Vitreo-macular interface disorders in retinitis pigmentosa.

PURPOSE: To investigate the prevalence and progression of vitreo-macular interface disorders (VMID) phenotypes and their natural history in retinitis pigmentosa (RP). METHODS: A total of 257 eyes of 145 RP patients with VMID were retrospectively evaluated. Patients were divided according to the VMID subtypes into epiretinal membranes (ERMs), vitreo-macular traction (VMT) group, and macular hole (MH). Serial eye-tracked spectral-domain optical coherence tomography (SD-OCT) and best-corrected visual acuity (BCVA) changes were analyzed for a mean follow-up of 36.95 months. The status of posterior vitreous cortex was also considered. A control group of 65 eyes belonging to 65 RP patients with no macular changes was also recruited. RESULTS: VMID and control groups had the same baseline BCVA (0.50 vs 0.44 LogMAR) and did not differ in terms of phakic status. Different VMID groups had similar BCVA at baseline (p = 0.98). ERM represented the most prevalent disorder (207/257 eyes, 80.5%), followed by 35/257 (13.6%) VMT, and 15/257 Lamellar MH (LMH) eyes (5.8%). There were no cases of full thickness MH. Throughout the 36.9 months of follow-up, BCVA decreased an average 0.09 LogMAR from 0.31 to 0.4 in VMID patients and 0.01 in controls. VMID subgroup analysis showed a significant BCVA decrease in ERM patients (- 20.29%, p < 0.001), while VMT and LMH did not change significantly. Foveal thickness also remained stable over time. Complete PVD was present in 11 eyes in ERM, VMT, and LMH. CONCLUSIONS: Our study confirms the high prevalence of VMID in RP patients; however, only ERMs determined a significant loss of vision over 24 months. The high prevalence of VMID in RP patients suggests that macular alteration other than edema represents part of disease spectrum.

Acute exudative polymorphous vitelliform maculopathy: To bolus or not to bolus?

Acute exudative polymorphous vitelliform maculopathy (AEPVM) is a rare bilateral maculopathy characterized by chronic and long-term course. We report a case of AEPVM with an unusual presentation and management in a middle-aged man. He presented with clinical features of bilateral AEPVM accompanied by multiple intraretinal cysts, with a sudden increase of intraretinal fluid and visual function deterioration over a span of few days. Therefore, we administered empirically an intravenous bolus injection of methylprednisolone. One week after, there was a full recovery of visual acuity and cystic intraretinal spaces completely disappeared.

Factors Influencing Fixation Stability Area: A Comparison of Two Methods of Recording.

SIGNIFICANCE: The authors analyze factors influencing bivariate contour ellipse area (BCEA) in healthy and pathologic eyes and how such factors may affect isolated (static BCEA) or microperimetric fixation (dynamic BCEA). They conclude that aging increases both dynamic BCEA and examination time, whereas static BCEA offers less variance and the maximum distinction with pathologic eyes. PURPOSE: The aim of this study was to assess factors influencing BCEA and the recording method that offer less variability and thus maximum distinction between healthy and pathologic eyes. METHODS: A total of 136 eyes were retrospectively reviewed, 85 eyes without ophthalmic disorders (logMAR acuity ≦0.0) and 51 eyes with late age-related macular degeneration (AMD) were enrolled. All patients underwent two consecutive examinations, a 30-second isolated fixation (static BCEA) and a microperimetric test with continuous fixation recording (dynamic BCEA). All the examinations were carried out using MP1 microperimeter (NAVIS software version 1.7.6; Nidek Technologies, Padova, Italy). RESULTS: Dynamic BCEA was significantly correlated with age (r = 0.38, P < .001), total (r = 0.32, P = .03), and tracking time (r = 0.33, P = .02) in controls, whereas no significant relationships were found in the AMD group. The greatest difference between static and dynamic BCEA was observed in 70- to 79-year decade in healthy subjects (P < .01). Logistic regression analysis showed that late AMD status was significantly predicted by ±2 SD and ±3 SD static BCEA (both P = .03). CONCLUSIONS: Dynamic BCEA is influenced by a certain degree of variability in advanced-age healthy subjects. In such cases, the use of ±2 SD and ±3 SD static BCEAs seems to offer a more accurate detection of fixation stability changes in the AMD group with respect to normal subjects.

