Comprehensive overview of IRVAN syndrome: a structured review of Case Reports and Case Series.

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare spectrum of retinal vasculitis, aneurysms, and neuroretinitis affecting young individuals in their third decade. Most of our current knowledge is based on case reports, case series, and a handful of collaborative studies. There is much diversity in treatment approaches and outcomes in the reported literature. We have aggregated published case reports and case series into quantitative and narrative synthesis to draw evidence-based conclusions toward clinical features, atypical and rare findings, systemic associations, disease course, and treatment outcomes. The analysis suggested the disease mostly affects young individuals with a female predilection. Anterior chamber and vitreous inflammation are common than previously believed. The most prevalent pattern of retinal vasculitis in IRVAN eyes is mixed vasculitis, followed by arteritis and phlebitis. Most eyes at the time of presentation have capillary nonperfusion and require treatment. Most eyes retain good visual acuity; however, treatment is required to maintain visual function. Intravitreal antivascular endothelial growth factors administered as an adjunct to retinal laser photocoagulation are more likely to improve visual outcomes. Besides, we have discussed the different hypotheses on the etiopathogenesis of the disease and stronger evidence suggests an inflammatory origin of the disease.

Optical coherence tomography angiography of retinal vasculature in recovered COVID-19 patients compared to age and ethnic matched controls.

CLINICAL RELEVANCE: Understanding the impact of the COVID-19 virus on the retinochoroidal vasculature can provide valuable information regarding potential multi-organ ischaemic sequelae in COVID-19 patients, and can thus be a useful tool for optometrists, ophthalmologists, pulmonologists, infectious disease specialists and others. BACKGROUND: Assessment of retinochoroidal vasculature alterations in recovered mild COVID-19 patients using optical coherence tomography angiography (OCTA) when compared to age and ethnic matched controls. METHODS: Multimodal imaging was performed using OCTA, spectral domain (SD)-OCT (Optovue RTVue XR Avanti; Optovue, Inc, Fremont, CA), and colour fundus photography (Compass; iCare Inc, Raleigh, NC). Vessel flow density, foveal avascular zone, foveal perimeter circumference and retinal thickness were calculated automatically by the OCTA software on 6 × 6mm angiograms. Morphologic changes in the retinochoroidal vasculature on OCTA were assessed and compared with the findings on fundoscopy, SD-OCT and fundus photography and were evaluated by two trained graders. RESULTS: Mean vessel parafoveal density, superior and inferior hemispheric vessel density and perifoveal temporal vessel density on 6 × 6 angiograms of the superficial capillary plexus were lower among the COVID-19 patients when compared to their age and ethnic matched controls. Vessel flow density of the deep capillary plexus, foveal avascular zone size and circumference and retinal thickness did not illustrate statistical significance between the groups. CONCLUSION: OCTA provides non-invasive high-resolution imaging of the retinochoroidal vascular network. Compared with conventional imaging, OCTA can demonstrate precise microvascular structural alterations in the retinal vessels before visible on SD-OCT or fundus examination. When matched for age and ethnicity, patients with a history of mild COVID illness manifested alterations in vessel density.

Case Report: Use of Multimodal Imaging to Document a Rare Complication of Torpedo Maculopathy.

SIGNIFICANCE: This study demonstrates the importance of routine multimodal imaging for the diagnosis of choroidal neovascular membrane secondary to torpedo maculopathy. CASE REPORT: A 63-year-old woman with a history of torpedo maculopathy presented with recent-onset symptoms of visual distortion in the right eye. Multimodal imaging modalities revealed the presence of a choroidal neovascular membrane. The choroidal neovascular membrane was treated, and the patient was subsequently followed up to monitor the retinal appearance over time. Significant structural restoration of the macular anatomy with overall functional improvement was seen. CONCLUSIONS: Although torpedo maculopathy is often a benign diagnosis, complications such as choroidal neovascular membrane can occur. Choroidal neovascular membrane formation is well documented in younger patients with torpedo maculopathy; however, we present a rare case of choroidal neovascular membrane in an elderly individual. Multimodal imaging proved instrumental in the early detection and management of this infrequently reported complication.

