecutive series. Phase 1 included 160,936 patients. Phase 2 included 1000 consecutive patients referred to the emergency department (ED) of our institution. In phase 1, the authors retrospectively analyzed EMRs of patients presenting to the ED, listing signs and symptoms most frequently associated with hospitalization. Redness, pain, loss of vision, and the risk for an open eye were identified and assigned a score ranging from 0 to 12. Color coding was assigned based on increasing scoring: 0-3 white, 4-7 green, 8-12 yellow code. In phase 2, 1000 consecutive ED patients were enrolled and prospectively coded according to RESCUE. After diagnosis and proper treatment, EMRs were retrospectively reviewed by a masked physician and patients recoded (Retro coding) according to clinical course. Correlation between Rescue and Retro coding was calculated., Main Outcome Measures: Prospective and retrospective ED color coding correlation., Results: A total of 160,936 EMR were retrospectively analyzed; 2407 (1.4%) patients required hospitalization. Loss of vision (90%), redness (76%), and pain (47%) were the most frequent complaints. Rescue significantly correlated to Retro coding (p<0.01): 841/1000 patients coded exactly the same color, 45/1000 were overestimated by one color class, none by two, 107/1000 underestimated by one, and 6/1000 by two classes. The 32/1000 hospitalized patients in the prospective cohort had a Rescue score significantly higher than non-admitted patients (p<0.01) and color coding among admitted and dismissed patients was significantly different as well (p<0.01)., Conclusions: The Rescue system seems promising in terms of usefulness and ease of implementation. The high correlation between Rescue code assigned prospectively and the post-diagnosis coding, as well as the prompt discrimination of cases that eventually required hospitalization, may lead to a wider use of the Rescue system. Further testing on larger samples and different institutions is warranted.