Factores asociados con el desprendimiento neurosensorial macular en pacientes miopes magnos con estafiloma inferior / Factors associated with serous retinal detachment in highly myopic eyes with inferior posterior staphyloma

OBJETIVO: Investigar los factores clínicos asociados con el desprendimiento neurosensorial macular en ojos de pacientes miopes magnos con estafiloma inferior. MATERIAL Y MÉTODO: Estudio transversal de 27 pacientes miopes magnos (44 ojos) con estafiloma inferior. Todos los pacientes fueron sometidos a un examen oftalmológico completo, incluyendo angiografía fluoresceína. El grosor coroideo, la interfaz vitreorretiniana y la altura del envainamiento macular se analizaron mediante el dispositivo DRI OCT Triton Swept-Source (Topcon Corp., Tokio, Japón). RESULTADOS: De los 44 ojos, 13 presentaron un desprendimiento de la retina neurosensorial y 31 carecían del mismo. No observamos diferencias estadísticamente significativas en ninguna de las variables estudiadas (edad, sexo, equivalente esférico, longitud axial, agudeza visual, tracción vitreomacular, membrana epirretiniana o desprendimiento de membrana limitante interna). Los ojos con desprendimiento de la retina neurosensorial presentaron una mayor altura del envainamiento macular (p = 0,01) y una reducción del espesor coroideo (p = 0,02). El desprendimiento neurosensorial macular siempre se produjo en el borde superior del estafiloma inferior y se caracterizó por una hiperfluorescencia granular en la angiografía fluoresceínica. CONCLUSIONES: Una mayor altura del envainamiento macular y un menor grosor coroideo podrían ser factores importantes en el desarrollo del desprendimiento neurosensorial macular en pacientes miopes magnos con estafiloma inferior

PURPOSE: To investigate the factors associated with persistent serous retinal detachment in highly myopic eyes with inferior posterior staphyloma. METHODS: A total of 27 highly myopic patients (44 eyes) with an inferior posterior staphyloma were recruited. Serous retinal detachment was investigated; 13 eyes had persistent sub-macular fluid (study group), and 31 eyes lacked sub-macular fluid (control group). All patients underwent complete ophthalmologic examinations, including axial length measurement and fluorescein angiography (FA). Triton Deep Range Imaging (DRI) optical coherence tomography (OCT) (Topcon Corp., Tokyo, Japan) scans through the fovea measured choroidal thicknesses, macular bend height, and vitreoretinal interface factors. RESULTS: Of the 44 eyes, 13 had neurosensory retinal detachment and 31 did not. No significant differences were found in any of the studied variables (age, gender, spherical equivalence, axial length, vitreomacular traction, epiretinal membrane, internal limiting membrane detachment), except a higher macular bend height (p = 0.01), and a reduced macular choroidal thickness (p = 0.02), which were associated with the risk of serous retinal detachment. No statistically significant differences in best-corrected visual acuity (BCVA) were observed between the study and control groups. Serous retinal detachment always occurred at the bisected retinal pigment epithelium of the macula corresponding to the upper edge of the staphyloma, and was characterised by multiple hyperfluorescent granular patches on fluorescein angiography. CONCLUSIONS: A higher macular bend height and a reduced macular choroidal thickness may be important factors in the development of serous retinal detachment in patients with inferior posterior staphyloma

Crosstalk between EP2 and PPARα Modulates Hypoxic Signaling and Myopia Development in Guinea Pigs.

Purpose: Cyclic adenosine monophosphate (cAMP) and peroxisome proliferator-activated receptor alpha (PPARα) levels mediate extracellular matrix (ECM) changes by altering the levels of hypoxia-inducible factor 1-alpha (HIF-1α) in various tissues. We aimed to determine, in the sclera of guinea pigs, whether a prostanoid receptor (EP2)-linked cAMP modulation affects PPARα and HIF-1α signaling during myopia. Methods: Three-week-old guinea pigs (n = 20 in each group), were monocularly injected with either an EP2 agonist (butaprost 1 µmol/L/10 µmol/L), an antagonist (AH6809 10 µmol/L/30 µmol/L) or a vehicle solution for two weeks during normal ocular growth. Separate sets of animals received these injections and underwent form deprivation (FD) simultaneously. Refraction and axial length (AL) were measured at two weeks, followed by scleral tissue isolation for quantitative PCR (qPCR) analysis (n = 10) and cAMP detection (n = 10) using a radioimmunoassay. Results: Butaprost induced myopia development during normal ocular growth, with proportional increases in AL and cAMP levels. FD did not augment the magnitude of myopia or cAMP elevations in these agonist-injected eyes. AH6809 suppressed cAMP increases and myopia progression during FD, but had no effect in a normal visual environment. Of the diverse set of 27 genes related to cAMP, PPARα and HIF-1α signaling and ECM remodeling, butaprost differentially regulated 15 of them during myopia development. AH6809 injections during FD negated such differential gene expressions. Conclusion: EP2 agonism increased cAMP and HIF-1α signaling subsequent to declines in PPARα and RXR mRNA levels, which in turn decreased scleral fibrosis and promoted myopia. EP2 antagonism instead inhibited each of these responses. Our data suggest that EP2 suppression may sustain scleral ECM structure and inhibit myopia development.

