Intravascular large B-cell lymphoma of the eye: Literature review and new findings.

PURPOSE: Intravascular large B-cell lymphoma (IVLBCL) is an extremely rare, aggressive, multi-system disease that can affect the eye. We describe the ophthalmic presentation, multimodal imaging and treatment response of uveal IVLBCL. METHODS: Review and case report. RESULTS: Twenty-five published cases of IVLBCL involving the eye including our own were identified. Of these, 15 patients (60%) had clinically-detectable intraocular involvement, 6 (24%) had extraocular ophthalmic involvement only and 4 (16%) had subclinical, undiagnosed intraocular involvement that was retrospectively detected on post-mortem ocular histopathology. The male to female ratio was 1.08:1 with a mean presenting age of 65.1 ± 11.7 years (range 38-82 years). The majority of cases had bilateral involvement (21/25 patients, 84%). Extraocular manifestations included diplopia, ptosis and ophthalmoplegia. Intraocular manifestations included serous retinal detachment (13/28, 46%), retinal hemorrhages (9/28, 32%), vascular changes (9/28, 32%), retinal pigment epithelial changes (7/28, 25%), thickened choroid (6/28, 21%), vitritis (5/28, 17%), cotton-wool spots (3/28, 10%), and a subretinal lesion (1/28, 3%). Histopathological diagnosis was most commonly confirmed on post-mortem enucleation (8/25 patients, 32%), skin (6/25 patients, 24%) or brain biopsy (6/25 patients, 24%). CONCLUSION: The presence of intra-retinal hemorrhages, cotton wool spots and/or Roth spots help differentiate IVLBCL from other similarly presenting diseases such as central serous chorioretinopathy and Vogt-Koyanagi-Harada disease. New signs not previously described in IVLBCL include macular bacillary layer detachment and hypo-cyanescent spots on ultra-wide field indocyanine green angiography. The diagnosis is elusive and requires tissue biopsy, but systemic chemotherapy and rituximab can lead to rapid improvement of the eye.

POLYPOIDAL LESIONS ASSOCIATED WITH CHOROIDAL NEVI.

PURPOSE: To investigate eyes with polypoidal lesions associated with choroidal nevi, their multimodal imaging characteristics, and long clinical follow-up. METHODS: Multicenter, retrospective case series study of patients with polypoidal lesions overlying choroidal nevi. Demographic and clinical information were recorded. Multimodal imaging including color fundus photography, optical coherence tomography, optical coherence tomography angiography, fundus fluorescein angiography, indocyanine angiography, and A- and B-scan ultrasonography were analyzed for nevus and polypoidal lesion characteristics. RESULTS: Fourteen eyes (14 patients; mean age: 70.3 ± 6.7 years) with polypoidal lesions overlying choroidal nevi were included. The mean follow-up duration was 50.0 ± 27.9 months (range 12-108). All nevi were pigmented on color fundus photography, flat on ultrasonography with a mean basal diameter of 3.8 ± 0.4 mm. In all but one eye, optical coherence tomography showed a shallow irregular pigment epithelium detachment overlying the nevus. A total of 11/14 eyes (78.6%) had exudative activity, 9 eyes received intravitreal anti-vascular endothelial growth factor injections, and one eye required intravitreal anti-vascular endothelial growth factor combined with photodynamic therapy. Mean visual acuity was 20/32 at baseline and 20/50 at final visit. CONCLUSION: We present the largest known cohort of eyes with polypoidal lesions associated with choroidal nevi with up to 9 years follow-up. The exudative degree of the polypoidal lesion in this condition is variable and treatment decisions should be taken on an individual basis. We hypothesize that choroidal ischemia because of altered choroidal vasculature rather than Haller layer hyperpermeability plays a role in the formation of polypoidal lesions overlying nevi.

Diabetic Retinopathy Screening at the Point of Care (DR SPOC): detecting undiagnosed and vision-threatening retinopathy by integrating portable technologies within existing services.

