Cilioretinal Artery Research Articles

IDIOPATHIC RETINAL VASCULITIS, ANEURYSMS, AND NEURORETINITIS COMPLICATED BY CILIORETINAL ARTERY OCCLUSION: MULTIMODAL IMAGING AND 5-YEAR FOLLOW-UP.

To describe a multimodal imaging and a 5-year follow-up of a case of cilioretinal artery occlusion (CLRAO) in a 40-year-old woman with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. Fundus photography, indocyanine green and fluorescein angiography, optical coherence tomography (OCT), OCT angiography (OCTA), and a literature review of retinal artery occlusions associated with IRVAN syndrome were performed. Fundus examination revealed bilateral blurred margins of the discs and peri-vascular hard exudates. Fluorescein angiography (FA) demonstrated peripheral capillary nonperfusion areas in both eyes (OU). It also revealed an aberrant cilioretinal artery in the right eye (OD). OCT showed attenuation of the inner retinal layers and disruption of the outer retinal layers secondary to CLRAO in OD. OCTA allowed a clear identification of macular hypoperfusion in OU. Laser photocoagulation, along with anti-VEGF and corticosteroid intravitreal injections, was administered in OU. Best-corrected visual acuity remained stable, with no evidence of progression or development of neovascularization after a 5-year follow-up. IRVAN syndrome may be complicated by CLRAO, resulting in poor final visual acuity despite reperfusion of the occluded artery. In such cases, multimodal imaging allows the identification of complications such as macular atrophy on OCT and hypoperfusion areas that may not be detected on FA on OCTA.

Teaching NeuroImages: Cilioretinal Artery Occlusion Secondary to Antiphospholipid Syndrome.

Teaching NeuroImages: Cilioretinal Artery Occlusion Secondary to Antiphospholipid Syndrome.

Outcomes of Hyperbaric Oxygen Treatment for Central Retinal Artery Occlusion: A Single Center Experience

PurposeTo describe the outcomes of hyperbaric oxygen therapy (HBOT) for patients with central retinal artery occlusion (CRAO) at a single tertiary care center. DesignRetrospective clinical cohort study. MethodsMedical records of all patients diagnosed with CRAO who received HBOT at Mayo Clinic in Rochester, Minnesota from 1/1/2009 to 12/31/2020 were reviewed to confirm diagnosis, time from onset to presentation, exam findings, treatments, and follow-up data. Main outcome measures included final visual acuity (VA) and number of lines of improvement. ResultsThere were 41 patients diagnosed with CRAO who received HBOT during the 12 year study period. Median time from symptom onset to HBOT treatment was 9.5 hours (interquartile range [IQR] 6.5, 14.0 hours), and patients received a median of 4 HBOT sessions (IQR 2.5, 6.0 sessions). There were 20 patients who received HBOT within 9 hours, 14 (70%) of which had clinically meaningful improvement in VA of ≥0.3 logMAR. In comparison, of the 21 patients treated after 9 hours, 6 (28.6%) had VA improvement of ≥0.3 logMAR (p=0.008). For all patients, the median logMAR VA at presentation was 2.00 (IQR 1.70, 2.30) and the median logMAR VA at follow-up was 1.94 (IQR 1.00, 2.00) (p<0.001), with median lines of improvement of 3.0 (IQR 0.0, 7.0). For patients treated within 9 hours, the median logMAR VA at presentation was 2.00 (IQR 1.93, 2.30) and the median logMAR VA at follow-up was 1.70 (IQR 0.54, 2.00). Patients treated within 9 hours had statistically significant greater median lines of VA improvement than cases that were treated after >9 hours from symptom onset at 5.9 (IQR 3.0, 10.0) and 0.0 (IQR 0.0, 3.0), respectively (p<0.001). There was no difference in VA recovery associated with specific retinal exam findings such as cherry-red spot (p=0.22) and cilioretinal artery perfusion (p=0.36) compared to patients without those findings. ConclusionThere was a statistically significant improvement in VA after HBOT treatment in CRAO patients among patients that received early HBOT, with patients receiving the most benefit when receiving treatment within 9 hours. Randomized control trials in patients with CRAO are required to confirm the efficacy of HBOT.