The diversified defocus profile of the near-work environment and myopia development.

PURPOSE: To quantify the defocus characteristics in the near-work environment at home and investigate the relationship with subsequent myopia progression. METHODS: Fifty subjects (aged 7-12 years) were recruited and followed for 1 year. The home near-work environment (writing desk) was measured at a baseline home-visit using the Kinect-for-Windows to capture a 3-dimensional image. The depth values of the image were then converted into scene defocus with respect to the subject's viewpoint. The defocus characteristics were quantified as the dioptric volume (the total amount of net defocus, or DV) and standard deviation of the defocus values (SDD ). Information on home size, time spent outdoors, and in front of a desk were also obtained. Univariate correlation, and multivariate regression were used to assess the association between myopia progression, defocus characteristics, and other co-variates. RESULTS: The baseline spherical equivalent refraction (M) and refraction change over 1 year (∆M) were - 1.51 ± 2.02 D and - 0.56 ± 0.45 D respectively. DV was not significantly correlated with ∆M (Spearman's ρ = -0.25, p = 0.08), while SDD was negatively correlated to ∆M (Spearman's ρ = -0.42, p = 0.003). Although SDD was not a significant predictor in multivariate analysis, the regional DV at 15°-20° eccentricity was significant (p = 0.001). Home size (F2,50  = 7.01, p = 0.002) and time spent outdoors (Independent t = -2.13, p = 0.04) were also associated with ∆M, but not time spent in front of desk (Independent t = 0.78, p = 0.44). CONCLUSION: The defocus profile in the home environment within the para-central field of view is associated with childhood refractive error development.

Management of coexistent choroidal neovascular membrane and macular hole with serous detachment in a case of pathological myopia: challenges and dilemmas.

Coexistent choroidal neovascular membrane and macular hole with serous retinal detachment in pathological myopia is a rare entity and may be quite challenging to manage. We present such a case managed with bevacizumab injection and vitrectomy. Although the retina was attached at the final follow-up, the visual outcome was poor, due to non-closure of hole.

Foveosquisis miópica asociada al Síndrome de Straatsma / Myopic foveoschisis associated with Straatsma's syndrome

RESUMEN Este reporte describe la presentación concomitante de foveosquisis miópica y fibras de mielina en una paciente con miopía elevada. La primera puede ser un hallazgo incidental, pero no infrecuente en pacientes con miopía elevada, y causa pérdida visual central severa. La segunda es infrecuente, y en casos muy raros se presenta asociada a miopía ipsilateral y estrabismo. Ambas entidades deben incluirse en el diagnóstico diferencial de un paciente alto miope con disminución de la visión central. La tomografía de coherencia óptica es el medio auxiliar de elección(AU)

ABSTRACT This case report describes the concomitant presentation of myopic foveoschisis and myelinated retinal nerve fibers in a patient with elevated myopia. The myopic foveoschisis may be an incidental but not uncommon finding in highly myopic eyes, leading to severe central vision loss. Myelinated retinal nerve fibers are infrequent and in rarely cases they are associated with ipsilateral myopia and strabismus. Both entities should be including as differential diagnosis of central vision loss in high myopia, and optic coherence tomography is the leading as auxiliary test(AU)

OLIMPIC: a 12-month study on the criteria driving retreatment with ranibizumab in patients with visual impairment due to myopic choroidal neovascularization.