INTRODUCTION: The aim of this study was to determine the prevalence of diabetic retinopathy (DR) in a low socioeconomic region of a high-income country, as well as determine the diagnostic utility of point-of-care screening for high-risk populations in tertiary care settings. RESEARCH DESIGN AND METHODS: This was a cross-sectional study of patients with diabetes attending foot ulcer or integrated care diabetes clinics at two Western Sydney hospitals (n=273). DR was assessed using portable, two-field, non-mydriatic fundus photography and combined electroretinogram/ pupillometry (ERG). With mydriatic photographs used as the reference standard, sensitivity and specificity of the devices were determined. Prevalence of DR and vision-threatening diabetic retinopathy (VTDR) were reported, with multivariate logistic regression used to identify predictors of DR. RESULTS: Among 273 patients, 39.6% had any DR, while 15.8% had VTDR, of whom 59.3% and 62.8% were previously undiagnosed, respectively. Non-mydriatic photography demonstrated 20.2% sensitivity and 99.5% specificity for any DR, with a 56.7% screening failure rate. Meanwhile, mydriatic photography produced high-quality images with a 7.6% failure rate. ERG demonstrated 72.5% sensitivity and 70.1% specificity, with a 15.0% failure rate. The RETeval ERG was noted to have an optimal DR cut-off score at 22. Multivariate logistic regression identified an eGFR of ≤29 mL/min/1.73 m2, HbA1c of ≥7.0%, pupil size of <4 mm diameter, diabetes duration of 5-24 years and RETeval score of ≥22 as strong predictors of DR. CONCLUSION: There is a high prevalence of vision-threatening and undiagnosed DR among patients attending high-risk tertiary clinics in Western Sydney. Point-of-care DR screening using portable, mydriatic photography demonstrates potential as a model of care which is easily accessible, targeted for high-risk populations and substantially enhances DR detection.

PIGMENTATION IN FOCAL SCLERAL NODULE.

PURPOSE: To report a case of pigmentation in focal scleral nodule (FSN). METHODS: This is a single case report. RESULT: An asymptomatic 61-year-old woman was referred with small, partially pigmented raised lesion located at the superior margin of the optic nerve head. The lesion's clinical and multimodal imaging features were consistent for FSN including a dome-shaped elevation confined to the sclera with overlying choroidal thinning. However, the pigmentation within our lesion is a novel finding in FSN that has not been described before. CONCLUSION: To our knowledge, we report the first case of pigmentation in FSN. It is likely that our case was a typical FSN that then became pigmented, with melanosomes involving the flanges of the lesion where thin choroid remains. The understanding that FSN can be partially pigmented may eventually help unravel the origins of this poorly understood lesion.

COVID-19 reduced scleral buckling training in fellows and shifted young ophthalmologists' preference toward vitrectomy: An Asia-Pacific survey.

BACKGROUND: To assess rhegmatogenous retinal detachment (RRD) surgery trends and training among young ophthalmologists (YOs, vitreoretinal fellows or attendings/consultants with ≤10 years of independent practice) and the impact of the COVID-19 pandemic. METHODS: An anonymous online survey was completed by 117 YOs in the Asia-Pacific regarding their RRD surgery experiences in 2021-2022. RESULTS: To achieve a 90% probability of surgical competency, 91 vitrectomy and 34 scleral buckling (SB) completions during fellowship were needed. In total, 49 (41.9%) YOs had fellowship affected by COVID-19. In the COVID versus pre-COVID era, however, the volume of SB completions per fellowship year decreased significantly (median [IQR] 3.3 [1.5, 9] vs. 13 [6.5, 23]; p < 0.001) and was lower than the required volume to achieve competency. YOs were less confident in conducting SB versus vitrectomy (3.5 ± 1.1 vs. 4.2 ± 0.8, p < 0.001), and they reported a decrease in the proportion of SB (-3.1%, p = 0.047) and an increase in the proportion of vitrectomy (+4.8%, p < 0.001) after the pandemic outbreak. Apart from RRD clinical characteristics, surgical confidence is among the main factors that affect surgical method decisions. During the pandemic, more YOs may have avoided SB due to the need for general anaesthesia, leading to longer surgical time and risk of viral transmission during intubation/extubation. CONCLUSIONS: SB surgical exposure is suboptimal in most fellowship programs in the 11 Asia-Pacific countries/regions we surveyed and further declined during the COVID-19 pandemic. YOs are less confident in performing SB, leading to a trend toward primary vitrectomy since the COVID-19 outbreak.

