Detección y caracterización de defectos del campo visual mediante perimetría Octopus en glaucoma congénito primario / Detection and characterisation of visual field defects using Octopus perimetry in congenital glaucoma

Detectar y caracterizar los defectos del campo visual (CV) mediante perimetría Octopus en pacientes con glaucoma congénito primario (GCP) y determinar la calidad y duración del CV. Material y métodos: Se incluyeron 88 ojos de 70 pacientes diagnosticados de GCP. Las evaluaciones se realizaron con un Octopus 900 y cada ojo se evaluó con el algoritmo de perimetría orientada por tendencias (G-TOP). Se recogieron datos cuantitativos de CV: datos de calidad (respuestas falsa positiva y negativa, y duración del tiempo) y resultados de desviación media (DM) y raíz cuadrada de la varianza de pérdida (sLV). También se recogieron datos cualitativos: presencia de defectos difusos y localizados, hemicampo afectado y grado de defectos utilizando la clasificación de Aulhorn y Karmeyer. Se analizaron las correlaciones entre los resultados perimétricos y las variables clínicas. Resultados: La mediana de edad fue de 11 (8-17) años. El 65,9% (58/88) de los ojos con GCP presentaban defectos de CV. Se observaron defectos difusos en 10/58 ojos (16,94%) (DM media=23,92 [DE: 2,52]) dB) y defectos localizados en 48/58 ojos (82,75%). El defecto más frecuente fue el escotoma incipiente paracentral (n=15), el escalón nasal (n=8), el defecto arciforme añadido (n=2), el semianular (n=13) y el defecto concéntrico con isla central (n=9). El hemicampo visual afectado con mayor frecuencia fue el inferior. La duración media de la prueba fue de 2min 12s (DE: 21,6s). Los valores MD y sLV se correlacionaron con la agudeza visual mejor corregida, la relación excavación/disco y el número de cirugías de glaucoma (todas p<0,001). Conclusión: Se identificó un alto número de defectos difusos y localizados utilizando la perimetría Octopus en pacientes con GCP. El defecto más frecuente fue el escotoma paracentral, y el hemicampo inferior fue el más afectado.(AU)

Purpose: To detect and characterise visual field (VF) defects using static Octopus perimetry in patients with primary congenital glaucoma (PCG) and to determine VF quality and time duration. Material and methods: Eighty-eight eyes of 70 patients diagnosed with PCG were included. Assessments were performed using an Octopus 900 and each eye was assessed with the tendency-oriented perimetry (G-TOP) algorithm. Quantitative VF data were collected: quality data (false positive and negative response, and time duration) and results of mean deviation (MD) and square root of loss variance (sLV). Qualitative data were collected: the presence of diffuse and localized defects, the affected hemifield and grade of defects using the Aulhorn and Karmeyer classification. Correlations between perimetric results and clinical variables were analysed. ResultsMedian age was 11 (8-17) years. 65.9% (58/88) of PCG eyes showed VF defects. Diffuse defects were observed in 10/58 eyes (16.94%) (mean MD=23.92 [SD: 2.52]) dB) and localized defects in 48/58 eyes (82.75%). The most frequent defect was spot-like/stroke-like/incipient paracentral scotoma (n=15), nasal step (n=8), adding arcuate defect (n=2), half ring-shaped (n=13) and concentric defect with a central island (n=9). And the most frequent affected visual hemifield was inferior hemifield. Mean test duration was 2min 12s (SD: 21.6s). MD and sLV values were correlated with best corrected visual acuity (BCVA), cup to disc ratio and number of antiglaucoma surgeries (all P<.001). Conclusion: A high number of diffuse and localized defects were identified using Octopus perimetry in PCG patients. The most frequent defect was paracentral scotoma and inferior hemifield was the most affected.(AU)

Congenital stromal corneal dystrophy in a Spanish family: Clinical, genetic and histological analysis.

PURPOSE: To present the clinical, genetic, and histopathological features of the ninth family affected by congenital stromal corneal dystrophy (CSCD) to date. METHODS: Twelve cases of a Spanish family affected by CSCD were analyzed regarding history, visual acuity (VA, decimal scale), an ophthalmologic exam and specular microscopy. Five eyes were treated by deep anterior lamellar keratoplasty (DALK), and thirteen eyes by penetrating keratoplasty (PK). In the two last generations, a genetic study was performed. RESULTS: Most of the patients affected were born with opaque corneas except for three, whose corneas were clear at birth. Biomicroscopy showed a whitish diffuse stromal opacity with an unaltered epithelium, causing poor VA (from hand motions to 0.4). Patients treated with PK presented mean postoperative VA of 0.19±0.20 over a follow-up time of 235.3±101.4months with 38% recurrences. Patients who underwent DALK experienced VA improvement to 0.17±0.11 over a follow-up time of 10.8±2.6months without signs of recurrence. In the latter, the big bubble technique was not achieved, so a manual technique was performed. The genetic study showed heterozygosis for a 1-bp deletion at nucleotide 962 in exon 8 of the decorin gene. CONCLUSIONS: CSCD is a rare entity, which should be treated by DALK whenever possible, obtaining better results than PK. Close monitoring of children of affected individuals is important, because CSCD can progress during the early years of life.