Case Report: Optical Coherence Tomography Angiography of Idiopathic Foveal Hypoplasia and Its Correlation With Visual Acuity.

SIGNIFICANCE: Foveal hypoplasia is described clinically by the absence of a foveal pit and subsequent reduction in visual acuity. Optical coherence tomography angiography provides precise segmentation of the retinal vascular supply demonstrating the vascular perfusion in affected patients. Preservation of perfusion is linked to visual acuity and function. PURPOSE: This case report describes a patient with foveal hypoplasia and preservation of visual acuity with preserved retinal capillary density of the superficial and deep capillary plexuses on optical coherence tomography angiography. In addition, the diagnostic findings of foveal hypoplasia as seen on optical coherence tomography angiography will be described. CASE REPORT: A 25-year-old Caucasian female with history of foveal hypoplasia presented to the clinic for evaluation. She had no other visual, ocular, or systemic complaints. Her ocular history included Duane syndrome, accommodative insufficiency, and traumatic brain injury. Her medical history included cardiac ablation secondary to supraventricular tachycardia, gall bladder removal, maxillary sinus cyst, and a history of migraines. Best-corrected visual acuity was 20/15 in the right and left eyes. Funduscopic examination was unremarkable. Spectral domain optical coherence tomography revealed absence of the anatomical foveal pit with normal inner retinal morphology. Optical coherence tomography angiography confirmed a decreased foveal avascular zone; however, a vascular density analysis showed normal perfusion to the inner retinal plexuses. CONCLUSIONS: Optical coherence tomography angiography is a rapid, noninvasive imaging modality that provides excellent insight into the microvasculature supply to the retina and choroid. As such, it allows for an in-depth analysis into the pathophysiology behind certain conditions such as foveal hypoplasia.

PREVALENCE OF VITREOMACULAR ADHESION IN PATIENTS WITHOUT MACULOPATHY OLDER THAN 40 YEARS.

PURPOSE: To determine the prevalence and factors influencing vitreomacular adhesion (VMA) or vitreomacular traction (VMT) in subjects without maculopathy older than age 40 years. METHODS: In a prospective cross-sectional study, 1,950 eyes in 1,090 participants aged 40 to 89 years representing various ethnic groups from 14 centers in the United States underwent a comprehensive eye examination, including spectral domain optical coherence tomography. A team of independent, masked readers classified the presence or absence of VMA/VMT on spectral domain optical coherence tomography based on the International Vitreomacular Traction Study Group rubric. RESULTS: Across all eyes, the prevalence of VMA or VMT was 39% or 1%, respectively. For every 1-year increase in age, there was a statistically significant 7% decreased odds of having VMA or VMT (95% confidence interval [CI]: 0.89-0.96; P < 0.001), whereas African Americans had 55% significantly reduced odds of having VMA or VMT when than whites (95% CI: 0.23-0.90; P = 0.025). Vitreomacular adhesion >1,500 µm was significantly more likely than VMA <1,500 µm in younger adults (95% CI: 0.70-0.86; P < 0.001), hyperopes versus emmetropes (95% CI: 1.49-35.9; P = 0.01), primary eye care versus tertiary practices (95% CI: 0.03-0.92; P = 0.04), and patients without hyperlipidemia (95% CI: 0.04-0.83; P = 0.03). CONCLUSION: Vitreomacular adhesion is highly prevalent among middle-aged adults. Diagnostic screening with spectral domain optical coherence tomography may help to accurately detect VMA or VMT, prompting routine monitoring and timely therapeutic intervention.

More sensitive correlation of afferent pupillary defect with ganglion cell complex.

