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.

A physical sign of pathological myopia: myopic scleral pit.

PURPOSE: Myopic scleral pit (MSP) is a rare physical sign of pathological myopia (PM). The aim of this study was to summarize the clinical characteristics of MSP and analyze its correlation with PM. METHODS: Eight cases with PM and MSP were enrolled in this study. Comprehensive ophthalmic examinations, including subjective refraction, slit-lamp biomicroscope, intraocular pressure, fundus photographs, A- and B-scan ultrasonography and spectral-domain optical coherence tomography, were performed. RESULTS: All the patients had a long history of PM with visual impairment, long axial length, and myopia-related fundus degeneration. Mean axial length was 31.48 ± 2.17 mm. Mean size of MSP was 0.69 ± 0.29 optic disc diameter (PD). Mean logMAR BCVA was 1.21 ± 0.88 logMAR. Spearman correlation analysis showed that the logMAR BCVA had no correlation with the size of pits (P = 0.34). Fundus examination revealed a focal pale concave located in the sclera exposed area of retinal choroid atrophy was found in all cases. OCT showed a deep scleral pit where the retinal choroid was thin or absent, without retinal sensory detachment or sensory defect. CONCLUSIONS: This study identified a rare scleral lesion in all eight individuals with PM, which was termed "myopic scleral pit". This phenomenon is different from focal choroidal excavation and posterior staphyloma.

[Advances on optic disc morphological features and peripapillary structure changes in high myopia].

High myopia is an important cause of low vision and blindness in the world, most of which are characterized by the prolongation of the axial length, accompanied by various degenerative changes of fundus posterior pole, especially in the optic disc area and peripapillary structures, such as optic disc tilt, optic cup and rim changes, chorioretinal atrophy, posterior staphyloma and intrachoroidal cavitation, and so on. This article reviews the optic disc morphological features and peripapillary structure changes of high myopia, in order to reveal the pathogenesis of high myopia and provide new ideas for finding more effective prevention and treatment methods.

Silicone Oil Tamponade-Retina Contact in Highly Myopic Eyes With and Without Encircling Bands: A Computational Fluid Dynamics Study.

Purpose: To investigate the behavior of silicone oil (SiO) at a steady equilibrium and during saccades in pseudophakic highly myopic eyes with posterior staphyloma with and without an encircling band and compare it to behavior in emmetropic eyes. The SiO-retina contact area and shear stress were calculated by computational fluid dynamics. Methods: A numerical model of an emmetropic eye and a myopic eye with and without scleral band underwent a saccade of 50°/0.137 s. The vitreous chamber surface was divided into superior and inferior 180° sectors: lens, pre-equator, post-equator, and macula. SiO-retina contact was evaluated as a function of fill percentages between 80% and 90% for standing, 45° upward tilt, and supine patients. Maximum and average shear stress were calculated. Results: Overall, SiO-retina contact ranged between 40% and 83%; fill percentage varied between 80% and 95%. Neither the encircling scleral band nor the staphyloma significantly affected the SiO-retina contact area, although the presence of a scleral band proved disadvantageous when gazing 45° upward. The inferior retina-SiO contact remained below 40% despite 95% SiO fill. The SS significantly increased at the scleral band indentation and decreased elsewhere. The staphyloma greatly reduced shear stress at the macula. Conclusions: The presence of a myopic staphyloma reduces shear stress at the macula but does not alter SiO-retina contact significantly. The apposition of a 360° scleral band may reduce SiO-retina contact at least in some postures and increases the SS at the indentation. Translational Relevance: Assessing SiO-retina contact when vitreous chamber geometry changes according to pathologic or iatrogenic modifications allows accurate prediction of real-life tamponade behavior and helps explain surgical outcomes.

Histopathological Characteristics of Limbal Stem Cell Deficiency Secondary to Chronic Vernal Keratoconjunctivitis.

