Clinical features of primary and compound forms of wide macular posterior staphyloma in high myopia.

BACKGROUND: To compare the ocular features of highly myopic eyes with posterior staphyloma of wide macular type according to its morphological complexity. METHODS: In this cross-sectional study, wide macular posterior staphyloma (WMPS) was classified into the primary (Curtin type I) and the compound (Curtin types VI to X) forms based on the configuration within the staphyloma. The grades of myopic maculopathy and the thicknesses of choroid and sclera were compared between the primary and compound forms of WMPS. RESULTS: A total of 154 eyes (103 patients) with primary WMPS and 65 eyes (49 patients) with compound WMPS were included. Eyes with compound WMPS had worse visual acuity (P = 0.001) and greater axial length (P < 0.001) than those with primary WMPS. Compared to primary WMPS, compound WMPS had a higher grade of myopic macular degeneration (P < 0.001) and a higher frequency of lamellar or full-thickness macular hole associated with myopic traction (21.5% vs. 10.4%; P = 0.028) and active or scarred myopic choroidal neovascularization (33.8% vs. 20.1%; P = 0.030). On swept-source optical coherence tomography, eyes with compound WMPS had significantly thinner choroid and sclera. CONCLUSIONS: The compound form of WMPS had more severe myopic macular changes and worse visual prognosis compared to the primary form of WMPS, and these were associated with more structural deformation in the posterior eyeball. Compound WMPS should be considered as an advanced form of staphyloma.

Imágenes multimodales y retromodo en la calcificación esclerocoroidea: reporte de un caso / Multimodal and retro-mode imaging in sclerochoroidal calcification: A case report

Presentamos un reporte de caso en una paciente de 70años sobre las calcificaciones esclerocoroideas, una rara condición que involucra depósitos de pirofosfato de calcio en el polo posterior del ojo. Proporcionamos un relato sobre la presentación clínica y su apariencia en imágenes multimodales, utilizando fotografías de color del fondo de ojo, tomografía de coherencia óptica de fuente de barrido (SS-OCT), ecografía ocular y la novedosa técnica de retromodo (RMI). Al examen la agudeza visual fue de 20/25 en el ojo derecho y de 20/20 en el ojo izquierdo. La fotografía de color del fondo de ojo reveló depósitos amarillentos localizados en la arcada temporal-superior de ambos ojos. SS-OCT demostró masas de origen escleral. Las ecografías oculares confirmaron la calcificación de estas masas. El RMI detectó imágenes hiperreflectivas con una marcada elevación superficial de las lesiones. El laboratorio sistémico no presentó alteraciones, lo que llevó al diagnóstico de calcificaciones esclerocoroideas bilaterales de origen idiopático.(AU)

We present a case report on sclerochoroidal calcification (SCC), a rare condition involving calcium pyrophosphate deposits in the posterior pole of the eye in a 70-year-old patient. We provide an account of the clinical presentation and its appearance in multimodal images, using color fundus photography, swept-source optical coherence tomography (SS-OCT), ocular ultrasound, and the novel retro-mode imaging (RMI) technique. Visual acuity was 20/25 in the right eye (OD) and 20/20 in the left eye (OS). Color fundus photography revealed yellowish deposits located in the upper temporal arcade of both eyes. SS-OCT demonstrated masses of scleral origin. Ocular ultrasounds confirmed the calcification of these masses. RMI detected hyper-reflective images with marked superficial elevation. Systemic laboratory results did not detect any abnormalities, leading to the diagnosis of bilateral idiopathic SCC.(AU)

Multimodal and retro-mode imaging in sclerochoroidal calcification: A case report.

We present a case report on sclerochoroidal calcification (SCC), a rare condition involving calcium pyrophosphate deposits in the posterior pole of the eye in a 70-year-old patient. We provide an account of the clinical presentation and its appearance in multimodal images, using color fundus photography, swept-source optical coherence tomography (SS-OCT), ocular ultrasound, and the novel retro-mode imaging (RMI) technique. Visual acuity was 20/25 in the right eye (OD) and 20/20 in the left eye (OS). Color fundus photography revealed yellowish deposits located in the upper temporal arcade of both eyes. SS-OCT demonstrated masses of scleral origin. Ocular ultrasounds confirmed the calcification of these masses. RMI detected hyper-reflective images with marked superficial elevation. Systemic laboratory results did not detect any abnormalities, leading to the diagnosis of bilateral idiopathic SCC.

Calcified Sclero-Choroidal Choristomas in Mosaic RASopathies: A Description of a New Imaging Sign.

