Retinal Pigment Epithelium Lesions Research Articles

Real-time OCT feedback-controlled RPE photodisruption in ex vivo porcine eyes using 8 microsecond laser pulses.

Selective retinal pigment epithelium (RPE) photodisruption requires reliable real-time feedback dosimetry (RFD) to prevent unwanted overexposure. In this study, optical coherence tomography (OCT) based RFD was investigated in ex vivo porcine eyes exposed to laser pulses of 8 µs duration (wavelength: 532 nm, exposure area: 90 × 90 µm2, radiant exposure: 247 to 1975 mJ/µm2). For RFD, fringe washouts in time-resolved OCT M-scans (central wavelength: 870 nm, scan rate: 85 kHz) were compared to an RPE cell viability assay. Statistical analysis revealed a moderate correlation between RPE lesion size and applied treatment energy, suggesting RFD adaptation to inter- and intraindividual RPE pigmentation and ocular transmission.

Pigment epithelium and choroid changes in pachychoroid pigment epitheliopathy: A descriptive study

AbstractPurpose: To describe characteristic elementary lesions of retinal pigment epithelium and choroidal vascular parameters determined by swept source optical coherence tomography (SS‐OCT) and OCT‐angiography (OCT‐A) in eyes with pachychoroid pigment epitheliopathy.Methods: Twenty‐seven patients with retinal pigment epithelium (RPE) abnormalities associated to pachychoroid features, with no history or findings indicative of subretinal fluid or soft drusen were evaluated. All eyes underwent comprehensive ophthalmic examination, fundus photography, SS‐OCT and OCT‐A. After binarization of B‐scan images with ImageJ Software, the choroidal vascularity index (CVI), the choroidal vascularity proportion (CVP) and flow voids area were calculated.Results: A total of 27 eyes of 23 patients, 19 of whom were male (70.37%), with a mean age of 59.11 ± 12.76 years were enrolled in this study. We detected six different types of RPE lesions: focal disruption in ellipsoid and interdigitation zones in 18 eyes (66.66%), RPE elevation with microbreak appearance in 12 eyes (44.44%), RPE thickening in 10 eyes (37.03%), small pigment epithelium detachment in seven eyes (25.92%), RPE spike in five eyes (18.51%) and outer nuclear layer thinning in five eyes (18.51%). The subfoveal choroidal thickness (SFCT) was 399, 78 ± 88, 13 μm. The mean inner choroidal thickness was 87.18 ± 36.67 μm. The CVI was 65.28 ± 3.35%. The CVP was 59.10 ± 0.58% in the choriocapillaris and 59.69 ± 1.42% in the Haller layer. The total choriocapillaris flow void area was 39.87 ± 1.21%.Conclusions: SS‐OCT and OCT‐A are valuable diagnostic tools to characterize pigment epithelium and choroidal changes in PPE eyes.

Color Doppler imaging characteristics of RPE neoplasms: A study of 17 cases

PurposeTo report the color Doppler imaging (CDI) features of 17 patients with retinal pigment epithelium (RPE) neoplasms.DesignRetrospective, observational case series.ParticipantsSeventeen patients with RPE adenoma/adenocarcinoma.MethodsContrast-enhanced color Doppler ultrasonography (CDU) was performed in 16 patients with RPE adenoma and in one patient with adenocarcinoma.ResultAll 17 RPE neoplasms showed well-defined margins. An oval mass was observed in 11 tumors, a lenticular-shaped mass in four tumors, and a flat mass in two tumors. On CDI, RPE lesions showed homogeneous reflectivity of moderate intensity with no choroidal excavation. After the contrast-enhanced administration of sulfur hexafluoride, mild enhancement was identified in six tumors and moderate enhancement in 11 tumors. In all 17 RPE neoplasms, contrast-enhanced CDU time–intensity curves (TICs) exhibited fast fill-in and slow washout-shaped curves with the dominant vessel within RPE lesions. A “black-linear” sign (defined as a low signal intensity-linear zone located between the tumor and enhanced choroid on CDI) was noted in all 17 patients with RPE neoplasms.ConclusionsThis preliminary study noted a “black-linear” sign on CDI of RPE lesions for the first time. The novel sign may be a diagnostic characteristic of RPE neoplasms and may help to distinguish this rare entity from uveal melanomas (UMs).

