Patchy Chorioretinal Atrophy Research Articles

International Photographic Classification and Grading System for Myopic Maculopathy

To develop a classification and grading system for myopic maculopathy. Development and evaluation of a classification system for myopic maculopathy based on observational case series. A comprehensive set of myopic macular lesions was defined via literature review and through consensus meetings among retinal specialists and clinician scientists. A classification of myopic maculopathy was formulated based on fundus photographs and a modified Delphi process and consensus. Inter- and intraobserver reproducibility, assessed as agreement (%) and weighted kappa values, were evaluated. One hundred retinal photographs with myopia and myopic macular lesions were selected from case series at the High Myopia Clinic of the Tokyo Medical and Dental University, Tokyo, Japan. We defined 5 categories of myopic maculopathy including "no myopic retinal degenerative lesion" (Category 0), "tessellated fundus" (Category 1), "diffuse chorioretinal atrophy" (Category 2), "patchy chorioretinal atrophy" (Category 3), and "macular atrophy" (Category 4). Three additional features to supplement these categories were defined as "plus" lesions, namely, lacquer cracks, myopic choroidal neovascularization, and Fuchs spot. Posterior staphyloma was considered as a further, important sign of myopic retinopathy. The intraobserver agreement was ≥85% and the corresponding weighted kappa statistic was ≥0.6 between observations. After a brief training session, interobserver kappa statistics reached the predefined satisfactory level (≥0.4), considered as above moderate agreement. We propose a classification system for myopic maculopathy that was found to be reproducible. Applying a uniform classification in different studies will facilitate communication and comparison of findings from clinical trials and epidemiologic studies.

Extreme Thinning or Loss of Inner Neural Retina Along the Staphyloma Edge in Eyes With Pathologic Myopia

To investigate the morphologic changes along the edge of posterior staphyloma in highly myopic eyes with patchy chorioretinal atrophy and to identify the relationship between retinal thinning and the visual field (VF) defects found in these eyes. Retrospective, observational case series. Twenty eyes (17 patients) with pathologic myopia (myopic refractive error ≥ 8 diopters or an axial length ≥ 26.5mm) and patchy atrophy along the staphyloma edge were analyzed retrospectively. The VFs were obtained by Goldmann kinetic perimetry. In the area of patchy atrophy, the inner retinal tissue was very thin or completely absent. Any remnants of the retinal tissue appeared to be adherent to the layer of large choroidal vessels or to the sclera. The mean thickness of the retina at its thinnest point in the area was 51.6 ± 29.0μm in 20 eyes. The retina was thinner than 50μm at its thinnest point in 12 (60.0%) of the 20 eyes, and the mean retinal thickness at its thinnest point in these 12 eyes was 32.1 ± 16.0μm (range, 4 to 49μm). Nineteen eyes (VF of 1 eye was not determined) had very similar VF findings by Goldmann perimetry, that is, a defect of the sector corresponding to the course of retinal nerve fiber over the patchy atrophy. The results show that the thickness of not only the outer retina, but also of most of the inner retina, including the nerve fiber layer, is significantly thinner at the staphyloma edge. Such damage to the inner retina along the staphyloma edge causes the VF defects in highly myopic eyes.

Chorioretinal atrophy after spontaneous resolution of myopic foveoschisis.

Myopic foveoschisis is one of the major complications of pathologic myopia, and it was most recently identified by new imaging modalities. During the natural evolution of this complication, anatomical and visual improvement without surgical intervention is an unusual course, and most of these eyes remain stable or progressively worsen. The authors report a case of a highly myopic eye that developed patchy chorioretinal atrophy after spontaneous resolution of myopic foveoschisis, which to the best of our knowledge has not been reported previously in the medical literature.

Complicated mechanism for axial elongation in eyes with pathologic myopia accompanying scleral thinning and deformity

