Ariko Kojima

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

OBJECTIVE To investigate the long-term progression pattern of myopic maculopathy and to determine the visual prognosis of each progression stage. DESIGN Retrospective, observational case series. PARTICIPANTS 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. METHODS 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. MAIN OUTCOME MEASURES The longitudinal long-term progression pattern and the visual prognosis of each type of fundus lesion. RESULTS 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. CONCLUSIONS 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.

Patchy atrophy and lacquer cracks predispose to the development of choroidal neovascularisation in pathological myopia

Aims: To determine the incidence and predisposing findings for choroidal neovascularisation (CNV) in a large series of highly myopic patients. Methods: The medical records of 218 consecutive patients (325 eyes) with myopic fundus changes in the macula were reviewed. The incidence of CNV during a follow up of at least 3 years of highly myopic patients and identification of predisposing findings for the development of myopic CNV were examined. Results: Among 325 highly myopic eyes examined, 33 eyes (10.2%) developed myopic CNV. The incidence was higher (34.8%) among the fellow eyes of patients with pre-existing CNV than among eyes of patients without pre-existing CNV (6.1%). CNV developed in 3.7% with diffuse chorioretinal atrophy, in 20.0% with patchy atrophy, and in 29.4% with lacquer cracks. Conclusion: Approximately one in 10 highly myopic eyes developed myopic CNV in average 130.2 months. Patchy atrophy and lacquer cracks were shown to be important predisposing findings for CNV development.

Prevalence and characteristics of foveal retinal detachment without macular hole in high myopia.

PURPOSE To report the prevalence of foveal retinal detachment without macular hole in a large number of highly myopic eyes using optical coherence tomography (OCT), and to clarify the demographic characteristics associated with foveal retinal detachment in these eyes. DESIGN A consecutive, prospective, observational case series. METHODS In 134 eyes of 78 consecutive patients with high myopia (refractive error of -8 diopters or more), we performed complete ophthalmic examinations and studied cross-sectional images of the macula with OCT. The patients were divided into two groups according to the presence (group 1, n = 78 eyes of 45 patients) or absence (group 2, n = 56 eyes of 33 patients) of posterior staphyloma. Slit-lamp examination with a Goldmann three-mirror lens indicated that none of the eyes had a macular hole. RESULTS In seven of 78 eyes (9.0%) with posterior staphyloma (group 1), OCT revealed foveal retinal detachment. Two of the seven eyes had foveal retinoschisis. Optical coherence tomography revealed no retinal detachment or retinoschisis in any eye without posterior staphyloma (group 2). Visual acuity of the seven eyes with foveal retinal detachment ranged from 20/40 to 20/200. Two of the seven eyes had visual acuity 20/50 or better. No patients complained of recent, progressive visual impairment. All seven eyes with foveal retinal detachment had severe myopic fundus changes (focal chorioretinal atrophy or bare sclera). CONCLUSIONS In highly myopic eyes with posterior staphyloma, the prevalence of foveal retinal detachment without macular hole was 9.0%. In eyes with this type of retinal detachment, visual acuity varies and foveal retinal detachment tends to be missed on routine examination. Periodic examination using OCT is recommended for highly myopic eyes with severe myopic degenerative changes and posterior staphyloma.

Long-term changes in axial length in adult eyes with pathologic myopia.

OBJECTIVE To examine the long-term changes of the axial length in adults with high myopia. DESIGN Open-label, consecutive, retrospective case series. METHODS The medical records of 101 patients (184 eyes) with high myopia (myopia ≥-6 diopters or axial length ≥26.5 mm) were studied. The axial length of the eye was measured by A-scan ultrasonography. The significance of the changes in the axial length during follow-up was determined. The effects of the age, axial length, and the presence of a posterior staphyloma at the initial examination on the axial length elongation were determined. RESULTS The mean follow-up period was 8.2 years. The median axial length increased significantly from 28.6 mm at the initial examination to 29.4 mm at the final examination in the 184 eyes. The axial length remained stable (≤1-mm difference) in 69%, whereas the axial length increased by more than 1 mm in 31% of the eyes. For these 31%, the median axial length increased by 1.55 mm. An increase of the axial length per year was significantly greater in older patients than their younger cohorts, and the increase in eyes with a posterior staphyloma was significantly greater than in eyes without a staphyloma. Multiple regression analyses showed that the axial length elongation was positively and significantly correlated with patient age at the initial examination. CONCLUSIONS In highly myopic adult patients, the axial length continued to increase. Older individuals with posterior staphyloma were more susceptible to having a larger increase in the axial length. A progression of posterior staphyloma with increasing age is considered a key factor for the continuous increase of axial length in adults with high myopia.