Benign Lobular Inner Nuclear Layer Proliferations of the Retina Associated with Congenital Hypertrophy of the Retinal Pigment Epithelium

To report the clinical and imaging findings of 4 patients with benign intraretinal tumors, 2 of which were associated with retinal pigment epithelium (RPE) hypertrophy. To our knowledge, this condition has not been described previously and should be distinguished from retinoblastoma and other malignant retinal neoplasms. Retrospective case series. Four patients from 3 institutions. Four patients with intraretinal tumors of the inner nuclear layer (INL) underwent a combination of ophthalmic examination, fundus photography, fluorescein angiography, OCT, OCT angiography, and whole exome sequencing. Description of multimodal imaging findings and systemic findings from 4 patients with benign intraretinal tumors and whole exome studies from 3 patients. Six eyes of 4 patients 5, 13, 32, and 27 years of age were found to have white intraretinal tumors that remained stable over the follow-up period (range, 9 months-4 years). The tumors were unilateral in 2 patients and bilateral in 2 patients. The tumors were white, centered on the posterior pole, and multifocal, with some consisting of multiple lobules with arching extensions that extended beyond the central tumor mass. OCT demonstrated these lesions to be centered within the INL at the border of the inner plexiform layer. In addition, 2 patients demonstrated congenital hypertrophy of the RPE (CHRPE) lesions. Three of 4 patients underwent whole exome sequencing of the blood that revealed no candidate variants that plausibly could account for the phenotype. We characterize a novel benign tumor of the INL that, in 2 patients, was associated with separate CHRPE lesions. We propose the term benign lobular inner nuclear layer proliferation to describe these lesions. Proprietary or commercial disclosure may be found after the references.

A Pigmented Fundus Lesion with Pseudohypopyon and Subretinal Fluid Resembling Unilateral Retinal Pigment Epithelium Dysgenesis (URPED).

To describe multimodal imaging findings in a patient with a rare, symptomatic fundus lesion arising from the retinal pigment epithelium (RPE). Case report. A 36-year-old female presented with photopsia in her left eye. Funduscopy revealed an 8 × 7 mm, dark brown lesion at the level of the RPE inferior to the macula. The lesion had an irregular, cauliflower-like border and a light grey subretinal pseudohypopyon. On fundus autofluorescence, the lesion was markedly hypoautofluorescent with an irregular hyperautofluorescent margin. It was generally hyopofluorescent on fluorescein angiography and moderately hypofluorescent on indocyanine green angiography. Spectral-domain optical coherence tomography revealed a fine layer of subretinal fluid over the entire lesion, thinning of the outer retinal layers with loss of photoreceptors, and an irregular RPE. Multiple drusen-like subretinal deposits were located along the lesion margin, and inferiorly, the pseudohypopyon appeared as a hyperreflective subretinal mass. During three years of follow-up, her symptoms remained unchanged and fundus photography showed minimal enlargement of the lesion. Multimodal imaging findings of a solitary pigmented RPE lesion with pseudohypopyon and subretinal fluid are shown. The lesion may represent an atypical variant of unilateral retinal pigment epithelium dysgenesis.

A Pigmented Fundus Lesion with Pseudohypopyon and Subretinal Fluid Resembling Unilateral Retinal Pigment Epithelium Dysgenesis (URPED).

To describe multimodal imaging findings in a patient with a rare, symptomatic fundus lesion arising from the retinal pigment epithelium (RPE). Case report. A 36-year-old female presented with photopsia in her left eye. Funduscopy revealed an 8 × 7 mm, dark brown lesion at the level of the RPE inferior to the macula. The lesion had an irregular, cauliflower-like border and a light grey subretinal pseudohypopyon. On fundus autofluorescence, the lesion was markedly hypoautofluorescent with an irregular hyperautofluorescent margin. It was generally hyopofluorescent on fluorescein angiography and moderately hypofluorescent on indocyanine green angiography. Spectral-domain optical coherence tomography revealed a fine layer of subretinal fluid over the entire lesion, thinning of the outer retinal layers with loss of photoreceptors, and an irregular RPE. Multiple drusen-like subretinal deposits were located along the lesion margin, and inferiorly, the pseudohypopyon appeared as a hyperreflective subretinal mass. During three years of follow-up, her symptoms remained unchanged and fundus photography showed minimal enlargement of the lesion. Multimodal imaging findings of a solitary pigmented RPE lesion with pseudohypopyon and subretinal fluid are shown. The lesion may represent an atypical variant of unilateral retinal pigment epithelium dysgenesis.

