Successful treatments of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome-related macular edema and exudation by intravitreal aflibercept injection in a senile patient: A case report.

RATIONALE: The prevalence of idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is <1/1,000,000, and it tends to occur in young women. Panretinal photocoagulation, focal laser photocoagulation, peripheral cryopexy, vitrectomy, and corticosteroid therapy are the traditional treatments. We reported a case of a senile patient with IRVAN syndrome who presented with severe exudation in both eyes and macular edema in the left eye, successfully treated by serial intravitreal aflibercept injections. PATIENT CONCERNS: A 77-year-old Taiwanese woman complained of progressive blurred vision in the left eye and ocular examinations revealed a visual acuity of 20/125 in the left eye. DIAGNOSIS: Indirect fundoscopy and fluorescein angiography showed bilateral multiple aneurysms, vasculitis, optic nerve staining, and neovascularizations. In addition, optical coherence tomography demonstrated macular edema with subretinal fluid and exudations in the left eye. INTERVENTIONS: Monthly intravitreal injections of antivascular endothelial growth factor with aflibercept 2.0 mg were administered in the left eye. OUTCOMES: The visual acuity in the left eye improved to 20/50 after 18 months of treatment. Macular edema and subretinal fluid regressed. Furthermore, vessel leakage and optic disc staining also improved. LESSONS: This case is the first to demonstrate successful treatment of IRVAN syndrome-related central macular edema and exudations, in the absence of neovascularization, using a series of intravitreal aflibercept injections as monotherapy.

Sea-fan retinal neovascularization associated with rickettsial retinitis: a case report.

BACKGROUND: Rickettsial disease has been commonly associated with retinitis, retinal vasculitis, and optic nerve involvement, but the development of retinal neovascularization has been very rarely reported. We herein describe a case of rickettsial retinitis complicated with the development of sea-fan retinal neovascularization documented with multimodal imaging, including fundus photography, SS-OCT, fluorescein angiography, and SS-OCT angiography. CASE PRESENTATION: A 26-year-old female with a history of fever one week earlier presented with sudden decreased vision in the left eye. Best-corrected visual acuity (BCVA) was 20/2000 and the patient was diagnosed with rickettsial retinitis along the superotemporal retinal vascular arcade associated with serous retinal detachment and retinal hard exudates. The indirect immunofluorescence test was positive for Rickettsia conorii, and the patient was treated with oral doxycycline (200 mg/day) and oral prednisone (0.75 mg/kg/day, with gradual tapering). Four weeks after presentation, the retinal infiltrate and associated serous retinal detachment had resolved, but retinal hard exudates had increased. A large sea-fan preretinal fibrovascular neovascularization became apparent along the superotemporal retinal vascular arcade, but there was no associated retinal ischemia on fluorescein angiography. The patient received an adjunctive single intravitreal injection of 1.25 bevacizumab. Sequential follow-up examinations showed shrinking of sea-fan retinal neovascularization, a complete resolution of retinal hard exudates, and the development of a self-limited vitreous hemorrhage. On last follow-up, 30 months after intravitreal bevacizumab injection, BCVA was 20/25. CONCLUSION: Patients with rickettsial retinitis may develop a sea-fan retinal neovascularization, with subsequent vitreous hemorrhage, putatively through inflammatory mechanisms. Multimodal imaging including OCT, fluorescein angiography, and OCT-angiography, is highly useful for accurate diagnosis and reliable monitoring of the evolution of retinitis, retinal neovascularization, and other retinal changes. The use of a combination therapy with oral doxycycline and corticosteroids and intravitreal anti-VEGF can improve outcomes.

Clinical Case Review of Bilateral Retinal Metastasis.

Bilateral retinal metastasis is a rare disease that represents less than 1% of ocular metastases. Additionally, the prevalence of ocular metastases overall is only 5% to 10%. It is uncommonly found due to the absence of a lymphatic system in the eye. Ocular metastasis is spread hematogenously and the retina only receives 5% of blood flow, contributing to the rarity of this condition. Retinal metastasis has been reported to mimic symptoms of retinitis which include watery eye discharge, conjunctival injection and pain with ocular movement which leads to a harder diagnosis. Treatment options for retinal metastasis include systemic chemotherapy, intravitreal chemotherapy, and plaque radiotherapy. However, despite treatment, retinal metastasis often has a poor prognosis. This is a case of a 65-year-old woman with a history of breast carcinoma status post mastectomy who initially presented with metastatic infiltration of the lung and liver. However, she later developed an interesting case of retinal metastasis, which presented as symptoms of retinitis and indicated widespread dissemination of an unknown primary neoplasm.

