Peripapillary Chorioretinal Atrophy Research Articles

Progression of Peripapillary and Macular Chorioretinal Atrophy in Multifocal Choroiditis Is Associated with Periatrophic Inflammatory Plumes.

To investigate peripapillary atrophy and macular chorioretinal scars in eyes affected by multifocal choroiditis and panuveitis (MCP). This retrospective cohort study reviewed the medical records, fundus photographs, and SD-OCT scans of 31 eyes from 19 patients. Patients had a mean age of 45 years (range 24-69 years). The average follow-up duration was 7 years (range 2.5-14.5 years), with 14 patients undergoing immunosuppressive treatment. In the group of 31 eyes, 20 showed peripapillary plumes of ill-defined hyperreflectivity at the termination border of the retinal pigment epithelium (RPE). These plumes, extending from bare Bruch's membrane to the outer nuclear layer, sometimes undermined the adjacent RPE. They responded to corticosteroid treatment and resembled the material under the RPE in acute lesions. Among 20 eyes with these peripapillary inflammatory lesions, 16 (80%) experienced increased atrophy, in contrast to none in the eyes without these lesions (P<0.001). Similar patterns were observed at the edges of macular chorioretinal atrophy. This observation occurred in patients using immunosuppressive medication who were otherwise thought to be under adequate control. In MCP patients, previously unrecognized plumes of smoldering inflammatory activity at the borders of chorioretinal atrophy appears to be linked to atrophy expansion. The recognition of this phenomenon may require a reappraisal of treatment of multifocal choroidopathies to help mitigate expansion of atrophy in these eyes.

Factors associated with intrachoroidal cavitation and sinkhole formation in eyes with glaucomatous visual-field defects

PurposeTo investigate factors associated with intrachoroidal cavitation (ICC) and sinkhole formation in eyes with glaucomatous visual-field defects.MethodsThis retrospective, cross-sectional study enrolled a total of 2808 eyes of 1482 patients who were diagnosed/treated for glaucoma and underwent swept-source optical coherence tomography (OCT). We first determined the prevalence of ICCs and sinkholes and their locations. Next, we selected one eye from each patient and compared the clinical characteristics of eyes with and without ICCs. Finally, in eyes with ICCs, we compared the clinical characteristics of eyes with and without sinkholes. Blood flow (BF), represented by laser speckle flowgraphy–measured tissue-area mean blur rate (MBR), was measured in the temporal optic nerve head (ONH), temporal peripapillary chorioretinal atrophy (PPA) zone, and in the ICC zone. ICC area and angle were analyzed in OCT en-face images. Mean deviation and total deviation in the central area (TD-central) were measured with Humphrey visual-field testing.ResultsA total of 86 eyes (3.1%) had ICCs and 52 eyes (1.9%) had sinkholes. ICC eyes had a lower spherical equivalent and longer axial length (AL) than non-ICC eyes (P < 0.05). Patients with eyes with sinkholes were more elderly and had worse best-corrected visual acuity, worse TD-central, a larger ICC, and lower tissue-area MBR in the temporal ONH, temporal PPA zone, and ICC zone (P < 0.05).ConclusionIn eyes with glaucoma, AL elongation might be linked to ICC formation. Sinkhole formation might be associated with ICC enlargement, impaired ocular BF, and impaired retinal structure and function involving the central area.

IDIOPATHIC MULTIFOCAL CHOROIDITIS WITH SERPIGINOUS-LIKE PERIPAPILLARY CHORIORETINAL ATROPHY.