Multimodal Imaging Characteristics and Correlation to Outcomes in Patients with CRAO Presenting to a Large Academic Center

PurposeTo characterize a large modern cohort of patients with central retinal artery occlusion (CRAO) by describing presenting features and outcomes relating to manually segmented optical coherence tomography (OCT) features, angiographic reperfusion, and visual recovery. DesignRetrospective clinical cohort study. MethodsPatients with CRAO (ICD-10: H34.1) initially presenting to a tertiary referral center between January 2017 and December 2021 were included. Demographics, eye exam findings, fundus photographs, OCT, and fluorescein angiography (FA) were analyzed. Main outcome measures included total and inner retinal thickness on macular OCT, reperfusion, visual outcomes, and development of neovascularization. Results145 eyes of 144 patients with mean age at of 69.4±13.6 years were included. The mean time to presentation was 1.6±4.2 days, with 19% examined within 4.5 hours and 26% within 6 hours of vision loss. 19% had cilioretinal artery (CLRA) sparing. Mean initial visual acuity (VA) was 1.68±1.10 LogMAR (CLRA sparing) compared to 2.53±0.58 LogMAR (non-CLRA sparing), P<0.001. 32% had elevated inflammatory makers. Out of 47 eyes with final fluorescein angiography, one-third showed some reperfusion. Final vision was 1.40±1.16 LogMAR (CLRA sparing) compared to 2.46±0.81 (non-CLRA sparing), P<0.001. A third of patients improved in VA in both groups, 27% of patients gained more than 2 lines of vision in the CLRA sparing group and 36% in the non-CLRA sparing group. 17% improved to better than 20/200 in CLRA-sparing and 4% in non-CLRA sparing. 11% developed neovascularization all in non-CLRA sparing. In a multiple linear regression, VA at presentation was associated with regaining vision of 2 lines or more (OR=2.603, P=0.007). OCT showed progressive thinning over time, reaching lowest measurements at 6 months, and stabilizing thereafter. ConclusionsIn this modern cohort of acute CRAO patients, presentation to a tertiary facility within 12 hours of symptoms was seen in almost half of the patients. Final visual acuity improved in almost a third of the patients, however, vision better than the legal blindness limit was rare (∼5%). Interestingly, a third of patients had some mild elevation of systemic inflammatory markers. Better visual acuity at presentation was associated with visual gain, while baseline OCT values had poor correlation with final outcome.

Essential thrombocythemia (ET) presenting as a combined cilioretinal artery occlusion (Cil.RAO) and central retinal vein occlusion (CRVO)

A 35-year-old male presented with sudden vision loss in his left eye for 1 day and was diagnosed with combined cilioretinal artery occlusion (Cil.RAO) and central retinal vein occlusion (CRVO). A multidisciplinary clinical work up was conducted to determine the underlying cause. A full blood count revealed a very high platelet count (1,147,000/mm3), prompting further hematologic investigation. Peripheral blood smear showed abundant platelet clumps, and bone marrow trephine biopsy indicated megakaryocytic hyperplasia and reticulin fibrosis (grade 1). The patient tested heterozygous for the JAK2 V617F mutation, a genetic marker for myeloproliferative disorders, and was diagnosed with essential thrombocythemia (ET). After a month of treatment with hydroxyurea 500 mg daily, his platelet count returned to normal. Thrombocythemia is associated with systemic and ocular thrombotic and embolic complications. A full blood count was pivotal in diagnosing and managing ET in this patient with combined retinal vascular disease.

Fine-Tuning SSL-Model to Enhance Detection of Cilioretinal Arteries on Colored Fundus Images.

