LONG-TERM PROGRESSION PATTERN OF MYOPIC TRACTIONAL MACULOPATHY: Outcomes and Risk Factors.

PURPOSE: To evaluate the long-term progression pattern of myopic tractional maculopathy and the risk factors. METHODS: The prevalence and grade of myopic tractional maculopathy were assessed with optical coherence tomography at enrollment and at the 2-year follow-up. The severity of posterior staphyloma and the presence of dome-shaped macula were also evaluated. RESULTS: In total, 610 highly myopic eyes of 610 patients were analyzed. The prevalence of epiretinal membrane, myopic retinoschisis, and macular hole increased from 26.7%, 12.1%, and 4.4% at enrollment to 41.1%, 18.2%, and 9.5% at the 2-year follow-up, respectively. Epiretinal membrane progressed in 21.8% of eyes, but visual acuity did not decline significantly in these eyes. Myopic retinoschisis progressed in 6.8% of eyes, and macular hole progressed in 14.8% of eyes. Significantly greater best-corrected visual acuity reduction was detected in the eyes with myopic retinoschisis or macular hole progression than the rest ( P < 0.05). Multivariate analysis showed that longer axial length, more-severe posterior staphyloma, and absence of dome-shaped macula were associated with myopic tractional maculopathy progression. CONCLUSION: In highly myopic eyes, long-term visual acuity was relatively stable in those with epiretinal membrane, but was significantly affected by myopic retinoschisis or macular hole progression. Longer axial length, more-severe posterior staphyloma, and absence of dome-shaped macula were risk factors for myopic tractional maculopathy progression.

[Occurrence of myopic maculopathy in eyes with staphylomas of various localization]. / Chastota formirovaniya miopicheskoi makulopatii pri stafilomakh razlichnoi lokalizatsii.

Complicated myopia develops in young people of working age, of which about 30% subsequently become visually impaired. Formation of ocular staphyloma affects the macula causing myopic maculopathy, which leads to significant and irreversible decrease in visual functions. PURPOSE: To study the occurrence frequency of various forms of myopic maculopathy in scleral staphylomas of different localization in patients with pathologic myopia. MATERIAL AND METHODS: The study included 105 patients with high myopia (105 eyes, axial length ≥26 mm). The main group consisted of 50 patients with myopic scleral staphylomas (50 eyes). The comparison group - 55 patients (55 eyes) without scleral staphylomas. RESULTS: In the main group, myopic maculopathy was detected in 82% of eyes, it was absent in 96% of eyes in the comparison group. In the main group there were: staphyloma of the posterior pole of the eye with involvement of the optic nerve head (ONH) - 44% of eyes; macular staphyloma - 28% of eyes; peripapillary staphyloma - 12% of eyes; staphyloma below the ONH - 16% of eyes. Atrophic, neovascular and traction maculopathy was revealed in 22 eyes with staphylomas of the posterior pole of the eye. Dome-shaped changes in the macula were revealed in 17 patients (19 eyes) of the main group (15 eyes with staphylomas of the posterior pole of the eye, 4 eyes with macular staphylomas). The highest average value of axial length (30.94±0.81 mm) was noted in eyes with macular staphylomas, and the lowest (28.58±1.62 mm) - with staphylomas near the ONH. CONCLUSION: The presence of myopic maculopathy is associated with the presence of staphylomas with the odds ratio of 120.7 (95%, confidence interval 24.7-589.3). Staphyloma of the posterior pole of the eye and macular staphyloma were associated with the most severe forms of myopic maculopathy and low visual acuity.

Inter-Eye Comparison in Highly Myopic Patients with Unilateral Myopic Traction Maculopathy.