Revisiting acute retinal pigment epitheliitis (Krill disease).

We reevaluate acute retinal pigment epitheliitis (ARPE) first described by Krill and Deutman in 1972, integrating a meticulous literature review with advanced multimodal imaging analyses. Our review included 98 eyes from 86 published cases diagnosed with ARPE. We scrutinized ARPE's clinical presentations, variability, and imaging characteristics, revealing that a large majority (90 %) of cases previously diagnosed as ARPE align more closely with other retinal disorders based on modern diagnostic criteria and imaging techniques. Only a small fraction (5 eyes) did not fit into any known categories, casting doubt on ARPE's distinct existence. This underscores the critical role of multimodal imaging in redefining our understanding of macular diseases and challenges the historical classification of ARPE as a unique clinical entity.

Long-term Outcomes of Surgeries for Retinal Detachment Secondary to Parasitic or Viral Infectious Retinitis.

PURPOSE: This study sought to compare the long-term outcomes of surgeries for retinal detachment (RD) secondary to viral or parasitic infectious retinitis. METHODS: A total of 47 eyes that received pars plana vitrectomy with or without scleral buckling due to RD secondary to polymerase chain reaction-proven viral (cytomegalovirus, varicella zoster virus, and herpes zoster virus) or parasitic (toxoplasma and toxocara) retinitis from October 1, 2006, to June 30, 2023, in a single medical center were retrospectively enrolled. RESULTS: Mean follow-up period was 59.03 ± 55.24 months in viral retinitis and 34.80 ± 33.78 months in parasitic retinitis after primary reattachment surgery. During follow-up, nine eyes (24.3%) with viral retinitis and five eyes (50.0%) with parasitic retinitis developed retinal redetachment. Visual acuity success at final follow-up was achieved in 19 eyes (51.4%) with viral retinitis and six eyes (60.0%) with parasitic retinitis (p = 0.64). The incidence of retinal redetachment during the 1st postoperative year was significantly higher in parasitic retinitis compared with viral retinitis (crude incidence, 0.21 vs. 0.85; p = 0.02). Hazard ratio analysis adjusted for age and sex showed 4.58-fold (95% confidence interval, 1.22-17.27; p = 0.03) increased risk of retinal redetachment in parasitic retinitis compared with viral retinitis during the 1st postoperative year. Tamponade with silicone oil and preoperative diagnostic vitrectomy were associated with significantly decreased risk of retinal redetachment in patients with parasitic retinitis. CONCLUSIONS: Compared with RD secondary to viral retinitis, RD secondary to parasitic retinitis showed higher incidence of retinal redetachment during the 1st postoperative year. Tamponade with silicone oil and preoperative diagnostic vitrectomy were associated with significantly decreased risk of retinal redetachment in patients with parasitic retinitis.

Vascular Changes in Eyes with Post-Fever Retinitis Pre and Post Treatment Demonstrated Using Optical Coherence Tomography Angiography.

PURPOSE: To document vascular changes in eyes with post-fever retinitis (PFR) pre and post treatment demonstrated using optical coherence tomography angiography (OCTA). METHODS: This is a retrospective observational case series wherein patients with PFR were retrospectively evaluated for changes in the retinal vasculature during the course of disease using OCTA. RESULTS: At presentation, OCTA revealed flow void areas in superficial and deep capillary plexus (SCP and DCP) corresponding to the areas of retinitis. Post treatment, OCTA showed a significant decrease in the flow void areas with the appearance of new capillary network in both SCP and DCP. The optical coherence tomography also demonstrated normalization of retinal architecture over time. It is speculated that the good visual outcome in PFR could be attributed to the normalization of retinal architecture and remodelling in retinal vasculature. CONCLUSION: OCTA being non-invasive can be used to understand and quantify the extent of vascular remodelling in PFR.

Choroidal and central macular thickness before and after treatment in post fever retinitis.