PURPOSE: This study investigated the correlation between the relative afferent pupillary defect (RAPD) and retinal nerve fiber layer thickness (RNFLT) in optic neuropathy. METHODS: RAPD assessment was performed using a log unit neutral density filter bar. Spectral domain optical coherence tomography RTVue-100 (Optovue) was used to examine the subjects. The optic nerve head pattern (ONH) was subdivided and identified for the purpose of the study into circumpapillary RNFLT (cpRNFLT) and peripheral circumpapillary RNFLT (pcpRNFLT). The cpRNFLT, pcpRNFLT and ganglion cell complex (GCC) parameters were analyzed. RESULTS: Eighteen females and twenty three males with asymmetric optic neuropathy and a RAPD participated. Thirty-three subjects had glaucoma and eight had optic neuropathy other than glaucoma. Significant correlations (p<0.02) were obtained for the RAPD and the percentage difference loss of the GCC and RNFLT parameters. The grouped mean percentage difference loss for RNFLT was significantly different from that of the GCC (p<0.001). At a 0.6log unit RAPD, the average mean percentage difference loss was 23% for the CRNFLT, 15% for the GCC, 12% for the global loss volume percentage and 6% for the focal loss volume percentage (FLV%). CONCLUSIONS: Significant correlations between RNFLT loss for cpRNFLT, pcpRNFLT and GCC parameters with RAPD were observed. Approximately a 35% higher sensitivity was obtained using GCC compared to CRNFL parameters. The expected change in GCC average for every 0.3log unit increment was approximately 8.49µm. The FLV% corresponded more sensitively to a RAPD but appeared to be influenced by disease severity.

Outer Retinal Tubulation: A Case Series.

PURPOSE: The advent of spectral domain optical coherence tomography has led to superb imaging capabilities in addition to enhanced visualization of the retinal layers. Such advancements have led to the identification of a variety of new retinal conditions, including outer retinal tubulations (ORTs). ORTs are ovoid hyporeflective spaces located in the outer retina. The pathogenesis is unclear but seems to involve sublethal injury to the photoreceptors leading to a compensatory reorganization of the photoreceptor layer with the neighboring ellipsoid zone resulting in a hyperreflective border surrounding a central lumen. Most ORTs have been linked to wet age-related macular degeneration; however, these peculiar structures are now seen in a myriad of retinal disorders. CASE REPORTS: Our cases will highlight the wide variety of clinical presentations associated with outer retinal tubulations. The clinical presentations include two cases of wet age-related macular degeneration, a case of presumed ocular histoplasmosis syndrome, a case of central areolar choroidal dystrophy, and a case of pathological myopia. CONCLUSIONS: By correctly differentiating outer retinal tubulations from other masqueraders, unnecessary referrals and interventions can be minimized. Understanding the various disease entities associated with outer retinal tubulation could give further insight into the mechanism and formation of these structures.

Variability in Presentation of Bilateral Vitreomacular Traction.

PURPOSE: Vitreomacular traction (VMT) is a condition characterized by an incomplete posterior vitreous detachment resulting in traction on the macula and possible subsequent decrease in visual acuity and/or other symptomatology. Vitreomacular traction often presents as a unilateral condition, with some cases developing bilaterality with disease progression. The natural course and presentation of VMT vary widely among individuals. There is no extensive literature regarding the prevalence of VMT in the general population; therefore, little is known on the laterality. CASE REPORTS: We present eight cases of bilateral VMT encountered in a primary care setting. These cases highlight the variability in presentation, symptomatology, and clinical outcome between and within eyes of patients with bilateral VMT. CONCLUSIONS: Spectral-domain optical coherence tomography has provided new insight into VMT by allowing better visualization of the vitreoretinal interface. Using spectral-domain optical coherence tomography, eight unique cases of bilateral VMT were confirmed and classified. However, despite the bilaterality noted in each case, the clinical presentation and course of disease varied among patients. Because of the paucity of epidemiological data regarding VMT, the laterality of the condition, risk factors for involvement, and overall prognosis remain unclear.

The "double-layer sign": in vivo imaging of polypoidal choroidal vasculopathy.