PURPOSE: To evaluate the risk factors for myopic traction maculopathy (MTM) through inter-eye comparisons of asymmetric ocular features in patients with unilateral MTM. MATERIALS AND METHOD: Highly myopic patients with unilateral MTM were enrolled in the study. The results of comprehensive ophthalmologic examinations, color fundus images, and optical coherence tomography (OCT) were reviewed. MTM and myopic atrophic maculopathy was evaluated according to the ATN classification system. The status of the posterior vitreous detachment (PVD), posterior precortical vitreous pocket, vitreoretinal interface abnormalities, posterior staphyloma height (PSH), and the location of the protrusion of the sclera were assessed through OCT. RESULTS: Among the 54 eyes of 27 patients 48.89 ± 12.78 years of age, the affected eyes had worse best-corrected visual acuity, a longer axial length (AXL), greater PSH, and higher rates of posterior staphylomas, vitreovascular traction and epiretinal membranes (ERMs) than the fellow eyes (P < .001; P < .001; P < .001; P = .010; P = .002; P < .001). Thirteen cases present obvious anisometropia with an inter-eye AXL difference of more than 1 mm. In the 14 cases without obvious anisometropia, the eyes with MTM still had longer AXL, greater PSH and a higher ERMs rate than the fellow eyes (P = .039; P = .017; P = .001). Besides, in the 7 cases with an inter-eye AXL difference of less than 1 mm and asymmetrical stages of PVD, 5 cases with greater PVD in the affected eyes. Multiple risk factors coexisted in 96% of cases. CONCLUSIONS: In patients with unilateral MTM, a longer AXL, greater PSH, posterior staphyloma, vitreovascular traction, and ERMs were the main factors contributing to the occurrence of MTM. The process of PVD might involve in MTM development.

Wide-field swept source optical coherence tomography evaluation of posterior segment changes in highly myopic eyes.

BACKGROUND: To investigate the features in the posterior pole of highly myopic (HM) eyes using a wide-field high-resolution swept source optical coherence tomography (SS OCT). METHODS: This observational cross-sectional study involved 262 eyes of 139 patients, who were diagnosed as HM and had consecutively been examined by SS OCT in the Ophthalmology Department of the Peking Union Medical College Hospital between March 2019 and December 2019. The characteristics of OCT images were documented and analyzed. RESULTS: In our study, SS OCT could demonstrate the entire layer of the choroid and detect the sclera in all eyes. The mean subfoveal retinal/choroidal/scleral thickness were 204.84 ± 119.86 µm, 92.80 ± 75.78 µm and 394.734 ± 123.09 µm, respectively. 138 eyes (52.67%) had posterior precortical vitreous pocket. Myopic foveoschisis was detected in 110 eyes (41.98%), and significantly associated with the presence of posterior staphyloma. 36 eyes (13.74%) had DSM in our study, of which 8 eyes (22.22%) showed a round dome, 16 (44.44%) were horizontal oval-shaped, 9 (25%) were vertical oval-shaped and 3 (8.34%) were oblique oval-shaped. Both SFCT and SFST were inversely and significantly associated with age and refractive errors. Macular neovascularization was significantly correlated with intrascleral vessels. Different deformation of the sclera and posterior staphyloma were vividly identified on SS OCT images. CONCLUSIONS: This study provided a relatively comprehensive picture of posterior pole in HM eyes. Such good visualization of ocular fundus provided by wide-field SS OCT could be useful for the therapy option, disease condition monitoring and pathogenesis investigation.

Severe retinitis pigmentosa with posterior staphyloma in a family with c.886C>A p.(Lys296Glu) RHO mutation.

Background: Posterior pole staphylomata (PSS) is an outward bulging of ocular wall, rarely reported in association with inherited retinal degenerations. Patients and methods: We report a large French family of Jewish ancestry with a peculiar form of dominant retinitis pigmentosa (RP) and posterior pole staphyloma (PPS). Eight members were clinically and genetically examined. Results: All affected members complained of night blindness from early childhood and their ERGs were extinguished in the first decade of life. Seven out of eight presented PPS on fundus examination and SD-OCT. The youngest patient did not present PPS at 11 months of age, but the signs of posterior pole bowing became evident at age 8 years. There was no association between the presence of PPS and refraction. Patients with PPS were either hyperopic or myopic, but all have a high with-the-rule astigmatism. A myopic shift was observed for all of them at follow-up. In this family, the disease segregated with the c.886A>G mutation in RHO gene. Conclusion: A PPS development was observed in initially non-myopic patients of a family with unusually severe dominant RP. The PPS concerned only the area with relatively preserved outer retinal layers (outer nuclear layer and ellipsoid zone). How the outer retina could guide choroid and scleral remodelling remains unclear.

