SCLERAL EXCAVATION AT OBLIQUE MUSCLE INSERTIONS: IMAGING CHARACTERISTICS AND CORRELATION WITH SCLERAL PLAQUES AND SCLEROCHOROIDAL CALCIFICATION.

PURPOSE: To compare the imaging features of lesions showing hyporeflective posterior scleral excavation found near the insertions of the oblique extraocular muscles to the features and the natural course of Cogan scleral plaques. METHODS: Multimodal imaging with color fundus photography, spectral-domain optical coherence tomography (OCT), swept-source optical coherence tomography, and B-scan ultrasonography. RESULTS: A 71-year-old man and an 89-year-old man presented with ring-shaped hypopigmented lesions measuring between 200 µm and 300 µm transversally, and located along the superior vascular arcade and temporal to the fovea. All lesions were identified near the insertion of oblique muscles, with one observed in the temporal macula, and two found along the superotemporal arcades. Enhanced depth imaging-optical coherence tomography showed hyporeflective boat-shaped areas of scleral excavation with reduced choroidal thickness along their margins. B-scan ultrasonography showed the lesions to be intensely reflective with varying degrees of posterior shadowing. CONCLUSION: To our knowledge, this is the first report of excavated hyporeflective scleral lesions found near the oblique muscle insertions. Imaging and clinical data support the diagnosis of a posterior form of Cogan scleral plaque and are consistent with the natural course of this entity.

Scleral Thickness in Autosomal Dominant Best Vitelliform Macular Dystrophy.

PURPOSE: To investigate the posterior and equatorial scleral thickness in patients with autosomal dominant Best disease, a condition that has chronic subretinal fluid. METHODS: Retrospective study involving patients with Best disease and age-matched controls. Participants were evaluated with contact B-scan ultrasonography and enhanced depth imaging optical coherence tomography to evaluate scleral thickness in the posterior pole and equator. Univariate analysis and generalized estimating equations were used. RESULTS: Of 9 patients with genetically proven Best disease and 23 age-matched controls, there was no significant difference in the age or the gender proportion between groups. Subfoveal choroidal thickness and axial length were not significantly different between groups. Both posterior scleral (OD; 1.38mm vs. 0.89mm, P<.001 and OS; 1.39mm vs. 0.83mm, P<.001) and equatorial scleral (OD; 0.61mm vs. 0.42mm, P=.003, and OS; 0.55mm vs. 0.41mm, P=.017) thicknesses were much greater in cases as compared with controls. Multivariate analysis showed male sex and having Best disease were each significant predictors of posterior scleral thickness and Best disease was the sole significant predictor for equatorial scleral thickness. CONCLUSION: BEST1 gene may have a developmental role leading to having a thicker sclera, influencing disease manifestation, and contributing to the accumulation of subretinal fluid in Best disease.

REGIONAL SCLERAL THICKNESS AS A RISK FACTOR FOR CENTRAL SEROUS CHORIORETINOPATHY.

PURPOSE: To evaluate regional sclera thicknesses as possible risk factors for central serous chorioretinopathy (CSC). METHODS: Patients with CSC and controls were evaluated with contact B-scan ultrasonography using a 20 Mhz concentric phased array ultrasound unit and enhanced depth imaging optical coherence tomography to measure the scleral thickness at the equator and posterior pole. The resultant data were evaluated using univariate analysis and generalized estimating equations. RESULTS: There were 40 patients with CSC with a mean age of 58 years and 23 controls with a mean age of 60.7 years (P = 0.31). The mean subfoveal scleral thicknesses were 1.3 mm in the CSC group and 0.86 mm in the control group (P < 0.001). The mean equatorial scleral thickness was 0.61 mm in the CSC group and 0.42 mm in the control group (P < 0.001). Using generalized estimating equations, the equatorial scleral thickness (P = 0.001), posterior scleral thickness (P < 0.001), and subfoveal choroidal thickness (P = 0.032) were independent predictors of CSC. Once these variables were entered into the equation, neither sex nor age were significant predictors. Generalized estimating equation analysis showed that equatorial, but not posterior, scleral thickness was a significant predictor of subfoveal choroidal thickness. CONCLUSION: Scleral thicknesses of the posterior and equatorial portions of the eye were found to be significant predictors of CSC, consistent with what was proposed in the theory of venous overload choroidopathy. Direct measurement by high resolution ultrasonography provides independent information about specific regions of the sclera and also avoids making speculative assumptions derived from anterior segment measurements.

