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The purpose of this study was to assess whether growth and remodeling (G&R) theory could explain staphyloma formation from a local scleral weakening—as could occur from age-related elastin degradation, myopia progression, or other factors. A finite element model of a healthy eye was reconstructed, including the lamina cribrosa, the peripapillary sclera, and the peripheral sclera. The homogenized constrained mixture model was employed to simulate the adaptation of the sclera to alterations in its biomechanical environment over a duration of 13.7 years. G&R processes were triggered by reducing the shear stiffness of the ground matrix in the peripapillary sclera and lamina cribrosa by 85%. Three distinct G&R scenarios were investigated: (1) low mass turnover rate in combination with transmural volumetric growth; (2) high mass turnover rate in combination with transmural volumetric growth; and (3) high mass turnover rate in combination with mass density growth. In scenario 1, we observed a significant outpouching of the posterior pole, closely resembling the shape of a Type-III staphyloma. Additionally, we found a notable change in scleral curvature and a thinning of the peripapillary sclera by 84%. In contrast, scenario 2 and 3 exhibited less drastic deformations, with stable posterior staphylomas after approximately 7 years. Our proposed framework suggests that local scleral weakening is sufficient to trigger staphyloma formation under a normal level of intraocular pressure. Our model also reproduced characteristics of Type-III staphylomas. With patient-specific scleral geometries (as could be obtained with wide-field optical coherence tomography), our framework could be clinically translated to help us identify those at risks of developing posterior staphylomas. [ABSTRACT FROM AUTHOR]

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High myopia is often associated with local ectasia and scleral thinning. The progression of myopia depends upon scleral biochemical and biomechanical properties. Scleral thinning is associated with decreased collagen fiber diameter, defective collagen fibrillogenesis, and collagen cross-linking. Reversing these abnormalities may make the sclera tougher and might serve as a treatment option for myopic progression. Collagen cross-linking is a natural process in the cornea and sclera, which makes the structure stiff. Exogenous collagen cross-linkage is artificially induced with the help of external mediators by using light and dark methods. In this systematic review, we discussed existing literature available on the internet on current evidence-based applications of scleral collagen cross-linking (SXL) by using different interventions. In addition, we compared them in tabular form in terms of their technique, mechanisms, cytotoxicity, and the stage of transition from preclinical to clinical development. Furthermore, we discussed the in-vivo technique to evaluate the post-SXL scleral biomechanical property and outcome in the human eye.

Adèle Ehongo Ophthalmology Department, Erasmus Hospital, Brussels, BelgiumCorrespondence: Adèle Ehongo, Ophthalmology Department, Erasmus Hospital, Route de Lennik 808, Brussels, 1070, Belgium, Tel +3225553114, Fax +3225556737, Email adele.ehongo@erasme.ulb.ac.beAbstract: Posterior staphyloma (PS) is considered the hallmark of pathologic myopia and is defined as an outpouching of a circumscribed portion of the eyeball with a radius of curvature smaller than that of the adjacent zone. Although more common in eyes with high myopia, it can affect those without it. The presence of PS is associated with a structurally and functionally worse course of high myopia that can lead to visual disability. Unfortunately, the pathogenesis of PS is unclear so far. Thus, due to the increasing prevalence of myopia which has been further exacerbated by the advent of COVID-19 lockdown, researchers are eager to elucidate the pathogenesis of pathologic myopia and that of its complications, especially PS, which will allow the development of preventive strategies. The aim of this work was to review the morphological characteristics of PS with emphasis on similarities with peripapillary staphyloma and to discuss the pathogenesis of PS considering recent suggestions about that of peripapillary staphyloma.Keywords: myopia, staphyloma, pathogenesis, optical coherence tomography, oblique muscles, optic nerve sheaths

High myopia is often associated with local ectasia and scleral thinning. The progression of myopia depends upon scleral biochemical and biomechanical properties. Scleral thinning is associated with decreased collagen fiber diameter, defective collagen fibrillogenesis, and collagen cross-linking. Reversing these abnormalities may make the sclera tougher and might serve as a treatment option for myopic progression. Collagen cross-linking is a natural process in the cornea and sclera, which makes the structure stiff. Exogenous collagen cross-linkage is artificially induced with the help of external mediators by using light and dark methods. In this systematic review, we discussed existing literature available on the internet on current evidence-based applications of scleral collagen cross-linking (SXL) by using different interventions. In addition, we compared them in tabular form in terms of their technique, mechanisms, cytotoxicity, and the stage of transition from preclinical to clinical development. Furthermore, we discussed the in-vivo technique to evaluate the post-SXL scleral biomechanical property and outcome in the human eye. [ABSTRACT FROM AUTHOR]