Outcomes of Anti-vascular Endothelial Growth Factor Treatment for Foveal Serous Retinal Detachment Associated with Inferior Staphyloma.

PURPOSE: To evaluate the efficacy of anti-vascular endothelial growth factor (VEGF) treatment of eyes with foveal serous retinal detachment (SRD) associated with inferior staphyloma and to investigate choroidal thickness changes following anti-VEGF therapy. METHODS: In this observational case series, eyes with inferior staphyloma accompanied by foveal SRD were treated with a single intravitreal anti-VEGF injection, followed by further injections as needed. Changes in height and width of subretinal fluid (SRF) and visual acuity after treatment were assessed. Choroidal thickness was measured at the subfovea, 1.5 mm superior and inferior to the fovea using enhanced depth imaging optical coherence tomography at baseline and 1 month after initial anti-VEGF therapy. RESULTS: Six eyes from six patients were included. One month after the initial injection, the mean SRF height and width had decreased significantly from 112.5 ± 40.1 to 44.5 ± 48.7 µm (p = 0.046) and from 1,401.8 ± 627.3 to 690.7 ± 634.7 µm (p = 0.028), respectively. Mean choroidal thickness at the superior point decreased from 218.7 ± 59.3 to 200.5 ± 61.0 µm (p = 0.046). SRF resolved completely in three of the six eyes (50%) with a mean of 6.8 ± 5.9 injections (range, 1 to 15). All eyes experienced at least one recurrence of exudation, at a mean interval of 4.8 months. Mean visual acuity improvement was 0.17 logarithm of the minimum angle of resolution units at a mean of 28.7 months follow-up. CONCLUSIONS: Anti-VEGF therapy resulted in an SRF decrease and modest visual improvement in eyes with foveal SRD associated with inferior staphyloma. Reduction in superior choroidal thickness appeared to contribute to the clinical improvements that were observed.

Presence of Posterior Staphyloma in Congenital Cataract Children.

Purpose: To investigate the prevalence of posterior staphyloma (PS) in congenital cataract children and its role in predicting postoperative axial elongation.Materials and Methods: Preoperative prevalence of PS in 520 congenital cataract patients was reviewed and compared with that of the healthy eyes of 300 unilateral traumatic cataract children after 1:1 propensity score matching. Then, 32 pseudophakic children with preoperative PS and 48 age-matched pseudophakic controls without preoperative PS were followed up after the surgery, to compare their axial growth rates and refractive changes.Results: Congenital cataract was significantly associated with the presence of PS (OR: 14.88, P = .009) after propensity score matching. Even in congenital cataract eyes with axial length <26 mm, 5% were identified with PS on B-scan: ≤22 mm: 3%, 22-24 mm: 5% and 24-26 mm: 13%. Eyes with preoperative PS exhibited faster postoperative axial growth than those without, especially in bilateral cases or in children undergoing surgery before 8 years old (≤4 years: 0.53 ± 0.33 vs 0.30 ± 0.21 mm/y P = .028; 4-8 years: 0.37 ± 0.26 vs 0.23 ± 0.15 mm/y P = .044). Myopic shift after surgery was also more significant in children with preoperative PS than in those without (-1.10 ± 0.50 vs -0.60 ± 0.47D/y, P < .001).Conclusions: Congenital cataract is a risk factor for PS. Preoperative PS in pediatric cataract eyes may be an indicator of excessive postoperative axial elongation, especially in bilateral cases or in cases undergoing cataract surgery at a younger age. Our findings may also promote better clinical decision-making in intraocular lens power selection for pediatric population.

