Miklós Schneider

MINERALOCORTICOID RECEPTOR ANTAGONIST TREATMENT IN BILATERAL CHRONIC CENTRAL SEROUS CHORIORETINOPATHY: A COMPARATIVE STUDY OF EXUDATIVE AND NONEXUDATIVE FELLOW EYES

Purpose: To evaluate the macular thickness, choroidal thickness, and visual acuity changes in eyes of patients with bilateral chronic central serous chorioretinopathy during eplerenone treatment. Methods: This prospective clinical trial was conducted on patients with bilateral chronic central serous chorioretinopathy, who had subretinal fluid (SRF) in 1 eye. Twenty-eight patients were treated with 50 mg/day of oral eplerenone for 3 months and were observed for another 3 months. Twenty-eight eyes with SRF were compared with the 28 fellow eyes with pachychoroid pigment epitheliopathy. Results: The central macular and choroidal thickness showed a significant decrease (P < 0.005) at 3 months in all eyes, but change in choroidal thickness was smaller in nonexudative fellow eyes (P > 0.05 at 6 months). In the exudative eyes, the decrease in choroidal thickness showed a significant correlation with the resolution of SRF (P < 0.001). Visual acuity remained stable in all eyes, with significant improvement only in exudative eyes at 6 months (P < 0.005). Baseline choroidal thickness was a significant positive predictor for SRF decrease (P = 0.003). Conclusion: Patients with chronic central serous chorioretinopathy can safely be treated with eplerenone as it can reverse choroidal vasodilation with an accompanying resolution of the SRF and improvement in visual acuity. These beneficial therapeutic effects are more pronounced in the exudative eyes.

Optical Coherence Tomography Features of POEMS Syndrome and Castleman Disease-associated Papillopathy

Abstract Purpose: To report ocular symptoms and optical coherence tomography (OCT) features of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. Methods: Case report of two Caucasian patients with POEMS, one of whom also had multicentric Castleman disease. Results: Both patients presented with edema on both optic disks, bilateral macular edema was seen in case 1, and peripapillary choroidal neovascularization was seen on the right side in case 2. OCT confirmed papilledema involving all retinal layers, with additional bilateral peripapillary serous retinal detachment involving the macula in both cases, and peripapillary CNV in case 2. Changes in retinal nerve fiber layer thickness, total peripapillary retinal thickness, and macular detachment were followed with OCT over the course of the disease and after administration of systemic therapy. Conclusion: OCT examination of the optic nerve head and the macula has an important additional role in the diagnosis and follow-up of POEMS syndrome and Castleman disease.

Central corneal thickness measurements with optical coherence tomography and ultrasound pachymetry in healthy subjects and in patients after photorefractive keratectomy.

Purpose To compare central corneal thickness measurements made using two different methods, optical coherence tomography (OCT) and ultrasound pachymetry, applied both in normal eyes and in eyes on which photorefractive keratectomy (PRK) had been performed. A second objective was to assess the intrasession variability of OCT measurements. Methods In this prospective study, central corneal thickness was measured in 20 normal subjects (normal group) and in 20 PRK patients using the StratusOCT instrument model 3000 (Carl Zeiss Meditec), and also with an ultrasound pachymeter. Five OCT measurements were performed using the Fast Macular Thickness protocol. Corneal thickness data were obtained with the Scan Profile analysis protocol. The OCT measurement results were compared with the mean value of three ultrasound pachymetry measurements for the same eye. Results The pachymetry–OCT correlation coefficients were 0.96 and 0.97 in the normal and PRK groups, respectively (p=0.14). Neither linear regression nor Bland-Altmann analysis revealed any significant systematic measurement error. Intrasession standard deviations in the normal and PRK groups were 4.9 μm and 3.8 μm, respectively. Conclusions Noncontact central corneal thickness measurements made using the StratusOCT instrument are accurate and reproducible, both in normal subjects and in post-PRK patients. The instrument system does not need any modifications to correctly detect and measure the center of the cornea.

Three-dimensional imaging of an optic disk pit using high resolution optical coherence tomography.

Purpose To clarify the origin of the coexisting fluid in an optic disc pit case by using optical coherence tomography (OCT). Methods High resolution OCT (Cirrus™ prototype, Carl Zeiss Meditec) was used for image acquisition; three dimensional segmentation was performed using Food and Drug Administration–approved imaging software (3D-Doctor V4.0, Able software Corp., Lexington, MA) to demonstrate the structural changes of the optic nerve head and the retina. Results Using high resolution OCT, the authors demonstrated that this case of optic pit had a possible connection between the subretinal and the intraretinal space. Conclusions The authors assume that the intraretinal space is progressively filled with subarachnoidal fluid, leading to a tearing force within the outer neurosensory layers. A connection between the outer nuclear layer and the subretinal space may lead to a serous retinal detachment as a secondary event. Vision loss could consecutively be induced by a serous retinal detachment. High resolution OCT technology is able to visualize discrete changes of the microarchitecture of the optic nerve as well as the retina when combined with appropriate imaging software.