A Review of Central Serous Chorioretinopathy: Clinical Presentation and Management.

Central serous chorioretinopathy (CSC) may be understood as a disease of the chorioretina with the serous detachment of the neurosensory retina, which is secondary to single or multiple localized defects in retinal pigment epithelium (RPE). CSC is one of the common forms of loss of vision, usually seen in people who do belong to the working-age group. The most common symptoms are blurring of vision, usually unilateral and which is perceived as a scotoma in the center of the field of vision with associated metamorphopsia and micropsia. The risk factor associated with CSC is psychosocial stress, type A personality, pregnancy, and hypercortisolism. Normal vision is often restored within a span of a few months. After around three months, if the resolution of acute CSC did not change or, let us say, in the case of CSC that is chronic, one should consider treatment. In acute CSC, to resolve symptoms, especially in individuals who work in a field where eyesight is of utmost importance, for example, pilots, focal photocoagulation of leaking RPE lesions can be performed. CSC is a prototype cause of serous neuroretinal detachment, which involves the fovea. CSC symptoms reflect the separation between the RPE and the photoreceptors and the bullous distension of the foveal retina. The effect of therapy as such on the long-term outcome of vision visual is not sufficiently documented. The management would largely be dependent on the appropriate diagnosis made based on clinical presentations, and thus it becomes very much necessary to have knowledge about the same and counsel the patient regarding the association between stress and disease pathology. In acute CSC, retinal photocoagulation is successful to a good extent in eliminating or reducing the leakage of RPE and hence it induces resolution of the serous detachment. This review article is made to make sure the reader is updated about the various clinical and management aspects of CSC by providing a comprehensive idea that is obtained from various well-acknowledged databases across the globe on CSC.

Mitochondrial Transplantation as a Novel Therapeutic for Retinal Metabolic Dysfunction‐Driven Disease

Metabolic dysfunction and mitochondria defect are implicated in several age‐associated diseases including age‐related macular degeneration (AMD), a leading cause of blindness in the elderly. In AMD, mitochondrial oxidative stress in the retinal pigment epithelium (RPE) drives disease progression and growth of atrophic lesions. Mitochondria release and uptake has been recently identified as a novel mechanism of intercellular communication but its implication in ocular diseases such as AMD has never been investigated. Here, we examined the role of mitochondrial transfer as a new mechanism of metabolic crosstalk between RPE cells. Diseased mitochondria were purified from RPE cells treated with the AMD‐associated cytokine TNFα (10 ng/mL), administered to host RPE cells and the effects of MitoTNFA compared to MitoCtrl, isolated from control RPE, or exposure to exogenous TNFα. We showed that treatment of healthy RPE with MitoTNFA, and not MitoCtrl, triggers mitochondrial network fragmentation and transcriptional upregulation of inflammatory factors RelB, IL6, IL8, and repression of PGC1α, mirroring the effect of direct TNFα treatment. ELISA assay confirmed that the effects observed were not caused by presence of soluble TNFα within the mitochondrial fraction. Metabolic profiling using the Seahorse XFe96 bioanalyzer further validated the ability of MitoTNFA to phenocopy TNFα‐induced metabolic reprograming in RPE. Finally, we demonstrated that transfer of MitoCtrl improved the bioenergetic functions of both healthy and diseased RPE with significant increases in basal, maximal, and spare respiratory functions. Our results showed that mitochondria transfer recapitulates the context‐specific phenotype from donor to host cells in RPE. This new paradigm in RPE biology may not only explain the centrifugal expansion of RPE lesions in AMD but also presents a promising therapeutic avenue for mitochondria‐driven disorders such as AMD.

Efficacy of Navigated Laser Photocoagulation for Chronic Central Serous Chorioretinopathy: A Retrospective Observational Study.

