Effectiveness and safety of XEN45 implant over 12 months of follow-up: data from the XEN-Glaucoma Treatment Registry.

OBJECTIVES: To evaluate the 1-year effectiveness and safety of the XEN45, either alone or in combination with phacoemulsification, in glaucoma patients. METHODS: This multicentre, prospective, observational study included consecutive eyes of glaucoma patients from the Italian XEN-Glaucoma Treatment Registry (XEN-GTR) who underwent XEN45 alone or in combination with phacoemulsification, with at least 1 year of follow-up. Surgical success was defined as intraocular pressure (IOP) < 18 mmHg and ≥20% reduction from preoperative IOP, over 1 year of follow-up. RESULTS: Two hundred thirty-nine eyes (239 patients) were analyzed, 144 (60.2%) eyes in the XEN-solo and 95 (39.8%) eyes in the XEN+Phaco groups. One hundred-sixty-eight (70.3%) eyes achieved overall success, without statistically significant differences between study groups (p = 0.07). Preoperative IOP dropped from a median (IQR) of 23.0 (20.0-26.0) mmHg to 14.0 (12.0-16.0) mmHg at month 12 (p < 0.001), with overall 39.9 ± 18.3% IOP reduction. The mean number of preoperative ocular hypotensive medications (OHM) was significantly reduced from 2.7 ± 0.9 to 0.5 ± 0.9 at month 12 (p < 0.001). Preoperative IOP < 15 mmHg (HR: 6.63; 95%CI: 2.61-16.84, p < 0.001) and temporal position of the surgeon (HR: 4.25; 95%CI: 2.62-6.88, p < 0.001) were significantly associated with surgery failure. One hundred-forty-six (61.1%) eyes had no intraoperative complications, whereas 91 (38.1%) and 56 (23.4%) eyes experienced at least one complication, respectively early (< month 1) and late (≥ month 1), all self-limiting or successfully treated without sequelae. Needling occurred in 55 (23.0%) eyes at least once during follow-up. CONCLUSION: Over 1-year follow-up, XEN45 alone or in combination with phacoemulsification, had comparable success rates and effectively and safely lowered IOP and the need for OHM.

The "Topography" of Glaucomatous Defect Using OCT and Visual Field Examination.

Aim: To describe the modifications in the superior and inferior retinal nerve fiber layer (RNFL) thickness regarding the distribution of the VF defects for the horizontal meridians in glaucomatous patients and the differences in the RNFL thickness topography between glaucomatous and healthy subjects. Methods: One hundred twenty eyes of 91 patients affected by glaucoma and 94 eyes of 51 normal patients were retrospectively reviewed. Computerized 30°VF (Octopus G1 Dynamic strategy) and optical coherence tomography (OCT) ONH and 3D disk analysis were performed in all cases. The RNFL thickness measures analyzed in both groups were superior-nasal (SN), superior-temporal (ST), inferior-nasal (IN), and inferior temporal (IT) sectors. The VFs were classified according to the distribution of the VF defect as for the horizontal meridian in the pattern deviation plot as superior, inferior, predominantly superior, or predominantly inferior. Result: In the glaucomatous group, 78 eyes (65%) showed a predominantly superior VF defect, while 38 eyes (32%) showed a predominantly inferior VF defect. Fifty-six eyes (46.7%) presented an exclusively superior, and 27/120 eyes (22.5%) presented an exclusively inferior VF defect. In the control group, the thickest RNFL sector was IT. The ST sector showed the thickest RNFL in presence of an exclusive superior VF defect. In case of an exclusive inferior VF defect, the thickest RNFL was the IT sector. VF showing superior defect presented a more altered MD than the VF with an inferior defect. Conclusion: Glaucomatous damage affects both the superior and inferior neural rim almost simultaneously. However, the neural rim loss seems to be asymmetric, involving the inferior or superior rim depending on the predominant involvement of the superior or inferior hemifield at the VF test. Particularly, the IT sector appears to be the most compromised in glaucomatous eyes. Therefore, the asymmetry between superior and inferior RNFL could support the diagnosis of glaucoma. How to cite this article: de Paula A, Perdicchi A, Pocobelli A, et al. The "Topography" of Glaucomatous Defect Using OCT and Visual Field Examination. J Curr Glaucoma Pract 2022;16(1):31-35.

