Central Retinal Artery Occlusion: Visual Outcomes from a Large Northern California Cohort.

PURPOSE: To assess visual acuity (VA) outcomes in a large cohort of patients diagnosed with nonarteritic central retinal artery occlusion (CRAO), and to ascertain whether time from symptom onset to presentation, presenting VA, or conservative treatment delivery (anterior chamber paracentesis, ocular massage, intraocular pressure lowering drugs, hyperventilation, or some combination of those) impacted ultimate VA outcomes. DESIGN: Retrospective cohort study. SUBJECTS: The study included 794 patients who presented with CRAO between 2011 and 2020. Within this cohort, 484 individuals presented within 30 days of symptom onset and had comprehensive documentation regarding the details of their presentation, management, and follow-up ≥ 90 days postdiagnosis. METHODS: Retrospective chart review was conducted for all patients with a diagnosis of CRAO initially identified via International Classification of Diseases coding, followed by confirmation of diagnosis by 2 retina specialists. Cases of arteritic CRAO were excluded. MAIN OUTCOME MEASURES: Visual acuity recovery, defined as improvement from ≤ 20/200 or worse at presentation to ≥ 20/100 ≥ 90 days after diagnosis. RESULTS: Of the 794 identified patients, 712 (89.7%) presented with VA of ≤ 20/200. Similarly, 447 (92.4%) of the 484-patient subset that presented within 30 days and had comprehensive documentation presented with VA ≤ 20/200. Of the 441 of those patients with documented follow-up, 380 (86.2%) remained at that level. Of the 244 patients who presented within 4.5 hours of symptom onset, 227 (93%) presented ≤ 20/200 and 201 (92.6%) of the 217 of those with follow-up data did not improve beyond that threshold. There was no significant difference (P < 0.05) in final VA between patients presenting before versus after 4.5 hours from time of vision loss. There was also no significant difference (P < 0.05) in VA outcomes between patients who did or did not receive conservative treatment. CONCLUSIONS: This large retrospective study further highlights the poor visual prognosis for patients with CRAO. Earlier time to presentation did not seem to impact final VA outcome, nor did conservative treatment efforts. Efficacious evidence-based treatment options are needed for this patient population. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Autoimmune Retinopathy in a Patient with Stiff-Person Syndrome: A Case Report.

PURPOSE: To describe a patient who developed retinal degeneration associated with autoimmune retinopathy (AIR) and who was also found to have anti-glutamic acid decarboxylase (GAD65) autoantibodies and the diagnosis of stiff-person syndrome (SPS). METHODS: Ophthalmologic workup consisted of clinical examination, multi-modality retinal imaging, and electrophysiologic testing. Further neurologic assessment including relevant serum and cerebrospinal fluid studies was also conducted. RESULTS: We highlight the case of a 45-year-old patient who developed subacute, sequential vision loss, along with bilateral lower extremity weakness. On initial presentation, optical coherence tomography (OCT) of the left eye was notable for diffuse attenuation of the outer retinal bands. Fundus autofluorescence demonstrated a ring of hyper-autofluorescence encircling the fovea of the left eye. At fifteen-month follow-up, the right eye also became similarly affected. He was found to have elevated serum and cerebrospinal anti-GAD65 autoantibodies and was diagnosed with both SPS and AIR. CONCLUSION: There is a potential association of anti-GAD65 autoantibodies with the development of AIR.

Retinal Dialysis and Associated Rhegmatogenous Retinal Detachment in Patients with Neurofibromatosis Type 1: A Case Series.

PURPOSE: While ophthalmic manifestations of neurofibromatosis type 1 (NF1), including iris Lisch nodules and optic gliomas have been well described, retinal involvement in these patients has yet to be established. Characterizing the relationship between NF1 and the retina is necessary to optimize outcomes for these patients. METHODS: Independent chart review of NF1 patients was conducted. RESULTS: Chart review yielded four patients, with a history of NF1, with subsequent retinal dialysis and rhegmatogenous retinal detachment. These four patients presented to our institution with a rhegmatogenous retinal detachment secondary to a retinal dialysis with no history of trauma. These patients also demonstrated hyperreflective choroidal abnormalities on near-infrared reflectance (NIR) imaging and optical coherence tomography (OCT). CONCLUSION: Seeing that patients diagnosed with NF1 are susceptible to various ocular manifestations and pathologies, routine ophthalmic examinations are essential in maintaining their ocular health and minimizing morbidity.

Central Retinal Artery Occlusion: Time to Presentation and Diagnosis.