Cilioretinal arteries are a common congenital anomaly of retinal blood supply. This paper presents a deep learning-based approach for the automated detection of a CRA from color fundus images. Leveraging the Vision Transformer architecture, a pre-trained model from RETFound was fine-tuned to transfer knowledge from a broader dataset to our specific task. An initial dataset of 85 was expanded to 170 images through data augmentation using self-supervised learning-driven techniques. To address the imbalance in the dataset and prevent overfitting, Focal Loss and Early Stopping were implemented. The model's performance was evaluated using a 70-30 split of the dataset for training and validation. The results showcase the potential of ophthalmic foundation models in enhancing detection of CRAs and reducing the effort required for labeling by retinal experts, as promising results could be achieved with only a small amount of training data through fine-tuning.

Non-syndromic OTX2-associated pattern dystrophy: a 10-year multimodal imaging study

PurposeTo report novel multimodal imaging features and long-term follow-up of Orthodenticle Homeobox 2 (OTX2)-associated pattern Gdystrophy.MethodsA 14-year-old boy referred with glaucoma suspect and macular pigmentation underwent fundus autofluorescence imaging, optical coherence tomography, fluorescein and indocyanine green angiography, visual field test, microperimetry and electrophysiology over a ten-year period. Next-generation sequencing panel identified a de novo heterozygous likely pathogenic OTX2 variant, c.259G>A, [p.(Glu87Lys)].ResultsVisual acuity was 20/40 OD and 20/30 OS. Examination showed bilateral enlarged optic nerve heads and increased disc cupping, multiple cilioretinal arteries, a pigmentary maculopathy with stellate-shaped region of hypoautofluorescence, shallow serous macular detachment, subretinal deposits and temporal avascular retina. Angiography showed no source of leakage and absence of retinal neovascularisation despite extensive peripheral non perfusion. Electrophysiological assessments demonstrated mild progressive rod and cone pathway abnormalities, reduced light-adapted b:a ratio, and reduced Arden ratio on electro-oculogram. Ten-year follow-up confirmed a stable disease course despite persistent submacular fluid. There was no associated pituitary structural abnormality or dysfunction.ConclusionsThis case study contributes to further understanding of OTX2-associated pattern dystrophy, highlighting its stability over 10 years. Further investigation into inter-individual and intrafamilial variability is warranted.

Non-Arteritic Anterior Ischemic Optic Neuropathy (NAION) associated with Paracentral Acute Middle Maculopathy (PAMM) and Cilioretinal Artery Occlusion (CILRAO): multimodal imaging of a newly described clinical association.

To report multimodal imaging features of a novel association of non-arteritic anterior ischemic optic neuropathy (NAION), paracentral acute middle maculopathy (PAMM), and cilioretinal artery occlusion (CILRAO). A 74-year-old male patient presented complaining of an acute subjective decline in visual acuity in the right eye over the previous days. This patient underwent a comprehensive ophthalmological assessment, including multimodal retinal imaging and visual field test. The eye examination revealed yellowish optic disc edema in the right eye with peripapillary hemorrhages and a whitish lesion extending from the temporal margin of the optic nerve head towards the macula. Multimodal imaging confirmed the diagnosis of NAION in the right eye. Notably, the structural B-scan and en face OCT images revealed hyperreflective areas in the inner and mid retinal layers, indicating an association with CILRAO and PAMM. We herein describe the clinical findings of a previously unreported association among NAION, CILRAO and PAMM.

The Spectrum of Isolated Retinal Artery Occlusion Secondary to Giant Cell Arteritis.

We systematically reviewed the literature to investigate the clinical features of isolated arteritic retinal artery occlusion (A-RAO) associated with giant cell arteritis (GCA). The four primary types of A-RAO were central retinal artery occlusion (CRAO), hemi-central retinal artery occlusion (hCRAO), branch retinal artery occlusion (BRAO), and cilioretinal artery occlusion (CLRAO). The most reported presentation was unilateral CRAO, followed by bilateral CRAO, unilateral CLRAO, and bilateral BRAO. Most RAOs were accompanied by typical GCA signs and symptoms, which can help distinguish them from non-arteritic RAOs. When reported, temporal artery biopsy confirmed GCA in most cases. Patients with GCA may present with a broad spectrum of isolated unilateral and bilateral A-RAOs. [Ophthalmic Surg Lasers Imaging Retina 2024;55:536-540.].

