Traumatic choroidal injuries - classification, incidence, diagnosis and prognosis twenty-year results of Eye Injury Vitrectomy Study.

PURPOSE: To characterize the classification, incidence, diagnosis and prognosis of traumatic choroidal injuries. METHODS: Subjects were selected from the database of the Eye Injury Vitrectomy Study (EIVS) and were examined for occurrences of different categories of choroidal injuries. Standard photographs were collected. Anatomical and visual outcomes were assessed in patients with greater than 1 year of follow-up. Eyes that had no light perception (NLP) and/or phthisis bulbi were defined as having had unfavourable outcomes. The percentage of eyes with an unfavourable outcome was analysed for different types of choroidal injuries. RESULTS: Nine categories of choroidal injuries with distinctive features were identified in the EIVS database. The incidence and the percentage of eyes with an unfavourable outcome in each injury category were as follows: suprachoroidal effusion, 21.2% (7.2%); suprachoroidal haemorrhage, 12.8% (11.2%); massive suprachoroidal haemorrhage, 4.0% (64.9%); choroidal avulsion, 4.2% (92.2%); traumatic chorioretinal rupture, 1.8% (13.3%); choroidal rupture, 4.8% (6.8%); choroidal loss, 1.6% (79.3%); choroidal hole, 1.1% (5.3%); and choroidal damage at the wound site, 39.2% (17.7%). CONCLUSIONS: Ocular trauma can cause a variety of choroidal injuries that have distinctive features, some of which are associated with a high frequency of unfavourable prognoses.

Characterizing Branching Vascular Network Morphology in Polypoidal Choroidal Vasculopathy by Optical Coherence Tomography Angiography.

This study analyze the morphological characteristics of branching vascular networks (BVN) in polypoidal choroidal vasculopathy (PCV) using optical coherence tomography angiography (OCTA), and correlate imaging characteristics with clinical presentations. We presented a retrospective observational case series for fifty cases of PCV confirmed by indocyanine green angiography. Macular OCTA were done by the AngioVue. The PCV cases were classified by distinct morphologic patterns of BVN by two retina specialists and clinical features were analyzed. The sensitivity of polyp detection by OCTA was 86% after manual segmentation and that of BVN was 90%. Three distinct morphologic patterns of BVN were identified. (1) The "Trunk" pattern (47%) exhibited major vessel trunk with features including presence of drusens, thin choroid, and larger BVN area. (2) The "Glomeruli" pattern (33%) showed anastomotic vascular network without major trunk. (3) The "Stick" pattern (20%) had localized BVN and the thickest choroid. Subtypes 2 and 3 held higher recurrence rate. In conclusions, the precise visualization of BVN on OCTA supported that OCTA might be a noninvasive tool to study the morphology of BVN in PCV, which exhibits three different morphological types. Identifying the morphology of BVN has the potential to prognosticate outcomes in PCV patients.

ANGIOGRAPHIC SUBTYPES OF POLYPOIDAL CHOROIDAL VASCULOPATHY IN TAIWAN: A Prospective Multicenter Study.

PURPOSE: To determine the incidence and clinical characteristics of angiographic subtypes of polypoidal choroidal vasculopathy (PCV). METHODS: It is a prospective, multicenter, cross-sectional study. Patients with newly diagnosed exudative macular degeneration are classified into PCV, age-related macular degeneration (AMD), and retinal angiomatous proliferation. Polypoidal choroidal vasculopathy is further classified into two subtypes depending on the presence (Type 1: polypoidal choroidal neovascularization) or absence (Type 2: typical PCV) of feeder vessels on indocyanine green angiography. RESULTS: We enrolled 169 patients: 76 (45%) with PCV, 75 (44.4%) with AMD, and 14 (8.3%) with retinal angiomatous proliferation. Of the patients with PCV, 20 (26%) were classified as Type 1 PCV and 56 (74%) were classified as Type 2 PCV. The Type 1 PCV had a similar mean age compared to the AMD group (73.1 ± 9.6 vs. 75.6 ± 8.8 years, P = 0.281) and the Type 2 PCV (68.8 ± 9.6 years) was younger than the AMD group (P < 0.001). Type 1 PCV presented with worse visual acuity compared with the AMD. Both PCV subtypes had a higher incidence of hemorrhagic complications (85% and 75% respectively). CONCLUSION: Type 2 PCV is more common than Type 1 PCV in Taiwan. Our results support the hypothesis that polypoidal choroidal neovascularization and typical PCV may be distinct entities.