Italian Candidates for the XEN Implant: An Overview from the Glaucoma Treatment Registry (XEN-GTR).

Background The Italian XEN Glaucoma Treatment Registry (XEN-GTR) was created to acquire a comprehensive prospective dataset that includes the patient characteristics, intraoperative variables, and postoperative management of glaucoma patients undergoing the XEN gel stent implantation. Methods This was a prospective observational, longitudinal clinical study involving 10 centres throughout Italy. The baseline examination included a comprehensive evaluation of demographic parameters (age, sex, ethnicity, and systemic condition), specific ophthalmological parameters, and quality of life questionnaire score collection. Results The baseline data of 273 patients were analysed. The median (IQR) age was 72 (65.0 to 78.0) years. Of the 273 patients, 123 (45%) were female and 150 (55%) were male. A total of 86% of the patients had open-angle glaucoma with a mean intraocular pressure of 24 ± 6 (range 12.0-60.0) mmHg. The mean number of medications was 2.7 ± 0.9 at baseline for the patients with a prevalence of prostaglandin analogues combined with a beta-blocker and anhydrase carbonic inhibitor (31.8%). The mean scores of the NEI-VFQ 25 and GSS questionnaires were 78 ± 18 (range 26.5-100) and 85 ± 14 (range 79-93), respectively. Combined XEN/cataract surgeries were scheduled in 73.7% of the patients. The preferred place for the XEN implant was the supero-nasal quadrant (91.6%). Conclusions Observing the baseline characteristics of the typical Italian candidates for the XEN gel implant shows that they are patients affected by POAG and cataracts, with moderate to severe glaucoma damage, all of which has an impact on their quality of life.

Understanding the role of microperimetry in glaucoma.

PURPOSE: The present narrative review attempts to provide an overview on the use of microperimetry or fundus-driven perimetry in glaucoma, considering the clinical use, the different strategies and limits compared to standard automated perimetry. METHODS: An electronic database (PubMed and Medline) search was performed of articles of any type published in the English language between 1998 and 2020 with a combination of the following terms: microperimetry, glaucoma, primary open-angle chronic glaucoma, visual field, Humphrey visual field, fundus automated perimetry. RESULTS: All the original articles, case reports, and short series analyzed were included in the present review, offering an excursus on the strengths and limitations characterizing the use of microperimetry in glaucomatous patients. The characteristics of a recently introduced fundus-driven perimetry Compass (CMP; Centervue, Padua, Italy) were also included. CONCLUSION: Although there remain several contradictions regarding routine use of microperimetry and the restricted research on this topic limits our ability to draw firm conclusions, microperimetry may be preferable in cases of localized retinal nerve fiber layer defects in patients with primary open-angle glaucoma and normal visual field. However, standard automated perimetry remains the gold standard for monitoring glaucoma, especially in patients with diffuse retinal nerve fiber layer impairment and visual field defects. The newly introduced Compass device can potentially provide a more accurate structural-functional evaluation than standard automated perimetry and can therefore produce superior testing reliability.

Do Additional Testing Locations Improve the Detection of Macular Perimetric Defects in Glaucoma?