Dear Editor Thank you very much for your interest concerning our recent paper reporting the changes of axial length in eyes of adults with pathologic myopia [1]. This study relates to our earlier study which examined the long-term change of axial length in eyes with pathologic myopia by using A-mode echography [2]. In that study, older individuals with posterior staphyloma were more susceptible to having a larger increase in the axial length, and we considered that a progression of posterior staphyloma with increasing age should be regarded as a key factor for the continuous increase of axial length in adults with high myopia [2]. As you suggested, peripheral hyperopia has been considered to be a cause of myopia progression. However, it is not clear whether axial length increase is regulated by the same mechanism between eyes with mild to moderate myopia and eyes with extreme myopia accompanied by scleral deformity and thinning. In fact, the patients in our study were extremely myopic; the mean axial length at the onset of the study was 29.35± 1.80 mm. In such extremely myopic cases, the sclera becomes very thin and deformed [3, 4]. Recently, we measured the scleral thickness of eyes with pathologic myopia (mean axial length; 29.9 mm) by using swept-source OCT, and found that the mean subfoveal scleral thickness of these patients was as thin as 227.9±82.0 μm, with a minimum value of 80 μm [3]. In 32.7 % of the patients, the curvature of the posterior eye segment was totally irregular and not spherical at all. It is doubtful whether peripheral hyperopia still plays some role in an expansion of sclera in such extremely myopic eyes. In addition to the scleral thinning and deformity, various other factors like myopic chorioretinal atrophy seem to affect the scleral contour in eyes with pathologic myopia. We recently found that a sclera was posteriorly dislocated in the area of patchy chorioretinal atrophy by developing intrachoroidal cavitation in the macula [5]. Thus, the globe expansion in extremely myopic eyes with deformed sclera is not simply explained by defocus theory. I consider that peripheral hyperopia theory might be more important in less myopic patients than in our patients in the present study. In our patients with extreme myopia with thin and deformed sclera, I think that peripheral hyperopia theory has less impact on the increase of axial length. However, further studies are necessary to confirm this.

Prevalence and Risk Factors for Myopic Retinopathy in a Japanese Population: The Hisayama Study

To examine the prevalence of myopic retinopathy and its risk factors in a general Japanese population.Population-based, cross-sectional study.In 2005, a total of 1969 Hisayama residents aged ≥ 40 years consented to participate in this study. Of these, 1892 subjects with adequate data were enrolled.Each participant underwent comprehensive physical and eye examinations that included measurements of refractive error, axial lengths, and color fundus photography. Myopic retinopathy was defined as the presence of diffuse chorioretinal atrophy, patchy chorioretinal atrophy, lacquer cracks, or macular atrophy.Prevalence of myopic retinopathy.Thirty-three participants had myopic retinopathy and the prevalence was 1.7% (2.2% in women and 1.2% in men). The prevalence of myopic retinopathy increased significantly with advancing age. Diffuse chorioretinal atrophy, patchy chorioretinal atrophy, lacquer cracks, and macular atrophy were present in 1.7%, 0.4%, 0.2%, and 0.4% of subjects, respectively. In multivariate analysis, myopic retinopathy was significantly associated with older age (per 1 year: odds ratio [OR], 1.12; 95% confidence interval [CI], 1.07-1.18), female gender (OR, 3.29; 95% CI, 1.09-9.92), and longer axial length (per 1 mm: OR, 4.20; 95% CI, 3.03-5.83).The prevalence of myopic retinopathy was 1.7% in a general Japanese population. Older age, female gender, and longer axial length were significant risk factors for myopic retinopathy.

Intrachoroidal Cavitation in Macular Area of Eyes With Pathologic Myopia

To determine the incidence and characteristics of intrachoroidal cavitations in the macular area of eyes with high myopia. Prospective, noninterventional case series. We evaluated 56 eyes of 44 patients with pathologic myopia (myopic spherical equivalent >8 diopters) and with patchy chorioretinal atrophy using a swept-source optical coherence tomographic (OCT) system with a center wavelength of 1050 nm. We focused on the changes in the scleral curvature in the area of patchy atrophy. The relationship of the macular intrachoroidal cavitation and retinoschisis was also analyzed. Sixty-eight consecutive patients with pathologic myopia but without patchy atrophy were analyzed as controls. In 31 of 56 eyes (55.4%) with patchy atrophy, the swept-source OCT images showed that the sclera was bowed posteriorly in and around the patchy atrophy compared to neighboring sclera, whereas none of the 68 patients without patchy atrophy showed this finding. Macular intrachoroidal cavitation had OCT features similar to peripapillary intrachoroidal cavitation; the choroid in the macular intrachoroidal cavitation area appeared thickened and the retina was caved into the cavitation. There was a direct communication between the vitreous and intrachoroidal cavitation in 3 eyes. Retinoschisis was observed significantly more frequently in or around the patchy atrophy in eyes with macular intrachoroidal cavitation than in those without cavitation. These findings suggest that patchy atrophy affects the scleral contour within posterior staphyloma beyond the funduscopically identified patchy atrophy by macular intrachoroidal cavitation. Such deformation of sclera may facilitate the development of retinoschisis in and around the patchy atrophy.

