Bilateral diffuse choroidal hemangioma in Sturge Weber syndrome: A case report highlighting the role of multimodal imaging and a brief review of the literature.

PURPOSE: To present a patient with bilateral choroidal hemangioma in Sturge-Weber syndrome (SWS) and highlight multimodal imaging techniques for early detection and management of ocular alterations. METHODS: A 37-year-old woman with diagnosis of SWS presented to our unit. The patient had been treated with pulsed dye laser for bilateral nevus flammeus and had right leptomeningeal angiomatosis. She had glaucoma, but ultrasound biomicroscopy did not show anterior chamber or ciliary body alterations. RESULTS: Enhanced depth imaging (EDI) spectral domain optical coherence tomography (SD-OCT) showed bilateral diffuse choroidal hemangiomas in both eyes with choroidal thickness above 1000 µm. B-scan ultrasound examination showed diffuse choroidal hemangioma in both eyes, with a choroidal thickness of 1.53 mm and 1.94 mm in the right and left eye (RE, LE), respectively. Peripapillary retinal nerve fiber evaluation showed thinning of the retinal nerve fiber layer in both eyes. CONCLUSIONS: This report highlights multimodal imaging techniques for the critical assessment of patients with SWS, especially in rare cases with bilateral choroidal hemangioma of the choroid. Novel imaging modalities enable optimal management and follow-up of rare conditions, and our case adds further evidence to the existing literature.

Fascicular Visual Field Defects in Open-Angle Glaucoma: Evaluation with Microperimetry.

Purpose. Use of microperimetry (Mp-1), correlating with Humphrey perimetry (30-2 program), in patients affected by primary open-angle glaucoma (POAG) with perimetric defects, in order to obtain an evaluation of the accuracy of the results obtained by Mp-1. Materials and Methods. In this study 40 eyes of 25 patients affected by POAG with perimetric defects were included. All patients underwent microperimetry test by Nidek Mp-1 (NAVIS software version 1.7.2, Nidek Technologies). Mean sensitivity values expressed in decibel (dB) of all tested dots and mean values for each quadrant obtained by microperimetric test were correlated with corresponding quadrants obtained by static perimetry analysis. Data were analyzed by Pearson's correlation and Bland-Altman analysis. Results. Interpolated data showed that mean sensitivity values in all spots tested by Mp-1 (11.98 dB, SD 4.31) may be significantly correlated with mean total values obtained by Humphrey 30-2 perimetry (17.95, SD 4.32), with correlation coefficient of 0.556. Conclusions. Topographic visualization of the perimetric alteration by microperimetry allows retesting areas with reduced sensitivity which are topographically visualized and displayable on the ocular fundus examination, avoiding worsening of the functional defect by better modulation of the antiglaucoma therapy and therefore it allows better monitoring of the pathologic functional damage.

Utility of retinal thickness analyzer in early diagnosis of glaucomatous damage.

AIM: The utility of retinal thickness analysis (RTA) in the primary open-angle glaucoma (POAG) early diagnosis and in the follow-up was evaluated. MATERIALS AND METHODS: The nerve fibre layer thickness was analysed with the RTA by repeated examinations in POAG, ocular hypertension (OH) and healthy subjects (H). RESULTS: In the POAG group, a statistically significant reduction of mean retinal thickness (MRT) compared to the H group was evidenced both in the total area examined and in each of the four quadrants. The visual field evaluation classified the POAG group's visual fields in stage 1 and the OH group's visual fields in stage 0. CONCLUSION: RTA reveals a variation of mean retinal thickness, even before a functional and/or morphological optic nerve alteration appears. RTA is a complementary tool with the exams usually applied in the diagnosis of glaucoma, even though the information obtained is not specific for glaucoma.

Acute monolateral proptosis and orbital myositis in a patient with discoid lupus erythematosus: a case report.