PURPOSE: To evaluate the ability of additional central testing locations to improve detection of macular visual field (VF) defects in glaucoma. DESIGN: Prospective cross-sectional study. PARTICIPANTS: Four hundred forty healthy people and 499 patients with glaucomatous optic neuropathy (GON) were tested with a fundus tracked perimeter (CMP; CenterVue) using a 24-2 grid with 12 additional macular locations (24-2+). METHODS: Glaucomatous optic neuropathy was identified based on expert evaluation of optic nerve head photographs and OCT scans, independently of the VF. We defined macular defects as locations with measurements outside the 5% and 2% normative limits on total deviation (TD) and pattern deviation (PD) maps within the VF central 10°. Classification was based on the total number of affected macular locations (overall detection) or the largest number of affected macular locations connected in a contiguous cluster (cluster detection). Criteria based on the number of locations and cluster size were used to obtain equivalent specificity between the 24-2 grid and the 24-2+ grids, calculated using false detections in the healthy cohort. Partial areas under the receiver operating characteristic curve (pAUCs) were also compared at specificities of 95% or more. MAIN OUTCOME MEASURES: Matched specificity comparison of the ability to detect glaucomatous macular defects between the 24-2 and 24-2+ grids. RESULTS: At matched specificity, cluster detection identified more macular defects with the 24-2+ grid compared with the 24-2 grid. For example, the mean increase in percentage of detection was 8% (95% confidence interval [CI], 5%-11%) and 10% (95% CI, 7%-13%) for 5% TD and PD maps, respectively, and 5% (95% CI, 2%-7%) and 6% (95% CI, 4%-8%) for the 2% TD and PD maps, respectively. Good agreement was found between the 2 grids. The improvement measured by pAUCs was also significant but generally small. The percentage of eyes with macular defects ranged from about 30% to 50%. Test time for the 24-2+ grid was longer (21% increase) for both cohorts. Between 74% and 98% of defects missed by the 24-2 grid had at least 1 location with sensitivity of < 20 dB. CONCLUSIONS: Visual field examinations with additional macular locations can improve the detection of macular defects in GON modestly without loss of specificity when appropriate criteria are selected.

Volume Rendering of Angiographic Optical Coherence Tomography Angiography in Fovea Plana and Normal Foveal Pit.

This paper aims to study adaptative vascular arrangements in idiopathic fovea plana with volume-rendered optical coherence tomography angiography (OCTA). A retrospective review of two cases of idiopathic fovea plana (mean age: 26.5 years) and two age-matched controls imaged with OCTA was conducted using spectral-domain OCTA (RTVue XR Avanti, Optovue, Inc., Fremont, CA) equipped with the AngioVue software. Both en face OCTA slabs and OCTA b scans were processed through Fiji software (http://fiji.sc; software version 2.0.0-rc-68/1.52e), and then extracted as image sequences for volume rendering reconstructions using the ImageVis3D volume rendering system (3.1.0 release). Eyes with idiopathic fovea plana demonstrated a regular superficial vascular plexus connecting to a single vascular monolayer representing the deeper vascular plexuses. At this location, several vertical short path connections were demonstrated, in contraposition with normal eyes where short path connections were infrequently observed. Advances in three-dimensional OCTA reconstruction increase the understanding of vascular connections and arrangement in retinal plexuses and possible anatomical variations that cannot be detected with conventional two-dimensional b scans.

Visual Field Endpoints Based on Subgroups of Points May Be Useful in Glaucoma Clinical Trials: A Study With the Humphrey Field Analyzer and Compass Perimeter.

PRECIS: Visual field (VF) endpoints based on average deviation of specific subsets of points rather than all points may offer a more homogeneous data set without necessarily worsening test-retest variability and so may be useful in clinical trials. PURPOSE: The purpose of this study was to characterize the outcome measures encompassing particular subsets of VF points and compare them as obtained with Humphrey [Humphrey visual field analyser (HVF)] and Compass perimeters. METHODS: Thirty patients with imaging-based glaucomatous neuropathy performed a pair of 24-2 tests with each of 2 perimeters. Nonweighted mean deviation (MD) was calculated for the whole field and separate vertical hemifields, and again after censoring of points with low sensitivity (MDc) and subsequently including only "abnormal" points with a total deviation probability of <5% (MDc5%) or <2% (MDc2%). Test-retest variability was assessed using Bland-Altman 95% limits of agreement (95%LoA). RESULTS: For the whole field, using HVF, MD was -7.5±6.9 dB, MDc -3.6±2.8 dB, MDc5% -6.4±1.7 dB, and MDc2% -7.3±1.5 dB. With Compass the MD was -7.5±6.6, MDc -2.9±1.7 dB, MDc5% -6.3±1.5, and MDC2% -7.9±1.6. The respective 95%LoA were 5.5, 5.3, 4.6, and 5.6 with HVF, and 4.8, 3.7, 7.1, and 7.1 with Compass. The respective number of eligible points were 52, 42±12, 20±11, and 15±9 with HVF, and 52, 41.2±12.6, 10±7, and 7±5 with Compass. With both machines, SD and 95%LoA increased in hemifields compared with the total field, but this increase was mitigated after censoring. CONCLUSION: Restricting analysis to particular subsets of points of interest in the VF after censoring points with low sensitivity, as compared with using the familiar total field MD, can provide outcome measures with a broader range of MD, a markedly reduced SD and therefore more homogeneous data set, without necessarily worsening test-retest variability.