To report nine cases of multifocal choroiditis with serpiginous-like peripapillary chorioretinal atrophy. A retrospective observational case series of eyes with multifocal choroiditis with serpiginous-like peripapillary chorioretinal atrophy. Multimodal imaging findings were reviewed and presented. Fifteen eyes of 9 patients (6 women and 3 men), with a mean age of 48.1 years (median, 46 years; range, 23-74 years), presented with multifocal choroiditis serpiginous-like peripapillary chorioretinal atrophy. All 15 eyes presented with serpiginoid peripapillary changes and had discrete patches of atrophy or punched-out scars in the posterior pole or periphery. Eleven eyes (73.3%) had cone-shaped retinal pigment epithelium elevations on optical coherence tomography, 10 eyes (66.7%) had mild vitritis, and 4 eyes (26.7%) had peripheral curvilinear streak lesions. Three eyes (20%) had choroidal neovascularization. All patients responded well to treatment with systemic immunosuppression, local corticosteroid injections, and/or intravitreal anti-vascular endothelial growth factor injections. Multifocal choroiditis may present with peripapillary chorioretinal changes resembling a serpiginous-like choroiditis in addition to the classic findings of patches of atrophy or punched-out scars in the posterior pole or periphery, cone-shaped retinal pigment epithelium elevated on optical coherence tomography and peripheral curvilinear streak lesions.

Posterior Segment Pathologies in Leprosy Patients with Visual Impairment: A Case Series

Leprosy is an important infectious disease, which is still prevalent in developing countries including Indonesia. This disease may affect the skin, extremities, peripheral nerves, and the eyes, causing disabilities of the patient. Leprosy is known to cause various ocular disorders, however, posterior segment abnormalities which involve the retina, choroid dan vitreous, have rarely been reported. Therefore, we present a case series of patients with posterior segment pathologies in visually impaired leprosy patients. These patients were identified during a community-based screening program on 99 leprosy patients, held in July 2019 in Alverno Hospital, Singkawang West Kalimantan. Patient comprised of inpatients and former patients who lived within the city and nearby cities who were invited for the program. All patients underwent comprehensive ophthalmology examination, performed by general ophthalmologists. Visual impairment was found in 15 patients, and their pupils were dilated, followed with posterior segment examination with indirect ophthalmoscopy and fundus photograph, using a handheld fundus camera, done by a vitreoretina specialist. Out of all patients screened, we did not find any leprosy-related posterior segment abnormalities. However, posterior segment pathologies were found in six patients, including peripapillary atrophy, myopic crescent, drusen and chorioretinal atrophy. One patient showed extensive chorioretinal atrophy with pigment clumping, which may be caused by leprosy- related chronic inflammatory process.

Factors associated with axial length elongation in high myopia in adults.

To investigate the association of axial length (AL) and ocular factors on AL elongation. A retrospective chart review of patients who underwent two or more AL examinations for more than two years. Totally 4 groups were divided according to initial AL (<24 mm, 24-26 mm, 26-28 mm, ≥28 mm). Initial fundus photograph was used to find risk factors associated AL elongation. The mean age of the patients was 47.21±7.79y. AL remained almost unchanged in the groups with AL<24 mm and 24≤AL<26 mm. On the contrary, AL increased by 0.011 mm/y in the group with 26≤AL<28 mm and 0.035 mm/y in the group with AL≥28 mm (P<0.001). In high myopia, AL elongation increased in eye with longer AL (r=0.003, P=0.024), female gender (r=0.014, P=0.019), eye with larger peripapillary chorioretinal atrophic area (r=0.002, P=0.019), and smaller vascular arcade angle (r=-0.004, P=0.006). The risk of elongation 0.03 mm/y in high myopia was increased in female gender (P=0.040), and gradually increased in eye with large peripapillary chorioretinal atrophy area (P<0.01). AL elongate significantly in the eye with longer AL, female gender, and the eye with larger atrophic area and smaller arcade angle on fundus photography.

The impact of optic disk morphological characteristics on peripapillary retinal nerve fiber layer thickness in non-glaucomatous subjects with high myopia