PURPOSE: To evaluate the imaging and clinical features of unusual calcified lesions seen in the fundus of patients with mosaic RASopathy. DESIGN: Single-center retrospective observational study. SUBJECTS: Ten eyes with calcified fundus lesions in 7 patients with mosaic RASopathy. METHODS: The lesions were evaluated with fundus photography, oral fundus fluorescein angiography, B-scan ultrasonography, magnetic resonance imaging (MRI), and computed tomography (CT) scan where available. MAIN OUTCOME MEASURES: The imaging characteristics of calcified fundus lesions were assessed. RESULTS: We found 7 patients with mosaic RASopathies, 5 men and 2 women (3 with linear sebaceous nevus syndrome, 3 with oculoectodermal syndrome, and 1 with encephalocraniocutaneous lipomatosis) with molecular confirmation in 5 cases, all 5 having KRAS-pathogenic variants. Calcified fundus lesions were identified in 10 eyes (bilateral in 3 patients), appearing as slightly elevated, creamy-yellow lesions around or adjacent to the optic nerve, extending supero-nasally; all but 2 of these lesions involved both the choroid and sclera, with 2 of them only involving the sclera at the time of examination. One case developed a choroidal neovascular membrane necessitating intravitreal bevacizumab injections. All 7 patients had B-scan ultrasonography, and the lesion appeared as a hyperechogenic area with an acoustic shadow posteriorly despite reduced gain. Five patients had MRI, and where fundus lesions were present, there was a focal defect in the sclero-choroidal layer. Four patients had a CT scan, and all 4 showed calcifications affecting both the posteromedial sclero-choroid and adjacent medial rectus muscle. Two of these patients had normal eye movements, 1 had a unilateral fixed adducted eye and a vestigial fibrous medial rectus muscle seen in imaging and intraoperatively, and the fourth had marked exotropia with a right gaze deficit affecting both eyes. CONCLUSIONS: We propose that the lesions seen in this cohort are calcified sclero-choroidal choristomas and should be suspected in mosaic RASopathies when creamy-yellow lesions are seen in the fundus. If identified, the possibility of choroidal neovascularization should be considered during follow-up. In all cases where a CT scan was performed, a novel sign of sclero-muscular calcification involving the medial rectus muscle was seen. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Diagnosis of posterior staphyloma using the radius of steepest curvature among retinal pigment epithelium segmentation line measured by optic coherent tomography.

PURPOSE: To investigate a novel marker to diagnose posterior staphylomas by measuring the radius of the steepest curvature on the retinal pigment epithelium (RPE) segmentation line using optical coherence tomography (OCT). STUDY DESIGN: Retrospective Cross-sectional Study. METHODS: The authors developed a prototype software to measure the radius of curvature on the RPE segmentation line of OCT. Twelve images of 9-mm radial OCT scans were used. The radius of curvature was measured at the steepest area of the RPE segmentation line, and the macular curvature (MC) index was calculated based on its reciprocal. Based on the wide-field fundus findings, the study sample was divided into three groups: definite posterior staphyloma, no posterior staphyloma, and undetermined. The differences of MC index among the groups and the correlation between the MC index, age, and axial length were analyzed. RESULTS: The present study analyzed 268 eyes, with 54 (20.1%) with definite posterior staphyloma, 202 (75.4%) with no posterior staphyloma, and 12 (4.5%) with undetermined disease status. A maximum MC index of 37.5 was observed in the group with no posterior staphyloma, which was less than the minimum MC index of 42.7 observed in the group with definite posterior staphyloma. The MC index had strong correlations with the axial length and age in eyes with high myopia. CONCLUSIONS: Eyes with posterior staphyloma have a steeper curvature than those with radius 8.44 mm, while eyes without posterior staphyloma do not. MC index 40 (radius 8.44 mm) might act as a reference to distinguish between those with and those without posterior staphyloma.

Peripheral and posterior pole retinal changes in highly myopic Chinese children and adolescents : Retinal changes in Chinese children and adolescents.

