Search Results (11)

Background/Objectives: Posterior staphyloma (PS) is a hallmark of pathological myopia, corresponding to a circumscribed outpouching of the eyeball with choroidal thinning and inward scleral deformation at its edges. Its pathogenesis is still unclear, thus constituting a research priority as the prevalence of myopia is increasing worldwide. Recently, it has been suggested that the optic nerve sheaths or oblique muscles are potential promoters of PS through the traction or compression effect that they apply to the eye wall. The inferior oblique muscle (IOM) inserts 1–2 mm from the macula. The projection of its insertion is accessible using Optical Coherence Tomography (OCT). Before launching prospective studies, we sought to detect any choroidal thinning (ChT) in the temporal vicinity of the macula and to measure the distance between it and the fovea (FT-distance). Methods: This retrospective cross-sectional pilot study included 120 eyes. Using Spectralis^®-OCT, the area centered by the Bruch’s membrane opening–fovea axis was analyzed for ChT and FT-distance. Results: Of the 112 defined eyes, 70% (78 eyes) had ChT. Pachymetry was significantly thinner (p = 0.018) in eyes with than without ChT. The mean FT-distance was 3601.9 ± 93.6 µm. Conclusions: The location of ChT coincided with the insertion distance of the IOM, suggesting a link between them. The association between the presence of ChT and a thinner pachymetry suggests a reduced scleral resistance, as a thinner pachymetry is related to a thinner sclera. Our results suggest a link between ocular deformation and the IOM, which may be relevant for the pathogenesis of PS, warranting further investigation. Full article

This study aimed to identify whether gyrate atrophy of the choroid and retina (GACR) heterozygous individuals have possible clinical manifestations and to explore the potential pathogenic mechanism. In this retrospective study, we surveyed a two-generation pedigree of an individual diagnosed with GACR. Two family members underwent ophthalmological, hematologic, and genetic tests. An arginine-restricted diet with vitamin B6 supplementation was implemented; clinical assessments were repeated every 3 months during follow-up. The relative OAT mRNA expression was determined using a real-time quantitative polymerase chain reaction. The 19-year-old compound heterozygous daughter (OAT: c.1186C>T; c.748C>T) had bilateral pathologic myopia, posterior staphyloma, chorioretinal atrophy, macular abnormalities, and elevated hematologic ornithine. The 54-year-old heterozygous mother (OAT: c.1186C>T) presented with bilateral pathologic myopia, asymmetric posterior staphyloma, retina and choroidal capillary layer atrophy, retinal pigment epithelium abnormalities, and mildly elevated hematologic ornithine. Compared to normal individuals, the daughter and mother had 29% and 46% relative OAT mRNA expression, respectively (p < 0.001). We believe that this is the first report of a carrier of one OAT variant allele exhibiting a mild phenotype, suggesting that family members should be aware of the possibility of clinical involvement in carriers with some autosomal recessive conditions. Additional data suggest that nonsense-mediated, decay-initiated mRNA degradation may cause GACR. Full article