Background. No consensus has been reached regarding the management of central serous chorioretinopathy (CSCR). We aimed to investigate the efficacy of navigated laser treatment for chronic CSCR and changes in the morphology of the retinal pigment epithelium (RPE). In this retrospective observational study, 19 patients with subjective symptoms admitted to the Nanjing Medical University Affiliated Eye Hospital were included between January 2021 and August 2021. All patients underwent visual acuity and optical coherence tomography (OCT) examination during follow-up. Fluorescein angiography (FA) was performed at baseline and at the final follow-up to confirm the dye leakage sites. The mean logMAR BCVA at baseline and at the end of follow-up was 0.49 ± 0.24 and 0.24 ± 0.15, respectively. The mean logMAR BCVA 3 months after treatment was significantly better than that before treatment (p = 0.002). Significant improvements were observed in central macular thickness (CMT) and subfoveal choroidal thickness (SFCT) after navigated laser photocoagulation (p < 0.0001). Subretinal fluid was completely resolved in 13 (68%) of 19 eyes at 3 months. RPE lesions on OCT images showed RPE detachment in 17 eyes (61.5%), small protrusion of the RPE layer in 5 eyes (7.5%), and a rough RPE layer in 4 eyes (31%). After laser treatment, 9 points (35%) showed retinal detachment, and 10 (38.5%) regions showed an irregular RPE layer. An irregularly protruded RPE layer was also observed in different regions of the leakage spot in 5 (19%) eyes, and RPE defects were seen in 2 (7.5%) eyes. Navigated laser photocoagulation for chronic CSCR can achieve substantial anatomical and visual improvement. OCT outcomes may provide new information to facilitate understanding of the mechanism of chronic CSCR. Navigated laser photocoagulation should be chosen as an optimal treatment option in patients with chronic CSCR who cannot afford photodynamic therapy.

Pentosan Polysulfate Maculopathy: Prevalence, Spectrum of Disease, and Choroidal Imaging Analysis Based on Prospective Screening

Purpose: To describe the prevalence and spectrum of disease of pentosan polysulfate (PPS) maculopathy in a large multimodal retinal imaging study and to report the results of choroidal vascularity index (CVI) analysis.Design: Prospective cohort studyMethods: Of 741 patients prescribed PPS within a large university database, 100 (13.4%) with any consumption agreed to participate in a prospective screening investigation. Multimodal retinal imaging including near-infrared reflectance (NIR), fundus autofluorescence (FAF), and spectral domain optical coherence tomography (SD-OCT) was performed in all patients. Characteristic findings of affected patients were identified, and affected and unaffected cohorts were compared. CVI, defined as stromal choroidal area (SCA) divided by the total choroidal area, was analyzed.Results: The prevalence of PPS maculopathy was 16%. NIR illustrated punctate hyperreflective lesions with early presentation. FAF illustrated a speckled macular network of hypo- and hyperautofluorescence colocalized with multifocal hyperreflective retinal pigment epithelial lesions on SD-OCT. Advanced cases demonstrated varying degrees of atrophy. The affected cohort exhibited significantly greater mean PPS therapy duration, mean daily dosage, and mean cumulative dosage (19.5±5.5 years, 433.9±137.6 mg, 3,103.1±1,402.2 g) compared with the unaffected cohort (7.1±6.6 years, 291.6±177.6 mg, 768.4±754.8 g). SCA was significantly lower and CVI was significantly greater in the affected vs the unaffected group.Conclusions: This prospective cohort study identified a prevalence of PPS maculopathy of 15%-20% among PPS users who agreed to participate. A spectrum of findings may be observed with multimodal retinal imaging. Significant choroidal abnormalities associated with this characteristic maculopathy may provide surrogate markers of macular toxicity.

