Heterozygous factor V Leiden mutation manifesting with combined central retinal vein occlusion, cilioretinal artery occlusion, branch retinal artery occlusion, and anterior ischaemic optic neuropathy: a case report.

BACKGROUND: Our purpose was to describe a patient who developed combined central retinal vein occlusion (CRVO), cilioretinal artery occlusion, branch retinal artery occlusion (BRAO), and anterior ischaemic optic neuropathy (AION) followed by CRVO in the second eye because of the heterozygous factor V Leiden (FVL) mutation. CASE PRESENTATION: A 39-year-old female with a history of recurrent pregnancy losses presented with acute blurred vision in the right eye (RE), with visual acuity limited to counting fingers. She was diagnosed with combined impending CRVO, cilioretinal artery occlusion, BRAO, and AION. The results of thrombophilia testing, not including the FVL mutation, were negative. Retinal atrophy with vascular attenuation and optic disc pallor developed after resolution of acute retinal findings. Nine months after initial presentation, the patient developed an impending CRVO in the left eye (LE), with a secondary progression to a complete CRVO causing a decrease in best corrected visual acuity (BCVA) to 20/40. The patient was determined to be heterozygous for the FVL mutation. She subsequently was treated with acenocoumarol. At the last follow-up visit, the BCVA was 20/400 in the RE and 20/20 in the LE, and there was a complete resolution of the acute CRVO findings in the LE. CONCLUSION: Our case shows that the heterozygous FVL mutation may manifest with combined retinal vascular occlusion involving multiple sites in both eyes. Early recognition of such an inherited thrombophilic disorder is important because it implies the need for long-term anticoagulative therapy to reduce the patient's risk of recurrent, sight-threatening and life-threatening thrombotic events.

Influence of segmental supply of Cilioretinal artery on morphology of diabetic macular edema.

BACKGROUND: The supply of Cilioretinal artery (CRA) to different layers of the retina influences retinal pathologies such as diabetic retinopathy (DR). Since the supply of CRA is segmental, our aim was to analyze the location of CRA with respect to non - center involving diabetic macular edema (DME) differentiated by various segments and center involving DME based on Early Treatment of Diabetic Retinopathy Study (ETDRS) scale using optical coherence tomography (OCT). METHODS: A retrospective study was conducted in which forty-three patients with various stages of DR and the presence of CRA were identified. Presence and location of CRA was recognized using fundus fluorescein angiography. Classification of DME was based on ETDRS subfields on OCT. RESULTS: Evaluation of 26 men and 17 women with varying degrees of severity involving DR revealed the presence of unilateral CRA in 40 subjects and bilateral CRA in 3 subjects. When CRA supplied the central area, maximum retinal thickness was noted at the temporal quadrant (271.67 ± 164.02 µm) along with non - center involving DME (194.87 ± 121.06 µm); when CRA supplied the lower area, maximum retinal thickness was noted at the superior quadrant (293.64 ± 159.36 µm) along with center involving DME (395 ± 285.75 µm) and when it supplied the upper area, maximum retinal thickness was noted at the nasal quadrant (293.49 ± 176.18 µm) along with center involving DME (292 ± 192.79 µm). CONCLUSION: The presence of CRA seems to influence the morphology of the retina amongst patients diagnosed with DR by altering the segments involved in DME based on its supply location. However, further studies with a larger sample size are warranted to strenghten this association.

Non-arteritic anterior ischemic optic neuropathy with Cilioretinal artery occlusion: a case report.

BACKGROUND: To describe a peculiar case of concurrent non-arteritic anterior ischemic optic neuropathy (NAION) and cilioretinal arteries occlusion (CLRAO) without other causative agents which responded well to intravenous and intravitreal injection of corticosteroids. CASE PRESENTATION: A 41-year-old woman presented with painless vision loss in the right eye for 1 week. Fundus examinations showed marked disc swelling, flame-shaped hemorrhage over the superior nerve fiber area, and well-demarcated retinal ischemia superior to the fovea in the right eye. Under the impression of NAION with branch retinal artery occlusion, the patient was treated with intravenous and intravitreal injection of corticosteroids. Two months later, as the disc swelling and retinal ischemia resolved, we found that the occluded artery was the cilioretinal artery and not the ordinary branch retinal artery. CONCLUSIONS: CLRAO may be concomitant with the setting of NAION, the physicians should be aware that CLRAO may be misinterpreted as BRAO owing to profound disc edema during the early stages of the disease.