Purpose: To report a case of peripapillary subretinal fluid associated with a ridge-shaped morphology surrounding the optic disk, which we termed ridge-shaped peripapilla. Methods: Case report. Results: A 6-year-old girl with mild-to-moderate myopia was referred for an abnormal fundus appearance of the left eye. Fundus examination of the left eye showed a vertical whitish elevation just temporal to the disk with pigment clumping. Spectral domain optical coherence tomography of the left eye showed an elevation of the fundus at the temporal edge of the disk with thinning of the choroid overlying the thickened scleral protrusion and a serous subretinal fluid. Fluorescein angiography of the left eye showed a hyperfluorescent area without leakage at the temporal edge of the disk, indicative of retinal pigment epithelium atrophy. There was no sign of choroidal neovascularization. Based on the fluorescein angiography and optical coherence tomography findings, the protrusion of the sclera seemed to result in overlying choroidal thinning with choroidal blood flow disturbances, and consequent retinal pigment epithelium atrophy, leading to the subretinal fluid. Conclusion: This case highlights an unusual presentation of ridge-shaped peripapilla, characterized by inward convexity of the peripapillary area with a ridge-shaped morphology and localized thickening of the peripapillary sclera, in eyes with myopia. [ABSTRACT FROM AUTHOR]

Background: Traboulsi syndrome is a sporadic autosomal recessive disorder. Very few cases reported and no surgical treatment has been described. Purpose: The purpose of the study was to describe the surgical treatment of a case of Traboulsi syndrome with intercalary staphyloma. Synopsis: Traboulsi syndrome[1-3] is a type of spontaneous filtering bleb. The case was of a 31-year-old lady with a flat anterior chamber and large intercalary staphyloma, with a subluxated clear lens. Liberal peritomy was done. The staphyloma was cut and the displaced lens was delivered through it. It was closed with 7-0 vicryl continuous suture. A cross-linked cornea was used after removing the Descemet membrane. It was modified to match the crescentic shape of the limbus by using 11 and 6 mm trephine. Two wedge-shaped defects were created and tissue was excised between them, which were then sutured to increase the arc radius. A peripheral flange at the corneal edge was created. A pocket was created in the host cornea to accommodate the donor corneal flange. The donor cornea was tucked in and the graft was sutured. The sclera was used to suture another side prophylactically, making a 360-degree encirclage. The patient got ambulatory vision postsurgery. The use of the cornea as biological encirclage has not been described previously. Highlights: Using a cross-linked cornea. Creating wedge-shaped defects in crescentic corneal graft to increase arc radius. Use of cornea as encirclage. Link of video: https://youtu.be/T3b5rkvFmlc

To present a case of anatomical success and visual improvement after the treatment of a long-standing foveal retinal detachment in a staphylomatous myopic eye with foveoschisis and macular hole. A 60-year-old woman with high myopia presented with foveoschisis and a lamellar macular hole in her right eye. After 2 years of follow-up without deterioration, her eye developed a full-thickness macular hole and a foveal retinal detachment which caused a severe reduction in visual acuity. However, the patient had no surgical treatment for her condition at that time. Vitrectomy was performed 2 years after the retinal detachment formation. Regardless of the longstanding detachment, anatomical success, and visual improvement were evident after the surgery. Despite a 2-year longstanding foveal detachment on a highly myopic eye with foveoschisis and macular hole, surgical repair could still be satisfactory.

Adèle Ehongo,1 Noélie Bacq,1 Nacima Kisma,1 Artemise Dugauquier,1 Yassir Alaoui Mhammedi,1 Kevin Coppens,2 Françoise Bremer,1 Karelle Leroy3 1Ophthalmology Department, Erasmus Hospital, Brussels, 1070, Belgium; 2Statistician, Cosma Consulting, Enghien 7850, Belgium; 3Histology Department, Erasmus Campus, CP 620, Université Libre de Bruxelles, Brussels, 1070, BelgiumCorrespondence: Adèle Ehongo, Ophthalmology Department, Erasmus Hospital, Route de Lennik 808, Brussels, 1070, Belgium, Tel +3225553114, Fax +3225556737, Email adele.ehongo@erasme.ulb.ac.beAbstract: Purpose: To compare the peripapillary polar characteristics in eyes combining peripapillary staphyloma and gamma peripapillary atrophy according to whether peripapillary intrachoroidal cavitation (PICC) was present or absent (combination-group).Patients and methods: This prospective non-interventional cross-sectional study included 667 eyes of 334 subjects. From the polar peripapillary regions to the opening of Bruch’s membrane, the following elements and their topographic relationships were analyzed using optical coherence tomography sections: configuration of the posterior curvature of the choroid, visibility of the subarachnoid space (SAS), and suprachoroidal detachment (SCD). Chi-squared and Fisher exact tests were used for statistical analysis.Results: The protrusion of the posterior choroidal wall, with anterior elevation on either side, observed in both groups progressed and transformed into a wedge-shaped deformity on the side of gamma peripapillary atrophy. This wedge configuration was significantly more frequent in PICC-group than in combination-group (p = 0.004 and p < 0.001) for the upper and lower poles, respectively. SAS was more frequently observed in PICC-group than in combination-group (p = 0.002 and p < 0.001) for the upper and lower poles, respectively. SCD was detected exclusively in PICC-group (p < 0.001, both poles). The wedge-shaped configuration and the SCD were aligned antero-posteriorly with the SAS.Conclusion: We confirmed that PICC is an SCD. We observed its constant alignment with the SAS. We suggest that the tensile forces of the optic nerve sheaths during adduction promote the collapse of the scleral flange onto the SAS, leading to PICC. Further studies are warranted to confirm this hypothesis.Keywords: peripapillary intrachoroidal cavitation, suprachoroidal detachment, gamma peripapillary atrophy, staphyloma, subarachnoid space, myopic complications