Evaluation of retinal pigment epithelium changes in serous pigment epithelial detachment in age-related macular degeneration

The purpose of this study was to quantitatively evaluate retinal pigment epithelium (RPE) changes in serous pigment epithelial detachment (PED) among patients with age-related macular degeneration by means of prototype multi-contrast optical coherence tomography (OCT), which is capable of simultaneous collection of OCT angiography, polarization-sensitive OCT, and standard OCT images. We evaluated 26 eyes of 21 patients with serous PED. RPE-melanin OCT images were calculated from the multi-contrast OCT dataset and compared with near-infrared autofluorescence images. An active RPE lesion was defined as an area of thickened RPE-melanin (≥ 70 μm; RPE70) on RPE-melanin OCT. Each PED area was divided into peak and slope regions. RPE70 area ratios were compared with the maximum PED height, PED area, PED volume, and slope area ratio (area of slope region/area of whole PED). RPE-melanin OCT images were consistent with near-infrared autofluorescence images. The RPE70 area ratio in the slope region was significantly negatively correlated with the slope area ratio. Development of active RPE lesions in the slope region was correlated with the PED configuration. Multi-contrast OCT is useful for objective evaluation of changes in the RPE in patients with age-related macular degeneration.

CONGENITAL HYPERTROPHY OF RETINAL PIGMENT EPITHELIUM (CHRPE) WITH TYPICAL ‘BEAR TRACK’ PRESENTATION

Congenital hypertrophy of retinal pigment epithelium (CHRPE) with typical ‘bear track’ presentationFamilial adenomatous polyposis (FAP) is an autosomal dominant condition caused by a mutation in APC tumor suppressor gene (chromosome 5q21).Affected patients have variable number of adenomatous polyps, mostly in the large intestine, that have nearly 100 percent progression to colorectal cancer with extracolonic manifestations such as desmoid tumours, osteomas, dental anomalies, and soft-tissue tumours.Solitary congenital hypertrophy of retinal pigment epithelium (CHRPE) and its multi focal variant, congenital grouped pigmentation (bear tracks), are isolated ophthalmic entities and are not associated with FAP and Gardner syndrome. Retinal pigment epithelium lesions seen with FAP are ovoid ,multifocal , bilateral with irregular borders, haphazardly distributed with peripheral location often with a white tail of depigmentation at margins.Patients found to have solitary or multifocal CHRPE on ophthalmoscopy can be counselled that they do not have an increased risk of colon cancer compared with that of the general population.

ASSOCIATION BETWEEN GENOTYPE AND DISEASE PROGRESSION IN ITALIAN STARGARDT PATIENTS: A Retrospective Natural History Study.

To investigate the natural history of Stargardt disease over a multiyear follow-up. We reviewed medical records of Stargardt disease patients, with clinical diagnosis of Stargardt disease at a single institution, which was also supported by molecular diagnosis. All patients underwent best-corrected visual acuity, fundus photography, optical coherence tomography, and full-field electroretinography. The study cohort consisted of 157 Stargardt disease patients aged 30.4 ± 1.1 years. Longitudinal analysis (mean follow-up: 3 years) showed a significant worsening of best-corrected visual acuity at an average rate of 1.5 Early Treatment Diabetic Retinopathy Study letters/year (P < 0.001), an enlargement of retinal pigment epithelium lesion area by optical coherence tomography at an average linear rate of 0.10 mm/year (P < 0.001), and a thinning of central macular thickness at a mean rate of -1.42 μm/year (P < 0.001). Survival analysis showed that patients with 2 alleles harboring likely-null variants, on average, reached most severe disease stage, i.e., legal blindness, alteration in both dark-adapted and light-adapted electroretinographic responses, and retinal pigment epithelium lesion area larger than 2.5 mm significantly earlier than patients with at least one allele harboring a missense variant. The current longitudinal study showed a significant genotype-phenotype correlation characterization, because patients harboring 2 likely-null alleles reach a severe disease stage about 10 years earlier than patients with at least one missense allele.

Selective retina therapy: toward an optically controlled automatic dosing.

Selective retina therapy (SRT) targets the retinal pigment epithelium (RPE) with pulsed laser irradiation by inducing microbubble formation (MBF) at the intracellular melanin granula, which leads to selective cell disruption. The following wound healing process rejuvenates the chorio-retinal junction. Pulse energy thresholds for selective RPE effects vary intra- and interindividually. We present the evaluation of an algorithm that processes backscattered treatment light to detect MBF as an indicator of RPE cell damage since these RPE lesions are invisible during treatment. Eleven patients with central serous chorioretinopathy and four with diabetic macula edema were treated with a SRT system, which uses a wavelength of 527nm, a repetition rate of 100Hz, and a pulse duration of 1.7 μs. Fifteen laser pulses with stepwise increasing pulse energy were applied per treatment spot. Overall, 4626 pulses were used for algorithm parameter optimization and testing. Sensitivity and specificity were the metrics maximized through an automatic optimization process. Data were verified by fluorescein angiography. A sensitivity of 1 and a specificity of 0.93 were achieved. The method introduced in this paper can be used for guidance or automatization of microbubble-related treatments like SRT or selective laser trabeculoplasty.