Presumed retinal pericapillary astrocytic hamartoma: multimodal imaging findings of a novel hamartomatous lesion.

PURPOSE: To describe the multimodal imaging findings of retinal lesions that clinically resemble retinal astrocytic hamartomas (RAHs), but also have unique characteristics that we believe represent a novel variant. METHODS: Observational study. Five eyes in five patients with solitary retinal lesion evaluated at the retina division of three institutions. We describe the multimodal imaging findings including fundus photography, fundus autofluorescence, fluorescein angiography, spectral-domain optical coherence tomography (OCT), swept-source OCT, swept-source OCT angiography and ultrasonography. RESULTS: The retinal lesions described shared similar appearance to RAHs but demonstrated unique features such as glistening granular appearance on fundus photographs with perivascular hyperreflectivity with OCT and OCT angiography. CONCLUSION: The lesions described herein appear to have unique characteristics that warrant a designation as a novel RAH variant. The name presumed retinal pericapillary astrocytic hamartoma is suggested.

Detection of Occult Arteriovenous Malformation With Annular Array Ultrasonography.

Retinal vascular tortuosity may occur in a wide range of ocular disorders. When retinal vascular tortuosity involves both arteries and veins, and presents unilaterally and without hemorrhage, a diagnosis of Wyburn Mason syndrome (WMS) should be considered due to the potential morbidity and mortality associated with cerebral involvement. Magnetic resonance imaging (MRI) and MRI angiography (MRA) are important tools for identifying cerebral arteriovenous malformations (AVMs), but these imaging modalities have limited spatial resolution to detect very small vascular lesions. Annular array contact ocular ultrasound is a new imaging modality capable of detecting small intraorbital AVMs. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:239-243.].

PRESUMED RETINAL PIGMENT EPITHELIUM TUMOR ORIGINATING FROM UNILATERAL RETINAL PIGMENT EPITHELIUM DYSGENESIS.

PURPOSE: To describe a patient with a presumed retinal pigment epithelium (RPE) tumor originating from unilateral RPE dysgenesis. METHODS: Case report. RESULTS: A 30-year-old woman with an unremarkable medical and ocular history was referred for an evaluation of progressive central metamorphopsia in her left eye. Visual acuity was 20/20 in her right eye and 20/25 in her left eye. Funduscopic examination of the left eye revealed an elevated mass within an area of unilateral RPE dysgenesis showing hyperpigmentation and hypopigmentation with scalloped margins. Fundus autofluorescence of the lesion showed a marginal pattern of hyperautofluorescence and hypoautofluorescence that was the inverse of the fluorescein angiography pattern. A well-circumscribed subretinal mass appeared to originate from the unilateral RPE dysgenesis lesion with surrounding subretinal fluid extending beneath the fovea. Ultrasonography showed medium-to-high reflectivity of the mass with no evidence of choroidal involvement. Optical coherence tomography showed a subretinal hyporeflective mass consistent with a tumor of RPE origin. The tumor appeared to invade the overlying retina where fluorescein angiography showed hyperfluorescent leakage and OCT angiography showed retinal vascular deformation. Indocyanine green angiography showed no evidence of choroidal neovascularization. Findings in the right fundus were normal. Over a 5-month follow-up, intravitreal anti-vascular endothelial growth factor therapy induced a resolution of subretinal exudation and modest reduction in tumor thickness. CONCLUSION: To the authors' knowledge, this is the first report of a presumed RPE tumor described as originating from unilateral RPE dysgenesis. Multimodal imaging was crucial for establishing the diagnosis and showing that the patient's visual symptoms were a product of the exudation produced by the tumor's invasion of the retina.

Panuveitis With Exudative Retinal Detachments After Vaccination Against Human Papilloma Virus.