ABSTRACT A 12-year-old boy with Donnai-Barrow syndrome diagnosed intra-uterus presented esotropia, high myopia, nystagmus, and optic disk staphyloma in an ophthalmologic examination. The patient had associated Fanconi syndrome and sensorineural hearing loss as well as facial manifestations as hypertelorism, downward slanting of palpebral fissures and low ear implantation. Magnetic resonance imaging revealed agenesis of the corpus callosum. To our knowledge, this is the first reported case associated with esotropia, nystagmus, and optic disk staphyloma.

To present a case of anatomical success and visual improvement after the treatment of a long-standing foveal retinal detachment in a staphylomatous myopic eye with foveoschisis and macular hole. A 60-year-old woman with high myopia presented with foveoschisis and a lamellar macular hole in her right eye. After 2 years of follow-up without deterioration, her eye developed a full-thickness macular hole and a foveal retinal detachment which caused a severe reduction in visual acuity. However, the patient had no surgical treatment for her condition at that time. Vitrectomy was performed 2 years after the retinal detachment formation. Regardless of the longstanding detachment, anatomical success, and visual improvement were evident after the surgery. Despite a 2-year longstanding foveal detachment on a highly myopic eye with foveoschisis and macular hole, surgical repair could still be satisfactory. [ABSTRACT FROM AUTHOR]

Leprosy is a chronic, granulomatous infectious disease commonly affecting the skin, nerves, mucosa and eyes. The stigma associated with the disease frequently leads to delay in presentation to health professionals. Treated patients, though considered cured presumptively, many continue to live with physical disabilities and deformities. Intact visual acuity prevents humans from trauma and any reduction in visual acuity, especially in leprosy, increases the risk of getting injured by many folds. Here, we present a case of leprosy with complete loss of vision due to bilateral anterior staphyloma secondary to keratitis and his physical deformities preventing him to take care of his eyes. This paper aims to emphasize on the importance of a baseline ophthalmology consultation in all newly diagnosed leprosy patients and repeat examination at onset of any new symptoms of the eye. [ABSTRACT FROM AUTHOR]

The aim of this study was to develop an eyewall curvature- and axial length (AxL)-based algorithm to automate detection (clinician-free) of staphyloma ridge and apex locations using ultrasound (US). Forty-six individuals (with emmetropia, high myopia or pathologic myopia) were enrolled in this study (AxL range: 22.3-39.3 mm), yielding 130 images in total. An intensity-based segmentation algorithm automatically tracked the posterior eyewall, calculating the posterior eyewall local curvature (K) and distance (L) to the transducer and the location of the staphyloma apex. By use of the area under the receiver operator characteristic (AUROC) curve to evaluate the diagnostic ability of eight local statistics derived from K, L and AxL, the algorithm successfully quantified non-uniformity of eye shape with an AUROC > 0.70 for most K-based parameters. The performance of binary classification (staphyloma absence vs. presence) was assessed with the best classifier (the combination of AxL, standard deviation of K and standard deviation of L) yielding a diagnostic validation performance of 0.897, which was comparable to the diagnostic performance of junior clinicians. The staphyloma apex was localized with an average error of 1.35 ± 1.34 mm. Combined with the real-time data acquisition capabilities of US, this method can be employed as a screening tool for clinician-free in vivo staphyloma detection. [ABSTRACT FROM AUTHOR]

Retinol binding protein (RBP4) is important for transport of vitamin A from liver to end organs. Variants in the RBP4 gene have been associated with a broad range of ocular phenotypes but only in a small number of patients. We describe the phenotypes in a multi-generation family with RPB4 variants. A sibling pair was found to be homozygous for a novel pathogenic variant (c.112-2A>G) in RBP4. Both had presented with early-onset atypical retinitis pigmentosa and they had rheumatoid arthritis and acne. The female sibling became the mother of a child, heterozygote for the variant. The child was born with ocular malformations including corneal opacities, microcornea, posterior staphyloma including the optic nerves. The child did not demonstrate any signs of night blindness or progressive retinal dystrophy. In addition, two older family members were reported to be night blind and two distant relatives were born with spina bifida but were not available for genetic testing. Homozygous variants were associated with severe retinal dystrophy, rheumatoid disease, and acne whereas malformations were likely associated with reduced intra-uterine vitamin A levels. It seems advisable to monitor and treat vitamin A deficiency in all patients carrying one or more variants in the RBP4 gene especially during pregnancy. [ABSTRACT FROM AUTHOR]