CLASSIFICATION OF SCLEROCHOROIDAL CALCIFICATION BASED ON ENHANCED DEPTH IMAGING OPTICAL COHERENCE TOMOGRAPHY "MOUNTAIN-LIKE" FEATURES.

PURPOSE: To describe distinct enhanced depth optical coherence tomography patterns of sclerochoroidal calcification and their correlation to clinical features. METHODS: Retrospective chart review of 67 eyes of 46 patients with spectral domain optical coherence tomography imaging. RESULTS: The mean patient age at diagnosis was 68 years. There were 20 (43%) men and 26 (57%) women of white (n = 45, 98%) or Hispanic (n = 1, 2%) heritage. The most prominent sclerochoroidal calcification lesions were located in the superotemporal quadrant (n = 57, 85%) between the temporal arcades and the equator (n = 58, 87%). On enhanced depth optical coherence tomography, the sclerochoroidal calcification was located within the sclera in all cases and the inner surface topography assumed specific "mountain-like" patterns, including flat (Type 1) (n = 9) at median thickness of 1.2 mm, rolling (Type 2) (n = 28) at 1.4 mm thickness, rocky-rolling (Type 3) (n = 21) at 2.1 mm thickness, and table mountain (Type 4) (n = 9) at a thickness of 1.9 mm. The retinal layers were undisturbed in flat lesions, and outer retinal abnormalities were found in all other types. A comparison of the 4 types revealed that Type 3 lesions were thickest (P < 0.001), showing abnormalities in the retinal pigment epithelium, ellipsoid region, and external limiting membrane most commonly (P < 0.05) and demonstrating the most dramatic thinning of the overlying choroid (P < 0.01) and retina (P < 0.05). Type 4 lesions showed greatest basal diameter (P < 0.01) and least outer retinal abnormalities (P < 0.05) or choroid thinning (P < 0.05). CONCLUSION: In this report, enhanced depth optical coherence tomography has demonstrated that sclerochoroidal calcification is a scleral-based disease and can be classified based on four "mountain-like" topographic patterns, associated with variable effects on the choroid and retina.

Choroidal hyperreflective foci in Stargardt disease shown by spectral-domain optical coherence tomography imaging: correlation with disease severity.

IMPORTANCE: The presence of choroidal hyperreflective foci in Stargardt disease is, to our knowledge, a potentially new finding. Evaluation of these foci may aid in better understanding of the disease process. OBJECTIVES: To report the presence of choroidal hyperreflective foci in spectral-domain optical coherence tomography (SD-OCT) images from eyes with Stargardt disease and investigate the relationship between the number of hyperreflective foci and disease severity. DESIGN, SETTING, AND PARTICIPANTS: Twenty-six eyes of 13 patients with a clinical diagnosis of Stargardt disease were evaluated in a retrospective case series. Patient data were collected between January 1, 2009, and August 31, 2014. MAIN OUTCOMES AND MEASURES: The number of choroidal hyperreflective foci in Stargardt disease as well as correlation with visual acuity, central macular thickness (CMT), and disease duration were the main outcomes. A total of 707 macular SD-OCT scans of 13 patients with Stargardt disease were reviewed and evaluated for the presence and number of retinal/choroidal hyperreflective foci, central macular thickness, visual acuity, and disease duration. Enhanced depth imaging with OCT (EDI-OCT) scans available for 2 patients were compared with SD-OCT scans. A PubMed/Google search was performed to identify SD-OCT images in Stargardt disease; these findings were reviewed for the presence of choroidal hyperreflective foci. RESULTS: The mean (SD) numbers of hyperreflective foci in each retinal/choroidal layer in decreasing frequency were as follows: Bruch membrane/retinal pigment epithelial (RPE) complex, 78.22 (24.39); choriocapillaris, 25.77 (17.57); Sattler layer, 18.59 (12.89); outer retina, 16.64 (6.96); inner retina, 0.95 (1.58); and Haller layer, 0.73 (0.87). The number of hyperreflective foci in the Bruch membrane/RPE complex increased exponentially with decreasing CMT (R2 = 0.99; P = .008). The number of hyperreflective foci in the Bruch membrane/RPE complex, choriocapillaris, and Sattler layer increased proportionally with decreasing visual acuity (R2 = 0.97, R2 = 0.95, and R2 = 0.99, respectively; and P = .007, P = .006, and P = .008, respectively). Direct correlation existed between the number of hyperreflective foci in the choriocapillaris and the Sattler layer and disease duration (R2 = 0.98 and R2 = 0.99, respectively; and P = .006 and P =.009, respectively). In the 10 studies on Stargardt disease, choroidal hyperreflective foci were present in 51 of 54 SD-OCT images (94%). CONCLUSIONS AND RELEVANCE: Based on the findings of the present study, choroidal hyperreflective foci in Stargardt disease, prominent at the Bruch membrane/RPE complex, choriocapillaris, and Sattler layer, correlate with disease severity in terms of retinal atrophy, decline in vision, and disease duration. Further studies are necessary to assess whether these findings are unique to Stargardt disease.