A 20-year-old white woman presented with bilateral acute visual loss (visual acuity: 20/60), panuveitis, and exudative retinal detachments 3 weeks after a second dose of quadrivalent human papillomavirus (HPV4) vaccine. She was treated with oral prednisolone for 6 weeks and responded rapidly. By week 4, vision had normalized and clinical signs resolved. Uveitis after HPV4 vaccination has been reported in two cases. Although the differential diagnosis includes Harada disease, temporal correlation with HPV4 and definitive response to a short course of treatment implicate the vaccine in this case. Vaccine-induced uveitis is rare and difficult to distinguish from coincidental autoimmune disease.

"Floater scotoma" demonstrated on spectral-domain optical coherence tomography and caused by vitreous opacification.

Patients with prominent vitreous opacities may demonstrate a characteristic shadowing on the volume-scanning protocols using spectral-domain optical coherence tomography. The authors present five cases of this characteristic shadowing artifact. This finding may be useful in objectively documenting the status of vitreous opacification and verifying the patient's complaint of symptomatic floaters, a so-called "floater scotoma".

Correlation between baseline echographic features of endophthalmitis, microbiological isolates, and visual outcomes.

BACKGROUND: The purpose of this study was to examine the baseline echographic features in culture-positive and culture-negative endophthalmitis and to correlate these echographic features with final visual outcomes. METHODS: We identified a retrospective noncomparative case series of patients with a clinical diagnosis of endophthalmitis and a baseline echographic examination between 1996 and 2010 at a single institution. Graded echographic features studied included: dense, moderate, and mild vitreous opacities; marked, moderate, and mild vitreous membranes; retinal detachment; and choroidal detachment. These were compared between culture-negative, coagulase-negative staphylococci, and other culture-positive cases of endophthalmitis, and were correlated with final visual outcomes. RESULTS: Of 149 patients reviewed, 60 were culture-negative, 26 grew coagulase-negative staphylococci, and 60 grew other culture-positive species. Three had multiple culture isolates. The presence of dense, moderate, and mild vitreous opacities, marked, moderate, and mild vitreous membranes, retinal detachment, and choroidal detachment was not significantly different between the three groups (P = 0.86, P = 0.56, P = 0.34, P = 0.45, respectively). The presence of advanced echographic features of dense vitreous opacities, marked vitreous membranes, retinal detachment, and choroidal detachment was correlated with poorer visual acuity outcomes (P = 0.005, P = 0.0001, P < 0.0001, and P < 0.0001, respectively). CONCLUSION: No correlation could be made between echographic features and the organism. The presence of advanced echographic features, such as dense vitreous opacities, marked vitreous membranes, retinal detachment, and choroidal detachment, was correlated with worse visual outcomes.

Management of intraocular foreign bodies: a clinical flight plan.

A systematic review of the literature on the diagnosis and management of traumatic intraocular foreign bodies (IOFBs) is presented together with a schematic "flight plan" to assist in clinical decision making when confronted with an IOFB. Several large retrospective series of IOFB have been published recently, with relevant observations regarding prognostic factors, endophthalmitis and retinal detachment incidence, timing of surgical intervention, and preventative measures. Eye trauma and IOFB, in particular, remain poorly suited to prospective study because of their variability. With few exceptions, retrospective observational series represent the strongest clinical evidence to guide our approach to IOFB. Synthesis of the available literature into a general guideline for management of IOFB would be helpful considering the typically hectic first few hours after such a patient presents to the ophthalmologist.

Clinical manifestations of optic pit maculopathy as demonstrated by spectral domain optical coherence tomography.