PURPOSE: Polypoidal choroidal vasculopathy (PCV), a disease of the choroidal vascular network, presents clinically with polyp-like reddish orange lesions, as well as retinal pigment epithelial (RPE) and/or neurosensory retinal detachments. Optical coherence tomography (OCT) is a useful diagnostic tool that provides cross-sectional and volumetric imaging of posterior segment microstructure. Typical OCT findings in PCV may reveal a classic hyperreflectivity in the choroidal layers known as the "double-layer" sign. This sign is indicative of an abnormal choroidal vascular network. Indocyanine green angiography is also useful for the diagnosis of PCV and clearly identifies the polyp-like lesions arising from the choroidal vasculature. CASE REPORT: A 53-year-old African American woman presented with complaints of gradually decreasing vision in the left eye (OS). Clinical examination revealed moderately reduced acuity OS with a small central scotoma found on Amsler grid testing. Dilated fundus examination revealed a hemorrhagic RPE detachment with suspicion of a choroidal neovascular membrane in the foveal region of the OS. Exudative leakage appeared circumferentially around the macula OS. Optical coherence tomography of the OS also revealed two highly reflective layers: one at the level of the RPE and another beneath the RPE ("double-layer" sign). The patient was referred to a retina subspecialist for angiography and treatment consideration. CONCLUSIONS: It is important to distinguish PCV from other variants of choroidal neovascularization. The polyp-like lesions found in the PCV are a unique and classic characteristic. Proper diagnostic workup includes stereoscopic posterior fundus evaluation and imaging studies, including OCT and indocyanine green angiography.

Intralenticular neovascularization in a cataractous crystalline lens.

BACKGROUND: Neovascularization can occur in various ocular structures including the retina, iris, anterior chamber angle and cornea; however, it rarely occurs in the crystalline lens. Neovascularization results secondary to hypoxic conditions within the eye. A natural balance of angiogenic and antiangiogenic factors are critical for the eye to prevent the formation of neovascularization. Various factors can upset this natural balance, resulting in angiogenesis. Due to the lack of an intrinsic blood supply, intralenticular neovascularization is rare. CASE REPORT: A 61-year-old black male presented with a previous diagnosis of proliferative diabetic retinopathy (PDR). His ocular history included cataract extraction in the left eye (OS) and tractional retinal detachment in both eyes that was surgically repaired OS. His entering visual acuities were NLP OD and 20/400 OS. Upon biomicroscopic evaluation OD, there was evidence of a hypermature cataract with iris neovascularization and neovascularization coursing over and within the anterior capsule of the lens. CONCLUSIONS: Intralenticular neovascularization is a rarely reported ocular complication. We present a patient with uncontrolled diabetes resulting in proliferative diabetic retinopathy, extreme ocular ischemia, and consequent intralenticular neovascularization.

Serpiginous choroiditis in a herpes-positive patient.

BACKGROUND: Viruses are one of the most common causes of infections involving the posterior segment of the eye. Viral infections can be congenital or acquired and can affect the retina, choroid, or optic nerve. Herpes simplex virus has been implicated in a number of posterior segment conditions, including serpiginous choroiditis (SC), which has generally been described as idiopathic. CASE REPORT: A 57-year-old black female presented with decreased and distorted vision in both eyes, of 10 months duration. Funduscopy revealed radial, deep grayish lesions emanating off of the optic disc in a peripapillary fashion. The clinical appearance was suggestive of SC, which was confirmed by fluorescein angiography. Serologic testing was positive for herpes simplex virus and thus may suggest a causative link between the virus and the choroiditis. CONCLUSIONS: SC has been historically cited as an idiopathic process. There have been only rare reports linking this process with a viral etiology. Laboratory testing and clinical work-up needs to be obtained in any patient suspected of having SC, to detect a viral etiology. Treatment of an underlying condition may lead to optimum resolution in these patients.

Cystoid macular edema as a result of immune-recovery uveitis.