Indocyanine green angiographic and optical coherence tomographic findings support classification of polypoidal choroidal vasculopathy into two types.

PURPOSE: We assessed the characteristic indocyanine green angiographic (ICGA) and spectral domain optical coherence tomographic (SD-OCT) findings of two types of polypoidal choroidal vasculopathy (PCV), distinguishable by different filling patterns on ICGA. METHODS: Thirty-one eyes with PCV were classified into types 1 and 2 based on ICGA findings of either the presence or absence of both a feeder and a draining vessel. Characteristic ICGA findings were evaluated for each type of PCV. Spectral domain optical coherence tomographic images of the 31 eyes were also used to compare the two types of PCV. RESULTS: Both a feeder and a draining vessel were observed in 13 eyes (type 1). Eighteen eyes had neither feeder nor draining vessels (type 2). In PCV type 1, a break in the highly reflective line thought to be Bruch's membrane was detected, corresponding to the feeder vessel in-growth site on SD-OCT. This line was straight. In PCV type 2, the highly reflective line exhibited irregular thickness and had highly reflective substances adhering to its lower portion. It curved downward and became increasingly obscure, ultimately disappearing at a point corresponding to the site at which network vessel filling began. The mean subfoveal choroidal thicknesses in eyes with PCV type 1 and PCV type 2 were 199 ± 65 and 288 ± 98 µm, respectively. CONCLUSIONS: Our observations support the existence of two distinct types of PCV. The first type represents choroidal neovascularization, whilst the second type involves choroidal vasculature abnormalities.

The association of age-related maculopathy susceptibility 2 (ARMS2) and complement factor H (CFH) variants with two angiographic subtypes of polypoidal choroidal vasculopathy.

PURPOSE: To compare the association of age-related maculopathy susceptibility 2 (ARMS2) and complement factor H (CFH) variants between two different angiographic phenotypes of polypoidal choroidal vasculopathy (PCV). METHODS: We included 175 Japanese patients with PCV and 150 age- and sex-matched controls. PCV was classified into two phenotypes (Type 1 and Type 2) according to the presence or absence of the feeding vessels found in indocyanine-green angiography. The single nucleotide polymorphism (SNP) at rs10490924 (A69S) in ARMS2 and rs800292 (I62V), rs1061170 (Y402H) in the CFH region were genotyped using the TaqMan assay. RESULTS: The minor allele frequency (MAF) of rs10490924 was significantly different between Type 1 PCV (n = 81) and control (p < 0.0001), while no difference was found between Type 2 PCV (n = 94) and control (p = 0.20). The MAF of rs800292 was significantly different between each type of PCV and control (p < 0.0001 and 0.0001 for Type 1 versus control and Type 2 versus control, respectively). The MAF of rs1061170 was not significantly different between either type of PCV and control (p = 0.084 and 0.15, respectively). CONCLUSIONS: There may be significantly different associations in the genetic variants of ARMS2 between two angiographic phenotypes of PCV.