The great escape- preserved cilioretinal artery with transient central retinal artery occlusion following snake bite

A 24-year female sustained a snake bite on the lateral side of the left ankle, followed by localized pain and blurring of vision in right eye. Her best corrected visual acuity was 20/20 in the both eyes. Relative afferent pupillary defect was noted in right eye. Fundus examination of right eye showed clear media, normal optic disc and retinal whitening with sparing of small area on macula, temporal to disc with small cilioretinal artery, suggestive of central retinal artery occlusion (CRAO) with presence of cilioretinal artery sparing. Ophthalmic manifestations following a snake bite are uncommon and can arise through diverse mechanism such as hematotoxicity from the snake bite, resulting in CRAO. Although CRAO following snake bite has been reported, we present a very rare case of snake bite induced transient CRAO with presence of spared cilioretinal artery.

A Review of Current Literature on Central Retinal Artery Occlusion: Its Pathogenesis, Clinical Management, and Treatment.

The ocular analogue of a cerebral stroke is central retinal artery occlusion (CRAO), a medical emergency concerning the eyes. Most patients experience substantial acute vision loss with a visual acuity of 20/400 or worse, resulting in decreased quality of life (QoL) and decreased functional ability. An impending cerebral stroke and ischemic heart disease are also more likely. The four distinct clinical entities that make up CRAO are non-arteritic CRAO, transitory non-arteritic CRAO, non-arteritic CRAO with cilioretinal artery sparing, and arteritic CRAO. Depending on the CRAO type, clinical traits, visual results, and treatment all vary greatly. Contrary to current belief, there is a spontaneous improvement in the optical field and vision, mainly in the first week. The likelihood of instinctive development in optical acuity in the first seven days varies greatly. The pathogenesis, epidemiology, and medical features of CRAO will be described in this review, along with present and potential management future options.

Central retinal vein occlusion with secondary cilioretinal artery occlusion following oral minoxidil use: A case report and literature review

Minoxidil, a drug that is widely used for hair loss treatment, is considered a safe medication. While there are minor side-effects such as scalp irritation or systemic effects like tachycardia, it is rarely associated with severe ocular complications. We report a case where a 35-year-old man, taking oral Minoxidil for alopecia, developed Central Retinal Vein Occlusion (CRVO) with Cilioretinal Artery Occlusion (CLRAO), leading to sudden vision loss. After Minoxidil discontinuation, the patient’s vision significantly improved. This incident emphasises the importance of recognizing potential ocular side effects of Minoxidil among healthcare professionals and necessitates further research to understand its comprehensive impact on health.

Ophthalmic giant cell arteritis in a patient with normal visual acuity

Abstract: In this study, we report a case of giant cell arteritis (GCA) and central retinal artery occlusion (CRAO) with cilioretinal artery sparing in a patient who presented with normal visual acuity and was treated with corticosteroids and tocilizumab. An 80-year-old male presented with restriction of his peripheral vision in the right eye along with fever, generalized headache, and jaw pain. His visual acuity was 20/20 in both eyes (OU) with an afferent pupillary defect in the right eye. Dilated fundoscopy revealed whitening of the retina around the vascular arcades with sparing of the central macula in the right eye, indicating CRAO sparing the cilioretinal artery. Laboratory evaluation revealed elevated inflammatory markers. The patient was treated initially with a pulse dose of intravenous corticosteroids, followed by an oral steroid taper, and he underwent a temporal artery biopsy (TAB). At 1-week follow-up, the patient reported improvement in his right eye visual symptoms. The TAB results confirmed the diagnosis of GCA, and the patient’s oral steroid was tapered with the addition of subcutaneous tocilizumab. One month later, the patient’s inflammatory markers returned to normal, and the patient’s vision remained stable. We conclude from this case that GCA may present with normal visual acuity, it can affect the central retinal artery without posterior ciliary artery involvement, and the combination of corticosteroids and subcutaneous tocilizumab may be an adequate treatment regimen.