NOVEL CLASSIFICATION SYSTEM FOR COMBINED HAMARTOMA OF THE RETINA AND RETINAL PIGMENT EPITHELIUM.

To develop an anatomical classification scheme for combined hamartoma of the retina and retinal pigment epithelium (RPE) and specify recommendations for follow-up interval. Retrospective review of patients with combined hamartoma of the retina and RPE examined during a 7-year period (2008-2015). The clinical presentation, fundus examination, and optical coherence tomography were analyzed. Lesions were classified based on location, fundus features, and optical coherence tomography findings. Lesion location: macular/peripapillary-Zone 1; mid-periphery-Zone 2; and far periphery-Zone 3. Associated fundus findings: no retinal traction-Stage 1; retinal traction and/or retinoschisis-Stage 2; and retinal detachment-Stage 3. Optical coherence tomography findings: epiretinal component only-A; partial retinal involvement-B; and complete retinal and RPE involvement-C. Complete ophthalmologic evaluation is recommended at least every 6 months for patients younger than 12 years, with more frequent follow-up in patients with: lesions in the macula/peripapillary (Zone 1) or with retinal traction, retinoschisis, or retinal detachment (Stage 2 and 3). Surgical intervention is recommended in patients with vision loss secondary to macular traction or retinal detachment. A new clinical classification system is proposed for evaluating and managing patients with combined hamartoma of the retina and RPE. The zone and stage of combined hamartoma of the retina and RPE lesion will assist in determining follow-up interval and surgical intervention. Application of a uniform classification scheme will facilitate assessment and comparison of findings across different studies.

Juxtapapillary tumors and pseudo‐tumors of the retinal pigmented epithelium

SummaryThe most conspicuous retinal pigment epithelium (RPE)‐associated lesion, most of which typically occur in the peripapillary region, is known as the combined hamartoma of the retina and the RPE although not all of them extend to the level of the RPE. This congenital lesion, which also has a vascular and an epiretinal component, often will slowly evolve during observation, not infrequently leading to reduced vision from distortion of the macula. It appears as a greyish‐brown, slightly elevated fundus lesion. It may be associated with neurofibromatosis type 2, although most seem to be isolated. None of the other RPE lesions occurs predominantly in the peripapillary region, although congenital hypertrophy of the RPE, simple hamartoma of the RPE, adenomas and adenocarcinomas of the RPE, and acquired reactive proliferations leading to RPE hyperplasia can do so. Except for the first one, these are slightly to moderately elevated lesions. Finally, rare congenital pigmentary changes such as pigmented optic disk, thought to be of RPE origin, can sometimes be observed.

Retinal pigment epithelium wound healing after traumatic choroidal rupture.

The aim of this study was to investigate wound healing in the retinal pigment epithelium (RPE) after traumatic indirect choroidal rupture using fundus autofluorescence (FAF) and spectral-domain optical coherence tomography (SD-OCT). A total of 14 eyes of 14 patients with traumatic indirect choroidal rupture were included. Baseline and last follow-up FAF images were compared to evaluate the extent of RPE healing after choroidal rupture, and associated morphologic characteristics were examined by SD-OCT. The size of the RPE lesion was reduced in five eyes. The change occurred in the fovea in four eyes and in the macula in three eyes. The change was noted in both the fovea and the macula in two eyes; in these cases, the changes were more prominent in the fovea than in the macula. Extra-macular lesions and lesions with deep choroidal involvement did not show any reduction in size. Choroidal neovascularization (CNV) developed in seven eyes. There was no extra-macular CNV. Retinal pigment epithelium (RPE) wound healing after traumatic choroidal rupture is affected by location and extent of the lesion.