A classification system for polypoidal choroidal vasculopathy.

PURPOSE: To describe the demographic features and clinical characteristics of polypoidal choroidal vasculopathy (PCV) in Chinese patients and define a new classification system. METHODS: Retrospective review of 138 eyes of 123 patients presenting to the Singapore National Eye Center with PCV. Patients underwent ophthalmologic examination including digital color fundus photography and stereoscopic indocyanine green angiography. Classification based on indocyanine green angiography findings. RESULTS: Mean age of patient 68.3 years and 62.4% were men. PCV was unilateral in 87.8% cases and age-related maculopathy was present in the unaffected fellow eye in 22.8%. Average largest size of polyp was 207 microm. PCV lesions were found in multiple discrete areas in 34.8%. Formation of lesion was cluster in 66.7%, single in 27.5%, and string in 5.8%. PCV lesions were found in the extrafoveal area in 63.0%, subfoveal in 29.7%, juxtafoveal in 15.9%, and peripapillary in 8.0%. CONCLUSIONS: Demographics of PCV, unilaterality and frequency of age-related maculopathy in fellow eye similar to other reports in Asians. We describe a classification system for PCV comprising polyp size, location, formation, and number of discrete polyp areas, which can be used for prospective interventional clinical studies and may aid in future prognosis and management of this condition.

Clinical features of early and late stage polypoidal choroidal vasculopathy characterized by lesion size and disease duration.

BACKGROUND: The pathogenesis of polypoidal choroidal vasculopathy (PCV), and even its clinical features, are controversial. Previous histopathological studies have identified different features; either dilated choroidal vessels or intra-Bruch's neovascularization. These differences might be partly attributable to the influence of the disease stage. We therefore evaluated the clinical features of early and late stage PCV. METHODS: The medical records of 110 eyes of 97 PCV patients were retrospectively reviewed. The time between the subjective onset of visual abnormality and examination at our clinic and the greatest linear dimension of the total lesion at the first examination were investigated. The period of disturbed vision and lesion size data were placed in ascending order to determine the first quartile point. Eyes with both values at or below the first quartile point were classified as 'small-short' (early stage). Eyes with both values equal to at least the third quartile point were classified as 'large-long' (late stage). Fundus photography, indocyanine green and fluorescein angiography, visual acuity, and clinical course were compared. RESULTS: Twelve eyes from 12 patients were small-short cases (period of disturbed vision of 1 month or less, lesion size 2.0 disc diameters or less). Eleven eyes from ten patients were large-long cases (period of disturbed vision 36 months or more, lesion size at least 5.0 disc diameters). The large-long eyes were characterized by occult choroidal neovascular membrane or scar tissue secondary to exudative age-related macular degeneration. Noticeable in the small-short eyes were atrophic changes in the retinal pigment epithelium, choroidal vessel hyperpermeability and pulsation. The visual prognosis and clinical course were different between the groups. CONCLUSIONS: The difference of clinical features between the groups might reflect different disease stages, although not all of the features observed in the small-short group appeared to represent the early stages of those recorded in the large-long group. Thus, the variation in histopathologic features among previous reports might be partly attributable to differences in disease stage.

[The study of the classification on polypoidal choroidal vasculopathy].

PURPOSE: To establish and explore the criteria of the classification on polypoidal choroidal vasculopathy (PCV). METHODS: The 42 patients (48 eyes) with PCV were classified according to the location, staining or not in late indocyanine green angiography (ICGA), with or without branching vascular network, and the extent of hemorrhagic or exudative. RESULTS: According to the location, staining or not in late ICGA, with or without branching vascular network, and the extent of hemorrhagic or exudative, the PCV were classified as macular (30 eyes, 62.5%), arcade (6 eyes, 12.5%), peripapillary (3 eyes, 6.3%), midperiphery and Combination PCV (9 eyes, 18.8%); active (34 eyes, 70.8%) and inactive PCV (14 eyes, 19.2%); typical (36 eyes, 75.0%) and atypical PCV (12 eyes, 25.0%); hemorrhagic (35 eyes, 72.9%) and exudative PCV (13 eyes, 27.1%), respectively. The classification on location of the polypoidal lesions and with or without branching vascular network of the lesions were the first suggested. CONCLUSIONS: The classification of PCV is beneficial for describing the clinical manifestations and for predicting the prognosis and for guiding the treatment of the disease.