22q11 MICRODELETION (DIGEORGE) SYNDROME WITH MICROVASCULAR MACULOPATHY.

PURPOSE: To describe a novel retinal finding of 22q11 microdeletion syndrome. METHODS: Retrospective chart review of a single patient. RESULTS: A 32-year-old man with genetically confirmed 22q11.2 microdeletion syndrome was found to have bilateral tortuous retinal vessels and right microvascular microangiopathy with microaneurysms, hard exudate, and cystoid macular oedema. Other underlying causes for this including diabetic and hypertensive retinopathy were excluded. No treatment was required because he was asymptomatic, and the visual acuity remained 20/30 in that eye with over one year of follow-up. CONCLUSION: 22q11 microdeletion syndrome can be associated with microvascular microangiopathy.

Central serous chorioretinopathy: A review.

Central serous chorioretinopathy (CSC) is the fourth most common non-surgical retinopathy associated with fluid leakage. The pathogenesis is not yet completely understood, but changes in the choroid, sclera and RPE have been described associated with venous congestion of choroidal outflow. CSC can be categorised into acute, chronic, and recurrent subtypes with recent classifications of simple and complex based on the area of RPE change seen on fundus autofluorescence. A multimodal imaging approach is helpful in the diagnosis and management of CSC and secondary complications such as type 1 neovascularisation. Although spontaneous resolution with relatively good visual outcomes is common, treatment should be considered in patients with persistent or recurrent SRF. Treatment options include laser, systemic medications, intravitreal therapy, and surgery. Of these, argon laser for focal extramacular fluid leaks and photodynamic therapy of leakage identified by indocyanine-green angiography currently have the greatest supportive evidence.

Choroidal Lymphoma: Diagnostic Value of Combined Indocyanine Green Angiography and Optical Coherence Tomography.

OBJECTIVE: To compare multimodal imaging findings in patients with choroidal lymphoma (CL). METHODS: Multicenter retrospective observational case series. Multimodal imaging features of patients with CL were reviewed with particular attention to the patterns of choroidal infiltration on indocyanine green angiography (ICGA) and optical coherence tomography (OCT). RESULTS: Eighteen eyes of 15 patients were included in this study. Average tumor thickness on ultrasonography was 2.6 mm (range, 1.2-5.7 mm). Choroidal infiltration on ICGA was characterized by multifocal, round areas (300-500 microns diameter) of hypocyanescence in all cases, whereas OCT at the same region disclosed diffuse choroidal infiltration. By OCT, the tumor surface contour was primarily placid (22%), dome-shaped (11%), or undulating (67%). CONCLUSIONS: In this analysis of eyes with CL, ICGA demonstrated multifocal sub-millimeter regions of choroidal hypocyanescence whereas OCT documented diffuse choroidal infiltration. This incongruence could be a distinctive diagnostic feature of choroidal lymphoma, assisting with differentiation from other pathological entities.

Acute zonal occult outer retinopathy-like presentation secondary to scleral buckle.

Purpose: To describe a case of acute zonal occult outer retinopathy-like (AZOOR-like) presentation following scleral buckle surgery for rhegmatogenous retinal detachment. Observations: A 48-year-old man underwent successful scleral buckle with cryotherapy for repair of a left eye inferior macula-on rhegmatogenous retinal detachment. Five years later he presented with a six-month history of left peripheral field restriction. Fundus autofluorescence and optical coherence tomography demonstrated degeneration of the photoreceptors in a ring pattern around the left macula. Humphrey visual fields showed functional loss corelating with the imaging, with a paracentral ring scotoma. Electrophysiology demonstrated a delayed 30 Hz flicker latency in the left eye confirming cone system dysfunction. Conclusion and Importance: Scleral buckling surgery for repair of a rhegmatogenous retinal detachment may be associated with a late AZOOR-like presentation.

Subretinal peripapillary biopsy-proven sarcoidosis: a case report.