INTRODUCTION: Discoid lupus erythematosus may lead to significant orbital inflammation syndrome. Ocular manifestations related to discoid lupus erythematosus are uncommon and few cases of eye inflammation are reported. CASE PRESENTATION: A 37-year-old Caucasian woman with 5-year history of discoid lupus erythematosus presented with exophthalmos, periorbital pain and blurred vision in her right eye. Orbital computed tomography and laboratory tests were performed. Computed tomography imaging revealed an enlargement of the right medial rectus muscle. Thyroid eye disease and orbital cellulites were excluded. Corticosteroid treatment completely resolved the symptoms. CONCLUSION: This is the first case of orbital myositis in a patient with discoid lupus erythematosus presenting with acute proptosis, diplopia and single extraocular muscle involvement.

Volumetric assessment of the space between the posterior hyaloid and internal limiting membrane using SD-OCT.

The purpose of this study was to measure, for clinical and surgical utility, the distance between the posterior hyaloid and the internal limiting membrane using a spectral domain optical-coherence tomography (SD-OCT) device. Eight eyes of eight patients with subclinical partial posterior vitreous detachment (PPVD) with vitreomacular traction syndrome were examined with SD-OCT. To quantify the vitreoretinal distance, the basic applications of SD-OCT (Spectralis HRA+OCT Heidelberg Engineering) were modified. We conducted a topographic study of the entire posterior pole by defining new maps, each generated from 19 raster scans. We obtained maps that represent the space between the residual vitreous and retina, expressed numerically and on a colour spectrum, and overlapped them on a posterior pole photograph. We also created a single map of the entire posterior pole. The mean PPVD volume in the eight eyes was 104.87±5.25 mm(3). This study produced detailed maps of the vitreoretinal distance, which may facilitate the study of the clinical progression of PPVD. In case of surgery the maps may help to accurately determine where surgical manipulation is needed safely apart from the retina. These maps could also assist in the assessment of pharmacological vitreolysis efficacy.

Acetazolamide-induced cilio-choroidal effusion after cataract surgery: unusual posterior involvement.

Idiosyncratic reactions to a large number of drugs have been reported to cause choroidal detachment and secondary angle-closure glaucoma (ACG). We report a case of bilateral acute ACG and peculiar choroidal effusion following administration of oral acetazolamide immediately after cataract surgery. Few cases of acute secondary ACG with choroidal effusion and anterior shift of the lens-iris diaphragm have been associated with acetazolamide compared with other sulfonamides. As far as we are aware, posterior involvement with retinal folds and papillary edema due to acetazolamide has not been described before.

Prospective microperimetry and OCT evaluation of efficacy of repeated intravitreal bevacizumab injections for persistent clinically significant diabetic macular edema.

To prospectively investigate morphologic and functional changes after intravitreal bevacizumab for persistent diffuse and clinically significant diabetic macular edema. In total, 26 eyes in 26 patients were treated with three intravitreal injections of bevacizumab (Avastin™) given at 4-week intervals. Study parameters included: visual acuity (VA), perceived visual improvement, central macular thickness as measured by Spectralis OCT, macular sensitivity and fixation pattern as measured by MP-1 microperimetry, and the incidence of ocular and systemic side-effects. At the time of follow-up, 76.9 % of eyes showed a significant improvement in VA (p = 0.012), 38.4 % showed a one-line improvement on the ETDRS chart compared with VA at day 0, 30.7 % showed a two-line improvement, and 7.6 % showed at least a three-line improvement. The mean central macular thickness was 447 microns at day 0 and 311.1 microns at follow-up (p = 0.003). The mean baseline macular sensitivity, by MP-1 microperimetry, was 8.29 dB; at follow -up, macular sensitivity had improved to 14.26 dB (p = 0.025). These results support further controlled trials on the efficacy of intravitreal bevacizumab in treating diabetic macular edema. Microperimetry and OCT are important tools in managing diabetic patients, providing a detailed study of the macular region, particularly when it is necessary to monitor the morphological and functional outcome after various interventions. A good correlation between retinal sensitivity and perceived visual performance was found.

Role of the intravitreal growth factors in the pathogenesis of idiopathic epiretinal membrane.