The objective of this paper was to determine how different types of posterior staphyloma (PS) may affect the appearance and degree of myopic maculopathy. A cross-sectional study was conducted, in which 467 eyes from 246 highly myopic patients [axial length (AL) ≥ 26 mm] were studied. A complete ophthalmic exploration was carried out on all patients, including imaging tests. The presence of macular PS was established as the main comparison variable between groups (macular PS vs. non-macular PS vs. non-PS). The variables analyzed included age, AL, decimal best-corrected visual acuity (BCVA), Atrophy (A)/Traction (T)/Neovascularization (N) components according to the ATN grading system, and the presence of severe pathologic myopia (PM). Out of the total, 179 eyes (38.3%) presented macular PS, 146 eyes presented non-macular PS (31.2%), and 142 eyes showed no PS (30.4%). The group without PS was significantly younger than macular PS and non-macular PS groups (53.85 vs. 66.57 vs. 65.20 years; p < 0.001 each, respectively). There were no age differences between PS groups. Eyes with macular PS (31.47 ± 2.30 mm) were significantly longer than those with non-macular PS (28.68 ± 1.78 mm, p < 0.001) and those without PS (27.47 ± 1.34 mm, p < 0.001). BCVA was significantly better in the non-PS group (0.75 ± 0.27) compared to the non-macular PS (0.56 ± 0.31) and macular PS groups (0.43 ± 0.33), with p < 0.001 each. Eyes without PS showed significantly lower A and T components (1.31 ± 0.96 and 0.30 ± 0.53, respectively) than non-macular PS (2.21 ± 0.75 and 0.71 ± 0.99, respectively, p < 0.001 each) and macular PS eyes (2.83 ± 0.64 and 1.11 ± 1.10, respectively, p < 0.001 each). The N component was lower in non-PS eyes vs. non-macular PS eyes (0.20 ± 0.59 vs. 0.47 ± 0.83, p < 0.001) and as compared to the macular PS group (0.68 ± 0.90, p < 0.01). Additionally, the N component was significantly lower in the non-macular PS group than in the macular PS one (p < 0.05). The prevalence of severe PM was different between groups (p < 0.001). It was higher among macular PS eyes (138/179) when compared to other groups (p < 0.001, each), followed by the non-macular PS eyes (40/146) and being the lowest in the non-PS group (20/142). To conclude, macular PS is associated with a more advanced maculopathy, worse vision, and higher rates of severe PM. Full article

Purpose: To report a case of macular hole and detachment occurring after the subretinal injection of Voretigene Neparvovec (VN) in a patient affected by atypical RPE65 retinal dystrophy with high myopia and its successful surgical management. Case description: We report a case of a 70-year-old man treated with VN in both eyes. The best corrected visual acuity (BCVA) was 0.7 LogMar in the right eye (RE) and 0.92 LogMar in the left eye (LE). Axial length was 29.60 mm in the RE and 30.28 mm in the LE. Both eyes were pseudophakic. In both eyes, fundus examination revealed high myopia, posterior staphyloma, and extended retinal atrophy areas at the posterior pole, circumscribing a central island of surviving retina. Both eyes were treated with VN subretinal injection, but a full-thickness macular hole and retinal detachment occurred in the LE three weeks after surgery. The patient underwent 23-gauge vitrectomy with internal limiting membrane (ILM) peeling and the inverted flap technique with sulfur hexafluoride (SF6) 20% tamponade. Postoperative follow-up showed that the macular hole was closed and the BCVA was maintained. Conclusions: Our experience suggests that patients with atypical RPE65 retinal dystrophy and high myopia undergoing VN subretinal injection require careful management to minimize the risk of macular hole and detachment occurrence and promptly detect and address these potential complications. Full article

Glaucoma secondary to Peters anomaly is an important factor affecting visual prognosis, but there are few reports on the condition. This study aimed to investigate the characteristics of glaucoma associated with Peters anomaly and glaucoma surgery outcomes. This retrospective study included 31 eyes of 20 patients with Peters anomaly. Peters anomaly was classified into three stages: Stage 1, with a posterior corneal defect only; Stage 2, a corneal defect with iridocorneal adhesion; and Stage 3, a corneal defect with lens abnormalities. The associations between glaucoma and anterior segment dysgenesis severity, visual prognosis, and glaucoma surgery outcomes were analyzed. Sixteen eyes of ten patients developed glaucoma. Stage 1 Peters anomaly had no glaucoma, 52% of Stage 2 had glaucoma, and 75% of Stage 3 had glaucoma. Of the 16 eyes with glaucoma, 11 underwent surgery. Eight of these eleven eyes achieved intraocular pressure (IOP) control. Five of the nine eyes that underwent trabeculotomy (TLO) succeeded, and none had corneal staphyloma. Three of the four eyes for which TLO was ineffective had corneal staphyloma (p = 0.0331). Patients with Peters anomaly are more likely to develop glaucoma as anterior segment dysgenesis progresses, and the effect of TLO is limited if corneal staphyloma is present. Full article