Effect of intraocular pressure lowering on the capillary density of optic nerve head and retinal nerve fiber layer in patients with glaucoma.

PURPOSE: To evaluate the effect of IOP lowering on the capillary density of optic nerve head and retinal nerve fiber layer in patients with primary open angle glaucoma. METHODS: Twenty eyes of 14 glaucomatous patients and 15 eyes of nine normal patients were enrolled. The most appropriate hypotonic treatment was applied to every patient. A HD Angio Disc 4.5 scan (Avanti-AngioVue) was performed at baseline and after a month in the glaucomatous eyes. The following parameters were analyzed: Radial Papillary Capillaries (RPC) density, inside disc, peripapillary, superior-hemi, inferior-hemi, quadrants, and peripapillary, hemi-superior, hemi-inferior, and quadrants RNFL thickness. Optic nerve head analysis was also evaluated. In addition, the RPC density and the RNFL were assessed in the eight sectors provided by the software. RESULTS: The RPC density did not significantly change after IOP reduction (p > 0.05). The inferior-temporal (p = 0.005) and inferior-nasal sectors (p < 0.001) showed a greater capillary density than the respective superior sectors in healthy eyes. In contrast in the glaucomatous eyes, the superior-nasal exhibited greater capillary density with respect to the inferior-nasal sectors. The aggregate RPC density of the inferior sectors was greater than the superior ones in the control group (p < 0.001). An improvement of the average disc area (p = 0.01) and the average cup volume (p = 0.059) were also observed along with increased RNFL thickness at different locations (all, p < 0.05) after IOP lowering therapy was initiated. CONCLUSION: The glaucomatous eyes presented rarefaction of the radial papillary capillaries density in the inferior sectors, but no significant changes in the density after IOP-lowering medications.

An Iconic Case of Pigmentary Glaucoma: Brief Review of the Literature.

Pigment dispersion syndrome and pigmentary glaucoma are two conditions characterized by pigment dispersion originating from the posterior part of the iris and its accumulation on the trabecular meshwork, corneal endothelium, and anterior surface of the lens. The pigment on the trabecular meshwork can cause chronic inflammation with a secondary reduction of its function and an increase in intraocular pressure. The case presented represents a typical example of pigmentary glaucoma in a myopic patient in which all the signs, symptoms, and complications typical of these pathologies were present. We report and describe an 8-year-long follow-up period with clinical and instrumental examinations.

Effect of fundus tracking on structure-function relationship in glaucoma.

AIMS: To investigate the effect of fundus tracking perimetry on structure-function relationship in glaucoma. METHODS: Perimetric data were acquired with the Humphrey Field Analyzer (HFA) and the Compass fundus perimeter (CMP, equipped with fundus tracking). We included data from 696 eyes from 360 healthy people and 711 eyes from 434 patients with glaucoma from the original study, for which the circumpapillary retinal nerve fibre layer optical coherence tomography scan (Cp-RNFL) was available. We explored the structure-function relationship using both global indices (mean deviation and average Cp-RNFL thickness loss) and anatomically defined visual field clusters comparing the R2 values from mixed-effect models. We then measured the diagnostic ability of a combined Structure Function Index (SFI) using perimetric data from either perimeter. The comparisons were based on partial receiver operating characteristic curves with a minimum specificity of 75% and their areas under the curve. RESULTS: The R2 for the global structure-function relationship was 0.50 for the CMP and 0.48 for the HFA. When visual field clusters were included in the model, the R2 was 0.29 for CMP and 0.30 for HFA. Overall, the discrimination ability of the SFI was not significantly higher than the Cp-RNFL for either the CMP (p=0.07) or the HFA (p=0.14). However, it was significantly better in eyes with perimetric damage (p<0.001), in which the CMP-SFI performed significantly better than the HFA-SFI (p=0.03). CONCLUSIONS: Structure-function relationship is similar between the two perimeters. Fundus tracking might improve discrimination ability with a combined SFI. TRIAL REGISTRATION NUMBER: ISRCTN13800424.