BackgroundTo analyze peripapillary retinal nerve fiber layer (pRNFL) thickness (pRNFLT) alterations in non-glaucomatous eyes with high myopia (HM), and to describe its relationship with some optic disk (OD) morphologic features. MethodsA cross-sectional case-control study includes 185 Caucasian subjects with HM and 122 healthy controls. Results of the pRNFLT analysis provided by optical coherence tomography were compared between the HM and control group, and the effects of some morphological features of OD, including tilt, OD radius, and peripapillary chorioretinal atrophy (pCRA) extension, on pRNFLT analysis were investigated. ResultsThe mean pRNFL of the HM group was significantly thinner than the control group in the inferior (p <0.001), superior (p <0.001), and nasal (p = 0.001) quadrants. In the HM group, the superior quadrant pRNFL was significantly thinner in the tilted OD subgroup compared to the non-tilted OD subgroup (p <0.001). The mean pRNFLT was negatively correlated with pCRA extension in the inferior (r =-0.209 and p = 0.020), superior (r =-0.308 and p <0.001), and nasal (r =-0.235 and p = 0.008) quadrants. ConclusionsIn non-glaucomatous Caucasian subjects with HM, pRNFL is thinner compared to controls in the inferior, superior, and nasal quadrants, and this thinning is negatively correlated with pCRA extension. Superior quadrant pRNFL is also thinner in tilted OD than non-tilted OD. The morphological characteristics of OD in non-glaucomatous eyes with HM are closely associated with the results of pRNFLT analysis.

Automatic identification of myopic maculopathy related imaging features in optic disc region via machine learning methods

BackgroundMyopic maculopathy (MM) is the most serious and irreversible complication of pathologic myopia, which is a major cause of visual impairment and blindness. Clinic proposed limited number of factors related to MM. To explore additional features strongly related with MM from optic disc region, we employ a machine learning based radiomics analysis method, which could explore and quantify more hidden or imperceptible MM-related features to the naked eyes and contribute to a more comprehensive understanding of MM and therefore may assist to distinguish the high-risk population in an early stage.MethodsA total of 457 eyes (313 patients) were enrolled and were divided into severe MM group and without severe MM group. Radiomics analysis was applied to depict features significantly correlated with severe MM from optic disc region. Receiver Operating Characteristic were used to evaluate these features’ performance of classifying severe MM.ResultsEight new MM-related image features were discovered from the optic disc region, which described the shapes, textural patterns and intensity distributions of optic disc region. Compared with clinically reported MM-related features, these newly discovered features exhibited better abilities on severe MM classification. And the mean values of most features were markedly changed between patients with peripapillary diffuse chorioretinal atrophy (PDCA) and macular diffuse chorioretinal atrophy (MDCA).ConclusionsMachine learning and radiomics method are useful tools for mining more MM-related features from the optic disc region, by which complex or even hidden MM-related features can be discovered and decoded. In this paper, eight new MM-related image features were found, which would be useful for further quantitative study of MM-progression. As a nontrivial byproduct, marked changes between PDCA and MDCA was discovered by both new image features and clinic features.

Long-term follow-up of a patient with JAG1-associated retinopathy

To report the long-term structural and functional changes in the posterior segments of an adult with an unusual retinal dystrophy caused by a novel mutation in JAG1. A 33-year-old female underwent comprehensive ophthalmic examination, including best corrected visual acuity (BCVA) measurement, dilated fundus imaging (wide-angle fundus colour and short wavelength autofluorescence imaging), macular and peripheral spectral-domain optical coherence tomography (SD-OCT) and electroretinography (ERG) at baseline and 10years later at the age of 43. The patient also underwent systemic review with detailed cardiac, brain and renal investigations. During follow-up, genetic analysis using whole-exome sequencing was performed on the patient and her parents to identify disease-causing variants. The patient's main complaint was of a recent onset of bilateral photophobia and blurred vision in the left eye. On examination, the most striking retinal finding was of bilateral well-demarcated, anterior circumferential chorioretinal atrophy with scattered pigment clumping from the mid periphery to the ora. In addition, she had posterior pole RPE hypopigmentation, peripapillary chorioretinal atrophy, left macular choroidal folds and retinal vasculature tortuosity with atypical branching. Her retinal electrophysiology was consistent with a cone rod photoreceptor dystrophy and left macular dysfunction. Ten years later, her BCVA, the anterior circumferential chorioretinal atrophy and her visual field constriction all remained stable. Her retinal electrophysiology demonstrated deterioration of left rod function, while cone dysfunction remained stable. Macular function deteriorated in both eyes. During follow-up, she was also noted to have progressive aortic root dilatation, posterior embryotoxon and an x ray diagnosis of butterfly vertebrae. Whole-exome sequencing revealed a novel c.2412C > A p.(Tyr804Ter) truncating mutation in JAG1 that was predicted to be pathogenic and suggested a diagnosis of Alagille syndrome. This is the first report of the long-term detailed follow-up of a patient with Alagille syndrome whose most striking ophthalmic finding was bilateral well-demarcated, anterior circumferential chorioretinal atrophy. During follow-up, this finding remained stable, suggesting that this may be developmental in origin. This is in contrast with the progressive deterioration in the posterior pole retinal and macular function.