A rare case of bilateral choroidal coloboma within deep posterior staphyloma associated with macular hole retinal detachment.

To report case of bilaterally symmetrical choroidal coloboma within posterior staphyloma with MHRD. This is a case report of a 50year old female presented with diminished vision in both eyes. On examination, she had Bilateral High Myopia with recent onset MHRD associated with symmetrically bilateral Choroidal Coloboma within posterior staphyloma and was operated for MHRD in left eye. Presence of choroidal coloboma within posterior staphyloma is rare and with coexisting macular hole makes pathophysiology of RD challenging to understand.

Progressive Intrascleral Epithelial Cyst With Intracorneal Extension.

The authors report a case of primary sclerolimbal cyst with corneal extension in a 3-year-old boy. The cyst enlarged and dissected into the cornea over time. Surgical excision with tectonic allograft was performed. Preoperative, intraoperative, and postoperative anterior segment findings were shown, and results from histologic and cytologic assays were presented. [J Pediatr Ophthalmol Strabismus. 2019;56:e20-e23.].

Ultrawide-Field OCT to Investigate Relationships between Myopic Macular Retinoschisis and Posterior Staphyloma.

PURPOSE: To investigate the relationships between myopic macular retinoschisis (MRS) and posterior staphylomas and to reveal the characteristics of other retinal lesions associated with MRS. DESIGN: Retrospective, observational case series. PARTICIPANTS: Seven hundred twenty-nine eyes of 420 patients with high myopia, which was defined as myopic refractive error of more than -8.0 diopters or an axial length longer than 26.5 mm. METHODS: Highly myopic eyes were examined by ultrawide-field (UWF) swept-source (SS) OCT with scan width of up to 23 mm and scan depth of 5 mm. The OCT features of MRS and posterior staphylomas and their spatial relationship were examined in UWF SS OCT images. MAIN OUTCOME MEASURES: Associations between MRS and staphylomas. RESULTS: In 729 eyes with mean axial length of 30.2±2.1 mm, posterior staphyloma was detected in 482 eyes (66.1%) and MRS was detected in 136 eyes (18.7%). All 136 eyes with an MRS showed outer retinoschisis, and 40 eyes (29.4%) also showed inner retinoschisis. Posterior staphyloma was detected significantly more frequently in eyes with MRS (117/136 [86.0%]) than in eyes without MRS (365/593 [61.6%]; P < 0.001). In all eyes with both staphyloma and outer retinoschisis, the area of the outer retinoschisis was restricted to the area within the staphyloma. In 1 of the 19 eyes with outer retinoschisis but without staphyloma, the outer retinoschisis extended beyond the range of the scanned fundus area. Among the 40 eyes with inner retinoschisis, the inner retinoschisis was located within the region of the outer retinoschisis in 39 eyes (97.5%). In all eyes with inner retinoschisis, retinal lesions causing an inward-directed tractional force were found within the area of the inner retinoschisis. CONCLUSIONS: In highly myopic eyes, the sites of the MRS and staphylomas were spatially related to each other. Posterior-directed force in association with staphylomas, and an inward-directed force resulting from epiretinal membranes or vitreoretinal attachments, may act as causative factors for MRS. However, the exact mechanisms related to the development of an MRS are probably diverse and complex.

Radius-Maumenee syndrome: a rare cause of glaucoma.

Case presentation of a 41-year-old woman with long-standing bilateral eye injection whose clinical findings included bilateral episcleral vessel engorgement and tortuosity, raised intraocular pressure with open iridocorneal angles and left optic disc cupping. All remaining objective examination was normal. She underwent cranial and orbits CT and CT-angiography scans which were unremarkable. Optical coherence tomography of the peripapillary nerve fibre layer and automated perimetry were compatible with advanced glaucomatous damage on the left eye. The patient was diagnosed with idiopathic elevated episcleral venous pressure or Radius-Maumenee syndrome. If medical therapy is unable to prevent disease progression, left eye filtering surgery will be proposed.

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."