PREDICTIVE FACTORS FOR DEVELOPMENT OF NEOVASCULAR AGE-RELATED MACULAR DEGENERATION: A Spectral-Domain Optical Coherence Tomography Study.

PURPOSE: To investigate the risk factors predictive for the development of neovascular age-related macular degeneration (NVAMD) by means of spectral-domain optical coherence tomography. METHODS: Retrospective study of 73 eyes graded Stage 2 and Stage 3 according to the AMD International Grading System with minimum follow-up of 24 months. Drusenoid pigment epithelial detachment, hyperreflective foci, external limiting membrane, inner ellipsoid band, and retinal pigment epithelium integrity were analyzed at baseline and last follow-up. Binary logistic regression model analyzed significant predictors of neovascular conversion. RESULTS: The discontinuity of external limiting membrane, inner ellipsoid band, and retinal pigment epithelium bands were significantly more prevalent in the NVAMD group at baseline and last follow-up (P < 0.001). Hyperreflective foci represented the single most important predictor of neovascular conversion (Exp [B], 15.15; P = 0.005) as confirmed by Kaplan-Meier curve (P = 0.002). Drusenoid pigment epithelial detachment width was significantly greater in NVAMD group than control subjects at baseline and last follow-up (P < 0.001), and its delta value also resulted a significant neovascular predictor (Exp [B], 0.99; P = 0.04). CONCLUSION: Hyperreflective foci significantly increase the risk of NVAMD progression. The delta width of drusenoid pigment epithelial detachment also predicts disease progression, integrating the stratification of NVAMD progression risk.

Microperimetric evaluation in patients with adult-onset foveomacular vitelliform dystrophy.

INTRODUCTION: To compare mean best-corrected visual acuity (BCVA), retinal sensitivity (RS), and bivariate contour ellipse area (BCEA) in patients with adult-onset foveomacular vitelliform dystrophy (AOFVD) and healthy subjects (HSs), reporting also functional disease-related changes in the different stages of the AOFVD disease. MATERIALS AND METHODS: In this observational cross-sectional study, a total of 19 patients (30 eyes; 12 female and 7 male) with AOFVD were enrolled, and 30 patients (30 eyes; 16 female and 14 male) were recruited as age-matched control group (74.36 ± 9.17 years vs. 71.83 ± 6.99 years respectively, P= 0.11). All patients underwent a complete ophthalmologic examination, fundus autofluorescence and fluorescein angiography, spectral-domain optical coherence tomography and microperimetry (MP)-1 analysis. The data collection included mean BCVA, mean RS measured by means of MP-1, BCEA, and central retinal thickness. RESULTS: All the functional parameters (BCVA, RS, and BCEA) were significantly worse in AOFVD group than HS. Subgroup analysis showed that the most significant functional changes, quantified by mean BCVA, RS, and BCEA, were in the atrophic stage (P = 0.03, P= 0.01, and P= 0.001, respectively). All the functional parameters were well correlated in the different stages. CONCLUSIONS: This study further confirms the good visual prognosis in the AOFVD eyes. Fixation stability measurement using BCEA demonstrates good evaluation of visual performance integrating traditional functional parameters. It may also serve for further rehabilitative purposes in atrophic eyes.

Correlation between retinal function and microstructural foveal changes in intermediate age related macular degeneration.