Quantitative OCT angiography of the retinal microvasculature and choriocapillaris in highly myopic eyes with myopic macular degeneration

PurposeTo quantify retinal and choriocapillaris (CC) microvasculature in highly myopic (HM) eyes with myopic macular degeneration (MMD) using swept-source optical coherence tomography angiography (SS-OCTA).Methods162 HM eyes (spherical equivalent ≤ −6.0...

Progression in Open-Angle Glaucoma with Myopic Disc and Blood Flow in the Optic Nerve Head and Peripapillary Chorioretinal Atrophy Zone

To investigate the effect of blood flow in the temporal optic nerve head (ONH) and peripapillary chorioretinal atrophy (PPA) zone on central visual field (VF) defects and progression in eyes with open-angle glaucoma (OAG) and myopic disc. Retrospective longitudinal medical chart review. This study comprised 366 eyes of 245 OAG patients with myopic disc, followed for at least 2 years with at least 5 reliable VF tests. OCT and laser speckle flowgraphy (LSFG) were performed at baseline. We analyzed the relationship between temporal ONH-tissue mean blur rate (MBR), temporal PPA-tissue MBR, total deviation (TD)-central, and TD-central slope with a linear mixed-effects model. Additionally, we investigated background factors influencing temporal PPA-tissue MBR. Main outcome measures were basic ophthalmic and systemic variables, baseline ONH-tissue MBR, baseline PPA-tissue MBR, baseline TD, and TD slope. Lower temporal ONH-tissue MBR was associated with both worse TD-central and faster TD-central slope (β= 0.30, P < 0.001; β= 0.18, P= 0.001, respectively). However, lower temporal PPA-tissue MBR was only associated with faster TD-central slope (β= 0.15, P= 0.005). Lower ONH-tissue MBR and lower PPA-tissue MBR were significant independent contributors to worse TD-central slope, after adjusting for potential confounding factors (β= 0.12 to 0.15, P < 0.05). The multivariate analysis showed that lower pulse rate, larger temporal PPA area, and lower circumpapillary retinal nerve fiber layer thickness were associated with lower PPA-tissue MBR (P < 0.05). Measurement of systemic variables and LSFG analysis might help clinicians to predict centralVF defect severity and progression in OAG eyes with myopic disc.

This issue of ACTA

This issue of ACTA

Factors associated with deep circulation in the peripapillary chorioretinal atrophy zone in normal-tension glaucoma with myopic disc.

To investigate factors associated with choroidal microcirculation in the peripapillary chorioretinal atrophy (PPA) zone in eyes with normal-tension glaucoma (NTG) and myopic disc. In 100 eyes of 100 NTG patients with myopic disc and 20 eyes of 20 age-matched myopic controls, 4.5×4.5mm scans were made of the optic nerve head with optical coherence tomography angiography (OCTA). Peripapillary chorioretinal atrophy (PPA) area and PPA superficial choroidal image intensity (PPA-CI) were calculated with image j software. Clinical characteristics, laser speckle flowgraphy-measured mean blur rate in the temporal tissue area (temporal MT), the central thresholds (the averaged standard automated perimetry-measured visual field thresholds in the four paracentral points) and 3D-OCT-measured ganglion cell complex thickness in the papillomacular bundle (PMB-GCCT) were recorded. The NTG patients had significantly lower intraocular pressure, PMB-GCCT and central threshold values, and a larger PPA area, than the controls. The area under the receiver operating characteristics curve to differentiate NTG with parafoveal scotoma (PFS) from controls was 0.76 for temporal MT, 0.85 for PPA-CI and 0.87 for PMB-GCCT. Univariate and multivariate analyses revealed that PPA-CI was negatively correlated with age, pulse rate, best-corrected visual acuity, axial length and PPA area, and positively correlated with temporal MT, PMB-GCCT and the central thresholds. Peripapillary chorioretinal atrophy (PPA)-CI was associated with temporal MT, ageing, bradycardia, axial length elongation and changes in central retinal structure and visual function in patients with NTG and myopic disc. Thus, microcirculation deep within the PPA zone might be a clinically useful biomarker of PFS in NTG.