Cilioretinal artery occlusion associated with cisplatin.

INTRODUCTION: Cancer is an important risk factor for venous and arterial thromboembolic events. Treatment with chemotherapy was associated with a 6.5-fold increase in the risk of thromboembolic events. Here, we present a patient with cilioretinal artery emboli during cisplatin-based therapy. CASE REPORT: A 54-year-old male patient with a diagnosis of metastatic small cell carcinoma was under cisplatin-based regimen. He presented with visual disturbance. Retinal fluorescein angiography showed multiple plaques located in cilioretinal artery and cilioretinal artery occlusion. After excluding other potential etiological factors, patient was diagnosed with cilioretinal artery occlusion associated with cisplatin. DISCUSSION: In oncology practice, patients are prone to thromboembolic events due to primary disease, underlying comorbidities and treatment modalities. In addition to numerous toxicities, cisplatin is an important risk factor for thromboembolic events. Clinicians caring patients with a diagnosis of cancer should be aware of this rare complication of cisplatin-based therapies.

Cilioretinal artery occlusion and anterior ischemic optic neuropathy as the initial presentation in a child female carrier of Fabry disease.

PURPOSE: To report the youngest female carrier of Fabry disease, complicated by cilioretinal artery occlusion and anterior ischemic optic neuropathy (AION). METHODS: Case report. RESULTS: An 11-year-old girl was referred to our clinic with painless, acute loss of vision in her right eye. Posterior segment examination and fluorescein angiography revealed cilioretinal artery occlusion and AION. Systemic evaluations were unremarkable, except for a low blood α-galactosidase A enzyme level of 242.27 pmol/spot*20 h (reference range: 450-2000 pmol/spot*20 h). The patient was diagnosed with female carrier of Fabry disease. CONCLUSION: Retinal vascular occlusions are rare in childhood, and Fabry disease may present with retinal vascular occlusion. Ophthalmological examinations may be contributing for early detection of the disease. To the best of our knowledge, this is the first report of a child female carrier of Fabry disease, complicated by cilioretinal artery occlusion and AION.

Concomitant Two-Temporal Cilioretinal Artery Occlusion (CLRAO) With Impending Central Retinal Vein Occlusion (CRVO) in a Young Adolescent With Protein C Deficiency: A Rare Case.

We report structural changes in the retina of an adolescent diagnosed with the concomitant two temporal cilioretinal artery occlusion (CLRAO) with impending central retinal vein occlusion (CRVO) along with mild protein C deficiency. An 18-year-old girl came to the emergency room with sudden onset painless loss of vision in her right eye. On comprehensive ophthalmic examination, she had a pale superior-temporal retina with spongy macular edema corresponding to two temporal CLRAO and blurred disc margins with mild disc swelling and mild tortuosity of retinal veins all over the retina with few superficial hemorrhages in the right eye corresponding to impending CRVO. Optimal coherence tomography (OCT) showed thickening of the nerve fiber layer in the superior-temporal quadrant involving some part of the macula in the right eye. Perimetry showed a right eye visual field defect in the inferior nasal quadrant. Her coagulation profile was normal but her autoimmune profile was suggestive of mild protein C deficiency. Immediately she was started on anticoagulants. After one month, her visual acuity improved from finger counting close to face to 6/9 with treatment. Over a period of one month, retinal and OCT changes recovered with the same perimetry findings as earlier. This case shows how prompt treatment resulted in dramatic improvement in the form of good visual outcomes.

Central Retinal Artery Occlusion With Triple Cilioretinal Artery Sparing.

We report a rare case of central retinal artery occlusion (CRAO) with triple cilioretinal artery sparing in a 76-year-old male with hypertension who presented with sudden diminution of vision in the left eye (OS) for one day. Optical coherence tomography angiography (OCTA) demonstrated the presence of three cilioretinal arteries and the absence of flow signals in the rest of the macula. Primary ophthalmic treatment was instituted immediately in the form of ocular massage, and acetazolamide 500 mg per oral (PO) stat was given. Systemic investigations revealed a significant blockage in coronary circulation on coronary angiography and an atheromatous plaque at the origin of the left internal carotid artery with 50% stenosis on digital subtraction angiography. Systemic anticoagulants and lipid-lowering agents (statins) were initiated by the cardiologist. Percutaneous transluminal coronary angioplasty was subsequently performed. At the eight-week follow-up visit, best-corrected visual acuity had improved to 2/60 OS. Fundus examination of the OS revealed optic disc pallor with normal retinal background. Spectral-domain optical coherence tomography showed diffuse retinal thinning except in the area supplied by the three patent cilioretinal arteries. En face OCTA OS showed restoration of retinal flow signal in the macula. Non-invasive imaging (OCTA) is critical in establishing early diagnosis and initiating prompt treatment in this ocular emergency with underlying potentially life-threatening systemic associations.