The purpose of this study was to examine bilaterality and symmetry of posterior staphyloma (PS) in high myopic eyes. Methods: This cross-sectional and non-interventional study assessed 473 high myopic eyes [axial length (AL) ≥ 26 mm] of 259 patients. Patients underwent an ophthalmological examination including multimodal-imaging and myopic maculopathy grading according to Atrophic/Tractional/Neovascular (ATN) system, presence and subtype of PS, and severe pathologic myopia (PM). Bilaterality of PS and subtype’s symmetry between eyes of the same patient was assessed. Four groups were analyzed: (1) bilateral vs. unilateral PS’s eyes. Within bilateral group, symmetric vs. asymmetric subtypes according to (2) Curtin’s classification, (3) Ohno-Matsui’s classification, and (4) primary/compound subtypes. Results: Out of the total, 334 myopic eyes of 167 patients were included. The 92.8% (n = 310/334) of the eyes presented PS and was bilateral in 85.6% (n = 143/167) of the patients. Bilateral eyes showed significantly (p < 0.01) greater AL, severe PM, A and N components vs. unilateral PS. AL-difference between both eyes was greater in unilateral PS (p < 0.01). Among bilateral PS, the subtype was symmetric in 79 (55.2%), 84 (58.7%), and 115 (80.4%) patients according to Curtin’s classification, Ohno-Matsui’s classification, and primary/compound; respectively. The asymmetric group presented worse best-corrected visual acuity (p < 0.01), higher AL (p < 0.01), incidence of PM, and severe PM (p < 0.05). Conclusions: PS was bilateral in most of the patients without clinical differences between both eyes, being symmetrical in more than half of bilateral cases. Patients with bilateral PS showed higher myopic maculopathy, AL, and incidence of severe PM than unilateral PS. Full article

Peripapillary intrachoroidal cavitation (PICC) is a yellow-orange lesion, located at the outer border of the myopic conus. First described as a localized detachment of the retinal pigment epithelium, its intrachoroidal location was later revealed, justifying its current name. PICC is related to other myopic complications such as posterior staphyloma, but its pathogenesis is not clear to date. Although it has been considered a benign condition, most eyes with PICC show visual field defects, which leads to diagnostic uncertainty as these deficits resemble those seen in glaucoma. Furthermore, eyes with PICC may develop macular detachment with retinoschisis. Finally, misdiagnosis of PICC as a metastatic choroidal tumor may lead to unnecessary and anxiety-inducing investigations. Advances in optical coherence tomography (OCT) imaging have improved the visualization of ocular structures, contributing to the understanding of PICC. Recently, high optic nerve sheath traction forces during eye movements in highly myopic eyes have been suggested as promoters of PICC, renewing interest around this condition. However, a review of PICC is still lacking. Therefore, we aimed to provide a concise yet comprehensive overview of the current state of the art, focusing on OCT illustrations, pathophysiology and potential future perspectives based on the biomechanics of the optic nerve. Full article

High myopia (HM) is defined as an axial length (AL) ≥ 26 mm that may result in various pathologies that constitute pathologic myopia (PM). The PLEX^® Elite 9000 (Carl Zeiss AC, Jena, Germany) is a new swept-source optical coherence tomography (SS-OCT) underdevelopment that allows wider, deeper and more detailed posterior-segment visualization; it can acquire ultra-wide OCT angiography (OCTA) or new ultra-wide high-density scans in one image. We assessed the technology’s ability to identify/characterize/quantify staphylomas and posterior pole lesions or image biomarkers in highly myopic Spanish patients and estimate the technology’s potential to detect macular pathology. The instrument acquired 6 × 6 OCTA, 12 × 12 or 6 × 6 OCT cubes, and at least two high-definition spotlight single scans. A hundred consecutive patients (179 eyes; age, 51.4 ± 16.8 years; AL, 28.8 ± 2.33 mm) were recruited in one center for this prospective observational study. Six eyes were excluded because images were not acquired. The most common alterations were perforating scleral vessels (88.8%), classifiable staphyloma (68.7%), vascular folds (43%), extrafoveal retinoschisis (24%), dome-shaped macula (15.6%), and more uncommonly, scleral dehiscence (4.46%), intrachoroidal cavitation (3.35%), and macular pit (2.2%). The retinal thickness of these patients decreased, and the foveal avascular zone increased in the superficial plexus compared with normal eyes. SS-OCT is a novel potent tool that can detect most main posterior pole complications in PM and may provide us with a better understanding of the associated pathologies; some pathologies were identifiable only with this new kind of equipment, such as perforating scleral vessels, which seem to be the most common finding and not so frequently related to choroidal neovascularization, as previously reported. Full article