PURPOSE: To assess foveal microstructural changes influencing retinal sensitivity (RS) and fixation stability using microperimeter MP-1 in intermediate age-related macular degeneration (AMD). METHODS: In this cross-sectional study, 22 eyes of 22 patients (mean age: 75 ± 9.02 years) with intermediate AMD were enrolled. Retinal sensitivity and bivariate contour ellipse area (BCEA) were obtained by microperimetry MP-1 (Humphrey 10-2 68-loci grid) under mesopic conditions. Drusen type, drusenoid pigment epithelial detachment, hyperreflective foci (HF), integrity of external limiting membrane (ELM), inner ellipsoid zone (ISel), RPE/Bruch's membrane complex (RPE/B) and subfoveal choroidal thickness were analyzed in the foveal region and compared with RS and BCEA. Spearman's rank correlation coefficient was used to evaluate the relationship between variables. Logistic regression analysis was also used to assess morphological predictor influencing RS or BCEA. RESULTS: RS was strongly and inversely related with the presence of HF (r = -0.66, P = 0.001), integrity of ELM (r = -0.70, P < 0.001), ellipsoid zone (r = -0.45, P = 0.03). Instead, BCEA is positively related to the ellipsoid zone integrity (r = 0.45, P = 0.03). Logistic regression analysis confirmed that disruption of ISel influenced fixation stability (ExpB: 9.69, P = 0.04) but not RS. Instead, the presence of HF and disruption of ELM predicted RS reduction (ExpB: 0.55, P = 0.02 and ExpB: 0.29, P = 0.04, respectively). CONCLUSIONS: The integrity of ELM and the presence of HF are both predictors of RS. The ELM status may be considered a new biomarker of retinal function together with HF. Instead, the integrity of ISel band seems to be a more selective predictor of BCEA than RS.

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.

Enhanced depth imaging optical coherence tomography in adult-onset foveomacular vitelliform dystrophy.

PURPOSE: To compare mean choroidal thickness in patients with adult-onset foveomacular vitelliform dystrophy (AOFVD) and healthy subjects, to analyze patients with AOFVD in order to evaluate choroidal thickness disease-related changes, also in relation to the different stages of AOFVD disease and to the retinal pigment epithelium (RPE) features, and to correlate mean choroidal thickness with age in both groups. METHODS: In this prospective observational cross-sectional study, a total of 63 eyes of 51 consecutive subjects were examined, consisting of a control group (n = 28 eyes) and the AOFVD group (n = 35 eyes). A complete ophthalmologic examination, fundus autofluorescence, and spectral-domain optical coherence tomography were performed in all patients. RESULTS: Mean subfoveal choroidal thickness was 214.78 ± 62.35 µm in healthy subjects and 222.31 ± 73.29 µm in the AOFVD group (p = 0.33). In the vitelliruptive group, the mean choroidal thickness was significantly thicker than in the control group at each choroidal location. Mean choroidal thickness was significantly increased in the pseudohypopyon stage when compared to the vitelliform one (+66.34 µm, p = 0.02). Eyes with subretinal fluid (SRF) showed significantly thicker choroid when compared with those without SRF. No significant correlations were found between age and choroidal thickness in the study group. CONCLUSIONS: The study of the choroid in patients with AOFVD suggested a possible role in the pathologic changes during the different stages of disease, and could help us to evaluate progression of the disease. Greater choroidal thickness associated with SRF and RPE bumps are signs of RPE alterations and could be related to evolution of the AOFVD lesion to a different stage.

Psychosis, Mood and Behavioral Disorders in Usher Syndrome: Review of the Literature.

The aim of this review is to focus the current knowledge about mental and behavioral disorders in Usher syndrome. Previous studies described the presence of various mental disorders associated with Usher syndrome, suggesting possible mechanisms of association between these disorders. The most common manifestations are schizophrenia-like disorder and psychotic symptoms. Mood and behavioral disorders are rarely described, and often are associated with more complex cases in co-occurrence with other psychiatric disorders. Neuroimaging studies reported diffuse involvement of central nervous system (CNS) in Usher patients, suggesting a possible role of CNS damage in the pathogenesis of psychiatric manifestations. Genetic hypothesis and stress-related theories have also been proposed.

Circulating levels of reactive oxygen species in patients with nonproliferative diabetic retinopathy and the influence of antioxidant supplementation: 6-month follow-up.