Detection and characterisation of visual field defects using Octopus perimetry in congenital glaucoma.

PURPOSE: To detect and characterise visual field (VF) defects using static Octopus perimetry in patients with primary congenital glaucoma (PCG) and to determine VF quality and time duration. MATERIAL AND METHODS: Eighty-eight eyes of 70 patients diagnosed with PCG were included. Assessments were performed using an Octopus 900 and each eye was assessed with the tendency-oriented perimetry (G-TOP) algorithm. Quantitative VF data were collected: quality data (false positive and negative response, and time duration) and results of mean deviation (MD) and square root of loss variance (sLV). Qualitative data were collected: the presence of diffuse and localized defects, the affected hemifield and grade of defects using the Aulhorn and Karmeyer classification. Correlations between perimetric results and clinical variables were analysed. RESULTS: Median age was 11 (8-17) years. 65.9% (58/88) of PCG eyes showed VF defects. Diffuse defects were observed in 10/58 eyes (16.94%) (mean MD = 23.92 [SD: 2.52]) dB) and localized defects in 48/58 eyes (82.75%). The most frequent defect was spot-like/stroke-like/incipient paracentral scotoma (n = 15), nasal step (n = 8), adding arcuate defect (n = 2), half ring-shaped (n = 13) and concentric defect with a central island (n = 9). And the most frequent affected visual hemifield was inferior hemifield. Mean test duration was 2 min 12 s (SD: 21.6 s). MD and sLV values were correlated with best corrected visual acuity (BCVA), cup to disc ratio and number of antiglaucoma surgeries (all P < .001). CONCLUSION: A high number of diffuse and localized defects were identified using Octopus perimetry in PCG patients. The most frequent defect was paracentral scotoma and inferior hemifield was the most affected.

Corneal endothelial validation in the eye bank: differences in automated methods and repeatability.

PURPOSE: To evaluate reproducibility of endothelial cell density (ECD) measurements using the Konan Cell Check D in donor corneas by two different ophthalmologists and to compare the two automated cell count methods (center and flex-center) available in the software of this specular microscope. METHODS: ECD values were quantified in 54 donor corneas by two independent investigators using the Cell Check D (Konan Medical USA Inc) with both automated cell count methods. In the center method, at least 30 contiguous cells are marked. For the flex-center method, an area is delineated and only the cells within the designated area are counted. RESULTS: The mean ECD was 2473.81±378.22 cells/mm2. Good ECD intergrader reproducibility for the center (ICC=0.821) and the flex-center method (ICC=0.784) were noted. Poor reliability was observed for coefficient of variation and hexagonality (ICC≤0.265). When both methods for ECD analysis were compared, a moderate correlation for the two independent graders using the two manual (center and flex-center) methods was detected (correlation coefficient of 0.678 and 0.745 for each of the investigators). Comparison between methods yielded significantly higher ECD with the flex-center method (P=0.013). When corneas were divided by ECD, those under 2200 cells/mm2 and those between 2200 and 2700 cells/mm2 also had significantly higher ECD with the flex-center method (P<0.022). CONCLUSIONS: ECD values are reproducible with both methods, although the flex-center method ECDs tend to be higher, particularly in cases of low ECD. Eye banks and surgeons should exercise caution in making decisions based only on small differences in ECD.

Reproducibility of SD-OCT inner macular layer thickness measurements in children with primary congenital glaucoma.