(1) Background: This paper investigates the incidence and risk factors of retinal manifestations in patients with Marfan syndrome (MFS) in a Chinese cohort. (2) Methods: This is a population-based cross-sectional study. In total, 344 eyes (172 MFS participants) were enrolled, each of whom underwent a detailed ocular examination. B-scan ultrasonography, ultra-wide-angle fundus images and optical coherence tomography images were conducted to assess posterior staphyloma, types of retinal damages and maculopathy. (3) Results: MFS patients have a high proportion (32.5%) of maculopathy, among which atrophy is the most common type (27.6%). Compared with participants without maculopathy, participants with maculopathy had a longer axial length (AL), higher incidence of posterior staphyloma, macular split and retinal detachment (RD) (p < 0.001, p < 0.001, p < 0.001 and p = 0.001). Moreover, the stage of RD has a significant correlation with longer AL and shallower anterior chamber depth (ACD) (p = 0.001 and p = 0.034, respectively). (4) Conclusions: A higher incidence and earlier onset of fundus lesions were found in MFS patients. Yearly systematic examination is recommended for MFS children with fundus manifestation until the cardiovascular and skeletal development is complete. Full article

This retrospective study investigated circulating immune cell alteration in patients with myopic retinopathy. Blood test results and demographic and ocular information of 392 myopic patients and 129 emmetropia controls who attended Changsha Aier Eye Hospital from May 2017 to April 2022 were used in this study. Compared with emmetropia, the percentages of neutrophils and basophils and neutrophil/lymphocyte ratio were significantly higher in myopic patients, whereas the percentages of monocytes and lymphocytes and the counts of lymphocytes and eosinophils were significantly lower in myopic patients. After adjusting for age and hypertension/diabetes, the difference remained. Interestingly, the platelet counts were significantly lower in myopic patients after the adjustments. Further subgroup analysis using multivariable linear regression showed that higher levels of neutrophils, neutrophil/lymphocyte ratio, and platelet/lymphocyte ratio, lower levels of monocytes, eosinophils, lymphocytes, and platelets, were related to myopic peripheral retinal degeneration (mPRD) and posterior staphyloma (PS). A higher level of basophils was linked to myopic choroidal neovascularization (mCNV). Our results suggest that higher levels of circulating neutrophils and neutrophil/lymphocyte ratio, lower monocytes, eosinophils, lymphocytes, and platelets are related to mild myopic retinopathy. A higher level of circulating basophils is related to the severe form of myopic retinopathy, such as mCNV. Full article

High myopia may develop to pathologic myopia, which brings severe visual impairment; however, the etiology is not fully understood. We, therefore, investigated the relationship between the presence of posterior staphyloma and posterior ocular disorders by assessing the patients with high myopia. A retrospective study was performed for the patients, who have more than 26 mm of the axial length and of whom fundus photography was taken with an ultra-widefield retinal imaging system. The objectives were 138 cases encompassing 229 eyes. In 138 cases, 91 were bilateral and 47 were unilateral. The averages ± SD of axial length of bilateral and unilateral were 28.8 ± 2.2 mm, 27.3 ± 1.2 mm, respectively, showing statistically significant difference. The number of eyes with and without posterior staphyloma were 107 (46.7%) and 122 (53.3%), respectively. Retinal detachment and retinal breaks are more observed in cases without posterior staphyloma (p = 0.017). Myopic choroidal neovascularization (mCNV) (p = 0.002), chorioretinal atrophy (p < 0.001), retinoschisis (p < 0.001), and optic neuropathy (p = 0.038) are more often seen in cases with posterior staphyloma. In conclusion, the prevalence rates of myopic choroidal neovascularization, retinal choroidal atrophy, and optic neuropathy were significantly higher with posterior staphyloma. The rate of periocular disorders such as retinal detachment was significantly higher without posterior staphyloma. These results indicate associations between types of pathological myopia and presence or absence of posterior staphyloma analyzed by ultra-widefield retinal imaging. Full article