Influence of Cilioretinal Arteries on Flow Density in Glaucoma Patients Measured Using Optical Coherence Tomography Angiography.

It has long been speculated whether the presence of a cilioretinal artery (CRA) can influence the development of glaucomatous damage in patients with open-angle glaucoma. Studies involving healthy patients have shown a change in flow density (FD) depending on the presence of a CRA. Similarly, studies that compared the optical coherence tomography angiography (OCTA) results of healthy controls and glaucoma cohorts identified a reduction in FD in certain retinal layers for glaucoma patients. These observations raise the question of whether FD is altered in glaucoma patients depending on the presence of CRA, with possible implications for the progression of glaucomatous damage. In this prospective study, 201 eyes of 134 primary and secondary open-angle glaucoma patients who visited the Department of Ophthalmology at the University of Muenster Medical Center, Germany were included. The patients were allocated to different groups according to the presence of CRAs and the level of glaucoma severity. The FD results obtained using OCTA for the CRA and non-CRA groups were compared. While FD differed noticeably between the CRA and non-CRA cohorts in the deep macular plexus, no differences in FD were observed between the two groups when adjusted for glaucoma severity. In both the CRA and non-CRA eyes, increasing glaucoma severity correlated most strongly with a reduction in peripapillary FD. Our results suggest that the presence of CRAs does not significantly affect retinal perfusion in glaucoma patients.

A case of cilioretinal artery occlusion: Diagnostic procedures.

Purpose: To evaluate characteristic imaging findings and functional outcomes of Cilioretinal Artery Occlusion (CLRAO) associated with giant cell arteritis (GCA). Observations: We report the case of a 70-year-old woman presenting with sudden vision loss caused by a GCA-associated-CLRAO in her left eye (LE). A thorough ophthalmologic examination together with optical coherence tomography (OCT), OCT-Angiography (OCT-A), fluorescein angiography and fundus autofluorescence were performed. At presentation, the best corrected visual acuity in the LE was 20/200 and funduscopic examination revealed optic disc edema associated with retinal whitening along the area perfused by the CLRA. After 1 month, OCT and OCT-A revealed an improvement of the retinal edema and a partial reduction of the non-perfused areas in the superficial and deep capillary plexuses, as well as in the outer retina and in the choriocapillaris. Fluorescein angiography showed a reduction in the perfusion of the affected area, a delayed perfusion of the temporal sector of the optic disc, as well as areas of choroidal hypoperfusion in the peripheral temporal retina. The patient's visual acuity did not change during the follow up. Conclusion and importance: Despite a partial recanalization of the occluded vasculature being possible after GCA-associated-CLRAO, the patient's visual prognosis remains poor.

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

INTRODUCTION: This case report aims to describe in detail the acute isolated cilioretinal artery occlusion (CLRAO) secondary to complicated therapeutic percutaneous coronary intervention (PCI). CASE DESCRIPTION: A 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. CONCLUSIONS: This 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.

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

BACKGROUND: The 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 PRESENTATION: A 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. CONCLUSIONS: We 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.

Combined central retinal vein occlusion and cilioretinal artery occlusion as the initial presentation of frosted branch angiitis: a case report and literature review.

PURPOSE: To report a case of combined central retinal vein occlusion (CRVO) with cilioretinal artery occlusion (CLRAO) that heralded the development of frosted branch angiitis (FBA). CASE REPORT: A 25-year-old healthy male presented with sudden painless visual loss in his left eye with a visual acuity (VA) of 20/300. Fundus exam and fluorescein angiography showed signs of combined CRVO and CLRAO. Without treatment, his vision gradually improved until it reached 20/30 within four months. Five months after initial presentation, he returned with severe visual loss (20/400) in the same eye and a clinical picture of severe occlusive periphlebitis resembling a frosted branch angiitis pattern associated with severe macular edema. This was promptly and successfully treated with systemic steroids and immunosuppressive medications. CONCLUSION: CRVO in young population can have an unusual course and one should carefully rule out underlying uveitic etiologies in each visit. Clinical suspicion and close follow­up are required for early detection and timely management of FBA.