PURPOSE: The purpose of this retrospective study was to evaluate the characteristic features, including spectral-domain optical coherence tomography (SD-OCT), clinical course, and outcome of treatment if given for patients with optic disc pit maculopathy. METHODS: We investigated a consecutive series of patients with a diagnosis of optic pit maculopathy treated between 2001 and 2012 at the Bascom Palmer Eye Institute. Patients were divided into two main groups, ie, patients who were observed without surgery and patients who received surgical intervention. The main outcome measures were presenting and final visual acuity, and changes in SD-OCT imaging were recorded. Other data including age, gender, eye, age of onset, length of follow-up, location of optic pit, and location of fluid by OCT were also recorded. RESULTS: On OCT, 67% (12/18) of the eyes showed schisis-like cavities, 22% (4/18) had only subretinal fluid, and 17% (3/18) had only a schisis-like cavity without subretinal fluid. In the patients managed by observation, visual acuity was ≥20/200 in 6/8 eyes initially and 6/8 eyes at last follow-up. Ten of 18 patients received either focal laser, surgery or both. Six of 10 eyes undergoing surgery had initial visual acuity ≥ 20/200, and 8 of 10 eyes undergoing surgery had a visual acuity of ≥20/200 at last follow-up. CONCLUSION: In this study, many eyes were observed and remained stable during follow-up. In eyes with reduced vision, surgical intervention produced variable outcomes, and persistent intraretinal/subretinal fluid was a common occurrence.

Solar retinopathy: a photobiologic and geophysical analysis. 1989.

A series of young adult patients developed solar retinopathy during sun exposure over a two-day period in a particular region of the United States during March of 1986. Evaluation of the photobiological and geophysical parameters involved in solar retinitis are presented. A multifactorial pathogenesis is proposed. Of interest, a possible increase in terrestrial ultra-violet B radiation secondary to a localized relatively low ozone column during the days involved may have contributed to the retinal damage. Recommendations for protection from solar retinitis are noted.

Peripheral retinal detachments and retinal pigment epithelial atrophic tracts secondary to central serous pigment epitheliopathy. 1984.

Twenty-five patients with central serous pigment epitheliopathy (CSP), also known as central serous chorioretinopathy, have been observed to have inferior hemispheric retinal pigment epithelial atrophic tracts, presumptive of antecedent retinal detachments. Five of these patients were noted to have clinically discernible, dependent peripheral retinal detachments. The clinical and fluorescein angiographic features of these patients are reviewed. Alterations in the retina, the retinal pigment epithelium (RPE) and the choroid are also described. They include the commonly associated manifestations of CSP such as RPE leaks and macular detachment as well as some newly recognized disturbances such as retinal capillary dilatation (telangiectasia), retinal capillary leakage, retinal lipid deposition, cystoid macular edema, choriocapillaris atrophy, choroidal neovascularization and disciform scarring.

Optical coherence tomography in the diagnosis and management of diabetic macular edema: time-domain versus spectral-domain.

Optical coherence tomography (OCT) is an important imaging modality in the setting of diabetic macular edema (DME). Its use allows more precise evaluation of retinal pathology in DME, including retinal thickness and edema, vitreomacular interface abnormalities, subretinal fluid, and foveal microstructural changes. Additional advantages include its ability to quantitatively monitor response to treatment of DME by laser, intravitreal pharmacotherapies, and vitreoretinal surgery. OCT measurements are now used in all major clinical studies of DME treatment as critical endpoints. This article presents a review of both time-domain and spectral-domain OCT in the diagnosis and management of DME. The authors discuss the various parameters evaluated by the OCT systems and provide an evidence-based evaluation of their accuracy, significance, reliability, and limitations. As the capability of OCT continues to advance, it appears that its use will play an increasingly important role in the understanding, evaluation, and treatment of DME.

Bilateral choroidal neovascularization in birdshot retinochoroidopathy treated with intravitreal injections of triamcinolone and bevacizumab.

PURPOSE: To describe a patient with birdshot retinochoroidopathy (BRC) with bilateral choroidal neovascularization (CNV) who was treated with intravitreal injection of bevacizumab and antiinflammatory medications. METHOD: Interventional case report. PATIENT: A 35-year-old woman with bilateral CNV associated with BRC. RESULTS: The patient was treated with intravitreal injection of triamcinolone, photodynamic therapy, and intravitreal injection of bevacizumab in one eye, while the fellow eye was treated with intravitreal injection of triamcinolone. Immunosuppressive therapy was performed in the course of the treatment. Not only did the neovascularization respond, but the birdshot lesions vanished as well. DISCUSSION: BRC can have secondary CNV that, as in the current case, responds favorably to treatment. We serendipitously observed regression of the choroidal inflammatory lesions with intravitreal injection of triamcinolone.