PURPOSE: The aim of the present study is to evaluate the roles of TGFs ß1 and ß2, glial cell line-derived neurotrophic factor (GDNF), and nerve growth factor (NGF) in the pathogenesis of idiopathic epiretinal membrane (ERM). METHODS: Eight patients, six males and two females, with an average age of 60.25 ± 17.16 years (range, 33-75 years) who were affected by idiopathic ERM were enrolled in the study. All patients underwent standard pars plana vitrectomy surgery with membrane removal and specific ELISA was performed to evaluate TGFß1, TGFß2, GDNF, and NGF in the vitreous samples. This was repeated after acidification of the samples with hydrochloric acid. RESULTS: Before acidification, ELISA analysis revealed a significant increase of TGFß2 in the samples with idiopathic ERM (327.98 ± 99.58 pg/mL; range, 206.864-466.235 pg/mL) compared to the control group (187.17 ± 58.20 pg/mL; range, 132.758-271.707 pg/mL; t = 3.4; P < 0.05). A statistically significant difference was also obtained after acidification of the samples (618.15 ± 201.43 pg/mL; range, 409.795-866.215 pg/mL compared to 265.04 ± 98.15 pg/mL; range, 152.478-352.101 pg/mL; t = 4.5; P < 0.05). Notably, before acidification the differences in NGF between the two groups were not statistically significant (t = 0.79; P = 0.46), while after acidification a significant increase of the NGF levels in ERM samples was found in comparison with the control group (723.41 ± 235.4 vs. 242.84 ± 104.61; t = 3; P < 0.05). CONCLUSIONS: The present study reveals that TGFß2 and NGF are associated with idiopathic ERMs, suggesting a novel compensatory mechanism so far never proposed.

Microperimetric biofeedback in AMD patients.

To analyse biofeedback training by microperimeter MP-1 (Nidek Technologies) on patients with Age Related Maculopathy (AMD). We enrolled 15 patients (10 female and 5 male) and examined total of 27 eyes with AMD. All the patient underwent 10 training sessions of 10 min for each eye, performed once a week using the MP-1 biofeedback examination. Statistical analysis was performed using Student's t-test. p values less than 0.05 were considered statistically significant. All patients displayed an improvement in visual acuity, fixation behaviour, retinal sensitivity an reading speed. The mean character size value improved from 36.4 to 11.7; this result was statistically significant (p = 0.031). A biofeedback examination using the MP-1 microperimeter can help the brain to memorize the final fixation location by increasing attention modulation, thereby providing an efficient preferred retinal locus for visual tasks in patients with macular disease and central scotoma.

Outer layer breaks and asymptomatic schisis detachment: clinical considerations.

BACKGROUND AND OBJECTIVE: To provide a detailed description of the clinical features that are considered forerunners of symptomatic complications in asymptomatic degenerative retinoschisis, and to show that in selected cases at this stage prophylactic photocoagulation may be a better choice than mere observation. MATERIALS AND METHODS: Forty-three eyes of 27 patients with asymptomatic bullous degenerative retinoschisis and outer layer breaks (OLBs) were studied through binocular indirect dynamic ophthalmoscopy and retinal biomicroscopy with the Goldmann 3-mirror lens, fundus drawings, and photographs where feasible. Argon laser treatment was performed on each eye: first, around the posterior border of the schisis to achieve a full-thickness retinal scar, and then on the schisis itself to promote scarring of the retinal pigment epithelium, thus avoiding retinal detachment. The follow up was 2 years minimum after treatment. RESULTS: OLBs usually involved the largest schises when multiple retinal splittings were present. Breaks were single in 18 eyes (peripheral in 16 and posterior in 2) and multiple in 25 (peripheral in 15 and posterior in 10). Overall, 23 eyes showed asymptomatic retinal detachment (schisis detachment): 20 with peripheral outer layer breaks and 3 with posterior breaks. Schisis detachment was localized to the schisis area in the first group, whereas it extended beyond the posterior boundary of retinoschisis in the latter. After treatment, no posterior progression of retinoschisis was noted nor did symptomatic retinal detachment arise. Only 1 eye had complications in the second step of the treatment that was later resolved with medical care. CONCLUSION: Prophylactic Argon laser photocoagulation can be used safely in the asymptomatic stage of bullous retinoschisis with outer layer breaks to avoid the onset of acute symptomatic retinal detachment.