Bilateral cystoid macular edema following docetaxel chemotherapy in a patient with retinitis pigmentosa: a case report

BackgroundDocetaxel is a chemotherapeutic agent of the taxane class of drugs for the treatment of breast cancer. We present a female patient who noted decreased vision after docetaxel treatment.Case presentationA 45-year-old female patient received docetaxel treatment after resection of a breast carcinoma. Funduscopy and optical coherence tomography (OCT) showed cystoid macular edema on both eyes. Dilated funduscopy also showed bone spicule-like pigmented deposits, typical for retinitis pigmentosa. Besides the fundus appearance restricted peripheral vision and scotopic electroretinogram confirmed the diagnosis of retinitis pigmentosa. Chemotherapy was discontinued following a consulation with the oncologist of the patient. After five weeks, visual acuity improved significantly along with decrease of retinal thickness measured by OCT.ConclusionDocetaxel may cause ocular adverse effects such as cystoid macular edema. Ophthalmological examination is warranted for patients with visual complaints during docetaxel chemotherapy.

Boundary Detection Errors on Optical Coherence Tomography Images in Patients With Diabetic Retinopathy

BACKGROUND AND OBJECTIVE To study the incidence of boundary detection errors produced by optical coherence tomography measurements in patients with diabetic retinopathy. PATIENTS AND METHODS One hundred sixteen eyes with diabetic retinopathy of 64 consecutive patients with diabetes mellitus were included in this retrospective study. The StratusOCT instrument (Carl Zeiss Meditec, Dublin, CA) with the macular thickness map protocol was used for the examinations. After data acquisition, each scan was analyzed using the retinal thickness (single eye) protocol to evaluate whether there was any misdetection of the retinal boundaries. RESULTS Boundary detection errors were found in 35.3% of eyes. The majority of artifacts were those caused by hard exudates (41.5%), followed by cystoid macular edema (31.7%) and proliferation (17.0%). CONCLUSION Occurrence of artifacts with optical coherence tomography measurements in cases of diabetic retinopathy is not a rare phenomenon and verification of quantitative measurements is strongly recommended.

Leber congenital amaurosis: First genotyped Hungarian patients and report of 2 novel mutations in the CRB1 and CEP290 genes

Purpose To introduce the first Hungarian patients with genetically defined Leber congenital amaurosis (LCA) and to report 2 novel mutations. Methods Seven otherwise healthy patients (4-29 years, 5 male and 2 female) who had an onset of severe visual impairment before age 2 years were investigated. The diagnosis was established in all individuals by medical history, funduscopy, and full-field electroretinogram (ERG). Ocular examination included visual acuity testing, digital fundus photography, and in 6 patients retinal imaging with optical coherence tomography (OCT). Arrayed primer extension microarray screening was performed in all probands. In 2 patients, further Sanger sequencing and targeted next-generation sequencing revealed the second disease allele. Results A cone-rod type LCA was revealed in 4 patients and a rod-cone type disease in 3 patients. Five patients presented with maculopathy. Optical coherence tomography (OCT) imaging showed diffuse retinal thickening in 3 probands with severe macular atrophy in one. Full-field ERGs were undetectable or residual in all patients. Genetic screening revealed AIPL1, CRB1, and CEP290 gene-related pathology in 6 patients; in 1 proband, no mutation was found. Three homozygous and 3 compound heterozygous mutations were identified. Two novel variants were detected: c.2536G>T (p.G846X) in the CRB1 gene and c.4929delA (p.Lys1643fsX2) in the CEP290 gene. Conclusions Genetic subtypes identified are among the most common ones in LCA; the phenotypes are consistent with those reported previously. Both novel mutations are predicted to result in a premature translation termination. The phenotype related to the novel CRB1 mutation results in severe atrophic maculopathy.