PURPOSE: To examine the reproducibility of spectral domain optical coherence tomography (SD-OCT) segmented ganglion cell complex and circumpapillary retinal nerve fiber layer (cpRNFL) measurements in children with primary congenital glaucoma (PCG) in comparison with healthy children. METHODS: 12 children with PCG (G1) and 24 healthy children (G2) were recruited. The following SD-OCT measurements (Spectralis, Heidelberg Engineering) were made in one eye per child: total macular thickness (MT), thicknesses in several subfields and volumes of the three inner macular layers, macular retinal nerve fiber layer (mRNFL), ganglion cell layer (GCL), and inner plexiform layer (IPL) and cpRNFL thickness. In a single day, an expert operator obtained 3 circumpapillary and 3 macular measurements in each participant to determine intraoperator variability. Intraoperator repeatability was defined by the coefficient of variation (CoV) and intraclass correlation (ICC). RESULTS: ICC was excellent in both groups for cpRNFL measurements (G1 ICC=0.950 and G2 ICC=0.995) and for MT was excellent in G1 (ICC=0.957) and moderate in G2 (ICC=552). For the inner macular layer measurements, all ICCs were better in PCG group (mRNFL-ICC: 0.915 vs. 0.765; ICC-GCL: 0.584 vs. 0.263 and ICC-IPL: 0.979 vs. 0.742; G1 and G2 respectively). Greater CoV were recorded for macular measurements (from 0.71% to 9.82%) compared to cpRNFL measurements (from 0.52% to 1.50%). CONCLUSION: In children with PCG, Spectralis SD-OCT showed excellent intrasession repeatability for cpRNFL, MT, mRNFL and IPL measurements and moderated for GCL measurements. For all macular measurements, ICC were higher in children with PCG than healthy children.

Comparison of corneal biomechanical properties of patients with dry eye secondary to Sjögren's syndrome and healthy subjects.

PURPOSE: The goal of this study is to determine whether any difference in corneal biomechanical properties exists between Sjögren's syndrome dry eye patients and healthy subjects. METHODS: Thirty-one patients diagnosed with Sjögren's syndrome and associated dry eye manifestations and 44 healthy individuals were included in the study. Ultrasonic pachymetry (UP) was used to measure central corneal thickness (CCT). Corneal biomechanical parameters were obtained using ocular response analyzer (ORA). The main parameters assessed were corneal hysteresis (CH), corneal resistance factor (CRF), Goldmann correlated intraocular pressure (IOPg) and corneal compensated IOP (IOPcc). A Student's t-test for independent groups was performed to compare the mean of these variables between both groups. RESULTS: Mean CH values in Sjögren's syndrome and healthy subject eyes were 10.1mmHg and 11.18mmHg respectively, representing a statistically significant difference (P=0.003). No other variable measured differed between cases and controls (P>0.05). Mean CRF values were 9.51mmHg and 10.37mmHg respectively, and mean CCT measured by UP in cases and controls was 527.41µm and 552.51µm respectively. CONCLUSIONS: Sjögren's syndrome can influence corneal biomechanical properties, specifically CH. ORA measurements should be considered of interest in the evaluation of Sjögren syndrome subjects.

Corneal Segmentation Analysis Increases Glaucoma Diagnostic Ability of Optic Nerve Head Examination, Heidelberg Retina Tomograph's Moorfield's Regression Analysis, and Glaucoma Probability Score.

Purpose. To study whether a corneal thickness segmentation model, consisting in a central circular zone of 1 mm radius centered at the corneal apex (zone I) and five concentric rings of 1 mm width (moving outwards: zones II to VI), could boost the diagnostic accuracy of Heidelberg Retina Tomograph's (HRT's) MRA and GPS. Material and Methods. Cross-sectional study. 121 healthy volunteers and 125 patients with primary open-angle glaucoma. Six binary multivariate logistic regression models were constructed (MOD-A1, MOD-A2, MOD-B1, MOD-B2, MOD-C1, and MOD-C2). The dependent variable was the presence of glaucoma. In MOD-A1, the predictor was the result (presence of glaucoma) of the analysis of the stereophotography of the optic nerve head (ONH). In MOD-B1 and MOD-C1, the predictor was the result of the MRA and GPS, respectively. In MOD-B2 and MOD-C2, the predictors were the same along with corneal variables: central, overall, and zones I to VI thicknesses. This scheme was reproduced for model MOD-A2 (stereophotography along with corneal variables). Models were compared using the area under the receiver operator characteristic curve (AUC). Results. MOD-A1-AUC: 0.771; MOD-A2-AUC: 0.88; MOD-B1-AUC: 0.736; MOD-B2-AUC: 0.845; MOD-C1-AUC: 0.712; MOD-C2-AUC: 0.838. Conclusion. Corneal thickness variables enhance ONH assessment and HRT's MRA and GPS diagnostic capacity.