PURPOSE: This study was conducted to evaluate the prevalence and related factors of peripheral and posterior pole retinal changes in highly myopic Chinese children and adolescents. METHODS: A hospital-based cross-sectional study was designed. A total of 120 subjects with high myopia were recruited and underwent cycloplegic refraction, dilated fundus examination, and optical coherence tomography. A statistical analysis was performed to evaluate the factors associated with peripheral and posterior pole retinal changes. RESULTS: The mean spherical equivalent refraction of the subjects was - 8.74 ± 2.86 D, and the mean age was 11.45 ± 3.02 years. Snowflake retinal degeneration (27.5%), white without pressure (27.5%), snail-track degeneration (15%), and lattice degeneration (15%) were the most common peripheral retinal changes, while tessellated fundus (84.17%), optic nerve crescents (78.3%), and posterior staphyloma (11.7%) were the most common posterior changes. Subjects with peripheral changes were significantly older, with thinner choroids (OR = 1.194, 95% CI: 1.045-1.363, p = 0.009; OR = 0.993, 95% CI: 0.987-0.999, p = 0.022, respectively). Optic nerve crescents, tessellated fundus, and posterior scleral staphyloma were all associated with thin choroids (OR = 0.990, 95% CI: 0.983-0.997, p = 0.008; OR = 0.983, 95% CI: 0.974-0.991, p < 0.001; OR = 0.974, 95% CI: 0.960-0.987, p < 0.001, respectively). CONCLUSION: A substantial proportion of the subjects had peripheral and posterior retinal changes. An increased risk of retinal changes was associated with high degrees of myopia, long axial lengths, thin choroids, and older ages among 7-16-year-old individuals.

Characteristics and Prevalence of Staphyloma Edges at Different Ages in Highly Myopic Eyes.

Purpose: The purpose of this study was to determine the characteristics of staphyloma edges in highly myopic eyes and how they progress. Methods: We conducted a cross-sectional analysis using baseline data and a longitudinal study with follow-up data from 256 patients (447 eyes) with high myopia, with a mean (SD) follow-up of 3.79 (0.78) years. Participants were divided into four age groups: children (<13), youth (13-24), mature (25-59), and elderly (>60). Ultrawide-field swept-source optical coherence tomography was used to analyze staphyloma edges, which were divided into four areas: nasal to the optic disc (OD), superior to the macula, inferior to the macula, and temporal to the macula. Results: Staphylomas were significantly more prevalent in the mature (42.49%) and the elderly (51.35%) groups than in the children (13%) and youth (9%) groups. Staphyloma edges were predominantly superior to the macula in the mature and elderly groups. In contrast, staphylomas were rare in children and youth, with their edges mainly located nasal to the OD. The edges of staphylomas located superior and temporal to the macula were more likely to be associated with myopic traction maculopathy. During the follow-up period, 11 new staphyloma edges developed primarily in the mature group (64%). Additionally, 12 edges had an increased degree of protrusion over time, with most cases occurring in the mature (75%) group. Conclusions: The prevalence and location of staphyloma edges show significant variations depending on age. As time progresses, staphyloma edges manifest at distinct sites and increase their protrusion, potentially playing a role in the emergence of fundus complications.

Influence of posterior staphyloma in myopic maculopathy and visual prognosis.

BACKGROUND/OBJECTIVES: Posterior staphyloma is a hallmark of high myopia and its presence associates to greater degrees of myopic maculopathy. Nonetheless, its development, repercussion on visual function and relationship with maculopathy components, is still unclear. The objective was to analyze the impact of posterior staphyloma on the incidence and severity of myopic maculopathy and its repercussion on visual prognosis. SUBJECTS/METHODS: Cross-sectional study conducted on 473 consecutive eyes of 259 highly myopic patients examined at Puerta de Hierro-Majadahonda University Hospital (Madrid, Spain). All patients underwent complete ophthalmologic examination including best corrected visual acuity (BCVA), axial length (AL), myopic maculopathy classification according to ATN system (atrophic/traction/neovascularization), determined the presence of posterior staphyloma, pathologic myopia (PM) and severe PM. Multimodal imaging were performed including fundus photography, optical coherence tomography (OCT), OCT-angiography, fundus autofluorescence and/ or fluorescein angiography. RESULTS: Out of the total, 70.65% were female patients (n = 173/259), mean BCVA was 0.41 ± 0.54 logMAR units and mean AL was 29.3 ± 2.6 mm (26-37.6). Posterior staphyloma was present in 69.4% of eyes. Eyes with posterior staphyloma compared to non-staphyloma were older (p < 0.05), had greater AL (p < 0.01), worse BCVA (p < 0.01) and higher stage in ATN components (p < 0.01). Moreover, compound subgroup showed worse BCVA (p < 0.01) and greater stage in each of the ATN components (p < 0.01). Staphylomas with macular involvement presented worse BCVA (p < 0.01), higher AL (p < 0.01), and greater ATN (p < 0.05). The risk of posterior staphyloma presence in eyes with PM and severe PM eyes was 89.8% and 96.7%, respectively. Posterior staphyloma was the best predictor for BCVA in myopic patients (p < 0.01). CONCLUSIONS: Posterior staphyloma's presence determines high risk of myopic maculopathy and therefore worse visual prognosis, especially those with macular involvement. Posterior staphyloma represented the best predictor for BCVA in highly myopic patients.