Reduction of retinal blood flow in high myopia

PurposeTo investigate changes in retinal vessel diameter and blood velocity in high myopia using laser Doppler velocimetry.MethodsThirty-nine subjects (39 eyes) were enrolled in the study. The subjects were divided into three groups according to their refractive status; 15 eyes (15 patients) with emmetropia (within ±3.0 diopters), 14 eyes (14 patients) with mild myopia (between −3.0 and −8.0 diopters), and 10 eyes (10 patients) with high myopia (>−8.0 diopters). Patient age was matched between groups. Blood velocity and vessel diameter of the upper or lower temporal retinal artery were measured using laser Doppler velocimetry with an eye-tracking system, and measurements were compared between groups.ResultsThe average retinal blood flow and vessel diameter in highly myopic eyes were significantly decreased compared with emmetropic eyes or mild myopic eyes (Mann-Whitney U test, p<0.05). Also, there was significant difference regarding retinal blood flow and vessel diameter between eyes with mild myopia and the other groups. In addition, there was no significant difference in blood velocity between the three groups.ConclusionsRetinal blood flow was decreased in high myopia, mainly due to the narrowing of the retinal vessel diameter. Impaired retinal blood flow might have a role in the development of chorioretinal atrophy in high myopia.

Long-term Development of Significant Visual Field Defects in Highly Myopic Eyes

PURPOSE To identify the characteristics that are associated significantly with visual field (VF) defects in highly myopic eyes. DESIGN Retrospective, observational series. METHODS The medical records of 492 eyes of 308 patients with high myopia (myopic refractive error > 8 diopters or axial length ≥ 26.5 mm) with a follow-up of 5 years or more were reviewed. The VFs were determined by Goldmann kinetic perimetry, and the VFs were quantified in 100 sectors within the V4 isopter. Eyes with loss of 10% or more of the sectors were classified as having significant VF defects, and a further loss of 10% or more during the follow-up period was classified as a significant progression. To avoid the influence of the posterior fundus changes resulting from the high myopia, eyes with any type of myopic macular or peripheral lesions that could cause visual field defects were excluded. RESULTS Significant VF defects were newly developed in 13.2% of these selected highly myopic eyes during a mean follow-up ± standard deviation of 11.6 ± 5.5 years. The incidence of significant VF defects in myopic eyes was significantly higher in eyes with an oval optic disc than that in eyes with a round optic disc. An oval optic disc was present significantly more frequently in the myopic eyes with VF defects. Temporal and nasal VF defects were present in the same eye. Among the eyes with significant VF defect, the temporal VF defects were observed in 61.5% of the eyes with round discs, in 75.0% of the eyes with vertically oval discs, and in 68.2% of the eyes with obliquely oval discs. During a mean follow-up ± standard deviation of 10.2 ± 3.4 years, 73.8% of the eyes showed a significant progression of the VF defects. An abrupt change of the scleral curvature (types VII and IX staphyloma by Curtin) was the only factor significantly associated with a progression of the VF defects. CONCLUSIONS Because the VF defects are progressive, we suggest that high myopia is a high risk factor for VF defects and that these eyes be examined at least once yearly. The combination of stretching and distortion of the optic nerve fibers resulting from an abrupt change of scleral curvature may be the factors that lead to the damage of the optic nerve fibers in highly myopic eyes.

Altered Function of Factor I Caused by Amyloid β: Implication for Pathogenesis of Age-Related Macular Degeneration from Drusen

The results of recent studies have implicated local inflammation and complement activation as the processes involved in the pathogenesis of age-related macular degeneration (AMD). We have demonstrated that amyloid β (Aβ), which is deposited in drusen, causes an imbalance in the angiogenesis-related factors in retinal pigment epithelial cells. We have also shown that neprilysin gene-disrupted mice accumulate Aβ, and develop several features of AMD. The purpose of this study was to investigate the mechanisms involved in the development of AMD that are triggered by Aβ. Our results showed that Aβ binds to complement factor I which inhibits the ability of factor I to cleave C3b to inactivated iC3b. Factor H and factor I are soluble complement-activation inhibitors, and preincubation of factor I with Aβ in the presence of factor H abolished the ability of Aβ to cleave C3b, and also abolished the ability of factor I to cleave FGR-AMC. In contrast, Aβ did not affect the function of factor H even after binding. The production of iC3b was significantly decreased when C3b and factor H were incubated with the eyes from neprilysin gene-disrupted mice as compared with when C3b and factor H were incubated with eyes from age-matched wild-type mice. These results suggest that Aβ activates the complement system within drusen by blocking the function of factor I leading to a low-grade, chronic inflammation in subretinal tissues. These findings link four factors that have been suggested to be associated with AMD: inflammation, complement activation, Aβ deposition, and drusen.