AIMS: The aim was to evaluate circulating levels of reactive oxygen species (ROS) and changes in central macular thickness (CMT) in patients with nonproliferative diabetic retinopathy (NPDR) after antioxidant supplementation. MATERIALS AND METHODS: A total of 68 patients (68 eyes) with NPDR were enrolled. Patients were randomly divided into two groups: Treated with antioxidant supplement (Group A) and untreated control group (Group B). Each tablet, for oral administration, containing pycnogenol 50 mg, Vitamin E 30 mg and coenzyme Q10 20 mg. CMT and free oxygen radical test (FORT) were analyzed at baseline (T0), 3 (T1) and 6 (T2) months in both groups. RESULTS: In Group A, FORT levels and CMT were significantly reduced over time (P < 0.001 for both). In Group B, FORT levels were increased (P < 0.001) and CMT did not vary significantly (P = 0.81) over 3 time points. CONCLUSIONS: This is the first study showing the reduction of ROS levels in patients with NPDR thanks to antioxidant therapy. Moreover, our findings have suggested also an influence on retinal thickness.

Evaluation of recurrent hyphema after trabeculectomy with ultrabiomicroscopy 50-80 MHz: a case report.

BACKGROUND: Hyphema is a complication that can occur after glaucoma filtering surgery. Biomicroscopic examination of the anterior segment is commonly used to diagnose it and gonioscopy may provide a useful support to find the source of the haemorrhage. Unfortunately, when the blood hides the structure of the anterior segment the gonioscopic examination fails. In this case we performed ultrabiomiscroscopy with 50-80 MHz probes to overcome the limits of gonioscopy. The use of this technique to study the anterior segment of the eye has previously been reported in literature, but we illustrates its importance for performing a correct diagnosis in a specific case of hyphema. CASE PRESENTATION: We report a case of a sixty-year-old caucasian male with recurrent hyphema in the left eye. The episodes of hyphema were four in two years and the patient came to the hospital for the first time in the last occasion. The past episodes were managed with topical corticosteroids and mydriatic drops. He referred surgical trabeculectomy in both eyes 5 years before the first symptoms and no specific eye trauma before the first episode. The examination of the anterior segment revealed a 2 mm hyphema in the left eye due to blood leakage through the superior iridectomy. Gonioscopy could not identify the source of the haemorrhage. B-scan ultrasound and ultrabiomiscroscopy, with 50-80 MHz probes, were performed. Ultrabiomiscroscopy, mainly with the probe of 80 MHz, provided images of high resolution of the structures of the anterior segment and it allowed the visualization of an abnormal vessel at the inner margin of the trabeculectomy opening, probably responsible of the recurrent hyphema. CONCLUSION: Ultrabiomicroscopy proved to be a useful diagnostic technique for identifying the cause of the recurrent hyphema when other examination techniques are not applicable.

Intravitreal ranibizumab and laser photocoagulation in the management of idiopathic juxtafoveolar retinal telangiectasia type 1: a case report.

BACKGROUND: Idiopathic juxtafoveolar retinal telangiectasia (IJRT) type 1 represents an uncommon cause of congenital unilateral visual loss and it typically affects males. Decrease in visual acuity is caused by serous and lipid exudation into the fovea with cystoid macular edema. In some cases, spontaneous resolution may be observed, but when there is a progressive loss of visual acuity, laser photocoagulation is often necessary. This treatment is not always successful and therapy for this condition is still controversial. CASE PRESENTATION: A 57-year-old man referred a 2-month history of blurred and distorted vision in the right eye. Best-corrected visual acuity was 20/50 in the right eye and 20/20 in the left eye. Fundus examination showed temporal macular edema, confirmed by optical coherence tomography. Fluorescein angiography showed a localized area of hyperfluorescence probably due to telangiectasia type 1 located below the inferior temporal area of the fovea. A combined therapy of intravitreal ranibizumab injection and laser photocoagulation was performed. Visual acuity improved from 20/50 to 20/32 and the therapy was well tolerated by the patient. After 3 years of follow-up, both visual acuity and fundus examination were stable. CONCLUSIONS: This case suggests that the combined use of ranibizumab and laser photocoagulation may be considered an effective treatment for JRT type 1, leading to an improvement in both visual acuity and macular edema. We believe that intravitreal ranibizumab injection associated with laser photocoagulation should be considered as treatment for IJRT type 1.