Classification of Early Dry-Type Myopic Maculopathy with Macular Choroidal Thickness

To compare the macular choroidal thickness in 2 types of early dry-type myopic maculopathy. Prospective, observational, comparative study. Patients with a refractive error of less than -8 diopters were included and were classified into 2 groups. Group 1 consisted of 24 eyes with a tessellated fundus, and group 2 consisted of 33 eyes with diffuse chorioretinal atrophy, but not to the extent of patchy chorioretinal atrophy. These 2 groups were compared with regard to their clinical characteristics, refractive error, axial length, macular choroidal thickness, and best-corrected visual acuity (BCVA). Linear regression was used to evaluate the explanatory variables in terms of macular choroidal thickness and BCVA. Patients in group 1 were significantly younger and had better BCVA, less myopia, shorter axial length, and less staphyloma than those in group 2. Refractive error, axial length, and BCVA correlated significantly with macular choroidal thickness in group 2. However, no such significant correlations were observed in group 1. Multiple linear regression analysis showed that age and macular choroidal thickness were the variables that associated most strongly with BCVA, whereas neither refractive error nor axial length was a significant predictor of BCVA. In group 2, eyes with lacquer cracks showed worse BCVA and thinner macular choroidal thickness than eyes without lacquer cracks. Macular choroidal thickness is an important factor in myopic maculopathy and can be a better indicator of its severity. These findings suggest that BCVA reduction in eyes with dry-type myopic maculopathy can be related to a thinner macular choroidal thickness and to the development of lacquer cracks.

The types and severity of high myopic maculopathy in Chinese patients

To describe the severity and morphological features of high myopic maculopathy in Chinese patients and to evaluate their association with age, refractive error and visual acuity. Six hundred and four eyes of 337 patients (ages 8-88 years) with high myopia (refractive error ≤ -6.0 D) presenting to the Zhongshan Ophthalmic Center were studied retrospectively. All patients underwent an ophthalmological examination including digital colour fundus photography. Fundus fluorescein angiography was performed in 251 patients (443 eyes) and indocyanine green angiography was performed in 35 patients (63 eyes). The type and severity of myopic degeneration were categorized in each eye. Risk factors associated with high myopic maculopathy were evaluated using General Estimating Equation models. Two hundred and fifty-one patients (443 eyes) presented with maculopathy in this study. The most common subtype of high myopic maculopathy was lacquer cracks (prevalence 29.1%). The most commonly observed degree was M(3) (choroidal pallor and tessellation, with posterior staphyloma and lacquer cracks). Older age was significantly associated with diffuse chorioretinal atrophy (p = 0.024), patchy chorioretinal atrophy (p < 0.001), choroidal neovascularisation (CNV, p < 0.001) and macular atrophy (p = 0.002). Younger age was associated with lacquer cracks (p < 0.001). A higher degree of myopia was a risk factor for almost all kinds of maculopathy: tessellated fundus (p = 0.001), lacquer cracks (p < 0.001), diffuse chorioretinal atrophy (p < 0.001), patchy chorioretinal atrophy (p = 0.025) and macular atrophy p < 0.001), whereas a lower degree of myopia was associated with CNV (p = 0.001). A worse visual acuity was associated with lacquer cracks (p = 0.001), macular atrophy (p < 0.001) and CNV p < 0.001), while better visual acuity was associated with tessellated fundus (p < 0.001) and diffuse atrophy (p = 0.002). Older age (p < 0.001), more myopic refractive error (p < 0.001) and worse visual acuity (p < 0.001) were significantly associated with a greater severity of maculopathy. There was no gender difference in the type and severity of myopic maculopathy. Age and refractive error are important risk factors playing different roles in the development of distinct subtypes and varying severities of high myopic maculopathy. Visual acuity is significantly associated with various subtypes and the severity of high myopic maculopathy.

Rhegmatogenous Retinal Detachment Due to Paravascular Linear Retinal Breaks Over Patchy Chorioretinal Atrophy in Pathologic Myopia

To characterize posterior paravascular linear retinal breaks over areas of patchy chorioretinal atrophy as a cause of retinal detachment among patients with pathologic myopia. In this retrospective case series, we evaluated 10 pathologically myopic eyes having rhegmatogenous retinal detachment associated with posterior paravascular linear retinal breaks. Ten eyes with posterior paravascular linear retinal breaks and retinal detachment were identified from January 1, 2008, to July 31, 2009. The retinal breaks were most frequently found along the inferotemporal vascular arcade, followed by the superotemporal arcade. The length of the breaks ranged from 0.25 to 1 disc diameter, and their distance from the optic disc ranged from 1 to 5 disc diameters. These paravascular linear retinal breaks have distinct clinical characteristics, including a strong propensity to occur over areas of patchy chorioretinal atrophy, a linear shape that is oriented parallel to the adjacent retinal vessels, and a tendency to result in progressive retinal detachment. Vitrectomy with gas tamponade was performed in all cases, and retinal reattachment was achieved in 9 cases by a single operation. Paravascular linear retinal breaks over areas of patchy chorioretinal atrophy represent a distinct clinical entity that can result in a special category of retinal detachment among patients with pathologic myopia. These breaks are apt to elude detection before surgery, and a careful search along the posterior vascular arcades during vitrectomy may help to detect these abnormalities. Pars plana vitrectomy with photocoagulation and intraocular tamponade may lead to a resolution of such rhegmatogenous retinal detachments.