Abstract Background Prevalence of high myopia is continuously increasing, thus, patients affected with staphyloma are abundant worldwide. Assessment of the quality of vision in these patients is mandatory for a proper clinical counselling, specially when undergoing surgical procedures that require intraocular lenses implantation. Thus, the purpose of the study was to assess monochromatic higher order aberrations (HOAs) in highly myopic eyes with staphyloma with or without a dome-shaped macula. Methods Participants underwent spectral-domain optical coherence tomography, ocular axial biometry, dual Scheimpflug photography and integrated Placido disk topography, and Hartmann-Shack wavefront analysis. Five groups were evaluated: a low-moderate myopia control group (

Background: Traboulsi syndrome is a sporadic autosomal recessive disorder. Very few cases reported and no surgical treatment has been described. Purpose: The purpose of the study was to describe the surgical treatment of a case of Traboulsi syndrome with intercalary staphyloma. Synopsis: Traboulsi syndrome[1‑3] is a type of spontaneous filtering bleb. The case was of a 31‑year‑old lady with a flat anterior chamber and large intercalary staphyloma, with a subluxated clear lens. Liberal peritomy was done. The staphyloma was cut and the displaced lens was delivered through it. It was closed with 7‑0 vicryl continuous suture. A cross‑linked cornea was used after removing the Descemet membrane. It was modified to match the crescentic shape of the limbus by using 11 and 6 mm trephine. Two wedge‑shaped defects were created and tissue was excised between them, which were then sutured to increase the arc radius. A peripheral flange at the corneal edge was created. A pocket was created in the host cornea to accommodate the donor corneal flange. The donor cornea was tucked in and the graft was sutured. The sclera was used to suture another side prophylactically, making a 360‑degree encirclage. The patient got ambulatory vision postsurgery. The use of the cornea as biological encirclage has not been described previously. Highlights: Using a cross‑linked cornea. Creating wedge‑shaped defects in crescentic corneal graft to increase arc radius. Use of cornea as encirclage. [ABSTRACT FROM AUTHOR]

Purpose: To compare morphological features of staphyloma (type and grade) with structural and functional outcomes in 11 patients with symptomatic myopic tractional maculopathy (MTM) who underwent macular buckle surgery. Methods: A retrospective observational case series, where a chart review was conducted of the type (Curtin classification) and grade of staphyloma (USG B-scan based). Optical coherence tomography (OCT) macula analysis done preoperatively and postoperatively 1 and 6–8 weeks), Visual acuity was recorded in log mar. Results: With an average myopia of −10.35 D (−5 to −14.5 D), there were four patients with types II and IX staphyloma and seven with type I staphyloma. The average axial length was 28.6 ± 0.98 mm in type II/IX and 26.2 ± 0.8 mm in type I. Preoperative OCT features were outer retinal layer schisis (11), retinal detachment (foveal: 2, macular: 8), macular hole (lamellar: 3, full thickness: 4), and taut posterior hyaloid (3). Postoperatively, patients with type II/IX staphyloma had significant gain in visual acuity from 1.05 ± 0.3 to 0.74 ± 0.2 Log Mar. The structural features also responded better in patients with types II/IX staphyloma, with all patients having more than 90% reduction in schisis and retinal attachment at 6 weeks. Whereas only two patients with type I staphyloma had similar reduction in schisis at 6 weeks and only one had complete retinal attachment. Conclusion: In patients with MTM, the staphyloma characteristics preoperatively can help us prognosticate about structural and functional success after macular buckle surgery. In our small case series, patients with type II/IX staphyloma and larger axial length had better structural and functional outcomes.

A 12-year-old boy with Donnai-Barrow syndrome diagnosed intra-uterus presented esotropia, high myopia, nystagmus, and optic disk staphyloma in an ophthalmologic examination. The patient had associated Fanconi syndrome and sensorineural hearing loss as well as facial manifestations as hypertelorism, downward slanting of palpebral fissures and low ear implantation. Magnetic resonance imaging revealed agenesis of the corpus callosum. To our knowledge, this is the first reported case associated with esotropia, nystagmus, and optic disk staphyloma. [ABSTRACT FROM AUTHOR]

Belén Alfonso-Bartolozzi,1 Eva Villota,1 Álvaro Fernández-Vega-González,1 Luis Fernández-Vega-Cueto,1 Álvaro Fernández-Vega,1 José F Alfonso1,2 1Fernández-Vega Ophthalmological Institute, Oviedo, Spain; 2Surgery Department, School of Medicine, University of Oviedo, Oviedo, SpainCorrespondence: José F AlfonsoInstituto Oftalmológico Fernández-Vega, Avda. Dres. Fernández-Vega 114, Oviedo, 33012 SpainTel +34 985 245533Fax +34 985 233288Email j.alfonso@fernandez-vega.comPurpose: To assess visual outcomes in high myopic eyes with nasal-inferior staphyloma implanted with a pseudophakic trifocal intraocular lens (IOL).Methods: We retrospectively analyzed the visual outcomes of 50 eyes of 45 patients who had cataract surgery after AT LISA trifocal IOL implantation. Twenty-five eyes diagnosed with posterior staphyloma (nasal-inferior, type IV and V), and 25 eyes as long eyes. Uncorrected distance visual acuity (UDVA) and corrected distance visual acuity (CDVA) values were used to assess the efficacy and safety of the surgery. Refraction and defocus curves were also evaluated at 6 months.Results: No intra- or post-operative problems occurred during the 6 months of follow-up. After the surgery, the mean Snellen decimal UDVA ranged from 0.50 to 1.00, and CDVA from 0.60 to 1.00 for both groups. CDVA was 0.91 and 0.74 for the long eye and nasal-inferior staphyloma groups, respectively. Efficacy and safety indexes were 1.22 and 1.32 for the long eye, and 1.26 and 1.43 for the nasal-inferior staphyloma group, respectively. All eyes of both groups showed a postoperative spherical equivalent within ± 1.00D. The long eye group showed the highest percentage of spherical equivalent between − 0.13D and +0.13D (56%) and the nasal-inferior staphyloma group was between − 0.51D and − 0.14D (40%).Conclusion: The outcomes of the present study show that a trifocal IOL provides good visual acuity in high myopic eyes, being worse for nasal-inferior staphyloma eyes. The degree of tilt of the macular plane is related with the expected visual acuity.Keywords: high myopia, multifocal intraocular lens, phacoemulsification, staphyloma