OBJECTIVE: To evaluate the presentation patterns of patients diagnosed with central retinal artery occlusion (CRAO) from 2011 to 2020. DESIGN: Retrospective cohort study SUBJECTS: The present study was conducted in 484 patients presenting within 30 days of symptom onset with accurate documentation of time of symptom onset, time of presentation to the health care system, and time of presentation to an ophthalmologist. METHODS: An independent chart review of patients with CRAO was conducted. MAIN OUTCOME MEASURES: Demographic information including age, sex, and race were collected. Presentation patterns such as time of first symptoms, time of first contact with the health care system, and time of evaluation by an ophthalmologist were analyzed. Additionally, information regarding the medical venue or specialty of initial patient contact was collected. RESULTS: A total of 247 (51%) patients contacted the health care system within 4.5 hours of system onset, whereas 86 (17.8%) patients waited over 24 hours. Only 81 (32.8%) of the 247 patients who presented within 4.5 hours saw an ophthalmologist within that time frame, whereas 172 (35.5%) of the entire cohort of 484 did not present to an ophthalmologist within 24 hours of vision loss. There was significant variability with regards to medical specialty of initial patient contact, with 292 (60.3%) patients first presenting to an emergency department and 133 (27.5%) patients first presenting to an ophthalmologist. Black and Hispanic patients presented later than patients of White, Asian, or other racial backgrounds (40.4 ± 10.2 hours versus 23.0 ± 3.4 hours, P = 0.05). CONCLUSIONS: Although no level 1 evidence-based treatment is currently available for CRAO, thrombolytic therapy may be promising. Even though over half of patients with CRAO within our institution connected with the health care system within a potential window for thrombolytic therapy, most did not receive a definitive ophthalmic diagnosis within that time frame. Public health educational campaigns and infrastructure optimization must speed up presentation times, decrease the time to ophthalmic diagnosis, and target vulnerable populations to offer and research timely administration of thrombolytic therapy. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any materials discussed in this article.

RESCUE INTRAVITREAL METHOTREXATE TREATMENT FOLLOWING EARLY RECOGNITION OF PROLIFERATIVE VITREORETINOPATHY.

PURPOSE: To report a case of proliferative vitreoretinopathy (PVR) in a man with recurrent retinal detachment successfully managed without surgical intervention following the initiation of intravitreal methotrexate injections to arrest progression of PVR. METHODS: Report of a case. RESULTS: A 60-year-old man presented to the retina clinic 4 weeks after undergoing vitrectomy for rhegmatogenous retinal detachment and was found to have an inferior recurrent retinal detachment. He underwent repeat vitrectomy and scleral buckling with successful reattachment of the retina in the immediate postoperative period. At postoperative Week 2, preretinal membranes were noted inferiorly with stretching of the causative retinal break and localized subretinal fluid, consistent with early PVR. The patient underwent immediate laser barricade, and a course of intravitreal methotrexate injections was started. At the final follow-up 7 months later, the retina was fully attached without progression of PVR. CONCLUSION: Intravitreal methotrexate may play a role in arresting progression of early postoperative PVR and obviating the need for surgical intervention.

IDIOPATHIC MULTIFOCAL CHOROIDITIS WITH SERPIGINOUS-LIKE PERIPAPILLARY CHORIORETINAL ATROPHY.

PURPOSE: To report nine cases of multifocal choroiditis with serpiginous-like peripapillary chorioretinal atrophy. METHODS: A retrospective observational case series of eyes with multifocal choroiditis with serpiginous-like peripapillary chorioretinal atrophy. Multimodal imaging findings were reviewed and presented. RESULTS: Fifteen eyes of 9 patients (6 women and 3 men), with a mean age of 48.1 years (median, 46 years; range, 23-74 years), presented with multifocal choroiditis serpiginous-like peripapillary chorioretinal atrophy. All 15 eyes presented with serpiginoid peripapillary changes and had discrete patches of atrophy or punched-out scars in the posterior pole or periphery. Eleven eyes (73.3%) had cone-shaped retinal pigment epithelium elevations on optical coherence tomography, 10 eyes (66.7%) had mild vitritis, and 4 eyes (26.7%) had peripheral curvilinear streak lesions. Three eyes (20%) had choroidal neovascularization. All patients responded well to treatment with systemic immunosuppression, local corticosteroid injections, and/or intravitreal anti-vascular endothelial growth factor injections. CONCLUSION: Multifocal choroiditis may present with peripapillary chorioretinal changes resembling a serpiginous-like choroiditis in addition to the classic findings of patches of atrophy or punched-out scars in the posterior pole or periphery, cone-shaped retinal pigment epithelium elevated on optical coherence tomography and peripheral curvilinear streak lesions.

Evaluation of a Series of Wrong Intravitreous Injections.