BACKGROUND: Potent antiretroviral therapy can lead to improved immunity in patients with acquired immune deficiency syndrome. However, ocular inflammation can occur in predisposed individuals. This inflammation is referred to as immune-recovery uveitis. Cystoid macular edema (CME) is a complication that can result from this inflammation and is emerging as a major cause of visual loss in human immunodeficiency virus (HIV)-infected patients. CASE REPORT: A 52-year-old man presented with complaints of intermittent decreased vision that had coincided with the initiation of antiviral treatment. He also reported metamorphopsia and floaters in both eyes of several years duration. He had a history of HIV for 20 years and was only recently started on Highly Active Antiretroviral Therapy (HAART). He reported a vague history of ocular problems involving his retina. Retinal examination revealed bilateral areas of peripheral scarring from presumed past ocular inflammation and thickening and irregularity at each macula. Ancillary testing was performed, and CME was diagnosed. CONCLUSIONS: Immune-recovery uveitis is a leading cause of visual disturbance in HIV-infected patients with a history of cytomegalovirus (CMV) retinitis on HAART. Although the immune recovery associated with the advent of HAART has decreased the need for potent CMV medications, the heightened immune response can be associated with sight-threatening inflammation.

Optical coherence tomography in adult-onset vitelliform dystrophy.

BACKGROUND: Adult-onset vitelliform dystrophy (AOVD) is as a bilateral macular dystrophy that presents as a subretinal, oval, or round yellowish deposition in the macula. The lesions may be elevated and measure approximately 1/3 to 1 disc diameter (DD) in size. The dystrophy usually manifests between 30 and 50 years of age and has been genetically linked. Features of AOVD have been evaluated by optical coherence tomography (OCT). OCT examination shows subfoveal hyperreflective lesions lying on the retinal pigment epithelium (RPE) with slight separation between the RPE and photoreceptor layers. OCT is a diagnostic tool that provides insight into the morphologic characteristics of AOVD. CASE REPORT: A 67-year-old black woman presented with complaints of decreased vision and metamorphopsia in her right eye of 1 year's duration. The patient's ocular history was positive for "macular degeneration" for which she was given eye vitamins. The patient's best-corrected visual acuities were 20/30 in the right eye and 20/20 in the left eye. Fundus examination found bilateral, subfoveal lesions. Optical coherence tomography demonstrated an elevated, well-circumscribed area of hyperreflectivity within the RPE in each eye. This description is that of a pattern-dystrophy of the RPE, namely, adult-onset vitelliform dystrophy. CONCLUSION: OCT is a noninvasive instrument that provides information on the morphology of AOVD. It not only isolates the location of the lesion but can be utilized to monitor the stability of the condition. OCT interpretation of macular lesions can aid in diagnosis and management.

Ocular perineuritis secondary to neurosyphilis.

BACKGROUND: Syphilis is a condition caused by the spirochete Treponema pallidum (T. pallidum). Because of its ubiquitous nature, it has been coined as "the great masquerader." Syphilis can present with a wide array of clinical symptoms and manifestations, making it difficult to diagnose. The spectrum of visual system manifestations is broad and involves the anterior and posterior segments. We report a rare case of ocular perineuritis in an HIV-positive patient with neurosyphilis. CASE REPORT: A 33-year-old HIV-positive white male presented with blurred vision and floaters of 2 weeks duration. He reported coinciding neurological symptoms including headaches, nausea, and extreme weight loss. He also manifested an erythematous rash on his scalp. Fundoscopy revealed bilateral disc edema. CONCLUSIONS: Syphilitic ocular perineuritis is a condition that manifests with swollen optic discs in the absence of raised intracranial pressure or visual disturbance. This condition is due to inflammation of the optic nerve sheath and is often mistaken for papilledema or papillitis. Optic perineuritis is an infrequently reported complication of syphilitic disease. Early diagnosis of neurosyphilis and its complications is critical, because it is easily treated with penicillin when initiated early on.