The global prevalence of myopia has brought to the attention of the different eye and vision specialists, who make way to control its progression. Evidence have shown that a proactive reshaping of the eyeball is the core point of myopia developing process, which particularly includes the weakening, thinning, and expanding of the sclera. Thus, the sclera is considered to be a prime target for therapeutic manipulation in halting progressive myopia. In the past decades, corneal collagen cross-linking has been applied in clinical practice for treating aberrant corneal remodeling diseases. In this article, we hypothesize that scleral collagen cross-linking (SXL) has a huge potential in stabilizing myopic process by shaping the eyeball and preventing the aberrant scleral remodeling. In contrast with the current methods of optometry correction, such as physiotherapy, pharmacotherapy, spectacles, contact lenses, refractive surgeries, etc., eyeball-shaping method using SXL is a fundamental intervention which aims at the pathogenesis of progressive visual loss of myopia. Compared with the current posterior scleral reinforcement, the most advantage of SXL is that there is no allotransplant into the myopic eye, which means less expenditure, lower risk, and easier to handle in operating.

Purpose: To compare morphological features of staphyloma (type and grade) with structural and functional outcomes in 11 patients with symptomatic myopic tractional maculopathy (MTM) who underwent macular buckle surgery.Methods: A retrospective observational case series, where a chart review was conducted of the type (Curtin classification) and grade of staphyloma (USG B-scan based). Optical coherence tomography (OCT) macula analysis done preoperatively and postoperatively 1 and 6-8 weeks), Visual acuity was recorded in log mar.Results: With an average myopia of -10.35 D (-5 to -14.5 D), there were four patients with types II and IX staphyloma and seven with type I staphyloma. The average axial length was 28.6 ± 0.98 mm in type II/IX and 26.2 ± 0.8 mm in type I. Preoperative OCT features were outer retinal layer schisis (11), retinal detachment (foveal: 2, macular: 8), macular hole (lamellar: 3, full thickness: 4), and taut posterior hyaloid (3). Postoperatively, patients with type II/IX staphyloma had significant gain in visual acuity from 1.05 ± 0.3 to 0.74 ± 0.2 Log Mar. The structural features also responded better in patients with types II/IX staphyloma, with all patients having more than 90% reduction in schisis and retinal attachment at 6 weeks. Whereas only two patients with type I staphyloma had similar reduction in schisis at 6 weeks and only one had complete retinal attachment.Conclusion: In patients with MTM, the staphyloma characteristics preoperatively can help us prognosticate about structural and functional success after macular buckle surgery. In our small case series, patients with type II/IX staphyloma and larger axial length had better structural and functional outcomes. [ABSTRACT FROM AUTHOR]

Purpose. To study the effects of corneal collagen cross-linking (CCL) using ultraviolet-A (UVA) irradiation with riboflavin on the eye’s anterior segment structures in corneal ulcers and other keratopathies in animals.Material and methods. The study involved 26 animals (39 eyes) — 10 cats, 10 dogs, and 6 horses with ulcers, corneal staphyloma, dry and autoimmune keratoconjunctivitis. Examination included slit-lamp biomicroscopy, ophthalmoscopy (in cases of transparent cornea), tear production test, and staining the eye surface with vital dyes. CCL was performed using UVA radiation of LEDs with a fiber optic output (wavelength 370 nm, radiation power 3 mW/cm2) combined with 0.1 % riboflavin instillation according to the protocol we developed, which specifies various numbers and durations depending on the diagnosis and therapeutic effect desired.Results. Restoration of the shape and transparency of the cornea, relief of anterior eye inflammation, and normalization of tear production occurred much sooner than using traditional treatment. The restoration of vision occurred 2 to 3 months earlier in eyes with corneal staphyloma; more than 4 to 5 months earlier in eyes with keratoconjunctivitis; no relapses were noted in eyes with ulcerative processes, and the restoration of the function of stratified squamous epithelium was achieved within 2 or 3 weeks.Conclusions. The inclusion of modified UFA-based CCL in the treatment scheme of corneal ulcers and staphylomas as well as dry and autoimmune keratoconjunctivitis enables a high therapeutic effect of anterior eye structure restoration. In future, the results obtained could serve as basis for using the developed technology in the treatment of similar eye pathologies in humans.