IMPORTANCE: This case series describes events associated with errors in intravitreous injections. Given the volume of injections performed worldwide, it is important to identify the factors associated with these wrong events to try to reduce their occurrences. OBJECTIVE: To evaluate a series of errors in intravitreous injections within Kaiser Permanente Northern California (KPNC). DESIGN, SETTING, AND PARTICIPANTS: In this retrospective small case series of a convenience sample at KPNC between January 1, 2019, and December 30, 2020, cases of errors in intravitreous injection were identified either as part of a formal institutional quality review or by self-report of the involved surgeon during quality improvement discussions. MAIN OUTCOMES AND MEASURES: Description of the medical errors and the circumstances surrounding these errors. RESULTS: During the 2 years of this evaluation, there were more than 147 000 injections performed within KPNC. Four cases of errors in intravitreous injection were identified. Mistakes were associated with inaccurate review of the electronic medical record, poor surgeon and staff focus, and inconsistent use of surgical checklists and timeouts. No long-term ocular morbidity occurred following any of these errors. CONCLUSIONS AND RELEVANCE: Medical errors related to intravitreous injections have occurred within KPNC. We trust these events are not unique to our practice. A standardized teams-based approach that incorporates rigorous safety protocols will likely be needed to reduce the risk of future wrong intravitreous injections.

Multiple Evanescent White Dot Syndrome: Findings from a Large Northern California Cohort.

PURPOSE: To report the clinical and imaging characteristics of multiple evanescent white dot syndrome (MEWDS) from a large single-center cohort. DESIGN: Single-center, retrospective cohort study. PARTICIPANTS: A total of 111 patients previously diagnosed with MEWDS in the Kaiser Permanente Northern California system from 2012 to 2019. METHODS: Two retina specialists reviewed the medical records and all available retinal imaging, including Humphrey visual field testing, fundus photography (FP), OCT, fluorescein angiogram (FA), and fundus autofluorescence (FAF). Patients were excluded from analysis if confirmatory imaging was unavailable. MAIN OUTCOME MEASURES: Patient characteristics, visual acuity, clinical examination and imaging findings, and final diagnosis. RESULTS: Seventy-three patients (65.8%) were confirmed to have the diagnosis of MEWDS. Fifty-eight (79.5%) were female, with a mean (standard deviation [SD]) age at presentation of 35.2 (14.2) years, and mean refractive error of -1.6 diopters. Initial mean (SD) visual acuity was logarithm of the minimum angle of resolution (logMAR) 0.39 (0.31) and improved to mean (SD) logMAR 0.07 (0.15) at final follow-up. Presenting symptoms included blurred vision (82%), scotomas (56%), photopsias (43%), and floaters (23%). Nine patients (12%) had a previously diagnosed autoimmune condition, and 2 patients (3%) had documentation of a recent vaccination. Antecedent upper respiratory infection was documented in 15 of 66 patients (23%). Noted clinical and imaging features include ellipsoid zone disruption (100%), white fundus lesions (92%), FA hyperfluorescence (92%), foveal granularity (74%), vitreous cell (53%), and optic disc edema (52%). Twenty-nine of the 111 patients (26%) were initially misdiagnosed with MEWDS and subsequently given an alternative diagnosis, including other white dot syndromes, syphilis, primary vitreoretinal lymphoma, myopic degeneration, and central serous chorioretinopathy. CONCLUSIONS: Multiple evanescent white dot syndrome is a rare self-limiting condition of the outer retina. Although a distinct set of clinical exam and imaging findings permit recognition of this disease, misdiagnosis is not uncommon.

Glioblastoma multiforme mimicking optic neuritis.

PURPOSE: To present a case of glioblastoma multiforme which initially presented with only ophthalmic manifestations. OBSERVATIONS: A 48-year-old man presented with decreased vision and pain with eye movements of the right eye. MRI of the brain showed increased T2/FLAIR signal involving the right optic nerve with no other identified abnormalities. He was treated with intravenous steroids for presumed optic neuritis. His visual acuity then rapidly worsened to no light perception, with new orbital apex symptoms including central retinal artery and vein occlusions and inferior division third and fourth nerve palsies. Repeat MRI with contrast showed perineural enhancement surrounding the right optic nerve and markedly reduced diffusion along its entire course. After an unrevealing initial workup and then a 7 month period during which the patient refused follow up, he re-presented with left sided weakness, headache, and confusion. Repeat brain MRI revealed a large mass involving the right optic nerve, optic chiasm, basal ganglia, corpus callosum and brainstem. Biopsy led to a diagnosis of WHO grade IV glioblastoma multiforme. The disease was poorly responsive to temozolomide, bevacizumab and external beam radiation, and the patient passed away 5 months later. CONCLUSIONS AND IMPORTANCE: Malignant optic glioma of adulthood is a challenging diagnosis with a poor prognosis. This rare case highlights the importance of maintaining neoplasm in the differential for optic neuritis masqueraders.

A case of pentosan polysulfate maculopathy originally diagnosed as stargardt disease.