PURPOSE: To study the choroidal thickness (CT) and central macular thickness (CMT) in post-fever retinitis (PFR) and their correlation with visual acuity and treatment. METHODS: A retrospective, observational study of patients presenting with PFR from 2013 to 2021 and with spectral domain optical coherence tomography (SD-OCT) (Heidelberg®, SpectralisTM, Heidelberg, Germany) images were included. The CT and CMT were measured at presentation and at the final visit. The CT was measured subfoveally and at points 2000 µm superior, inferior, medial, and lateral from the fovea using the caliper tool. RESULTS: Seventy-nine eyes of 65 patients were included for this study. The mean age was 39.03 (±16.00) years with female preponderance of 53.84% (n = 35). Mean follow-up duration was 30 days. Mean CT at presentation and at follow-up was 254.12 µm and 241.51 µm, respectively. CT was decreased in majority of the eyes 67.1% (n = 53) from their baseline value. Mean CMTs at presentation and final visit were 454.8 µm and 223.7 µm, respectively. Best corrected visual acuity had a positive correlation with CMT (r = 0.340; P = 0.002) and negligible correlation with CT. A significant decrease in the mean CT was noted in patients who received doxycycline either alone or in combination with a steroid as compared to those who did not receive any treatment (P < 0.001). The significance of which is unknown presently. CONCLUSION: CMT has a greater role in determining the final visual outcome than CT. CT can be reduced post-treatment with no effect on vision.

Acute retinal pigment epitheliitis during treatment of hyperprolactinaemia.

BACKGROUND: Acute retinal pigment epitheliitis (ARPE) is a rare, idiopathic and self-limiting disease. The article aims to present ARPE in a patient using D2 dopamine receptor agonists for the treatment of hyperprolactinemia. CASE PRESENTATION: A 28-year-old female during hyperprolactinaemia treatment suffered from a dyschromatopsia and a central visual field defect in the left eye. She noticed a deterioration of vision and discontinued the cabergoline administration. The woman had not been diagnosed with other chronic conditions and exhibited no symptoms of infection. Upon admission, the patient was subjected to a test for COVID-19, which was negative. The ophthalmological examination revealed a decrease in visual acuity to distance in the left eye, which amounted to 18/20 on the Snellen chart. A central scotoma was noted on the Amsler chart and a loss of pigment epithelium was visible on the fundus of the left eye. Fluorescein angiography showed a discrete window defect in the left one, with no signs of leakage. Optical coherence tomography (OCT) scans of the maculae revealed a characteristic change in the photoreceptor layer and retinal pigment epithelium (RPE) in the fovea in the left eye. The electrophysiological tests revealed decreased function of cells in macular region. A magnetic resonance imaging (MRI) of the head and orbits demonstrated an asymmetric pituitary gland without chiasm compression and discrete signal enhancement from the left optic nerve. The patient underwent observation during hospitalisation. She reported improved colour vision and a decreased scotoma in the centre of her visual field. In regular outpatient follow-ups, successive improvements in visual acuity, as well as a decreased RPE damage and outer photoreceptor layer loss during an OCT test were observed. CONCLUSIONS: A case of ARPE is reported in a patient taking medications for hyperprolactinemia. The role of dopamine receptor antagonists in the photoreceptor function and causation of ARPE needs further evaluation.

Case series of neuroretinitis in Korea.

BACKGROUND: To present the clinical characteristics of neuroretinitis in Korea. METHODS: Twelve patients with neuroretinitis between January 2009 and September 2020 were retrospectively reviewed. Neuroretinitis was diagnosed based on fundus findings, optical coherence tomography, and fluorescein angiography. The serological findings of each patient were reviewed. RESULTS: Fifteen eyes of 12 patients (9 male and 3 female), with a mean age of 46.0 ± 10.7 years were included. Of the nine patients who underwent serological testing for Toxocara antibodies, six (66.6%) were positive. One patient had high titers of Toxoplasma immunoglobulins M and G. One patient diagnosed with dengue fever was suspected to have neuroretinitis in both eyes. There were no related abnormalities in the serological findings in four patients (33.3%) out of 12 patients. There were no suspected cases of cat-scratch disease. The six patients who were positive for Toxocara antibodies were older (mean age: 54.5 ± 9.1 years) than the others (mean age: 37.5 ± 4.4 years, p = 0.004). The four patients without any abnormal serological findings were relatively younger (mean age: 35.7 ± 3.0 years) than the other 8 patients (mean age: 51.1 ± 10.1 years, p = 0.008). CONCLUSIONS: Two-thirds of neuroretinitis patients were seropositive for Toxocara in the current cohort from Koreans. Causative factors in cases of neuroretinitis may vary according to age and region.

Ocular syphilis masquerading as CMV retinitis in a transplanted patient.