Peripapillary Atrophy in High Myopia

ABSTRACTPurpose: To evaluate peripapillary atrophy (PPA) in high myopia (HM) as a function of extent of myopia, axial length (AL), and age.Methods: Medical records from patients (n = 88) seen in the Ophthalmology Department of the Third Affiliated Hospital of Nanchang University, Nanchang, Jiangxi, China, and diagnosed with high myopia (HM)—defined as spherical equivalent (SE) of >6.00 diopters (D) of myopia in either eye—were included in this study. Eyes were classified according to SE and age. For the SE classification, individual eyes (n = 169) were arranged as follows: groups A1 (SE −6.00to −9.00D), A2 (−9.25 to −16.50D), and A3 (−16.75 to −27.00D). For the age classification, eyes (n = 154) were arranged as follows: groups B1 (5–18 years old), B2 (18.1–40 years old), and B3 (40.1–69 years old). Outcome measures included correlation of areas of optic nerve head (ONH), PPA, and peripapillary chorioretinal atrophy (PCA) with SE, age, and AL. Morphological parameters were measured using the 1000-edition program of 3D-OCT 2000 (http://eyecare.topcon.com.cn/index.php?optionid = 748&autoid = 8) clinical auto-analyzer module.Results: Average areas—ONH (PPA, PCA)—in groups A1, A2, and A3 were 1.95 (2.05, 0.42), 2.42 (4.44, 1.39), and 2.52 (7.19, 3.64) mm2, respectively; average areas were 1.99 (1.40, 0.03), 2.49 (4.68, 1.92), and 2.28 (5.99, 2.78) mm2 in groups B1, B2, and B3, respectively. There were statistically significant differences among groups A1, A2, and A3 (F = 6.03, 18.91, 13.86, all p < 0.01), and among B1, B2, and B3 (F = 5.46, 21.89, 13.06, all p < 0.01). ONH correlated only with SE (r = −0.30, p < 0.01). PPA and PCA correlated with SE, age, and AL (PPA: r = −0.53, 0.47, 0.61; PCA: r = −0.42, 0.46, 0.48; all p < 0.01).Conclusions: PPA and PCA correlated with SE, age, and AL; ONH correlated with SE, may be with age.

Optic disc, rim and peripapillary chorioretinal atrophy in normal Japanese eyes: the Kumejima Study.

To report reference data for optic disc, neuroretinal rim and β-peripapillary atrophy (β-PPA) in normal Japanese. In the Kumejima study, all participants underwent disc stereo-photography; 2208 normal subjects had good-quality disc stereo and β-PPA photographs OU. The disc and rim area and the area and angular extent of β-PPA were evaluated by computer-assisted planimetry. The disc, rim, and β-PPA area and its angular extent OD (OS) averaged 2.54±0.50 (2.53±0.49), 1.67±0.29 (1.67±0.30), 0.47±0.66 (0.42±0.59)mm2, and 99±85 (92±82) degrees (mean±standard deviation) with bilateral Pearson's correlation coefficient of 0.890, 0.769, 0.679 and 0.632 (P<0.001), respectively, while the 95th percentiles of the absolute inter-eye differences were 0.48, 0.41, 0.96mm2, and 153°. The correlation coefficient between β-PPA area and its angular extent was 0.963 (P<0.001). Age showed negative correlation with the rim area and positive correlation with β-PPA area (P<0.001). Among ocular factors, disc area showed positive correlation and intraocular pressure negative correlation with the rim area (P≤0.001), axial length and disc area positive correlation with β-PPA area (P<0.001 and P=0.051). The averages and distribution of the optic disc and rim area, β-PPA area and its angular extent in normal Japanese are reported, which will facilitate screening of glaucoma in Japanese. In normal Japanese eyes, the disc size and intraocular pressure significantly correlated with the rim area, and the axial length with β-PPA area.