PURPOSE: To evaluate criteria driving retreatment with ranibizumab in Italian patients with myopic choroidal neovascularization (mCNV). METHODS: OLIMPIC was a 12-month, phase IIIb, open-label study. Patients with active mCNV were treated with ranibizumab 0.5 mg according to the European label. The study assessed local criteria in Italy driving retreatment decisions with ranibizumab; and the efficacy, safety, and tolerability of ranibizumab. RESULTS: The mean (standard deviation [SD]) age of treated patients (N = 200) was 61.8 (12.7) years; range 22-85 years. The multivariate regression model indicated that presence of active leakage (odds ratio [OR] 95% confidence interval [CI]: 11.30 [1.03-124.14]), presence of intraretinal fluid (OR [95%CI]: 28.21 [1.55-513.73]), and an improvement in best-corrected visual acuity (BCVA) from baseline < 10 letters (OR [95%CI]: 17.60 [1.39-222.75]) were the factors with the greatest effect on retreatment with ranibizumab. The mean (SD) BCVA gain from baseline to month 12 was 8.4 (12.8) letters (P < 0.0001). The mean (SD) number of injections was 2.41 (1.53); range 1-9. Ocular and non-ocular adverse events were reported in 41 (20.5%) and 30 (15.0%) patients, respectively. CONCLUSIONS: Individualized treatment with ranibizumab was effective in improving BCVA in patients with mCNV over 12 months. Both anatomical and functional variables had significant effects on causing retreatment. There were no new safety findings. TRIAL REGISTRATION: www.ClinicalTrials.Gov (NCT No: NCT02034006).

Acquired myopia in Vogt-Koyanagi-Harada disease.

PURPOSE: To analyze the change in refractive error and the axial length of chronic Vogt-Koyanagi-Harada (VKH) disease. METHODS: Medical records of 106 eyes of 54 adult VKH patients were analyzed. The refractive error and the axial length were compared between the baseline (defined as the time point at least 2 weeks after acute stage of VKH) and the final visit. The rate of the eyes with significant myopia progression [defined as refraction change toward myopia > 1 diopter (D)] was examined. The correlation of the degree of sunset glow fundus/choroidal thickness with the change in refractive error was also evaluated. RESULTS: At the final visit, the mean refractive error was more myopic and the axial length was longer than at baseline. Seventeen of the 106 eyes (16.0%) showed significant myopia progression. The mean change in refractive error during a follow-up in these 17 eyes was - 2.7 D (range - 7.5 to - 1.1 D). The axial length data both at baseline and at the final visit were obtained only in 8 eyes. The mean change in axial length during a follow-up in these 8 eyes was 1.3 mm (range 0-3.7 mm). In the cases with myopia progression, sunset glow fundus was more frequent and subfoveal choroid was thinner than those without myopia progression. CONCLUSIONS: Myopia progression as well as increase in axial length occurs in VKH disease. The link between choroidal thinning and axial length elongation in VKH patients gives some insights into axial length increase of pathologic myopia.

Ocular and systemic manifestations of beta-propeller protein-associated neurodegeneration.

Beta-propeller protein-associated neurodegeneration (BPAN) is a rare genetic disorder characterized by neurodegeneration with brain iron accumulation (NBIA). We report an infant diagnosed with BPAN who was found to have high myopia and astigmatism, strabismus, and bilateral retinal pigmentary changes. While retinal pigmentary changes have been described in other disorders of NBIA, it has been only rarely reported in BPAN.

Knobby Eye Syndrome.

INTRODUCTION: A spherical globe is traditionally assumed, but this study employed magnetic resonance imaging (MRI) to demonstrate frequent occurrence of non-spherical staphylomata in strabismic patients. METHODS: High-resolution, surface coil MRI was obtained in multiple image planes in 21 highly myopic subjects (36 eyes) and compared with 17 normal controls (33 eyes). Images were analyzed for axial length, aspect ratio of eye shape, and deflection of muscle paths. RESULTS: All but two high myopes had strabismus. While myopic globes were generally spherical in 10 myopic eyes including both orthotropic subjects, 15 globes exhibited diffuse posterior staphylomata, 16 equatorial staphylomata, and 4 both posterior and equatorial staphylomata. Equatorial scleral ectasias were positioned to contact and elongate paths of horizontal rectus muscles in some gaze positions. Axial length in myopes averaged 28.8 ± 3.8 (SD) mm and did not differ significantly between regular vs. irregular staphylomata. Globe aspect ratios in the coronal, axial, and sagittal planes were significantly greater than normal in myopes (P < 0.005), but correlated significantly with axial length only in the axial and sagittal planes (P < 0.03). While myopes with irregular staphylomata were older at 57 ± 11 years than subjects with spherical globes at 24 ± 8 years (P < 0.0005), other clinical features were similar. CONCLUSION: Irregular equatorial or posterior staphylomata are common in strabismic axial high myopes, acting, like "cams" affixed to the normally spherical globe so that they may have no mechanical effect until rotating eccentrically against muscles. After rotational contact, staphylomata would nonlinearly increase muscle tension with further duction. Imaging may be clinically informative about this "knobby eye syndrome."