BACKGROUND: Syphilis has historically been referred to as "the great imitator", for the extent of disease manifestations secondary to infection. Ocular manifestations include a wide range of intra-ocular inflammation. METHODS: In this study, we report the case of a 52 years-old male patient with syphilitic hemorrhagic necrotizing retinitis. RESULTS: The patient presented to the emergency room for rapid and progressive vision loss and ocular redness lasting three weeks and was under immunosuppressive treatment. The diagnosis was syphilitic hemorrhagic necrotizing retinitis mimicking the typical clinical picture of retinitis caused by Cytomegalovirus infection in immunocompromised patients. CONCLUSIONS: The presented case highlights the need to consider ocular syphilis as a great masquerader even in the presence of atypical presentations such as hemorrhagic retinitis. Syphilis should be tested for treponemal and non-treponemal tests, and it should be ruled out as an etiological agent in every case of new-onset intra-ocular inflammation.

Enlarging aneurysm with paracentral acute middle maculopathy in idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) - a case report.

INTRODUCTION: Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare clinical entity affecting young healthy individuals. Treatment primarily involves pan retinal photocoagulation (PRP) to capillary non perfusion areas. Intravitreal anti-VEGF or steroids are given in the presence of macula edema. Oral steroids do not alter the course of the disease. Arterial occlusions have been reported in IRVAN. METHODS: Retrospective case review. RESULT: A twenty seven year old male presented to us with mild blurring of vision for one week. His BCVA was OU 20/20. Anterior segment examination was normal. Fundus examination showed bilateral disc aneurysm with OS arterial aneurysm along the inferior arcade. Fundhus fluorescein angiography and OCT angiography were confirmatory of the disc and retinal aneurysm. Capillary non perfusion (CNP) areas were noted in the periphery. Two days later he presented with paracentral scotoma in his left eye which was confirmed by Amsler chart. Fundus, OCT and OCTA were confirmatory of Paracentral Acute Middle Maculopathy (PAMM). The retinal aneurysm had increased in size from 333 micron diameter to 566 micron diameter. Panretinal photocoagulation to the CNP areas was done and intravitreal antiVEGF was given. At 6 months follow up, retinal aneurysm had disappeared. DISCUSSION: Our case describes a unique event with sudden increase in size of the aneurysm leading to acute blockage in the deep capillary plexus thus being the first report of PAMM in IRVAN. The patient was treated with PRP and intravitreal anti-VEGF for the enlarging aneurysm which reduced in size within a week.

Incidental multiple myeloma in a patient with neuroretinitis: a case report.

BACKGROUND: Neuroretinitis is classically defined as a clinical triad of unilateral, painless vision loss, accompanied by optic disc edema and characteristic macular star formation. The causes of neuroretinitis can be categorized as infectious, non-infectious, and idiopathic, therefore differential diagnosis and careful evaluation are required, owing to the various etiologies and masqueraders. CASE PRESENTATION: A 54-year-old woman presented to the clinic with blurred vision in both eyes. A complete ophthalmic examination revealed optic disc edema with blurred margins and macular exudates, intraretinal edema in the temporal peripapillary area, and subretinal fluid with neurosensory retinal detachment in the macular area. Systemic laboratory investigations showed no signs of infection or inflammation. However, bone marrow suppression was suspected based on the results of the complete blood count test, and the patient was diagnosed with multiple myeloma. CONCLUSION: Although neuroretinitis is rarely accompanied by hematological malignancy, it is important to be mindful of the latter because ophthalmic manifestations are a common feature of hematological malignancies and lesions occur in nearly every ocular structure.

CMV neuroretinitis in a post-transplant patient.

This report presents an atypical case of neuroretinitis in a post-transplant patient. A man in his 40s presented to a provincial ophthalmology service with unilateral left visual loss. He was immunosuppressed following orthotopic liver transplantation for end-stage liver cirrhosis secondary to primary sclerosing cholangitis. He had received his third Pfizer-BioNTech COVID-19 booster vaccine 34 days prior to symptom onset. His presenting left visual acuity was 6/36. His left optic nerve head was grossly swollen with peripapillary haemorrhage, intraretinal and subretinal fluid extending to involve the fovea. His serological and radiological investigations were all negative except for serum IgG and IgM positivity to cytomegalovirus (CMV). Following the commencement of antiviral treatment and systemic steroids, his neuroretinitis improved with visual recovery to 6/4.5. This report describes an atypical presentation of neuroretinitis in an immunocompromised patient without AIDS but with evidence of CMV exposure.