Ganglion Cell Complex Analysis in Glaucoma Patients: What Can It Tell Us?

Glaucoma is a group of optic neuropathies characterized by a progressive degeneration of retina ganglion cells (RGCs) and their axons that precedes functional changes detected on the visual field. The macular ganglion cell complex (GCC), available in commercial Fourier-domain optical coherence tomography, allows the quantification of the innermost retinal layers that are potentially involved in the glaucomatous damage, including the retinal nerve fiber (RNFL), ganglion cell and inner plexiform layers. The average GCC thickness and its related parameters represent a reliable biomarker in detecting preperimetric glaucomatous damage. The most accurate GCC parameters are represented by average and inferior GCC thicknesses, and they can be associated with progressive visual field loss. Although the diagnostic accuracy increases with more severe glaucomatous damage and higher signal strength values, it is not affected by increasing axial length, resulting in a more accurate discrimination of glaucomatous damage in myopic eyes with respect to the traditional RNFL thickness. The analysis of the structure-function relationship revealed a good agreement between the loss in retinal sensitivity and GCC thickness. The use of a 10-2° visual field grid, adjusted for the anatomical RGCs displacement, describes more accurately the relationship between RGCs thickness and visual field sensitivity loss.

Cluster analysis of computerized visual field and optical coherence tomography-ganglion cell complex defects in high intraocular pressure patients or early stage glaucoma.

PURPOSE: The aim of the study is to evaluate the relationship between functional defects shown by cluster analysis of computerized visual field and anatomic defects from optical coherence tomography-ganglion cell complex examination in ocular hypertension or eyes affected by glaucoma. METHODS: 205 eyes affected by ocular hypertension (intraocular pressure > 22 mmHg) or early stage glaucoma were enrolled. The age of the patients ranged from 26 to 87 years (average: 61.83 ± 1.54 years). Computerized 30° visual field (Octopus G1x Dynamic strategy) and optical coherence tomography-ganglion cell complex (I-Vue Optovue) analyses were performed for each eye selected; 68 eyes were tested and retested from two to seven times for a total of 320 visual fields and 320 optical coherence tomography-ganglion cell complex examinations. The visual field was considered abnormal with a mean defect < -2 and loss variance > 6. The optical coherence tomography-ganglion cell complex was considered abnormal with a significant focal loss volume (p < 5%) and/or a significant thinning of total, superior, or inferior thickness (p < 5%). Four different groups of examinations were created according to the results of visual field and ganglion cell complex: normal visual field and normal ganglion cell complex (group 1), abnormal visual field and abnormal ganglion cell complex (group 2), normal visual field and abnormal ganglion cell complex (group 3), and abnormal visual field and normal ganglion cell complex (group 4). The cluster analysis of visual fields (EyeSuite software Interzeag CH) was performed only in the visual field of group 3, and the correlation between cluster values and topographical changes at optical coherence tomography-ganglion cell complex was analyzed. RESULTS: The results of the ganglion cell complex and visual field examinations matched 247 (77.19%) times. In 143 cases, the examinations belonged to group 1, in 104 to group 2, in 23 to group 3, and, finally, in 50 to group 4. The visual field cluster analysis performed on group 3 showed that the correlation between optical coherence tomography-ganglion cell complex and visual field cluster analysis defects was 100% (both the exams altered). In 72% of them, there was also a topographical correspondence between the visual field and optical coherence tomography-ganglion cell complex defects. CONCLUSION: In the early stages of glaucoma, the visual field cluster analysis seems to be useful to detect some focal defects that can be otherwise underestimated when globally considering the visual field. In group 3, where the conventional analysis of visual field was normal while the optical coherence tomography-ganglion cell complex exam was abnormal, the visual field cluster analysis showed a topographical correlation with optical coherence tomography-ganglion cell complex defects in more than 70% of the examinations performed. In addition, the patients with abnormal visual field and normal optical coherence tomography-ganglion cell complex were older than those with normal visual field and abnormal optical coherence tomography-ganglion cell complex (66.44 ± 3.51 vs 57.04 ± 5.96 years, p < 0.001 (0.0002)). These results confirm that the reliability of a visual field examination is subjective and decreases with age because of its difficulty and the personal compliance of the patient toward this examination.