Stargardt disease phenotype‐genotype correlation – first results of a Lithuanian cohort study

PurposeTo describe the first phenotype‐genotype correlation results of the patients with Stargardt disease in Lithuanian population.MethodsFrom May 2015 till April 2016 a prospective study was performed, 16 patients with Stargardt disease were involved. Ophthalmological examination included best‐corrected visual acuity (BCVA), ophthalmoscopy, fundus photography, fundus autofluorescence (FAF), spectral‐domain optical coherence tomography (SD‐OCT) and specular microscopy. Different DNA variants in several genes have been identified for all patients.ResultsThe age of patients ranged from 18 to 66 years. Initial ophthalmoscopy revealed either no abnormalities or foveal retinal pigment epithelium (RPE) changes, foveal atrophy, atrophic RPE lesions, and/or irregular yellow‐white fundus flecks. Different FAF patterns were observed: central atrophy surrounded by ring of increased AF, irregular hypo/hyper AF or spots of hypo AF in the posterior pole. SD‐OCT revealed photoreceptor damage followed by atrophy of the outer retina, RPE, and choroid. The mean central retinal thickness of the right eye was 96.5 ± 57.004 μm and 110.9245 ± 54.84872 of the left eye. The mean subfoveal choroidal thickness, the endothelial cell count and corneal thickness were also evaluated.ConclusionsThese are the first results of Stargadt disease in Lithuanian population. All patients had a different phenotypic expression. Further investigations conserning individual mutations contribution to phenotype are necessary.

The status and progress of studies of idiopathic parafoveal telangiectasis

Idiopathic parafoveal telangiectasis (IPT) is a retinal vascular disease which is characterized by foveal and parafoveal telangiectasia. The main clinical manifestations are retinal telangiectasis, reduced retinal transparency, retinal venular dilatation, yellow exudation, retinal pigment epithelial lesions, retinal hemorrhage, macular atrophy, macular hole or lamellar hole, subretinal neovascularization and retinal detachment. According to the clinical characteristics and features of fluorescein angiography, IPT can be divided into 3 types and 6 subtypes. Laser photocoagulation, photodynamic therapy, and intravitreal injection of glucocorticoid or anti -vascular endothelial growth factor drugs, can reduce the macular edema and neovascularization. However, due to the unclear etiology of IPT, the existing treatment measures are not specific for its etiology. We need to work hard to understand further the clinical features and pathogenesis of IPT and search the targeted treatments based on its pathogenesis mechanism. Key words: Telangiectasis/diagnosis; Telangiectasis /therapy; Macula lutea/injuries; Review

Annular Lesions and Catenary Forms in Chronic Central Serous Chorioretinopathy

To describe a series of patients exhibiting annular retinal pigment epithelial (RPE) lesions in the context of chronic central serous chorioretinopathy. Retrospective comparative case series. Consecutive patients with chronic central serous chorioretinopathy were identified from the clinical practices of 3 retina specialists. A subset of patients exhibiting annular RPE lesions on fundus autofluorescence was included for chart review and examination of multimodal imaging (study group). Patients with alternative etiologies for neurosensory detachment or pigment epitheliopathy were excluded. A second consecutive cohort of patients, with acute central serous chorioretinopathy, was also examined for the presence of annular lesions (comparative group). Sixty-seven patients with chronic central serous chorioretinopathy were identified. Fourteen eyes of 12 patients exhibited annular lesions (study eyes). Mean visual acuity of study eyes was 20/27 (logMAR 0.13, SD 0.11). Annular lesions were composed of hyperautofluorescent stellate lesions arranged in an open or closed ring with intervening foci of punctate hypoautofluorescence. Optical coherence tomography showed RPE hyperplasia at the perimeters of annular lesions with loss of ellipsoid reflectivity and preserved RPE at the lesion center. Annular lesions were confined to the posterior poles and appeared to have developed at the margins of chronic neurosensory detachment. Forty-three eyes of 30 patients with acute central serous chorioretinopathy comprised the comparative group and none of these eyes exhibited annular lesions. Annular lesions occur in up to a fifth of patients with chronic central serous chorioretinopathy but carry a relatively good visual prognosis. Curvilinear RPE figures and demarcation lines are seen in various retinal conditions but the characteristics of annular lesions described here suggest that they are specific to chronic central serous chorioretinopathy.