Clinical and Genetic Features of Choroideremia in Childhood

To review the functional and anatomic characteristics of choroideremia in the pediatric population, aiming to describe the earliest features of the disease and to identify biomarkers useful for monitoring disease progression. Retrospective case series. Children diagnosed with choroideremia at a single institution. Patients were identified using an electronic patient record system. Case notes and retinal imaging (color fundus photography [CFP], spectral-domain [SD] optical coherence tomography [OCT], and fundus autofluorescence [FAF]) then were reviewed. The results of genetic testing also were recorded. Presenting symptoms, visual acuity, fundus changes (CFP, SD OCT, FAF), and CHM sequencing results. Twenty-nine patients were identified with a mean age at referral of 9 years (range, 3-16 years). CHM mutations were identified in 15 of 19 patients tested. Nyctalopia was the predominant symptom (66%). Five of 29 patients were asymptomatic at presentation. At the final follow-up visit (mean age, 16 years; range, 7-26 years), most maintained excellent visual acuity (mean, 0.98±0.13 decimalized Snellen acuity). The first sign of retinopathy was widespread pigment clumping at the level of the retinal pigment epithelium (RPE). This later evolved to chorioretinal atrophy, most marked in the mid-peripheral retina. Peripapillary atrophy also was an early feature and was progressive in nature. Three different zones of FAF change were visible. Persistence of the inner retinal layers, detected by SD OCT, was visible at presentation in 15 of 27 patients. Subfoveal choroidal thickness decreased with age, whereas central retinal thickness increased over a similar interval. Four patients in whom visual acuity decreased over the follow-up period recorded a reduction in central retinal thickness. Progressive structural changes occur at a time when central visual function is maintained. Pigmentary changes at the level of the RPE occur early in the disease course. Peripapillary chorioretinal atrophy, central retinal thickness, and subfoveal choroidal thickness are likely to be valuable in monitoring disease progression and should be considered as potential biomarkers in future therapeutic trials.

Peripapillary Diffuse Chorioretinal Atrophy in Children as a Sign of Eventual Pathologic Myopia in Adults

To search for a morphologic biomarker to differentiate between pathologic myopia and simple childhood myopia. Retrospective case series. The study included children (age ≤15 years) with high myopia (as defined by the Japanese Ministry of Health and Welfare) who attended the High Myopia Clinic between April 1982 and March 1994, had undergone fundus photography, and had a follow-up of 20 years or more. Fundus photographs obtained in childhood and adulthood were examined for presence of pathologic myopia, defined by high myopia (myopic refractive error >8 diopters or axial length ≥26.5 mm) and the presence of stage 2 or higher myopic maculopathy. Myopic maculopathy in childhood. The study included 56 eyes of 29 patients with a mean age of 10.2±3.6 years at the initial visit and an age of 36.0±7.6 years at the last visit. Mean axial length was 27.0±1.4 mm at baseline and 29.7±2.0 mm at the last visit. At the last visit, 19 eyes (34%) had tessellated fundus alone, 31 eyes (55%) had diffuse chorioretinal atrophy, 3 eyes (5%) showed patchy chorioretinal atrophy, and 1 eye (2%) had macular atrophy. Thus, 35 eyes (63%) had pathologic myopia in adulthood. Among the 35 eyes, 29 (83%) already had diffuse chorioretinal atrophy at the initial visit in childhood and the remaining 6 eyes (17%) showed tessellated fundus in childhood. The diffuse chorioretinal atrophy seen in childhood was restricted to the area temporal to the peripapillary region. The presence of peripapillary diffuse chorioretinal atrophy in children with high axial myopia may be an indicator for the eventual development of advanced myopic chorioretinal atrophy in later life. These features in children may be helpful for differentiating simple childhood myopia from eventual pathologic myopia.

Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: A case report

Clinical caseA five-year-old patient, with a diagnosis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency, was referred for an ophthalmological examination. He had a history of acute metabolic crises precipitated by intercurrent infections, as well as rhabdomyolysis. The fundoscopic examination revealed a peripapillary chorioretinal atrophy and a diffuse granular appearance of the macular retinal pigment epithelium. Best corrected visual acuity was 6/6 in both eyes, and he had a normal electroretinography test. DiscussionWe perform a review of the literature and recent findings in relation to this disease through the description of a clinical case in order to improve the knowledge of this uncommon disorder.