Postural changes revealing orbital venous malformation using ultrasound in blue rubber bleb nevus syndrome.

A 62-year-old white woman presented with a diagnosis of blue rubber bleb nevus syndrome (BRBNS). The right eye appeared enophthalmic, yet the patient complained of episodes of right proptosis on bending forward. The remainder of the examination was unremarkable. Orbital ultrasound (US) in an upright posture revealed a single low reflectivity cavity (4.27 mm x 2.82 mm) of uncertain interpretation. In a forward-leaning posture the lesion increased in size (maximum thickness of 13.72 mm), demonstrating multiple low reflectivity spaces with highly reflective septae. This case first reports the use of US with postural changes to assess the presence of orbital venous malformation in BRBNS. The expansile nature upon postural changes supports the venous origin of the orbital lesion.

A Comparison between the Compass Fundus Perimeter and the Humphrey Field Analyzer.

PURPOSE: To evaluate relative diagnostic precision and test-retest variability of 2 devices, the Compass (CMP, CenterVue, Padova, Italy) fundus perimeter and the Humphrey Field Analyzer (HFA, Zeiss, Dublin, CA), in detecting glaucomatous optic neuropathy (GON). DESIGN: Multicenter, cross-sectional, case-control study. PARTICIPANTS: We sequentially enrolled 499 patients with glaucoma and 444 normal subjects to analyze relative precision. A separate group of 44 patients with glaucoma and 54 normal subjects was analyzed to assess test-retest variability. METHODS: One eye of recruited subjects was tested with the index tests: HFA (Swedish interactive thresholding algorithm [SITA] standard strategy) and CMP (Zippy Estimation by Sequential Testing [ZEST] strategy), 24-2 grid. The reference test for GON was specialist evaluation of fundus photographs or OCT, independent of the visual field (VF). For both devices, linear regression was used to calculate the sensitivity decrease with age in the normal group to compute pointwise total deviation (TD) values and mean deviation (MD). We derived 5% and 1% pointwise normative limits. The MD and the total number of TD values below 5% (TD 5%) or 1% (TD 1%) limits per field were used as classifiers. MAIN OUTCOME MEASURES: We used partial receiver operating characteristic (pROC) curves and partial area under the curve (pAUC) to compare the diagnostic precision of the devices. Pointwise mean absolute deviation and Bland-Altman plots for the mean sensitivity (MS) were computed to assess test-retest variability. RESULTS: Retinal sensitivity was generally lower with CMP, with an average mean difference of 1.85±0.06 decibels (dB) (mean ± standard error, P < 0.001) in healthy subjects and 1.46±0.05 dB (mean ± standard error, P < 0.001) in patients with glaucoma. Both devices showed similar discriminative power. The MD metric had marginally better discrimination with CMP (pAUC difference ± standard error, 0.019±0.009, P = 0.035). The 95% limits of agreement for the MS were reduced by 13% in CMP compared with HFA in participants with glaucoma and by 49% in normal participants. Mean absolute deviation was similar, with no significant differences. CONCLUSIONS: Relative diagnostic precision of the 2 devices is equivalent. Test-retest variability of MS for CMP was better than for HFA.

Retinal pigment epithelium-photoreceptor layer alterations in a patient with Sturge-Weber syndrome with diffuse choroidal hemangioma.