PURPOSE: To describe the histopathological characteristics of limbal stem cell deficiency (LSCD) due to chronic vernal keratoconjunctivitis (VKC). METHODS: This retrospective study included 14 eyes of 13 patients who underwent simple limbal epithelial transplantation for total LSCD from 2017 to 2018. The histological characteristics of the excised fibrovascular pannus were compared between 2 groups of 7 eyes, each with LSCD due to VKC and chemical burns (CB). Histological characteristics and type of inflammation were studied using special stains and immunohistochemistry. Fisher exact test was used to detect the statistical significance of the histological differences between both groups. RESULTS: Epithelial hypertrophy, epithelial downgrowth, and eosinophilic infiltration were noted in all eyes in the VKC group (7/7, 100%). Epithelial hypertrophy was noted in 3 of the 7 (42.8%) eyes in the CB group, whereas epithelial downgrowth and eosinophilic infiltrates were absent. The average chronic inflammatory score of the pannus (5.28) was higher in VKC than in CB (3.85; P = 0.1080). The presence of goblet cells was higher in the CB group (5/7, 1.4%) than in the VKC group (3/4, 2.8%), although not statistically significant. Other histological differences between the groups were not statistically significant. CONCLUSIONS: The histopathological features of LSCD in VKC reveal some distinctive characteristics. These include the presence of epithelial downgrowth, eosinophilic infiltration, and epithelial solid and cystic implants. Although this information may be used to establish the diagnostic criteria for VKC as the cause of LSCD, further studies are needed to elucidate the reasons behind these unique findings.

Aggressive-fungating retinal hemangioblastoma.

Retinal hemangioblastomas are one of the most common and early manifestations of Von Hippel-Lindau disease. Early detection is the key in their management. When left untreated, these benign neoplasms may continue to grow and result in scleral infiltration and extraocular extension warranting enucleation of the globe.

A 10-year review of histopathological findings of enucleated eyes from a tertiary-care center, Pakistan.

OBJECTIVE: To determine the histopathology of enucleated eye specimens. METHODS: The 10-year inventory was conducted at Aga Khan University Hospital, Karachi, and comprised all histopathology reports of enucleated eye specimens received from January 2007 to December 2016 by the Section of Histopathology, Department of Pathology. SPSS 19 was used for data analysis. RESULTS: A total of 232 ocular tissue specimens from 231 patients were evaluated. Ocular tumours were the most common histopathological diagnosis 186(80%). Among tumours, retinoblastoma was the most common 137(59%) followed by malignant melanoma 31(13%) and squamous cell carcinoma 15(6.5%). Overall, 16(6.9%) specimens had degenerative changes secondary to different ocular disorders. Staphyloma and Coat's disease was diagnosed in 3(1.3%) cases each. In 4(1.7%) cases, there was no formal diagnosis. CONCLUSIONS: Early identification of tumours may allow for conservative management and limit the need for enucleations.

Multifocal choroidal melanoma in oculodermal melanocytosis in an Asian Indian.

A 51-year-old Asian Indian male with right oculodermal melanocytosis presented with a blurred visual acuity of 20/40. Upon fundus examination, he was discovered to have two independent pigmented choroidal melanomas in the temporal juxtapapillary region and inferiorly, in the region of trace clinical sectoral choroidal melanocytosis. The patient underwent enucleation, and on histopathology the two tumors were found to be in the area of choroidal melanocytosis, in continuity with each other. Multifocal choroidal melanoma is an exceedingly rare diagnosis. Ocular melanocytosis is a known predisposing factor for both unifocal and multifocal melanomas. Lifelong monitoring for uveal melanomas must be done in all patients with ocular melanocytosis.

Trifocal choroidal melanoma in an eye with oculodermal melanocytosis: A case report.

We report a case of trifocal choroidal melanoma (three separate tumors) in a 48-year-old Caucasian female who had been followed for oculodermal melanocytosis since childhood. At presentation, no tumor was present and annual examination was advised. Seventeen years later, three choroidal melanocytic lesions were detected in the right eye. Growth of each was documented, enucleation was performed, and histopathology revealed three independent choroidal melanomas. The patient developed extensive liver and bone metastases and subsequently died. Oculodermal melanocytosis is a risk factor for the development of uveal melanoma and a potential marker for worse prognosis. Careful long-term follow-up is required.

Congenital choroidal melanocytoma in a child with ocular melanocytosis.

A 1-day-old child was brought to the clinic for evaluation of enlarged right eye (OD). On examination, OD showed buphthalmos with diffuse scleral melanocytosis, fleshy blackish-brown extrascleral mass with corneal extension, and secondary glaucoma. Anterior segment evaluation revealed darkly pigmented iris and fundus evaluation OD revealed a darkly pigmented choroidal lesion. The left eye was within normal limits. A clinical diagnosis of choroidal melanocytoma with ocular melanocytosis was made. Enucleation OD followed by orbital implant was performed. Histopathology showed features of diffuse ocular melanocytosis involving limbus, iris, ciliary body, choroid, sclera, optic nerve head, optic nerve sheath, along with choroidal melanocytoma with extrascleral tumour extension. We presume that choroidal melanocytoma may have arisen from ocular melanocytosis.

Reaction on "Ocular ultrasound versus MRI in the detection of extrascleral extension in a patient with choroidal melanoma".