Giant anterior scleral staphyloma caused by blunt ocular trauma: a case report.

BACKGROUND: Anterior scleral staphyloma is a relatively rare disease characterized by thinning and expansion of sclera. We described the clinical presentation, diagnosis and treatment of a case with giant anterior scleral staphyloma caused by blunt ocular trauma. CASE PRESENTATION: A 24-years-old male, presented with a black cyst-like mass protruding from the right eyeball for 9 years after a history of glass crush contusion. The ultrasound biomicroscopy examination showed two cysts in the right eyeball. The larger one was about 5.92 mm*4.69 mm in size and the scleral lacerations were connected to the posterior chamber below the cyst. For treatment, resection of the anterior scleral staphyloma and the scleral patch graft transplantation was performed. The vision of the patient was improved compared with that before surgery. There were no obvious complications. CONCLUSION: The clinical presentation, diagnosis, and treatment of the case with giant anterior scleral staphyloma can provide a reference for the management of anterior scleral staphyloma. Surgical resection and scleral patch graft should be a good option for the treatment of giant anterior scleral staphyloma.

Ultrasound Assessment of Gaze-induced Posterior Eyewall Deformation in Highly Myopic Eyes.

Purpose: To establish a quantitative metric of posterior eyewall deformability in different directions of gaze in highly myopic eyes with and without posterior staphyloma. Methods: A prospective study was performed on 53 highly myopic patients (106 eyes). Ultrasound scans were acquired in primary, up, downward, nasal, and temporal gazes. A validated intensity-based segmentation algorithm was used to quantify the posterior eyewall geometry on digitalized B-scan images. Posterior eyewall local curvature (K) and distance (L) to the transducer were calculated. The associations between directions of gaze, axial length (AL), and presence of staphyloma with the K and L parameters were assessed. Results: A total of 53 participants (106 eyes) were studied. Multivariate regression analysis demonstrated that, after accounting for longer AL, and presence of staphyloma, eccentric gaze was often independently associated with various K and L parameters. Specifically, downward gaze was associated with increased posterior eyewall concavity as reflected in the maximum of K (KMax) (ß = 0.050, P < 0.001) and absolute value of KMax (ß = 0.041, P = 0.011). Both downward gaze and upgaze were independently associated with increase in the derivative of absolute KMax (which is consistent with more apparent, steeper staphyloma ridges), local KMax (which detects KMax at smaller intervals), and Kstd (which represents likelihood of staphyloma presence) and decrease in maximum of L (which represents movement of the staphyloma apex) with all P < 0.05. The ß coefficients for downward gaze were consistently greater in magnitude compared with those in upgaze. After accounting for AL and presence of staphyloma, horizontal gazes were independently associated only with decrease in the standard deviation of L (which also represents likelihood of staphyloma presence) and maximum of L. Conclusions: Downward gaze results in a significant increase in posterior eyewall concavity in highly myopic eyes after accounting for AL and staphyloma presence. In comparison with downward gaze, upgaze resulted in a lower magnitude, but significant changes in staphyloma ridge steepness and the likelihood of staphyloma presence. Horizontal gazes seemed to be associated with less posterior eyewall geometric parameters. Studies are required to further assess the association between downward gaze during near work on posterior eyewall concavity and possible effects on myopia development and progression.

Effects of posterior staphyloma on choroidal structure in myopic adults: a retrospective study.

BACKGROUND: Studies on the choroid of myopic eyes with posterior staphyloma have shown that choroidal thickness decreased. This retrospective study further analysed the effects of posterior scleral staphyloma on choroidal blood vessels and matrix components compared to non-pathological myopia. METHODS: In this cross-sectional study, ninety-one eyes were divided into pathological (posterior staphyloma) and non-pathological myopia. The latter was further divided into three groups (Group 1: 26 mm ≤ axial length; Group 2: 24 mm ≤ axial length < 26 mm; Group 3: 22 mm ≤ axial length < 24 mm). Choroidal thickness, total choroidal area, luminal area, stromal area, and choroidal vascularity index were calculated. RESULTS: The CVI in N1, N2, I1, S2 of the posterior staphyloma group were lower than those of group 1 (both P < 0.05). The mean height of posterior staphyloma was associated with mean CT (Pearson correlation: r = -0.578, P = 0.039) but not with the mean CVI in posterior staphyloma group. In all groups, the mean choroidal thickness, total choroidal area, luminal area, and stromal area were significantly associated with axial length (P < 0.001), and the mean choroidal vascularity index was significantly associated with the mean choroidal thickness (P < 0.001). CONCLUSION: The choroidal structure of pathological myopia with posterior staphyloma and non-pathological myopia with longer axial length demonstrates alterations in which choroidal vessels are more impaired than the stroma. A lower choroidal vascularity index should be alert to pathological changes for myopia with axial length > 26 mm.