An eye for an eye; a tooth for a tooth.

A 24-year-old man experienced the sudden onset of a painless superior-temporal visual field defect of the left eye. Fundoscopy showed peripapillary pigmentary changes and a few nasal retinal white spots. Automated perimetry demonstrated an enlarged blind spot. The differential diagnosis of the various presumed inflammatory retinopathies and choroidopathies associated with an enlarged blind spot are reviewed and the classification of the white spot syndromes of the retina discussed.

[Hereditary retinochoroidal dystrophies. Part 2: differential diagnosis]. / Hereditäre Netzhaut-Aderhaut-DystrophienTeil 2: Differenzialdiagnose.

A generally accepted classification for inherited retinochoroidal dystrophies does not exist. The names given to certain disorders are either based on ophthalmoscopic findings, or on histologic, electrophysiologic and genetic findings. Future research on the molecular genetic background will result in better definition of clinical entities. The purpose of this project is to outline a practical approach to inherited retinochoroidal dystrophies. For this reason, disorders with similar clinical symptoms are grouped together. Generalized retinochoroidal dystrophies affecting all retinal areas can be distinguished from regional dystrophies. Generalized dystrophies can be subdivided into those with peripheral onset, usually associated with initial rod function loss (night blindness, peripheral field loss: e.g. retinitis pigmentosa, choroideremia) and those with central onset associated with cone function loss (visual acuity loss, central scotoma, color vision deficits: e.g. cone or cone-rod dystrophies). Regionally limited dystrophies include the multitude of macular dystrophies and the autosomal dominant vitreoretinochoroidopathy, which remains limited to the periphery. It is important for a differential diagnosis to exclude involvement of other organ systems in syndromic disorders. Stationary inherited retinal dysfunction (e.g. monochromatism, congenital stationary night blindness) and other inherited or acquired diseases have to be excluded as well. Guidelines for differential diagnosis are presented.

[Risk factors for the degeneration of the choroid naevi: a retrospective study of 135 cases]. / Facteurs de risque pour la dégénérescence des naevi choroïdiens: étude rétrospective sur 135cas.

INTRODUCTION: We have carried out a retrospective study of 135 patients followed at Curie Institute for choroidal naevus between March 1983 and June 1997. PATIENTS AND METHODS: 54 patients presented naevi considered as benign and 81 patients presented suspicious choroidal naevi (with at least one of the following findings: visual symptoms; serious detachment of the retina; orange pigment; thickness greater than 2mm or a diameter greater than 7mm). These suspicious naevi were followed more carefully. The median follow-up was 49 months. The median diameter of the lesions was 6mm and the median thickness was 1.5mm. We studied the age of the patients, clinical symptoms, the presence or absence of orange pigment, drusen and serious detachment, and the angiographic and echographic findings. RESULTS: 3 patients died of unrelated cause; 7 patients were lost to follow-up; 30 patients presented documented growth; 4 of them belonged to the group considered as benign and 26 to the group considered as suspicious, with a significant difference between the groups. The lesions that grew were treated by proton beam or I125 patches. The risk factors for growth that were statistically significant were the presence of visual symptoms, pin points, orange pigment, serous detachment and thickness greater than 2mm. With drusen, the risk for growth was significantly less. DISCUSSION: These results are very similar to those published in the literature concerning risk factors for growth of choroidal nevi. The absence of metastatic spread in patients whose nevi have grown show that it is possible to monitor choroidal naevi if there is a doubt as to the malignant or benign nature of the lesion. CONCLUSION: It is important to determine if a choroidal naevus is suspicious or benign and to propose closer follow-up for suspicious lesions.

[Choroid cysts--laser therapy]. / Cysty cievovky--liecba laserom.