Trombosis venosa retiniana en paciente joven con déficit de factor XII de la coagulación / Retinal venous thrombosis in a young patient with coagulation factor XII deficiency

CASO CLÍNICO: Mujer de 35 años enviada a la consulta por disminución de la visión en ojo izquierdo. En la oftalmoscopia se apreció una trombosis venosa de rama en ese mismo ojo. La paciente fue derivada a la unidad de hematología para estudio de trombofilia, siendo diagnosticada de déficit de factor XII de la coagulación o factor de Hageman. DISCUSIÓN: El desarrollo de fenómenos trombóticos oculares en pacientes sanos menores de 50 años debe hacer sospechar la presencia de trombofilia o estados de hipercoagulabilidad. El déficit de factor XII es poco frecuente y su presencia contribuye al desarrollo de procesos trombóticos

CASE REPORT: A 35-year-old woman, with no relevant medical history, was referred for sudden vision loss in the left eye. Ophthalmological examination showed best corrected visual acuity of 1.0 in the right eye and 0.3 in left eye, with normal anterior pole and intraocular pressure. Fundus examination of the left eye revealed a venous thrombosis in the superior temporal branch, with dilated and tortuous retinal veins. The patient was referred to the hematology unit for thrombophilia study, and was diagnosed with a coagulation XII or Hageman factor deficiency. DISCUSSION: THE development of retinal vessel occlusions, in patients under 50 years of age, is frequently associated with thrombophilia or hypercoagulability disorders. Factor XII deficiency is a rare condition, and its presence could contribute to a higher risk of thromboembolic events

Agreement between central corneal thickness measured using Pentacam, ultrasound pachymetry, specular microscopy and optic biometer Lenstar LS 900 and the influence of intraocular pressure.

PURPOSE: To compare central corneal thickness (CCT) values obtained by Lenstar (LE), Pentacam (PC), specular microscopy (SM) and ultrasound pachymetry (UP) in healthy corneas and study their influence on intraocular pressure (IOP) readings determined by Goldmann applanation tonometry (GAT). METHODS: CCT was measured in 76 healthy subjects by LE, PC, SM and UP. We established Lin's concordance correlation coefficient (ρ-C) between different techniques. The influence of CCT on GAT was established through univariate linear regression models, IOP being the dependent variable. RESULTS: The highest ρ-C was found between LE and SM at 0.94 (95% CI: 0.91-0.96) and between LE and UP at 0.95 (95% CI: 0.94-0.97). IOP readings showed less variability when CCT was determined using LE (7.7%, B = 0.16; 95% CI: 0.004-0.28). CONCLUSIONS: Although CCT values obtained with UP, PC, SM and LE show good correlation, these methods are not completely interchangeable. The amount of IOP variation differs when CCT is determined using LE or SM.

Retinal venous thrombosis in a young patient with coagulation factor XII deficiency.

CASE REPORT: A 35-year-old woman, with no relevant medical history, was referred for sudden vision loss in the left eye. Ophthalmological examination showed best corrected visual acuity of 1.0 in the right eye and 0.3 in left eye, with normal anterior pole and intraocular pressure. Fundus examination of the left eye revealed a venous thrombosis in the superior temporal branch, with dilated and tortuous retinal veins. The patient was referred to the hematology unit for thrombophilia study, and was diagnosed with a coagulation XII or Hageman factor deficiency. DISCUSSION: The development of retinal vessel occlusions, in patients under 50 years of age, is frequently associated with thrombophilia or hypercoagulability disorders. Factor XII deficiency is a rare condition, and its presence could contribute to a higher risk of thromboembolic events.

[Comparing central corneal thickness measured using ultrasound pachymetry and the Pentacam in healthy subjects and patients with primary open-angle glaucoma]. / Comparaison de l'épaisseur cornéenne centrale mesurée par pachymétrie ultrasonore et par Pentacam chez les patients sains et les patients avec glaucome primaire à angle ouvert.

PURPOSE: To determine the capacity for measurement of central corneal thickness by ultrasound pachymetry and by Pentacam (pachymetry at the pupillary axis and minimum corneal pachymetry) to discriminate between healthy controls and patients with primary open-angle glaucoma. METHODS: Measurements of the left eyes of 123 control subjects and 128 glaucomatous patients were taken. Differences between the two data sets for each variable were identified using a t-test for independent samples. Three univariate binary logistic regression models (to discriminate between glaucoma and controls) were constructed, in which the predictive variables were the determinations of corneal thickness. RESULTS: All variables showed a normal distribution. Controls and patients failed to differ significantly in terms of ultrasound pachymetry but did differ in terms of pupillary axis thickness (mean difference 22.01µm in favour of controls; 95% CI: 5.61-34.15) and minimum corneal thickness (mean difference 21.65µm in favour of controls; 95% CI: 3.05-32.25). The logistic regression model for ultrasound pachymetry was not significant (P=0.051) but significant discriminatory capacity was shown for pupillary axis thickness (expB=0.987; 95% CI: 0.99-0.993; sensitivity: 58.5%; specificity: 64.8%) and minimum corneal thickness (expB=0.987; 95% CI: 0.981-0.994; sensitivity: 61.9%; specificity: 63.1%). CONCLUSIONS: Central corneal thickness measurement by Pentacam, while not an ad hoc diagnostic test for glaucoma, showed a similar yet not inappreciable capacity to discriminate between glaucoma patients and controls.