Abnormal retinal vessel filling in central retinal artery occlusion

ABSTRACT Clinical relevance Central retinal artery occlusion (CRAO) is an ophthalmic emergency with a poor prognosis. Several initial CRAO presentation factors can influence the final visual outcome. Fluorescein angiography filling of retinal vessels in CRAO has not been studied in great detail. Background The aim of this paper is to study the aberrant filling of the retinal vessels on fluorescein angiography in patients with CRAO and understand its clinical relevance. Methods Cases of CRAO diagnosed between June 2017 and May 2022 and who had undergone a fundus fluorescein angiography were included. Comparisons were made between the cases with and without aberrant filling of the retinal vessel. Results Twenty-two eyes of 22 patients (14 males and 8 females) with CRAO underwent fluorescein angiography. Ages of the patients ranged from 18 to 73 years, while time interval between development of acute onset vision problems and presentation to the retinal clinic ranged from a minimum of 1 day to a maximum of 30 days. Snellen visual acuity at presentation ranged from perception of light (PL +) to 6/6. On fluorescein angiography, aberrant flow of the fluorescein dye into the retinal vein was observed in four of the 22 (18%) eyes with CRAO. All these cases showed a patient's temporal cilioretinal artery. The cases with aberrant filling of the retinal vessels showed a better presenting visual acuity (6/6–6/60), lesser severity of inner retinal damage and a better final visual acuity (6/6–6/60) compared to CRAO cases without aberrant filling. Conclusion Aberrant filling of the retinal vein can be seen on fluorescein angiography in eyes with CRAO and a patent temporal cilioretinal artery. Such eyes with aberrant retinal vessel filling have a better visual prognosis.

Combined central retinal vein occlusion and cilioretinal artery occlusion in an MTHFR C677T homozygous 26-year-old patient with hyperhomocysteinemia: Case report

A 26-year-old female patient presented with sudden, painless visual disturbances in her right eye (RE). Fundus examination revealed signs of a central retinal vein occlusion (CRVO) (hemorrhages, mild venous tortuosity, mild disc swelling, and blurred disc margins), as well as a white ischemic area nasal to the optic nerve, suggesting a possible cilioretinal artery occlusion (CLRAO). A diagnosis of combined CRVO and nasal CLRAO with homozygosity for the C677T mutation in the methylenetetrahydrofolate reductase (MTHFR) gene and elevated levels of serum homocysteine was made. The patient was prescribed folic and acetylsalicylic acid and subsequent follow-up demonstrated gradual improvement.

Central retinal artery occlusion following pediatric blunt ocular trauma.

To analyze the clinical profile, presentation, possible pathophysiology, and outcomes of central retinal artery occlusion (CRAO) following blunt trauma in pediatric subjects. The medical charts of subjects aged 18 years or less with a diagnosis of CRAO following blunt ocular trauma were analyzed retrospectively for demography, details of the trauma, ocular findings, additional imaging reports if any, and final outcome. A Medline search was done (key words like central retinal artery occlusion, blunt trauma, children, pediatric subjects, and adolescents) to gather information available in the literature on the subject. A total of 11 patients (11 eyes), mean age of 14.3 ± 3.4 years, and 100% male preponderance, with an average time duration from trauma to presentation to the hospital of 8.1 days were included. Visual acuity ranged from no light perception (four eyes) to finger count at a 1 m distance. Intraocular pressure was raised in three patients, of which two were suffering from sickle cell disease. In two eyes, the CRAO coexisted with optic nerve avulsion and the cilioretinal artery was spared. Disk pallor was seen in six eyes as early as 12 days from the trauma. None of the cases revealed any bony fracture in the CT scan. CRAO was observed to be an important primary or contributory cause of visual loss in children following blunt trauma, reflex vasospasm being the most common etiology. Early onset disk pallor could suggest an underlying vascular compromise of both retinal and optic disk circulation in addition to direct disk damage.