Déficit de 3-hidroxiacil-CoA-deshidrogenasa de cadena larga: a propósito de un caso

Clinical caseA five-year-old patient, with a diagnosis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency, was referred for an ophthalmological examination. He had a history of acute metabolic crises precipitated by intercurrent infections,as well as rhabdomyolysis. The fundoscopic examination revealed a peripapillary chorioretinal atrophy and a diffuse granular appearance of the macular retinal pigment epithelium. Best corrected visual acuity was 6/6 in both eyes, and he had a normal electroretinography test. DiscussionWe perform a review of the literature and recent findings in relation to this disease through the description of a clinical case in order to improve the knowledge of this uncommon disorder.

Myopia and diabetes mellitus as modificatory factors of glaucomatous optic neuropathy

Myopic deformation of the eye and metabolic alterations of the nerve tissue of patients with diabetes may modify glaucomatous optic neuropathy (GON). Blockage of axonal transport of neurotrophic factors (NTFs) is the event crucial to understanding the factors that affect GON. The primary, but not sole, blockage site is at the lamina cribrosa (LC). Other than this primary site of damage at the LC, 7 other factors may explain atypical nerve fiber layer (NFL) defects and the vulnerability of the nerve fibers in eyes with high myopia and glaucoma: a second point of blockage at the edge of the posterior scleral foramen; ectatic strain on the NFL; ectasia and distortion of the LC; association of a hypoplastic optic disc; thin and weak collagen fibers; peripapillary chorioretinal atrophy; and myopic neuropathy. Among diabetic patients, diabetic neuropathy in the retinal NFL is present initially, and increased resistance to aqueous outflow leads to ocular hypertension. Superimposition of GON on diabetic neuropathy and ocular hypertension in patients with diabetes may enhance their susceptibility to nerve damage. Results of a meta-analysis study suggested a positive association between diabetes mellitus and glaucoma whereas other reports suggested that leakage of vascular endothelial growth factor, a survival mechanism of ischemic neural tissue, and enhanced stiffness of the LC as a result of diabetic glycation may protect neurons from apoptosis. Thus, modification of GON as a result of diabetes remains controversial.

Evaluation of retinal nerve fiber layer thickness parameters in myopic population using scanning laser polarimetry (GDxVCC)

Myopia presents a significant challenge to the ophthalmologist as myopic discs are often large, tilted, with deep cups and have a thinner neuroretinal rim all of which may mimic glaucomatous optic nerve head changes causing an error in diagnosis. To evaluate the retinal fiber layer (RNFL) thickness in low, moderate and high myopia using scanning laser polarimetry with variable corneal compensation (GDxVCC). One hundred eyes of 100 emmetropes, 30 eyes of low myopes (0 to - 4 D spherical equivalent(SE), 45 eyes with moderate myopia (- 4 to - 8D SE), and 30 eyes with high myopia (- 8 to - 15D SE) were subjected to retinal nerve fiber layer assessment using the scanning laser polarimetry (GDxVCC) in all subjects using the standard protocol. Subjects with IOP > 21 mm Hg, optic nerve head or visual field changes suggestive of glaucoma were excluded from the study. The major outcome parameters were temporal-superior-nasal-inferiortemporal (TSNIT) average, the superior and inferior average and the nerve fibre indicator (NFI). The TSNIT average (p = 0.009), superior (p = 0.001) and inferior average (p = 0.008) were significantly lower; the NFI was higher (P less than 0.001) in moderate myopes as compared to that in emmetropes. In high myopia the RNFL showed supranormal values; the TSNIT average, superior and inferior average was significantly higher(p less than 0.001) as compared to that in emmetropes. The RNFL measurements on scanning laser polarimetry are affected by the myopic refractive error. Moderate myopes show a significant thinning of the RNFL. In high myopia due to peripapillary chorioretinal atrophy and contribution of scleral birefringence, the RNFL values are abnormally high. These findings need to be taken into account while assessing and monitoring glaucoma damage in moderate to high myopes on GDxVCC.