Optic disc morphology in unilateral branch retinal vein occlusion using spectral domain optical coherence tomography

BackgroundThe aim of this study was to evaluate the association between optic nerve head (ONH) parameters and branch retinal vein occlusion (BRVO) using spectral domain optical coherence tomography (SD-OCT).MethodsBoth eyes of 40 patients with unilateral BRVO (mean age: 67.4 ± 11.4 years, male: female - 18:22) were enrolled in this study. Control group consisted of randomly selected single healthy eyes of 40 age and gender matched volunteers (mean age: 64.7 ± 15.4 years, male: female - 16:24). ONH parameters (including optic disc area, optic cup area, neuroretinal rim area, cup volume, rim volume, cup-disc area ratio, horizontal and vertical cup-disc ratio, average retinal nerve fiber layer) were measured by SD-OCT. Axial length (AL) of the eyes was measured by non-contact optical low coherence reflectometry. The ONH parameters of eyes with BRVO were compared with those of fellow eyes using mixed model, one-way between-groups analysis of covariance was conducted to compare the ONH parameters of affected and unaffected fellow eyes in BRVO patients with those of the control eyes keeping confounding factors, including AL, age and gender under control in the statistical analysis.ResultsNone of the investigated ONH parameters of affected BRVO eyes, unaffected fellow eyes and control eyes were statistically different after controlling for AL, age and gender.ConclusionOptic disc morphology might not be a potential anatomical predisposing factor for development of BRVO.

Evaluation of lymphatic vessel dilatations by anterior segment swept-source optical coherence tomography: case report

BackgroundConjunctival lymphangiectasia is a rare condition presumably caused by the obstruction of lymphatic channels or by an abnormal connection between conjunctival lymphatic and blood vessels. Diagnosis is based on clinical appearance and histology. We report a case of conjunctival lymphangiectasia in which anterior segment optical coherence tomography (OCT) was used to assist the diagnosis and the planning of the biopsy location.Case presentationA 31-year-old woman was referred with repeated episodes of conjunctival “hemorrhages” and chemosis with extended recovery periods over the last months. Other symptoms were dryness, redness, burning sensation and itching. Photo documentation, anterior segment OCT, ultrasound, computer tomography (CT) and magnetic resonance imaging (MRI) of the brain were performed. MRI revealed dilated atypical Virchow-Robin space (VRS). Conjunctival biopsy was taken and the location of the biopsy was selected based on OCT findings.Based on the clinical appearance we suspected the case to be conjunctival lymphangiectasia or lymphangioma. Histology and immunhistochemistry confirmed the diagnosis of conjunctival lymphangiectasia.ConclusionsAnterior segment OCT is a non-invasive tool, useful in the evaluation of conjunctival lesions and planning surgery.

Stromal Cell-Derived Factor 1 Polymorphism in Retinal Vein Occlusion

Background Stromal cell-derived factor 1 (SDF1) has crucial role in the regulation of angiogenesis and ocular neovascularisation (NV). The purpose of this study was to evaluate the association between SDF1-3’G(801)A polymorphism and NV complications of retinal vein occlusion (RVO). Methods 130 patients with RVO (median age: 69.0, range 35–93 years; male/female– 58/72; 55 patients had central RVO, 75 patients had branch RVO) were enrolled in this study. In the RVO group, 40 (30.8%) patients were diagnosed with NV complications of RVO and 90 (69.2%) patients without NVs. The median follow up period was 40.3 months (range: 18–57 months). The SDF1-3’G(801)A polymorphism was detected by PCR-RFLP. Allelic prevalence was related to reference values obtained in the control group consisted of 125 randomly selected, age and gender matched, unrelated volunteers (median age: 68.0, range 36–95 years; male/female– 53/72). Statistical analysis of the allele and genotype differences between groups (RVO patients vs controls; RVO patients with NV vs RVO patients without NV) was determined by chi-squared test. P value of <0.05 was considered statistically significant. Results Hardy-Weinberg criteria was fulfilled in all groups. The SDF1-3’G(801)A allele and genotype frequencies of RVO patients were similar to controls (SDF1-3’A allele: 22.3% vs 20.8%; SDF1-3’(801)AA: 5.4% vs 4.8%, SDF1-3’(801)GG: 60.8% vs 63.2%). The frequency of SDF1-3’(801)AA and SDF1-3’(801)GA genotypes, as well as the SDF1-3’(801)A allele frequency were higher in RVO patients with NV versus in patients without NV complication (SDF1-3’(801)AA+AG genotypes: 57.5% vs 31.1%, p = 0.008; SDF1-3’(801)A allele: 35.0% vs 16.7%, p = 0.002) or versus controls (SDF1-3’(801)AA+AG genotypes 57.5% vs 36.8%, p = 0.021; SDF1-3’(801)A allele: 35.0% vs 20.8% p = 0.01). Carrying of SDF1-3’(801)A allele increased the risk of neovascularisation complications of RVO by 2.69 (OR, 95% CI = 1.47–4.93). Conclusion These findings suggest that carrying SDF1-3’(801)A allele plays a role in the development of neovascular complications in retinal vein occlusion.