The author presents the survey of cysts of choroid classification. He covers a case of serous cyst of iris of a young man. The cyst caused the vision disorder because of pressure on the front capsule of lens. The successful therapeutical method was a laser photocoagulation. There was not find any recurrent disease during the six years of monitoring.

[Value of optical coherence tomography in diagnosis of age-related macular degeneration. Correlation of fluorescein angiography and OCT findings]. / Die Bedeutung der optischen Kohärenz-Tomographie in der Diagnostik der altersbezogenen Makuladegeneration. Korrelation von fluoreszenzangiographischen mit OCT-Befunden.

BACKGROUND: Age-related macular degeneration (AMD) is the leading cause of blindness in industrialized countries. In this study, we used optical coherence tomography for evaluation of patients with AMD. METHODS: Optical coherence tomography imaging is analogous to ultrasound, except that reflected light instead of sound is used. The analysis of the reflected light is processed with the technique of low-coherence interferometry. In this study, 33 patients with different stages of AMD were examined with optical coherence tomography. The classification of AMD was according to the guidelines as proposed by the "International ARM Epidemiological Study group". RESULTS: With this method we were able to identify drusen, alteration of the retinal pigment epithelium, and secondary retinal changes. Other structures such as basal laminar (linear) deposits could not be identified with this method. Choroidal neovascularization was evident in the tomogram. Classic choroidal neovascular membranes presented with well-defined boundaries on optical coherence tomography and occult choroidal neovascular membranes had a less delineable structure with optical coherence tomography. CONCLUSION: Optical coherence tomography cannot replace conventional diagnostic techniques. This method provides no additional information in patients with non-exsudative AMD. In patients with choroidal neovascular membranes secondary to AMD optical coherence tomography may be able to characterize the relation of the membrane to the retinal pigment epithelium and imaging may be possible through hemorrhage. The interpretation of the optical coherence tomogram needs further studies including clinicopathologic correlation.

[The retinochoroidal ischemia syndrome. Its classification, etiopathogenesis and clinical aspects]. / Sindromul de ischemie retinocoroidiana. Clasificare, etiopatogenie, aspect clinic.

The analysis of some clinical cases of retinochoroidal ischemia permitted to the authors the elaboration of some considerations, looking classification, etiopathogenesis and clinical aspect of the cases. At the base of ischemic vascular syndrome classification were two factors: the place of vascular obstacle (extra/intraocular) and the predominant clinic syndrome (retinal/choroidal). Going from this reasons the proposed classification includes three principal parts: The predominant retinal ischemic syndrome (extraocular/intraocular retinal obliteration). The predominant choroidal ischemic syndrome (intraocular obliteration-choriocapillary/ACSP). The ischemic retinochoroidal syndrome (mixed). Are commented in great detail, etiopathogenic and clinical aspects by the principal ischemia syndrome.

Indocyanine green angiographic findings in multifocal choroidopathies.

With high definition videoangiography (TOPCON IMAGEnet H1024) the Authors studied 41 patients affected by multifocal choroidopathies (MC) (68 eyes with ophthalmoscopic or indocyanine green angiographic evidences): 29 females and 12 males; age 21-51 years with a follow up of 6-29 months. In the light of the evidence provided by FA and ICG the Authors present a classification of MC in three stages: Stage 1 of subclinical choroidal activity (5 eyes): characterised by the presence of hypofluorescent or hyperfluorescent spots visible only in the late phases of ICGA; stage 2 of clinically evident choroidal activity (45 eyes): in FA the spots are hypofluorescent in the early phases and hyperfluorescent with a slight diffusion in the late phases, in ICGA either hypofluorescent spots or less frequently hyperfluorescent spots and choroidal permeability alterations can be observed; stage 3 or healed stage (18 eyes): in FA the spots are hyperfluorescent without late leakage, in ICGA hypofluorescence can be observed during all angiographic phases. In 5 patients in stage 1 of subclinical activity, a systemic steroid therapy induced the regression of the hypofluorescent sports in ICGA, in 2 cases the regression of hyperfluorescent spots in ICGA was observed after systemic antibiotic therapy. The authors underline that ICGA could be a particularly useful tool for an early diagnosis and clinical monitoring of MC.