The purpose of the study was to find out how age-related macular degeneration and medium-high degree myopia affect the structural and anatomical parameters of the eyeball, and to find whether a relationship exists between various types of myopic staphylomas and changes of visual functions.Material and methods. 120 patients (236 eyes), with medium and high degree myopia and age-related retinal changes, aged 44 to 81 years were tested for two groups of parameters: quantitative and qualitative. The former included the patients’ age, best corrected visual acuity (BCVA), computer perimetry data, the axial length, and the thickness of the retina in the central zone. The latter included retinal changes, in particular pigment epithelium (PE) defects, drusen, PE detachment, choroidal neovascular membrane (CNV), Fuchs spot and diffuse chorioretinal atrophy. The impact of various types of staphylomas on visual functions was evaluated. Results. Visual functions were found to directly depend on retinal PE integrity. If defects, detachment, or drusen are present, computer perimetry parameters and BVCA are reducing. Fuchs spot presence and diffuse chorioretinal atrophy reduces BCVA and causes an MD decrease. The lowest visual acuity and considerable light sensitivity loss was noted in patients with combined staphylomas. Conclusion. The obtained data confirm that retinal dystrophic processes directly affect visual functions: BCVA and retinal photosensitivity levels drop in all retinal changes studied. The presence of staphyloma, being a factor that aggravates myopia, undoubtedly worsens the most of the parameters studied.

Abstract Purpose To measure the choroidal thickness in patients with high myopia from staphyloma using swept source OCT (SS-OCT) in Early Treatment Diabetic Retinopathy Study (ETDRS) fields and compare to normal cohort. The study also evaluated the correlation between choroidal thickness with axial length and best-corrected visual acuity (BCVA). Methods In this prospective cross sectional study, 37 eyes of 20 patients with high myopia from staphyloma and 86 eyes of 43 normal subjects were included. In each eye, horizontal scans centered on the fovea (12 × 9 mm) were performed using SS-OCT (DRI-OCT, Topcon, Japan). Choroidal thickness in 9 ETDRS subfields, including central subfield (CSF) was analyzed and correlated with axial length as well as BCVA. Results The axial length and BCVA in the high myopia from staphyloma group ranged from 25.12 to 33.54 mm (28.4 ± 1.2) and 20/20–20/400, respectively. The choroidal thickness in staphyloma group was 85.53 ± 48.61 μm as compared to 250.24 ± 71.01 μm in the normal group (p

Background: Prevalence of high myopia is continuously increasing, thus, patients affected with staphyloma are abundant worldwide. Assessment of the quality of vision in these patients is mandatory for a proper clinical counselling, specially when undergoing surgical procedures that require intraocular lenses implantation. Thus, the purpose of the study was to assess monochromatic higher order aberrations (HOAs) in highly myopic eyes with staphyloma with or without a dome-shaped macula.Methods: Participants underwent spectral-domain optical coherence tomography, ocular axial biometry, dual Scheimpflug photography and integrated Placido disk topography, and Hartmann-Shack wavefront analysis. Five groups were evaluated: a low-moderate myopia control group (< 6.00 diopters, n = 31) and four high myopia (≥6.00 diopters) groups: eyes without staphyloma (n = 18), eyes with inferior staphyloma (n = 14), eyes with posterior staphyloma without dome-shaped macula (n = 15) and eyes with posterior staphyloma with dome-shaped macula (n = 17). Subsequently, two new groups (including all participants) were created to assess differences between myopia with and without staphyloma. One-way analysis of covariance was performed using age and lens densitometry as covariates.Results: Statistically significant (p ≤ 0.05) differences in anterior corneal fourth-order HOAs were observed between the low-moderate myopia and no-dome-shaped macula (Mean: 0.16 μm) and dome-shaped macula posterior staphyloma groups (Mean: 0.12 μm) in younger patients (≤45 years old). The same groups also showed (p ≤ 0.05) significant differences for anterior corneal primary spherical aberration (Mean: 0.19 and 0.13 μm, respectively). In addition, anterior corneal tetrafoil was significantly higher (p = 0.04) in dome-shaped macula compared to no-dome-shaped macula (Mean: 0.18 vs 0.06 μm, respectively). When all participants were grouped together, significantly lower mean anterior corneal primary spherical aberration (0.15 μm vs. 0.27 μm, p = 0.004) and higher internal primary spherical aberration (0.04 μm vs. -0.06 μm, p = 0.04) was observed in staphyloma compared to no-staphyloma myopic patients.Conclusions: Eyes with high myopia and staphyloma have less positive anterior corneal primary spherical aberration and less negative internal primary spherical aberration, suggesting that the anterior corneal surface tends to mimic in a specular fashion the posterior pole profile. This corneal behaviour appears to change in patients older than 45 years. [ABSTRACT FROM AUTHOR]