Knobloch syndrome associated with Polymicrogyria and early onset of retinal detachment: two case reports.

BACKGROUND: Knobloch Syndrome (KS) is a rare congenital syndrome characterized by occipital skull defects and vitreoretinal degeneration. Retinal detachment (RD) often occurs at the end of the first decade of life or later. Aside from occipital skull defects, central nervous system abnormalities are uncommon. CASE PRESENTATIONS: We report on two siblings with KS. The first, a seven month old male, presented with nystagmus and was found to have a serous RD and a tessellated retinal appearance. His sister had a history of multiple visual abnormalities and had a similar retinal appearance although no signs of RD, but retina staphylomas. Genetic testing performed on both siblings showed a mutation in COL18A1, diagnostic of KS. MRI of both siblings demonstrated polymicrogyria but did not show occipital defects. CONCLUSIONS: Although several families with KS have been described previously, our case is noteworthy for several reasons. The RD observed in our first patient occurred at an early age, and we find evidence of only one patient with KS who had an RD identified at an earlier age. The findings of polymicrogyria are not characteristic of KS, and we found only a few previous reports of this association. Additionally, we review potential treatment options for this condition.

[Intravitreal ranibizumab therapy for choroidal neovascularization secondary to pathological myopia]. / Patológiás myopia talaján kialakult érújdonképzodés intravitrealis ranibizumabkezelése.

INTRODUCTION: Pathological myopia is one of the leading causes of vision loss worldwide, especially among young people of working age. Choroidal neovascularization is one of the most important cause of visual impairment in pathological myopia. AIM: To evaluate the efficacy of intravitreal ranibizumab for the treatment of myopic choroidal neovascularization. METHOD: In this retrospective analysis 14 eyes of 14 patients (mean age: 61 ± 17 years) with myopic choroidal neovascularization were treated with intravitreal ranibizumab as needed. Best-corrected visual acuity, thickness of choroidal neovascularization lesion and the number of injections were assessed. RESULTS: The mean visual acuity changed from 55.8 ± 19.3 letters to 64.8 + 15.5 at 12 months (p = 0.0414), and 62.6 ± 16.3 during follow-up time (p = 0.2896). Mean follow-up time was 19.7 ± 23.9 months, average number of injections was 2.8 ± 2.1. Visual acuity declined in four patients despite the treatment. CONCLUSIONS: Intravitreal ranibizumab is an effective therapy in pathological myopia. Some patients experience deterioration of visual acuity despite of treatment. Orv. Hetil., 2017, 158(15), 579-586.

Pressureless Orbital Decompression for Myopic Proptosis.

Orbital decompression surgery increases the orbital volume. It has rarely been used for proptosis of the large highly myopic globe. However, external decompression surgery carries significant risks because of the large thin-walled globe. The authors report the first use of endoscopic medial wall orbital decompression surgery in this setting to obviate the risk of globe pressure.Endoscopic medial wall decompression brought about a 4 mm reduction of proptosis, correction of exotropia and elimination of retrobulbar ache providing good symmetry with the fellow eye.Endoscopic medial wall orbital decompression can be very effective for correcting the proptosis of high myopia and minimizes the risk of damage to the very large, thin-walled globe.

EPIDEMIC OF PATHOLOGIC MYOPIA: What Can Laboratory Studies and Epidemiology Tell Us?

PURPOSE: To systematically review epidemiologic and laboratory studies on the etiology of high myopia and its links to pathologic myopia. METHODS: Regular Medline searches have been performed for the past 20 years, using "myopia" as the basic search term. The abstracts of all articles have been scrutinized for relevance, and where necessary, translations of articles in languages other than English were obtained. RESULTS: Systematic review shows that there is an epidemic of myopia and high myopia in young adults in East and Southeast Asia, with similar but smaller trends in other parts of the world. This suggests an impending epidemic of pathologic myopia. High myopia in young adults in East and Southeast Asia is now predominantly associated with environmental factors, rather than genetic background. Recent clinical trials show that the onset of myopia can be reduced by increasing the time children spend outdoors, and methods to slow the progression of myopia are now available. CONCLUSION: High myopia is now largely associated with environmental factors that have caused the epidemic of myopia in East and Southeast Asia. An important clinical question is whether the pathologic consequences of acquired high myopia are similar to those associated with classic genetic high myopia. Increased time outdoors can be used to slow the onset of myopia, whereas methods for slowing progression are now available clinically. These approaches should enable the current epidemics of myopia and high myopia to be turned around, preventing an explosion of pathologic myopia.