Prevalence of Dome-Shaped Macula and Tilted Disc Syndrome in High Myopia.

BACKGROUND AND OBJECTIVE: Dome-shaped macula (DSM) and tilted disc syndrome (TDS) are two macular abnormalities that may occur in eyes with high myopia. The aim of this study was to determine the prevalence of both entities in our population. PATIENTS AND METHODS: This was a prospective and observational study. Optical coherence tomography of the macula was performed in eyes with high myopia (spherical equivalent [SE] of -8D or greater) to assess the prevalence of DSM and TDS. RESULTS: Sixty-eight eyes were included. Three eyes (4.41%) had DSM and 8 (11.76%) eyes had TDS. The most common macular anomaly was posterior staphyloma (PS) (12 [17.65%]). From the eyes with DSM (n = 3), only two presented PS. An older age and a higher SE were predisposing factors for PS (P = 0.003). CONCLUSIONS: A lower prevalence of DSM and a higher prevalence of TDS was observed in our population compared to those reported in literature. [Ophthalmic Surg Lasers Imaging Retina 2023;54:568-572.].

Successful Medical and Surgical Management of Recalcitrant Acanthamoeba Keratitis, Scleritis, and Culture-Positive Scleral Abscess.

PURPOSE: The purpose of this study was to describe the management of a case of recurrent scleritis and Acanthamoeba -positive scleral abscess in a patient after the use of miltefosine for recalcitrant Acanthamoeba keratitis. METHODS: This is a case study. RESULTS: In this study, we report a case of advanced Acanthamoeba keratitis with resultant corneal perforation with therapeutic keratoplasty and associated scleritis who later developed a scleral abscess after treatment with oral miltefosine. The scleral abscess was positive for Acanthamoeba cysts and trophozoites, and after treatment for an additional several months, the patient had complete resolution of her disease. CONCLUSIONS: Acanthamoeba scleritis is a rare complication associated with Acanthamoeba keratitis. It has traditionally been treated as an immune reaction and associated inflammation, especially with the use of miltefosine. Management can require a multitude of different approaches, and in this situation, it has been demonstrated that scleritis can be infectious and that conservative management can be effective.

Visualization of Short Posterior Ciliary Artery Insertion Due to Posterior Staphyloma and Myopic Chorioretinal Atrophy.

This case report describes the visualization of a short posterior ciliary artery insertion as a result of posterior staphyloma and chorioretinal atrophy in a patient in their 50s with pathologic myopia.

Posterior Staphyloma as Determining Factor for Myopic Maculopathy.

PURPOSE: The aim of this study was to determine the influence of posterior staphyloma (PS) on the development of myopic maculopathy. DESIGN: Cross-sectional study. METHODS: A total of 467 highly myopic eyes (axial length [AL] ≥26 mm) of 246 patients were included. Patients underwent a complete ophthalmological examination, including multimodal imaging. Presence of PS was defined as the main variable analyzed between groups (PS vs non-PS): age, AL, best-corrected visual acuity (BCVA), atrophy/traction/neovascularization (ATN) components, and presence of severe pathologic myopia (PM). Two different cohorts were studied (age-matched and AL-matched) comparing PS vs non-PS eyes. RESULTS: In all, 325 eyes (69.59%) presented with PS. Eyes without PS were younger and had lower AL, ATN components, and prevalence of severe PM than those with PS (P < .001). Moreover, non-PS eyes had better BCVA (P < .001). Analyzing the age-matched cohort (P = .96); mean AL, A, and T components, and severe PM were significantly higher in the PS group (P < .001), as well as the N component (P < .005), showing worse BCVA (P < .001). Regarding the AL-matched cohort (P = .93), the PS group showed worse BCVA (P < .01), older age (P < .001), A (P < .001), and T components (P < .01), and severe PM (P < .01). The risk of PS increased by 10% per year of age (odds ratio = 1.109, P < .001) and by 132% per each millimeter of growth of AL (odds ratio = 2.318, P < .001). CONCLUSIONS: Posterior staphyloma is associated with myopic maculopathy, worse visual acuity, and higher prevalence of severe PM. AL and age, in this order, constitute the main factors associated with the onset of PS.