Purpose: To characterize a population of high myopes with myopic traction maculopathy (MTM), to assess their retinal function, and to correlate it with anatomic status. Patients and Methods: This was an observational cross-sectional study including 50 eyes from 27 patients. Demographic and clinical data were analyzed. Macular structure was assessed with spectral domain optical coherence tomography (SD-OCT, Heidelberg®) and macular function was studied with Microperimeter MP-3, NIDEK®. Results: The average for central foveal thickness (CFT) and choroid thickness (CT) was 213± 151 μm and 36± 23 μm, respectively, in a total of 50 eyes from 27 patients. In the microperimetry analysis, the average sensitivity on the foveal-centered 12º polygon (CPS) was 14.37± 9.1 dB. CT was negatively associated with the bivariate contour ellipse areas (BCEA) 1 (r=− 0.314; p=0.034), 2 (r=− 0.314; p=0.034), and 3 (r=− 0.316; p=0.033). CPS had a strong positive correlation with best-corrected visual acuity (BCVA) (r=0.661; p=0.000). We found a trend to worse microperimetric results in eyes with schisis (n=19) (p> 0.05) but eyes with atrophic areas (n=33) presented significant inferior CPS (p< 0.001). The presence of staphyloma showed significant impact on macular sensitivities in eyes with areas of macular atrophy/fibrosis (p< 0.05). Conclusion: Macular microperimetry analysis can have a role as part of a multimodal anatomo-functional assessment for a more precise characterization of the high myopic patients with MTM, optimizing medical and surgical decisions. [ABSTRACT FROM AUTHOR]

Purpose: To report a case of bilateral idiopathic chorioretinal folds that seemed to be related to an atypical staphyloma. Observations: A 49-year old man without medical history consulted for slight vision loss and metamorphopsia in the left eye. The ophthalmologic examination revealed moderate myopia and bilateral chorioretinal folds in the posterior pole, confirmed by multimodal imaging. Orbital and systemic examinations ruled out all the known etiologies of chorioretinal folds. 3-D optical coherence tomography and B-scan suggested that the folds were related to an atypical staphyloma that developed in the temporal part of the fundus, while sparing the peripapillary area. The peripapillary area, spared by the staphyloma process, appeared as a “dome-shaped disc” compared to the staphylomatous area. Conclusion and importance: This case suggests that myopic patients with unusual staphyloma located outside the peripapillary area could develop chorioretinal folds.

Abstract Background Rhinosporidiosis is a rare chronic infection of the mucous membranes caused by the Rhinosporidium seeberi. Approximately 15% of cases of rhinosporidiosis are ocular, occurring mainly in the tarsal conjunctiva. There are only 11 cases of scleral melt with staphyloma formation associated with bulbar conjuctival oculosporidiosis and none of them was associated with partial regression of the scleral ectasia after a corneoscleral tectonic graft. Case presentation a 13-year-old girl with a progressively increasing black mass in the upper nasal part above the cornea of the left eye. The biomicroscopy revealed an oval, bluish mass measuring 10x10x5 mm with congestion of the overlying conjunctiva. Conjunctival biopsy showed sporoblasts of Rinosporidium seeberi. Treatment was conducted by conjunctival resection and tectonic corneoscleral graft (13x13mm) over the staphyloma. Within 1 year of follow-up the patient presented a partial staphyloma reduction, 9x9x2.5 mm, and the patch detached from the lesion. A novel surgical approach was done reducing the corneal patch and no recurrence was seen after 9 months. Conclusions This case is one of the largest anterior scleral staphylomas secondary to rhinosporidiosis described in the literature. Scleral anterior staphyloma partial regression is an unusual outcome after a tectonic corneoscleral graft. Infection resolution and graft covering of thinned area contributed to scleral reepithelization.

Purpose: Uncorrected refractive errors are the first cause of vision impairment worldwide. High myopia is a frequent cause of sight‐threatening chorioretinal complications. The aim of this study was to evaluate the prevalence of macular complications, visual impairment and blindness in patients with myopia. Methods: A cross‐sectional multicenter study carried out in French eye clinics mainly dedicated to refractive errors. Myopia severity was defined as mild (−0.5 to −3 D), moderate (−3 to −6 D), high (−6 to −10 D) and very high (more than −10 D). Macular complications related to myopia included lacquer cracks, myopic choroidal neovascularization, chorioretinal atrophy and retinoschisis. The prevalences of macular complications, blindness and vision impairment were estimated with respect to degree of myopia and age. Eligibility criteria were myopia on the left eye of −0.5 D or more. Exclusion criteria included any missing data related to subjective refractive error, age, gender and any history of cataract or refractive surgery. Results: Data files from 198 641 myopic individuals with a mean age of 34 years (SD: 15 years) were analysed. The prevalence of mild, moderate, high and very high myopia was, respectively, 65.95%, 26.14%, 6.72% and 1.19%. The prevalence of macular complications in the high and very high myopia groups was 0.5% [0.39–0.64] and 4.27% [3.49–5.17]. The prevalence of blindness or vision impairment was observed in 10.10% [8.91–11.39%] of the very high myopic group. At 60 years old or over, the prevalences of blindness or vision impairment were, respectively, 9.75% [7.91–11.85%] and 25.71% [21.00–30.87%] in the high and very high myopia groups. Conclusions: This multicenter cross‐sectional study provides new insights in terms of prevalence of macular complications related to myopia. To our knowledge, this is one of the largest European studies focusing on individuals with myopia, particularly on the macular complications and the functional consequences in relation to myopia. [ABSTRACT FROM AUTHOR]