Does the Presence of the Cilioretinal Artery Affect the Incidence, Clinical Picture and Progression of Age-Related Macular Degeneration?

The aims of this study were to analyze the relationship between the presence of the cilioretinal artery (CRA) and the incidence, severity and progression of age-related macular degeneration (AMD) and to estimate the influence of the CRA on choroidal and retinal parameters. A total of 287 patients with AMD and 110 healthy controls were enrolled in the study. CRA occurrence was determined using color fundus images. AMD progression was assessed after 3 years. There was no difference in the incidence of CRA between the AMD and control groups (23.34% vs. 24.55%; p = 0.8). Lower-stage AMD was more frequently observed in eyes with the CRA than in eyes without the artery (p = 0.016). The CRA did not influence disease progression (p = 0.79). The CRA did not influence retinal and choroidal thickness and volume parameters or the retinal vessel caliber and functionality in either the AMD or control groups. There was no relationship between CRA presence and CFH Y402H and ARMS2 A69S risk variants. The results did not show a protective effect of the CRA on the incidence and progression of AMD. The CRA may affect the severity of AMD; however, the mechanism of this phenomenon is unclear.

Neovascular Glaucoma Complicating Central Retinal Artery Occlusion Despite Cilioretinal Artery Sparing.

This case report aims to describe a case of unilateral central retinal artery occlusion (CRAO) with cilioretinal artery sparing, which was complicated by neovascular glaucoma (NVG). A 75-year-old Indian woman with underlying normal tension glaucoma presented with the sudden onset of painless generalized blurring of the right eye's vision for a week. Her right eye vision was hand motion with the presence of a right relative afferent pupillary defect. Fundus examination revealed retinal whitening over the macula sparing the papillomacular bundle with generalized retinal arteriolar attenuation, which was suggestive of right CRAO with cilioretinal artery sparing. Systemic examination revealed high blood pressure (175/75 mmHg) without ocular bruit or audible murmur on auscultation. Optical coherence tomography of the macula showed inner retinal thickening over the temporal macula. Ultrasound carotid Doppler and computed tomography angiography of the carotid showed more than 75% stenosis over the right distal internal carotid artery. Unfortunately, she developed rubeosis iridis over her right eye two weeks after her presentation, which required pan-retinal photocoagulation. She subsequently progressed to NVG, requiring maximum anti-glaucoma medications to stabilize intraocular pressure. In conclusion, CRAO is a sight-threatening medical emergency. Thorough investigations are required to determine the underlying cause so that early intervention can be done to reduce the risk of a similar attack in the fellow eye and the risk of a cerebrovascular event or cardiac ischemia, which could be life-threatening. The presence of a cilioretinal artery does not prevent ocular neovascularization in CRAO. Hence, patients should also be closely monitored after the initial diagnosis to prevent devastating complications such as NVG.

Cilioretinal Artery Occlusion as the Presenting Manifestation of Left Atrial Myxoma.

Purpose: This work reports a case of left atrial myxoma presenting with cilioretinal artery occlusion. Methods: A case report is discussed. Results: A 57-year-old man was referred for acute vision loss in the left eye after a workup including electrocardiogram, magnetic resonance imaging of the brain without contrast, computed tomography angiography of the head and neck, erythrocyte sedimentation rate, and C-reactive protein had negative results. Examination revealed cilioretinal artery occlusion with visible emboli. Because an echocardiogram was not performed, the patient was referred to the emergency department. Echocardiogram revealed a large left atrial mass prolapsing into the left ventricle. The mass was excised, and pathology showed myxoma. Conclusions: This is the first case to our knowledge of isolated cilioretinal artery occlusion as the initial presentation of an atrial myxoma. Thorough and complete workup was crucial to averting further morbidity and mortality.

Clinical Features and Treatment Outcome of a Concurrent Central Retinal Vein Occlusion and Cilioretinal Artery Occlusion.

We describe the clinical features and treatment outcome of a patient with combined central retinal vein occlusion and cilioretinal artery occlusion. A 52-year-old female presented to our clinic with decreased vision in the right eye for 4 days. Visual acuity and intraocular pressure were count fingers at 2&1/2M and 14 mm Hg in the right and 20/20 and 16 mm Hg in the left eye, respectively. Funduscopic exam and optical coherence tomography (OCT) of the right eye confirmed the diagnosis of concurrent cilioretinal artery occlusion and central retinal vein occlusion with segmental macular pallor in the territory of the cilioretinal artery, corresponding marked inner retina thickening on OCT and signs of vein occlusion. The patient received an intravitreal injection of bevacizumab and at 1-month follow-up, vision improved to 20/30 with corresponding anatomical improvement. It is very important to recognize combined central retinal vein occlusion and cilioretinal artery occlusion as they could be treated with intravitreal injections of anti-vascular endothelial growth factors with favorable treatment outcomes.