BACKGROUND: In the recently published article entitled "Ocular ultrasound versus MRI in the detection of extrascleral extension in a patient with choroidal melanoma" Jacobsen et al. describe a case in which a hyper-intense extra-ocular lesion on MRI was erroneously diagnosed as an extrascleral extension of the tumor. Based upon this the authors conclude "the superiority of ocular ultrasound in the diagnostic management of extra scleral extension in choroidal melanoma". In our view, there are numerous flaws in the investigation that cast doubt on this message. MAIN: First of all, this is quite a bold statement when only one patient has been evaluated. Secondly, the manuscript only presents a post-contrast T1-weighted image, whereas multiple MRI-sequences need to be included to determine if a hyperintense region is an extrascleral invasion. Moreover, no modern MRI-techniques such Dynamic Contrast Enhanced (DCE) or Diffusion Weighted Imaging (DWI) have been included in the evaluation of this patient, making it hard to use this single case to compare the efficacy of MRI and Ultrasound. The presented data do, however, give clear clues that the hyperintense lesion is likely to be inflammatory. CONCLUSION: Although the study falls short in providing a comprehensive comparison between current MRI techniques and ultrasound, it does show that the evaluation of ocular MR-images should be made in a multi-disciplinary setting involving both ophthalmologist and radiologists, since the field of ocular MRI is continuously progressing.

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.

[Local, regional and systemic dissemination of choroidal melanoma : Clinical and pathological correlation from 2 cases]. / Dissémination locorégionale et systémique du mélanome choroïdien : corrélation anatomoclinique à partir de 2 cas.

PURPOSE: To describe didactically the local, regional and systemic spread of choroidal melanoma. PATIENTS AND METHODS: Two patients who had undergone primary enucleation for the management of choroidal melanoma in 2018 at the University Hospital of Nice were included. Extrascleral extension and invasion of the vortex veins were evaluated, as well as synchronous and metachronous metastases, based on our database. RESULTS: Patient 1 was diagnosed with large choroidal melanoma with partial scleral invasion and vortex vein involvement. Cytogenetic analysis demonstrated a loss of chromosome 3, and a gain of chromosome 8q. Systemic work-up was unremarkable. Patient 2 was diagnosed with a large choroidal melanoma with extrascleral extension and vortex vein involvement. Cytogenetic analysis demonstrated a loss of chromosome 3 and a gain of chromosome 8q. Systemic work-up revealed several liver metastases. A total of 1762 patients were included in our database. Eighty-five patients (4.8 %) and 46 patients (2.6 %) experienced vortex vein invasion and extrascleral extension respectively. Patients with vortex vein invasion were diagnosed with synchronous and metachronous liver metastases in 1.2 % and 18.8 % respectively. Patients with extrascleral extension had synchronous and metachronous liver metastases in 6.5 % and 30.4 % respectively. The mean follow-up was 49.4 months (1-180). CONCLUSION: Extrascleral extension and vortex vein invasion illustrate the local, regional and systemic spread of choroidal melanoma. The latter are often associated with genetically aggressive tumours associated with high metastatic risk.

Posterior staphylomas and scleral curvature in highly myopic children and adolescents investigated by ultra-widefield optical coherence tomography.

PURPOSE: To determine the early signs of posterior staphylomas in highly myopic eyes of younger subjects by swept-source ultra-widefield optical coherence tomography (WF-OCT). METHODS: This was an observational case series study. Highly myopic subjects younger than 20 years old who were examined consecutively by prototype WF-OCT were studied. High myopia was defined according to the Ministry of Health and Welfare, Japan classification. A posterior displacement of the sclera and two OCT features indicating the staphyloma edges were used as markers of a staphyloma. RESULTS: Fifty-five eyes of 30 patients with the mean age of 12.3 years, and the mean axial length of 27.9 mm were studied. Seven of the 55 eyes (12.7%) had a posterior displacement of the sclera and were diagnosed as having a staphyloma. Among the two OCT features of the staphyloma edges, a gradual thinning of the choroid toward the staphyloma edge and gradual re-thickening of choroid from the staphyloma edge toward the posterior pole were found in these 7 eyes. However, the other feature of an inward protrusion of the sclera at the staphyloma edge, was obvious in only 2 eyes. The subfoveal choroid and choroid nasal to the optic disc were significantly thinner in eyes with a staphyloma than those without it. CONCLUSIONS: The changes of the choroidal thickness toward the staphyloma edge with the posterior displacement of the sclera were considered an early sign which precedes an inward protrusion of sclera at the staphyloma edge.