BACKGROUND: To report a case of a subretinal, unilateral, peripapillary granuloma that was diagnosed as sarcoidosis by a 27-gauge pars plana vitrectomy subretinal biopsy. Sarcoidosis is a chronic idiopathic granulomatous inflammatory disease, that has ocular involvement in 10-80% of patients. It is often mistaken for many other primary ocular diseases because the condition can involve any structure in or around the eye. Previous case reports of peripapillary sarcoidosis have either been limited to the choroid or presented with additional ocular and systemic signs, hence have not required an intraocular biopsy. CASE PRESENTATION: A 54-year-old Filipino male presented with a 6-month history of painless blurred vision in his right eye. Fundus examination revealed a large white peripapillary lesion. Enhanced-depth imaging optical coherence tomography confirmed the subretinal location of the mass. Indocyanine green angiography demonstrated absolute hypofluorescent blockage with satellite lesions. A whole-body positron emission tomography scan demonstrated widespread lymphadenopathy, but investigations including an inguinal lymph node biopsy were inconclusive. Following growth of the peripapillary lesion and worsening vision, a 27-gauge pars plana vitrectomy subretinal biopsy was performed which confirmed sarcoidosis. He was treated with oral corticosteroids and transitioned to long term immunotherapy with methotrexate. CONCLUSIONS: Sarcoidosis can present in the subretinal space, around the optic nerve without other ocular findings.

Polypoidal Choroidal Vasculopathy Based on Non-ICGA Criteria in White Patients With Neovascular Age-Related Macular Degeneration.

PURPOSE: To determine prevalence of probable polypoidal choroidal vasculopathy (PCV) among White patients with neovascular age-related macular degeneration (nAMD) using non-indocyanine green angiography (ICGA) criteria DESIGN: Multicenter, multinational, retrospective, cross-sectional study. METHODS: A total of 208 treatment-naive eyes from Hispanic and non-Hispanic White individuals diagnosed with nAMD were included. All underwent color fundus photography (CFP), optical coherence tomography (OCT), and fluorescein angiography (FFA). De-identified images of study eyes were sent to 2 groups of graders. Group 1 reviewed CFP, OCT, and FFA to confirm nAMD diagnosis. Group 2 reviewed CFP and OCT to determine highly suggestive features for PCV. Probable PCV diagnosis defined as the presence of ≥2 of 4 highly suggestive features for PCV: notched or fibrovascular pigment epithelial detachment (PED) on CFP, sharply-peaked PED, notched PED, and hyperreflective ring on OCT. RESULTS: Eleven eyes were excluded because of poor image quality (6) or non-nAMD diagnosis (5). Of 197 eligible eyes (197 patients), the mean age (SD) was 78.8 years (8.9), 44.2% were men, 26.4% were Hispanic, and 73.6% were non-Hispanic White individuals; 41.1%, 23.4%, 9.1%, and 2.5% had ≥1, ≥2, ≥3, and 4 highly suggestive features. Results showed that 23.4% (95% CI, 17.6%-29.9%) had probable PCV diagnosis. Predominantly occult CNV was more frequently found in probable PCV than nAMD subgroup (84.8% vs 64.9%, P = .01). Hispanic White individuals had a lower prevalence of probable PCV than non-Hispanic White individuals (9.6% vs 28.2%, P = .006) CONCLUSIONS: These findings suggest that probable PCV occurs between 17.6% and 29.9% in White individuals with nAMD, and more commonly in non-Hispanic than in Hispanic White individuals.

Cryotherapy versus Laser Does Not Influence Anatomic Success after Vitrectomy for Primary Rhegmatogenous Retinal Detachment Repair: Registry Analysis of 2413 Patients.