Long-term Pattern of Progression of Myopic Maculopathy: A Natural History Study

To investigate the long-term progression pattern of myopic maculopathy and to determine the visual prognosis of each progression stage.Retrospective, observational case series.The medical records of 806 eyes of 429 consecutive patients with high myopia (refractive error more than -8.00 diopters [D] or axial length > or =26.5 mm) who were followed for 5-32 years were reviewed.Participants had complete ophthalmological examinations including best-corrected visual acuity, axial length measurements, fluorescein angiography, and color fundus photography, at least once a year. The presence and type of posterior staphyloma was determined by binocular stereoscopic ophthalmoscopy. The types of myopic maculopathy included tessellated fundus, lacquer cracks, diffuse chorioretinal atrophy, patchy chorioretinal atrophy, choroidal neovascularization (CNV), and macular atrophy. None of the patients had received any type of treatment for the maculopathy.The longitudinal long-term progression pattern and the visual prognosis of each type of fundus lesion.During the mean follow-up of 12.7 years, 327 of the 806 highly myopic eyes (40.6%) showed a progression of the myopic maculopathy. The most commonly observed patterns were from tessellated fundus to the development of diffuse atrophy and lacquer cracks, an increase in the width and progression to patchy atrophy in eyes with lacquer cracks, an enlargement of the diffuse atrophy, and the development of patchy atrophy in eyes with diffuse atrophy, and an enlargement and fusion of patches of atrophic areas in eyes with patchy atrophy. Eyes with tessellated fundus, lacquer cracks, diffuse atrophy and patchy atrophy at the initial examination progressed to the development of CNV. Eyes with CNV developed macular atrophy. The fusion of patchy atrophy, the development of CNV, and macular atrophy all led to significant visual decreases. A posterior staphyloma was observed more frequently in eyes that showed progression from tessellated fundus, diffuse atrophy, and patchy atrophy than those without a progression.These findings indicate that myopic maculopathy tends to progress in approximately 40% of highly myopic eyes, and the pattern of progression affects the visual prognosis. Preventive therapy targeting posterior staphyloma should be considered to prevent the visual impairment caused by the progression of myopic maculopathy.

A far advanced case of gyrate atrophy in a 12‐year‐old girl

Gyrate atrophy (GA; McKusick 25887) is a progressive chorioretinal dystrophy with an autosomal recessive mode of inheritance. In this disorder there is a deficiency of the mitochondrial matrix enzyme ornithine-f-aminotransferase (OAT). Ocular symptoms usually present late in the first decade of life. Ophthalmological findings include myopia, constricted visual fields, elevated dark adaptation thresholds (night blindness), very small or absent electroretinographic responses, and characteristic patchy chorioretinal atrophy distributed circumferentially around the peripheral fundus. Gradually the round patches of atrophy coalesce to form widespread chorioretinal atrophy extending to the posterior pole. Cataracts develop requiring surgical intervention, and eventually blindness occurs due to progressive loss of functional retina at the age of 40 to 50 (Valle and Simell, 1989). Plasma ornithine levels range from 400 to 1400/~mol/L, and 0.5 to 10mmol of ornithine is excreted daily. Glutamate, glutamine, lysine, creatine and creatinine concentrations in plasma are modestly reduced. The pathophysiological mechanism responsible for the chorioretinal dystrophy in GA is not known. Hyperornithinaemia or associated secondary metabolic abnormalities may play an important role, but until the pathophysiology is understood, there is no assurance that correction of biochemical abnormalities in plasma will actually be beneficial (Berson et al., 1981). Four general therapeutic approaches have been used: pharmacological doses of pyridoxine, reducing the intake of arginine and administration of creatine or proline. In this paper we described a 12-year-old patient with night blindness and myopia as the first symptoms of gyrate atrophy with ornithinaemia.

Progessive retino-choroidal atrophy with spinocerebellar ataxia

The clinical course and ophthalmological findings in the five affected subjects of a kindred with an autosomal dominant, progressive retino-choroidal atrophy and spinocerebellar ataxia varied considerably during the 13–18 year follow-up. The symptoms started most often with visual disturbances suggesting affection of the cones and papillomacular nerve fiber layer. Macular pigmentary dispersion preceded macular degeneration, generalized patchy chorioretinal atrophy and complete optic atrophy, and extinction of ERG and VEP. The late fundus findings of one patient resembled gyrate atrophy of the choroid and retina, but plasma ornithine concentrations were within normal limits in all patients.