PURPOSE: To describe an 8-year-old girl with diagnosis of Sturge-Weber syndrome (SWS) presenting with glaucoma, abnormal iris vessels, diffuse choroidal hemangioma, and small white dot-shaped "microdrusen-like" alterations of the retina in the right eye. PATIENT AND METHODS: Complete ophthalmological examination was performed with slit-lamp anterior segment assessment and fundus ophthalmoscopy. Near infrared reflectance (NIR) and enhanced depth imaging (EDI) spectral domain optical coherence tomography (SD-OCT) were carried out. RESULTS: Microdrusen-like retinal alterations observed with fundus ophthalmoscopy appeared as multiple hyperreflective dots surrounded by a hyporeflective ring on NIR. EDI SD-OCT showed increased choroidal thickness exceeding 1000 µm. B-scan cross-sectional examination on the hyperreflective dots revealed focal alterations of the retinal pigment epithelial (RPE)-photoreceptor layer. CONCLUSIONS: The increase of the choroidal thickness due to the diffuse choroidal hemangioma caused alterations of the RPE-photoreceptor layer similar to reticular pseudodrusen or pachychoroid pigment epitheliopathy.

Evaluation of the progression of visual field damage in patients suffering from early manifest glaucoma.

BACKGROUND: This retrospective study aimed to determine how often a perimetric examination should be carried out in order to identify visual field (VF) changes in patients with relatively early manifestation glaucoma. MATERIALS AND METHODS: Patients included had a relatively recent manifestation of primary open-angle glaucoma. Patients with a minimum follow-up of 5 years and a minimum of seven VF tests were included. Statistical analysis was performed to verify the trend of variations in mean defect (MD) over time (PeriData). The results were subjected to a t-test for a comparative analysis of progression of VF changes over time. The annual rate of progression provided by PeriData considering all the VFs analyzed was compared with that obtained on half of the VF examinations during the same follow-up period. An analysis of the MD trend over time was also carried out in relationship to the number of VF tests done and by dividing the sample into a high-frequency group (more than eight VFs) and a low-frequency group (fewer than eight VFs) in the follow-up period. RESULTS: A total of 96 eyes of 96 patients were included, and overall 846 VFs were examined. The paired t-test performed comparing the MD index of all the VFs against half of them did not show statistical significance (P=0.537). The high-frequency group comprised 39 eyes (average VF 11.05±1.91, average time interval 0.76 years) while the low-frequency group comprised 57 eyes (average VF 6.95±0.6, average time interval 1.21 years). The analysis of the MD trend in the high-frequency patients showed significance (P=0.017); the low-frequency group did not show statistical significance (P=0.08). CONCLUSION: The number of VFs in a determined time interval was not significant. However, a greater frequency of tests provides a predictive evaluation of the rate of progression of early manifestation open-angle glaucoma.

Glaucoma Management in Carotid Cavernous Fistula.

Carotid cavernous fistulas (CCF) are vascular communications between the carotid artery and the cavernous sinus. Ophthalmologists are called to diagnose and manage the condition in cases that present with ocular features. A 73-year-old female was referred to our glaucoma center clinic. Eight years before, she had started receiving medication for glaucoma and had undergone laser iridotomy, but a satisfactory management of intraocular pressure (IOP) had not been achieved. The patient was complaining of intermittent diplopia, bilateral proptosis, and conjunctival chemosis over the past 6 months. Best-corrected visual acuity in the right (OD) and left eye (OS) was 9/10 and 10/10, respectively. Visual field testing showed slight paracentral field defects mostly in OS. IOP was 20 mm Hg in OD and 34 mm Hg in OS. We referred the patient to neuroradiology, and MRI angiography revealed a CCF with angiographic classification of Cognard grade 2. Closure of the CCF by transarterial embolization was performed in the neuroradiology department. One week following the procedure, the clinical signs of diplopia, proptosis, and conjunctival chemosis had greatly improved, and IOP was reduced to 12 mm Hg OD and 19 mm Hg in OS. Glaucoma treatment was maintained with topical brimatoprost, brinzolamide, and timolol. Owing to the risk of vision loss associated with vascular stasis, retinal ischemia, and high IOP, ophthalmologists must be aware of the clinical features of CCF and should request appropriate imaging studies such as MRI angiography in order to confirm the diagnosis and plan multidisciplinary treatment.

SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY EVIDENCE OF RETINAL NERVE FIBER LAYER AND GANGLION CELL LOSS IN ADULT PATIENTS WITH NEUROFIBROMATOSIS TYPE 1.