PURPOSE: To determine whether a difference exists between cryotherapy and endolaser photocoagulation retinopexy in terms of the rates of anatomic success after primary rhegmatogenous retinal detachment (RRD) repair with pars plana vitrectomy (PPV). DESIGN: Registry-based, nonrandomized, observational cohort study. SUBJECTS: Eyes with primary RRD in a binational retinal surgery registry that underwent repair with PPV and had a minimum of 3 months of follow-up. METHODS: Cases were recorded prospectively by participating surgeons, and the outcomes were recorded at 3 months. A generalized mixed model approach was used to compare the effect of retinopexy type on the outcomes. Variables known to be associated with retinal surgical success were considered as covariates for the final model. The eyes were divided into 2 groups: Cryo group (receiving cryotherapy as the only form of retinopexy) and Laser group (receiving endolaser as the only form of retinopexy). MAIN OUTCOME MEASURES: The primary endpoint was the proportion of patients with stable retinal reattachment without the need for further retinal detachment surgery, assessed 3 months after the surgery. Failure was defined as either retinal redetachment or foveal attachment with long-term silicone oil tamponade at the endpoint. The secondary outcome measured was visual acuity at 3 months compared with baseline measurements. RESULTS: A total of 2413 patients were included, and the overall single-procedure success rate was 85%. There was no statistically significant difference in surgical success between the adjusted proportion of successful reattachment for the Cryo group (87%) and that of successful reattachment for the Laser group (82%) (P = 0.84; odds ratio, 1.04; 95% confidence interval, 0.74-1.46). There was no difference in the mean change in the visual outcomes at 3 months between the groups (adjusted mean change of -0.48 logarithm of the minimum angle of resolution [logMAR] for the Cryo group vs. -0.50 logMAR for the Laser group, P = 0.82). CONCLUSIONS: The choice of cryotherapy versus endolaser retinopexy was observed to influence neither the anatomic success of PPV for RRD nor the visual acuity outcomes at 3 months after the surgery.

Relentlessly Progressive Sweet Syndrome of the Eye with Scleritis and Choroidal Infiltration.

PURPOSE: To describe a case of Sweet syndrome, a dermatologic inflammatory disease, with progressive, unrelenting ocular findings. METHODS: Case report. RESULTS: A 73-year-old male was evaluated with a six-month history of Sweet syndrome, manifesting as cutaneous erythematous edematous papules on the dorsal arms and shins and confirmed with biopsy demonstrating neutrophil infiltration with nuclei fragmentation and lack of vasculitis. He initially noted a unilateral red eye with ocular pain and was found to have scleritis and choroidal infiltration. The patient's ocular disease progressed despite treatment with systemic corticosteroids, intraocular Ozurdex ®, systemic dapsone, and subtenons triamcinolone. Systemic evaluation was negative for malignancy or other inflammatory syndromes. Following 7 months of non-manageable ocular pain enucleation was offered to the patient, but he declined. CONCLUSION: Sweet syndrome, a dermatologic condition, can be associated with unilateral scleritis and choroidal infiltration that are relentlessly progressive despite maximal systemic and ocular corticosteroid therapy.

Implantation and long-term assessment of the stability and biocompatibility of a novel 98 channel suprachoroidal visual prosthesis in sheep.

Severe visual impairment can result from retinal degenerative diseases such as retinitis pigmentosa, which lead to photoreceptor cell death. These pathologies result in extensive neural and glial remodelling, with survival of excitable retinal neurons that can be electrically stimulated to elicit visual percepts and restore a form of useful vision. The Phoenix99 Bionic Eye is a fully implantable visual prosthesis, designed to stimulate the retina from the suprachoroidal space. In the current study, nine passive devices were implanted in an ovine model from two days to three months. The impact of the intervention and implant stability were assessed using indirect ophthalmoscopy, infrared imaging, and optical coherence tomography to establish the safety profile of the surgery and the device. The biocompatibility of the device was evaluated using histopathological analysis of the tissue surrounding the electrode array, with a focus on the health of the retinal cells required to convey signals to the brain. Appropriate stability of the electrode array was demonstrated, and histological analysis shows that the fibrotic and inflammatory response to the array was mild. Promising evidence of the safety and potential of the Phoenix99 Bionic Eye to restore a sense of vision to the severely visually impaired was obtained.

Missing Internal Limiting Membrane during Macular Hole Repair in Alport Syndrome.

The aim of this manuscript is to describe a novel retinal finding of Alport syndrome during surgical management of an associated macular hole. A retrospective chart review of a 65-year-old man with a diagnosis of Alport syndrome confirmed by renal biopsy was found to have an associated full-thickness macular hole. Pars-plana vitrectomy surgery with internal limiting membrane (ILM) peeling was attempted, but intraoperatively the ILM was found to be absent at the macula. Alport syndrome may be associated with the absence of the ILM. This can complicate attempts at macular hole repair.