Natsuko Nagaoka

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.

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.

Glaucomatous-Type Optic Discs in High Myopia

Purpose To assess the prevalence of glaucoma in patients with high myopia defined as myopic refractive error of >-8 diopters or axial length ≥26.5 mm. Methods The hospital-based observational study included 172 patients (336 eyes) with a mean age of 61.9±12.3 years and mean axial length of 30.1±2.3 mm (range: 24.7–39.1mm). Glaucomatous-type optic discs were defined by glaucomatous optic disc appearance. Glaucoma was defined by glaucomatous optic disc appearance and glaucomatous Goldmann visual field defects not corresponding with myopic macular changes. Results Larger disc area (mean: 3.18±1.94 mm2) was associated with longer axial length (P<0.001; standardized correlation coefficient: 0.45). Glaucoma was detected in 94 (28%; 95% Confidence intervals: 23%, 33%) eyes. In multivariate analysis, glaucoma prevalence was 3.2 times higher (P<0.001) in megalodiscs (>3.79 mm2) than in normal-sized discs or small discs (<1.51 mm2) after adjusting for older age. Axial length was not significantly (P = 0.38) associated with glaucoma prevalence in that model. Glaucoma prevalence increased by a factor of 1.39 for each increase in optic disc area by one mm2. Again, axial length was not significantly (P = 0.38) associated with glaucoma prevalence when added to this multivariate model. Conclusion Within highly myopic individuals, glaucoma prevalence increased with larger optic disc size beyond a disc area of 3.8 mm2. Highly myopic megalodiscs as compared to normal sized discs or small discs had a 3.2 times higher risk for glaucomatous optic nerve neuropathy. The increased glaucoma prevalence in axial high myopia was primarily associated with axial myopia associated disc enlargement and not with axial elongation itself.

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

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

Characteristics of Periconus Choroidal Neovascularization in Pathologic Myopia

PURPOSE To examine the clinical characteristics of periconus choroidal neovascularizations (CNVs) in eyes with pathologic myopia. DESIGN Retrospective observational case series. METHODS We reviewed the medical records of 260 eyes of 254 patients with a myopic CNV and selected those with a periconus CNV. A periconus CNV was defined as a CNV that is located next to a myopic conus. To differentiate a periconus CNV from a subfoveal CNV that has expanded to the edge of the disc, eyes whose CNV involved the fovea were excluded. The data of the eyes with a subfoveal CNV were used for comparison. RESULTS Eleven eyes (4.2%) of 11 patients had a periconus CNV. These CNVs had a triangular or oval shape whose base was directed toward the myopic conus. The eyes with a periconus CNV had a significantly larger myopic conus than eyes with a subfoveal myopic CNV. The degree of myopia and axial length were not significantly associated with the incidence of periconus CNV. In 5 of the 11patients, the periconus CNV had a spontaneous regression without treatment, and none had a recurrence. The other 6 patients received treatment and obtained an angiographic closure after a single treatment. A chorioretinal atrophy (ChRA) developed in 3 eyes, and the ChRA enlarged over the fovea in 2 of these eyes, which led to a significant decrease of vision. CONCLUSION A periconus CNV is rare (4.2%) among the eyes with a myopic CNV and is more likely to develop in eyes with a large myopic conus. The absence of a significant association between the degree of myopia and incidence of periconus CNV suggests that the morphologic characteristics of the eye are not the causes of the periconus CNV. Although an angiographic closure can be easily attained with or without the treatment, the later development and progression of ChRA can impair vision.

SIX-YEAR OUTCOMES OF INTRAVITREAL BEVACIZUMAB FOR CHOROIDAL NEOVASCULARIZATION IN PATIENTS WITH PATHOLOGIC MYOPIA.

Purpose: To investigate the 6-year outcome of intravitreal bevacizumab (IVB) to treat eyes with active choroidal neovascularization (CNV) due to pathologic myopia. Methods: Medical records of 36 eyes of 35 consecutive patients with high myopia (refractive error ≥8 D or axial length ≥26.5 mm) and active CNV, who had been treated with IVB and followed for ≥6 years were analyzed. The factors that predicted the best-corrected visual acuity (BCVA) at 6 years after IVB were determined by multiple regression analyses. Results: The mean age of the subjects was 58 years, and the mean axial length was 29 mm. Twenty-one eyes had subfoveal CNV and 15 eyes had nonsubfoveal CNV. During the 6-year follow-up, the mean number of IVB was 1.78. The mean BCVA logMAR (equivalent Snellen visual acuity) was 0.50 (20/63), 0.31 (20/40), 0.39 (20/50), and 0.45 (20/63) at the baseline, and at 2, 4, and 6 years after the IVB. The BCVA was significantly improved at 2 and 4 years compared with baseline values but not at 6 years. Stepwise multiple regression analyses showed that the BVCA at 6 years was significantly correlated with the size of the CNV-related macular atrophy, and the baseline BCVA and CNV size. Conclusion: The significant correlation between the BCVA at 6 years and the size of the macular atrophy indicates that treatments to prevent the development of macular atrophy are important for the long-term visual outcome in eyes with active CNV.