Environmental Factors and Myopia: Paradoxes and Prospects for Prevention.

The prevalence of myopia in developed countries in East and Southeast Asia has increased to more than 80% in children completing schooling, whereas that of high myopia has increased to 10%-20%. This poses significant challenges for correction of refractive errors and the management of pathological high myopia. Prevention is therefore an important priority. Myopia is etiologically heterogeneous, with a low level of myopia of clearly genetic origins that appears without exposure to risk factors. The big increases have occurred in school myopia, driven by increasing educational pressures in combination with limited amounts of time spent outdoors. The rise in prevalence of high myopia has an unusual pattern of development, with increases in prevalence first appearing at approximately age 11. This pattern suggests that the increasing prevalence of high myopia is because of progression of myopia in children who became myopic at approximately age 6 or 7 because age-specific progression rates typical of East Asia will take these children to the threshold for high myopia in 5 to 6 years. This high myopia seems to be acquired, having an association with educational parameters, whereas high myopia in previous generations tended to be genetic in origin. Increased time outdoors can counter the effects of increased nearwork and reduce the impact of parental myopia, reducing the onset of myopia, and this approach has been validated in 3 randomized controlled trials. Other proposed risk factors need further work to demonstrate that they are independent and can be modified to reduce the onset of myopia.

Preserved retinal sensitivity in spatial correspondence to an intrachoroidal cavitation area with full thickness retinal defect: a case report.

BACKGROUND: Intrachoroidal cavitation (ICC) in the temporal peripapillary area is a relatively frequent finding in high myopia. However, ICC associated with a full thickness retinal defect rarely observed. We report an unusual case of ICC combined with a full thickness retinal defect in the papillo-macular bundle, in which the spatially corresponding visual field sensitivity was preserved. CASE PRESENTATION: A high myopic and pseudophakic left eye of a 79-year-old Japanese woman was evaluated with swept source optical coherence tomography (SS-OCT) and Humphrey 30-2 visual field testing for moderate glaucoma. The best-corrected visual acuity was 20/20, the axial length was 28.77 mm, the mean deviation -8.94 dB, and the intraocular pressure was 15 mmHg without medication. The horizontal SS-OCT scans showed a wide ICC with a full thickness retinal defect in the papillo-macular area at the outer border of the myopic peripapillary beta zone atrophy. The retina was herniated into the ICC area. While no sensitivity loss was seen in the central visual field corresponding to the full thickness retinal defect, a glaucomatous visual field deterioration spatially corresponding to the glaucomatous disc damage was present. The preserved retinal sensitivity spatially corresponding to the full thickness retinal defect was confirmed with microperimetry. CONCLUSIONS: Our case suggests that retina herniated in peripapillary ICC temporal to the disc may preserve some function despite the presence of a retinal defect.

Intraocular lens exchange for high myopia in pseudophakic children.

PurposeThe purpose of this study was to examine the preoperative factors and postoperative outcomes following intraocular lens (IOL) exchange for high myopia in pseudophakic children.MethodsThe medical records of all patients undergoing IOL exchange for high myopia were retrospectively reviewed.ResultsA total of 15 eyes were identified that had undergone an IOL exchange for myopic shift. Average age of cataract extraction (CE) was 5.4 months. In all, 10/15 had a unilateral cataract. IOL exchange usually occurred at an average of 6 years following cataract surgery. The average spherical equivalent (SE) of the refractive error at that time was -9.6 D. Following IOL exchange, SE was -1.3 D. A two-line reduction in best-corrected visual acuity was observed in 1/13 of our patients for whom pre- and post-exchange data were available. The average axial length (AL) of the eye undergoing the IOL exchange was 24.0 mm, average AL in the non-operative eye was 22.1 mm. On average, the operative eyes grew 4.4 mm and the non-operative eyes 3.02 mm. No adverse events were seen in the operative eyes.ConclusionYounger age at the time of CE creates a greater likelihood of AL elongation and predisposes a child to myopic shift. IOL exchange should be considered an option to reduce anisometropia and associated aniseikonia to improve visual outcomes. Successful visual rehabilitation and predictable post-exchange refractions were seen with our patients.