Myopia is a highly frequent ocular disorder worldwide and pathologic myopia is the 4th most common cause of irreversible blindness in developed countries. Pathologic myopia is especially common in East Asian countries. Ocular alterations associated with pathologic myopia, especially those involving the macular area—defined as myopic maculopathy—are the leading causes of vision loss in patients with pathologic myopia. High myopia is defined as the presence of a highly negative refractive error (>−6 to −8 diopters) in the context of eye elongation (26–26.5 mm). Although the terms high myopia and pathologic myopia are often used interchangeably, they do not refer to the same eye disease. The two key factors driving the development of pathologic myopia are: 1) elongation of the axial length and 2) posterior staphyloma. The presence of posterior staphyloma, which is the most common finding in patients with pathologic myopia, is the key differentiating factor between high and pathologic myopia. The occurrence of staphyloma will, in most cases, eventually lead to other conditions such as atrophic, traction, or neovascular maculopathy. Posterior staphyloma is for instance, responsible for the differences between a myopic macular hole (MH)—with and without retinal detachment—and idiopathic MH. Posterior staphyloma typically induces retinal layer splitting, leading to foveoschisis in myopic MH, an important differentiating factor between myopic and emmetropic MH. Myopic maculopathy is a highly complex disease and current classification systems do not fully account for the numerous changes that occur in the macula of these patients. Therefore, a more comprehensive classification system is needed, for several important reasons. First, to more precisely define the disease stage to improve follow-up by enabling clinicians to more accurately monitor changes over time, which is essential given the progressive nature of this condition. Second, unification of the currently-available classification systems would establish standardized classification criteria that could be used to compare the findings from international multicentric studies. Finally, a more comprehensive classification system could help to improve our understanding of the genetic origins of this disease, which is clearly relevant given the interchangeable—but erroneous—use of the terms high and pathologic myopia in genetic research. • Progression of high myopia and the appearance of the staphyloma are the most relevant factors leading to myopic maculopathy. • Myopic maculopathy is a complex disease that comprises atrophic, tractional and neovascular changes that lead to vision loss. • None of current systems allow us to classify every patient as they do not include all three main components of the disease. • We propose a new grading system (ATN classification) that includes atrophic (A), tractional (T) and neovascular (N) changes. [ABSTRACT FROM AUTHOR]

We report a case of a 38-year-old man who presented with a recently self-detected lump under his left eyebrow. Previous ophthalmological history was unremarkable except for unilateral high myopia (left eye) since childhood. The appearance of the left eye was seemingly normal; however, with the top lid pulled up on downward gaze, a dark brown bulge emerged. The bulge was 10 × 7 mm and approximately 4 mm in height, and was covered by the extended superior rectus muscle. The diagnosis of equatorial staphyloma was made after coronal T1-weighted magnetic resonance imaging of the orbit revealed the dilatation of the vitreous cavity. Ocular movements were fully maintained and visual acuity was largely spared: 20/15 in the right eye without correction and 20/25 in the left eye with –10.00 spheres and –4.00 × 80 degrees cylinders. His past and family histories were unremarkable; however, small neurofibromas and café au lait spots all over his body led to the diagnosis of neurofibromatosis type 1 (NF1). From this case, similar to previous reports, we suggest that manifestations of NF1 are extremely variable and unpredictable.

PURPOSE: Posterior staphyloma is an ocular complication associated with high myopia and reflects degenerative changes on the sclera. Its morphology is associated with chorioretinal atrophy and myopic maculopathy. The purpose of this study was to validate the efficacy of optical coherence tomography (OCT) in providing a simple estimation of the staphyloma pattern. MATERIALS AND METHODS: Observational case-series study of high myopic patients with posterior staphylomas. Patients were examined using the star scan pattern OCT in different radial planes. Three-dimensional (3D) magnetic resonance tomography was also performed to visualize the anatomical characteristics of the posterior pole. 3D-segmentation and curvature analysis were also performed. RESULTS: Eight patients were totally enrolled in this pilot study. Our study pool consisted of 2 wide macular staphylomas, 2 narrow macular staphylomas, and 4 barrel-shaped staphylomas. Our preliminary results revealed that patients displayed mirror-image distortion in the steeper staphyloma axis. In the barrel-shaped subtype, no image distortion was displayed in any plane. CONCLUSION: We estimated the axis of the smaller base curvature by noting the distortion pattern in the different radial axis. The recognition of pathologic axial myopia is important since there is a risk of permanent vision loss from vision to threatening sequelae. [ABSTRACT FROM AUTHOR]

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.” [ABSTRACT FROM AUTHOR]

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