Choroidal folds radiating from the edge of an inferior staphyloma in an eye with tilted disc syndrome

This patient showed no vitreous remnants on the retina, different from what is usual in highly myopic eyes. This might have been caused by the mutated CHM gene, leading to abnormal cell adhesion to the extracellular matrix. The association between retinal degenerative diseases and vitreous abnormality may be an interesting topic for future investigations. The thinness of the retina may have caused the separation of the retinal tissue at the macula (which lacks a tangential synaptic network) without as much tractional force as the adherent vitreous cortex exerts on the ILM. Since the RPE was atrophic and the retina–RPE adhesion was probably fragile, retinal detachment might have developed over time. Therefore, the pathogenic mechanism in this case was not related mainly to the patient’s high myopia but to the characteristics of choroideremia, although the infl uence of tangential force could not be eliminated. The ILM in this case was very thin. The presence of chronic chorioretinal infl ammation in choroideremia is suggested by post mortem retina fi ndings in a 30-year-old male choroideremia patient. Thus, the ILM, which is generated by Müller glial cells as their basement membrane, may also degenerate in this disease. These perioperative fi ndings not only reveal the pathology of choroideremia but also show the value of conducting a follow-up of choroideremia patients for the detection and treatment of secondary changes associated with this disease.

Long-term Pattern of Progression of Myopic Maculopathy

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.

Clinical characteristics of patients with congenital high myopia

BackgroundFour cases of congenital high myopia showing similar myopic fundus changes.CasesThe clinical characteristics of four cases of congenital high myopia are presented, and the long-term course of one of these cases is described.ObservationsOne patient had a history of preterm delivery; however, the remaining patients had no ocular or systemic abnormalities that could account for the congenital myopia. All of the cases showed similar fundus findings, including tilting of the optic disc, temporal conus, and tessellated fundus. A type II posterior staphyloma was found in three of the cases. All of the patients had best-corrected visual acuity (BCVA) ≥0.4. One patient was followed for 30 years (from 6 to 36 years of age), and during those 30 years, the posterior fundus showed only a slight enlargement of a myopic conus and slight increase of the tilting of the optic disc despite a 3.0-mm increase in the axial length, and final BCVA was 1.0 OU.ConclusionsLong-term follow-up showed that the posterior fundus did not change radically and good vision as maintained in spite of a significant increase in the axial length.

Glaucoma in high myopia and parapapillary delta zone

Purpose To examine the prevalence of glaucomatous optic neuropathy (GON) in a medium myopic to highly myopic group of patients and its association with parapapillary gamma zone and parapapillary delta zone. Methods The retrospective observational hospital-based study included patients who had attended the Tokyo High Myopia Clinics within January 2012 and December 2012 and for whom fundus photographs were available. GON was defined based on the appearance of the optic nerve head on the fundus photographs. Results The study included 519 eyes (262 individuals) with a mean age of 62.0±14.3 years (range:13–89 years) and mean axial length of 29.5±2.2 mm (range:23.2–35.3mm). GON was present in 141 (27.2%; 95% confidence intervals (CI): 23.3, 31.0%) eyes. Prevalence of GON increased from 12.2% (1.7, 22.7) in eyes with an axial length of <26.5mm to 28.5% (24.4, 32.5) in eyes with an axial length of ≥26.5mm, to 32.6% (27.9, 37.2) in eyes with an axial length of ≥28mm, to 36.0% (30.5, 41.4) in eyes with an axial length of ≥29mm, and GON prevalence increased to 42.1% (35.5, 48.8) in eyes with an axial length of ≥30mm. In multivariate analysis, higher GON prevalence was associated (Nagelkerke r2: 0.28) with larger parapapillary delta zone diameter (P<0.001; odds ratio (OR):1.86;95%CI:1.33,2.61), longer axial length (P<0.001;OR:1.45;95%CI:1.26,1.67) and older age (P = 0.01;OR:1.03;95%CI:1.01,1.05). If parapapillary delta zone width was replaced by the vertical disc diameter, higher GON prevalence was associated (r2:0.24) with larger vertical optic disc diameter (P = 0.04;OR:1.70;95%CI:1.03,2.81), after adjusting for longer axial length (P<0.001;OR:1.44;95%CI:1.26,1.64) and older age (P<0.001;OR:1.04;95%CI:1.02,1.06). Conclusions Axial elongation associated increase in GON prevalence (mean: 28.1% in a medium to highly myopic study population) was associated with parapapillary delta zone as surrogate for an elongated peripapillary scleral flange and with larger optic disc size.