Case report: acute isolated cilioretinal artery occlusion secondary to percutaneous coronary intervention

IntroductionThis case report aims to describe in detail the acute isolated cilioretinal artery occlusion (CLRAO) secondary to complicated therapeutic percutaneous coronary intervention (PCI).Case descriptionA 68-year-old Chinese man with coronary artery disease (CAD) complained of sudden, sharp chest pain. Coronary angiography revealed severe stenoses of the coronary arteries. The patient was then treated with PCI. One hour after the procedure, the patient presented with a sudden reduction in vision in the right eye. The patient was diagnosed with acute isolated CLRAO and treated with Salvia miltiorrhiza injections.ConclusionsThis is the report to provide a detailed description of acute isolated CLRAO secondary to therapeutic PCI treated with Salvia miltiorrhiza. The visual prognosis of the untreated patients is poor. Suitable management and prevention are essential for interventional cardiologists to prevent these complications.

Cilioretinal artery occlusion secondary to central retinal vein occlusion

Cilioretinal artery occlusion secondary to central retinal vein occlusion

Clinical, Radiological, and Histopathological Findings of Post-Exenterated Mucormycosis Specimens

Introduction: A sharp upsurge in the number of coronavirus disease-associated mucormycosis cases was noted during the second wave of coronavirus disease in India. The fungal hyphae spread from the nasal mucosa, orbit to the brain, hence otherwise called rhino-orbito-cerebral mucormycosis (ROCM). Prompt diagnosis and early initiation of treatment with amphotericin B, aggressive surgical debridement of the PNS, and orbital exenteration, where indicated, are essential for successful outcome. This study is done to enumerate the clinical, radiological, and histopathological findings of post-exenterated specimens of ROCM. Methods: This is a non-randomized cross-sectional study conducted at a tertiary care center. Ten post-exenterated specimens were examined histopathologically for the involvement of the central retinal artery, superior ophthalmic vein (SOV), optic nerve, muscle, and orbital fat showing necrosis. Clinical findings of these 10 patients were studied retrospectively for visual loss, proptosis, extraocular movements for muscle involvement, scleral necrosis, and fundus findings for artery or vein occlusions. Preoperative magnetic resonance imaging (MRI) findings like the number of orbital quadrants showing soft tissue thickening or tissue enhancement, diffusion restriction in the optic nerve, soft tissue enhancement seen at the orbital apex or superior orbital fissure, loss of flow void in the left internal carotid artery, enlargement of the SOV containing filling defect on post-contrast images were noted. Results: Optic nerve involvement was seen in 100% patients clinically, 70% on MRI, and 50% on histopathological examination (HPE). Muscle involvement was seen in 100% patients clinically, 90% on MRI, and 80% on HPE. None of the patients had scleral necrosis clinically. Scleral tenting was seen in 20% patients on MRI and 20% on HPE. Clinically, central retinal artery involvement was seen in 40%, and cilioretinal artery involvement was seen in 10%. MRI could not give much information on artery involvement. Clinically, none of them had central retinal vein occlusion. 20% had an enlarged SOV seen on MRI. HPE showed vascular involvement in 90% of the specimens. Conclusion: Clinical, radiological, and histopathological findings play a major role in the diagnosis, staging, and follow-up of ROCM.

Henle fiber layer hemorrhage associated with combined central retinal vein occlusion and cilioretinal artery occlusion: a case report

BackgroundThe purpose of this study is to describe a patient who experienced simultaneous central retinal vein and cilioretinal artery occlusions, as well as perifoveal hemorrhage in the Henle fiber.Case presentationA 67-year-old Iranian woman presented with a 3-day history of reduced vision in her left eye. Venous tortuosity and retinal hemorrhage were observed in the retina, together with whitened regions around the fovea, consistent with the diagnosis of central retinal vein occlusion in conjunction with cilioretinal artery occlusion. In structural and en face optical coherence tomography, star-shaped hemorrhages were observed around the fovea, which looked hyperreflective in the Henle fiber layer.ConclusionsWe present a case of central retinal vein occlusion exacerbated by cilioretinal occlusion and hemorrhage in the Henle fiber layer. The hemorrhage is most likely the result of increased intraluminal pressure in the deep capillary plexus.