Cilioretinal Artery Obstruction as a Complication of Retinitis Caused by Chikungunya Virus.

PURPOSE: The purpose of this case report is to describe the obstruction of cilioretinal artery in a patient with acute focal retinitis occurring due to Chikungunya virus (CHIKV) fever. CASE REPORT: A 45-year-old female presented with sudden and unilateral visual impairment from 2 days, which was preceded by a flu-like syndrome 2 weeks before. Multimodal imaging revealed focal retinitis in both eyes, which was complicated by cilioretinal artery obstruction in the left eye. Laboratory examinations confirmed the diagnosis of CHIKV fever and showed positive results (anti-CHIKV IgM and reverse transcriptase-polymerase chain reaction). The patient was treated with oral steroids. Following which, the patient showed a limited visual and anatomical recovery in the affected eye. CONCLUSIONS AND IMPORTANCE: CHIKV fever has been currently associated with atypical manifestations. This indexed report describes a unique perspective on CHIKV focal retinitis, complicated by cilioretinal artery obstruction, further leading to limited visual recovery.

Paracentral Acute Middle Maculopathy Associated With Cilioretinal Artery Insufficiency Following COVID-19 Vaccination in a Young Patient.

We aim to present a unique case of unilateral paracentral acute middle maculopathy (PAMM) associated with cilioretinal artery insufficiency following the coronavirus disease 2019 (COVID-19) vaccination. A 28-year-old male complained of a sudden blurring of vision in his left eye 40 days after receiving the second dose of COVID-19 immunization. The optical coherence tomography revealed a diffuse paracentral area of hyper-reflective change in the inner plexiform layer and an increase in the inner nuclear layer volume, consistent with PAMM along the course of the cilioretinal artery. PAMM has been connected to an assortment of retinal vasculature anomalies. Considering COVID-19 vaccination, we hypothesize that the immunogenic cascade following vaccination dysregulated coagulation and led to retinal vascular thrombosis. However, the link between COVID-19 vaccination and retinal vascular occlusion disease remains unknown.

Surgical mobilization of an arterial embolus in cilioretinal artery occlusion.

We describe an effective surgical approach for the management of cilioretinal artery occlusion. A 23-G pars plana vitrectomy assisted with two soft tip cannulas was performed. One cannula pressed the cilioretinal artery branch directed toward the macula, distal to the location of the embolus, whereas the other cannula was used to gently swipe over the cilioretinal artery proximal to the occlusion. Anatomical and functional outcomes were evaluated by fundus examination, fluorescein angiography, Goldmann visual field, and best-corrected visual acuity (BCVA). It was possible to mobilize the embolus by mechanical displacement with 23-G soft-tip cannulas and disintegrate it, preventing the passage toward the branch directed to the macula. Restoration of retinal circulation was confirmed by fluorescein angiogram. The patient recovered his previous documented BCVA and visual field. The described technique can be considered as a new possibility for achieving a solution to cilioretinal artery occlusion or any other retinal artery occlusion.

Spontaneous Visual Acuity Recovery After Cilioretinal Artery Occlusion Combined With Central Retinal Vein Occlusion in a Young Patient.

This work illustrates the case of cilioretinal artery occlusion (CilRAO) combined with central retinal vein occlusion (CRVO) in a young patient that resolved spontaneously. A 17-year-old male with an unremarkable medical history presented with acute painless loss of vision unilaterally. Upon ophthalmologic examination, retinal hemorrhages in all four quadrants and edema extending from the optic disc to the macula were reported. Using optical coherence technology (OCT) imaging and fundus fluorescein angiography (FFA), combined CilRAO/CRVO was diagnosed. The full medical evaluation was unremarkable. Within the next month, the patient had regained full visual acuity (VA) in the affected eye, and the retinal findings resolved without intervention. Combined CilRAO/CRVO is a common vascular pathology in young, otherwise healthy patients. It is commonly considered a hemodynamic block in the capillary bed, hence its hopeful prognosis. Nonetheless, several risk factors have been proposed that need to be eliminated. Despite the initial alarming symptoms, young patients with CilRAO/CRVO should be monitored closely, and intervention should be resorted to when necessary.