Characteristics of patients with a favorable natural course of myopic choroidal neovascularization

ObjectiveTo identify the characteristics of patients with myopic choroidal neovascularization (CNV) who had a favorable visual prognosis without treatment.MethodsWe reviewed the medical records of 52 consecutive patients (57 eyes) with myopic CNV who were followed for at least 5 years after the onset of CNV. Clinical characteristics (patient age, CNV size and location, visual acuity at onset, chorioretinal atrophy development around CNV, and degree of myopia) were compared between patients whose visual acuity 5 years after CNV onset was better than 20/40 and those whose visual acuity was worse than 20/200.ResultsAmong 57 eyes, eight eyes (14.0%; 8 patients) had a final visual acuity better than 20/40. On the other hand, 37 eyes (64.9%; 33 patients) had a final visual acuity worse than 20/200. Statistical analysis revealed that the patients with a good prognosis (final visual acuity better than 20/40) were significantly younger, had significantly smaller CNV, and significantly better initial visual acuity (Mann–Whitney U-test, p<0.05). Juxtafoveal CNV was more frequently observed in patients with a good prognosis than in those with a poor prognosis (Fisher’s exact probability test, p<0.05). Only one patient (12.5%) in the good prognosis group developed a very limited area of chorioretinal atrophy around the regressed CNV, while 91.9% of the patients in the poor prognosis group developed chorioretinal atrophy. Refractive status and the axial length measurements did not differ between the two groups.ConclusionsSome young patients with myopic CNV retain favorable vision over the long term without active treatment. These information might be useful to predict the visual outcome of patients with myopic CNV.

Fundus Characteristics of High Myopia in Children

PurposeTo evaluate the fundus characteristics of highly myopic eyes in children.MethodsWe reviewed the medical records of 46 children (1 to 8 years old; mean age, 6.8 years) (80 eyes) with high myopia (4 D or more for children younger than 5 years, 6 D or more for children aged 6–8 years) seen consecutively during a 10-year period at the high-myopia clinic in our hospital. Children of up to 8 years of age at the initial visit were included in the study.ResultsFundus examination revealed posterior staphyloma in only one eye (1.3%) and mild chorioretinal atrophy around the optic disc in 13 eyes (16.3%). There were no patients with choroidal neovascularization or geographic atrophy in the posterior fundus. Myopic peripapillary crescent was observed in 26 eyes (33.8%), but the area of the crescent was relatively small (mean, 0.5 disc area).ConclusionsThe results of the present study showed that myopic fundus changes are uncommon and mild in children. They suggest that aging, in addition to mechanical stretching of the eyeball, might be important for the development of myopic fundus changes. Jpn J Ophthalmol 2005;49:306–311© Japanese Ophthalmological Society 2005

Factors associated with the development of chorioretinal atrophy around choroidal neovascularization in pathologic myopia

PurposeTo examine the influencing factors on the development of chorioretinal atrophy, which is the main cause of long-term visual decrease in myopic choroidal neovascularization (CNV), in a large series of highly myopic patients.MethodsSixty-five patients (81 eyes) with myopic CNV were studied retrospectively. The influence of the patient’s age, refractive error, axial length, visual acuity at onset of CNV, size of CNV, and grade of myopic retinopathy on the extent of chorioretinal atrophy more than 3 years after CNV onset was investigated by means of multiple linear regression analysis.ResultsSeventy-seven of 81 eyes (95.1%) developed chorioretinal atrophy around myopic CNV during the follow-up period. Multiple linear regression revealed that age was the most influencing factor for the development of chorioretinal atrophy in all the subjects. When we divided the subjects into two groups according to their age, however, CNV size was the only factor to influence the development of chorioretinal atrophy in the patients younger than 40 years, whereas age was still the only influencing factor in those older than 40 years.ConclusionsThe factors influencing the development of chorioretinal atrophy differ according to patient age. Local factors, such as CNV size, determine the tendency to develop chorioretinal atrophy in young patients. Systemic factors, such as patient age, play a greater part in older subjects.