PURPOSE: To evaluate peripapillary retinal nerve fiber layer, macular retinal nerve fiber layer, and ganglion cell layer-inner plexiform layer thickness and analyze their correlations in adult patients with neurofibromatosis Type 1 (NF1) and disease-free controls. METHODS: This cross-sectional study was performed at the Azienda Policlinico Umberto I, University of Rome "La Sapienza." All participants underwent complete ophthalmologic examination. Spectral domain optical coherence tomography was used to evaluate peripapillary retinal nerve fiber layer and obtain retinal segmentation measurements to assess macular retinal nerve fiber layer and ganglion cell layer-inner plexiform layer at 1,000 µm nasal, temporal, superior, and inferior to the fovea. RESULTS: Thirty-four eyes of 17 patients with NF1 (mean age, 42.2 ± 14.3 years) and 34 eyes of 17 disease-free control subjects (mean age, 41.4 ± 12.2 years) were included. All participants had best-corrected visual acuity of 20/20. The mean thickness of peripapillary retinal nerve fiber layer, macular retinal nerve fiber layer, and ganglion cell layer-inner plexiform layer was lower in patients with NF1 with respect to controls (P = 0.003, P = 0.022, P < 0.001, respectively). Regression analysis showed a significant correlation (P < 0.001) between mean ganglion cell layer-inner plexiform layer thickness and mean peripapillary retinal nerve fiber layer thickness in patients with NF1. CONCLUSION: Retinal nerve fiber layer and ganglion cell loss correlate well with each other in adult patients with NF1 in comparison with a healthy control population.

Rare Diseases Leading to Childhood Glaucoma: Epidemiology, Pathophysiogenesis, and Management.

Noteworthy heterogeneity exists in the rare diseases associated with childhood glaucoma. Primary congenital glaucoma is mostly sporadic; however, 10% to 40% of cases are familial. CYP1B1 gene mutations seem to account for 87% of familial cases and 27% of sporadic cases. Childhood glaucoma is classified in primary and secondary congenital glaucoma, further divided as glaucoma arising in dysgenesis associated with neural crest anomalies, phakomatoses, metabolic disorders, mitotic diseases, congenital disorders, and acquired conditions. Neural crest alterations lead to the wide spectrum of iridocorneal trabeculodysgenesis. Systemic diseases associated with childhood glaucoma include the heterogenous group of phakomatoses where glaucoma is frequently encountered in the Sturge-Weber syndrome and its variants, in phakomatosis pigmentovascularis associated with oculodermal melanocytosis, and more rarely in neurofibromatosis type 1. Childhood glaucoma is also described in systemic disorders of mitotic and metabolic activity. Acquired secondary glaucoma has been associated with uveitis, trauma, drugs, and neoplastic diseases. A database research revealed reports of childhood glaucoma in rare diseases, which do not include glaucoma in their manifestation. These are otopalatodigital syndrome, complete androgen insensitivity, pseudotrisomy 13, Brachmann-de Lange syndrome, acrofrontofacionasal dysostosis, caudal regression syndrome, and Wolf-Hirschhorn syndrome.

Ganglion Cell Complex Evaluation in Exudative Age-Related Macular Degeneration after Repeated Intravitreal Injections of Ranibizumab.

Purpose. To detect the effects of intravitreal ranibizumab injections on GCC in patients with wet AMD. Methods. 32 wet AMD eyes were selected and submitted at three ranibizumab injections. RTVue-OCT GCC and MM5 protocol were performed before treatment and twenty days after each injection. Results. At baseline mean GCC thickness was 93.9 ± 18.5 µm. Twenty days after each intravitreal injection it was, respectively, 85.8 ± 10.1, 86.5 ± 9.3, and 91.1 ± 11.5 µm, without statistical significance. A significant improvement in visual acuity (P = 0.031) and a reduction of mean foveal (P = 0.001) and macular thickness (P = 0.001) were observed. Conclusion. The clinical results confirm therapeutic efficacy of intravitreal injections of ranibizumab in wet AMD. A contemporary not statistically significant reduction of GCC thickness suggests that the loading phase of ranibizumab does not have any toxic effects on ganglion cell complex.