PURPOSE: To describe a patient with a past diagnosis of Stargardt disease that was later determined to be pentosan polysulfate (PPS) maculopathy. OBSERVATIONS: The patient had clinical and imaging findings uncharacteristic of Stargardt disease. Rather, her fundus resembled the recently described maculopathy ascribed to PPS. After genetic testing was found to be negative for pathologic variants, the patient was asked to cease usage of PPS. CONCLUSIONS AND IMPORTANCE: This case emphasizes the importance of reviewing patient medication profiles prior to rendering a diagnosis of a retinal dystrophy. It is essential that ophthalmologists catch drug toxicities as early as possible, to minimize risk of further irreversible vision loss due to continued medication exposure.

TRANSTHYRETIN V30M FAMILIAL AMYLOIDOSIS PRESENTING AS ISOLATED RETINAL ANGIOPATHY.

PURPOSE: To describe a patient with confirmed transthyretin V30M form of familial amyloidosis who presented initially with isolated retinal angiopathy. METHODS: Retrospective chart review. RESULTS: A 66-year-old woman presented with bilateral retinal angiopathy. Extensive workup for an infectious, inflammatory, or hypercoagulable cause was unrevealing. The patient subsequently developed bilateral neovascularization of the optic nerve and iris complicated by recurrent vitreous hemorrhages, which were treated with intravitreal bevacizumab and panretinal photocoagulation. The development of cardiac and gastrointestinal symptoms 5 years after presentation led to tissue biopsies that revealed both Congo red staining and apple-green birefringence in polarized light, confirming the diagnosis of systemic amyloidosis. Sequencing of the transthyretin gene confirmed the patient to be heterozygous for the common amyloidogenic V30M mutation. CONCLUSION: The common transthyretin V30M form of familial amyloidotic polyneuropathy can rarely present with retinal angiopathy. Recurrent vitreous hemorrhages were treated successfully with intravitreal bevacizumab and panretinal photocoagulation.

MULTIFOCAL RETINAL INFILTRATES WITH PHLEBITIS AND OPTIC NEUROPATHY IN AN HIV-POSITIVE PEDIATRIC PATIENT.

PURPOSE: To describe an unusual presentation of bilateral HIV-associated multifocal retinal infiltrates with phlebitis and optic neuropathy in a pediatric patient from Zimbabwe, Africa. METHODS: Retrospective case report of a 15-year-old boy from Zimbabwe, Africa. RESULTS: The patient was found to have bilateral vitritis, multifocal retinitis with phlebitis, and optic neuropathy in the setting of previously unrecognized HIV infection. Vision improved and the clinical findings resolved after treatment with intravenous corticosteroids and highly active retroviral therapy (HAART). CONCLUSION: The authors describe the occurrence and treatment of bilateral, HIV-associated multifocal retinal infiltrates with phlebitis and HIV-associated optic neuropathy in a pediatric patient from Zimbabwe, Africa.

Spectral domain optical coherence tomography findings in eyes with acute ischaemic retinal whitening.

Acute retinal ischaemia presents in various forms depending on the type and location of the associated vascular occlusion. Cotton wool spots have been considered one manifestation of ischaemia and represent swelling in the nerve fibre layer. However, clinical retinal whitening also occurs in areas not affected by cotton wool spots, and has distinguishing spectral domain optical coherence tomography (SD-OCT) features. We present SD-OCT findings of hyper-reflectivity and thickening in four eyes with representative retinal arterial or retinal venous occlusions, specifically branch retinal artery occlusion, central retinal vein occlusion, Purtscher-like retinopathy and ophthalmic artery occlusion. The spectrum of retinal ischaemia from various causes was found to manifest in inner nuclear layer hyper-reflectivity and thickening on SD-OCT. En Face OCT imaging further characterises the topographical distribution of ischaemia, and reveals patterns which provide insight into the pathological processes involved.

Leaking choroidal nevus treated with focal laser photocoagulation.

PURPOSE: To present a case of leaky choroidal nevus that responded favorably to treatment with focal laser photocoagulation, shown by spectral domain optical coherence tomography, fundus autofluorescence, and fundus photography. METHODS: Descriptive case report of a 40-year-old male patient with decreased visual acuity because of subretinal fluid associated with a choroidal nevus, which was treated with focal laser photocoagulation. RESULTS: Treatment with focal laser photocoagulation to the surface of the choroidal nevus resulted in the resolution of subretinal fluid by 6 weeks. The therapeutic effect remained after 1 year of follow-up with continued improvement in vision and with no growth of the lesion. CONCLUSION: Laser photocoagulation can be an effective treatment for symptomatic subretinal fluid associated with a choroidal nevus. Spectral domain optical coherence tomography, fundus autofluorescence, and fundus photography can be helpful in monitoring the treatment response.