Cilioretinal Artery Occlusion Associated with Progressive Optic Nerve Head Ischemic Involvement: A Case Report

Summary

This case report describes cilioretinal artery occlusion associated with progressive optic nerve head ischemic involvement. It highlights the value of repeated optic disc assessment, OCT, FFA, and visual function monitoring when optic nerve involvement is suspected in the setting of retinal arterial ischemia.

Abstract

Cilioretinal artery occlusion is an uncommon retinal vascular emergency that can cause acute monocular visual loss, particularly when the macular region is involved. Non-arteritic anterior ischemic optic neuropathy is another ischemic disorder involving the optic nerve head and is usually associated with optic disc edema and sudden painless visual impairment. Although both conditions involve vascular territories related to the posterior ciliary circulation, the temporal and pathophysiological relationship between cilioretinal artery occlusion and optic nerve head ischemia remains incompletely defined. This case describes a 62-year-old woman with poorly controlled type 2 diabetes mellitus who presented with acute painless visual loss in the right eye. Initial fundus examination showed optic disc pallor and edema, grayish-white retinal edema extending from the optic disc to the macular region, macular involvement, and arterial attenuation. Multimodal ophthalmic evaluation, including fundus photography, Humphrey visual field testing, optical coherence tomography, visual evoked potential testing, and fundus fluorescein angiography, supported a diagnosis of cilioretinal artery occlusion with concurrent and progressive optic nerve head ischemic involvement, clinically consistent with suspected non-arteritic anterior ischemic optic neuropathy. Because the time window for thrombolysis had passed, conservative management for retinal arterial occlusion was initiated. After progression of optic disc edema and angiographic evidence of optic nerve head leakage, parabulbar triamcinolone was administered after consideration of systemic steroid-related risks. Partial reduction of optic disc edema and limited visual improvement were observed during follow-up; however, the single-case design and concurrent treatments preclude any conclusion regarding treatment efficacy. This case emphasizes that optic disc changes in cilioretinal artery occlusion should be monitored dynamically and interpreted using multimodal imaging rather than assumed to represent a fixed sequential diagnostic pattern.

Introduction

Cilioretinal artery occlusion (CLRAO) is an ophthalmic emergency that may result in acute monocular visual loss. The severity of visual impairment depends on the caliber of the occluded vessel and the extent of its perfusion territory. The cilioretinal artery is an anatomical variant present in approximately 15%–30% of individuals. It originates from the posterior ciliary circulation and supplies the inner retinal layers; in some patients, it also contributes to macular perfusion¹. The reported causes of CLRAO are heterogeneous and include embolism, trauma, coagulation abnormalities, and collagen vascular diseases².

Non-arteritic anterior ischemic optic neuropathy (NAION) is characterized by sudden, painless, unilateral visual loss and is typically accompanied by segmental or diffuse optic disc edema on fundus examination^3,4,5. NAION is generally considered to result from hypoperfusion or infarction of the anterior portion of the optic nerve head, which is mainly supplied by the short posterior ciliary arteries. Although the precise pathogenesis remains incompletely understood, small-vessel insufficiency affecting the anterior optic nerve circulation is thought to play a central role⁶.

CLRAO and NAION involve closely related vascular territories because both are associated with the posterior ciliary circulation. The anatomical and hemodynamic relationship between the cilioretinal and choroidal vasculature suggests that impaired perfusion within this shared circulation may theoretically cause sequential or simultaneous ischemic injury to the inner retina and optic nerve head⁷. Previous reports have described an association between CLRAO and NAION, but such cases remain rare, and the available evidence is largely limited to individual case observations⁸. Therefore, the temporal relationship and potential pathogenic link between these two entities remain insufficiently defined. In particular, it is often difficult to determine whether optic nerve head involvement represents a concurrent ischemic process, a progressive change after retinal arterial occlusion, or an overlapping manifestation of a shared vascular disturbance. The present case contributes additional clinical insight by documenting serial fundus findings, OCT changes, FFA features, and visual function assessment in a patient with CLRAO and progressive optic nerve head ischemic involvement.

This case report presents the clinical evaluation, multimodal ophthalmic imaging findings, treatment considerations, and follow-up of a patient with cilioretinal artery occlusion and progressive optic nerve head ischemic involvement. This case was considered appropriate for presentation because optic disc edema and pallor were present at the initial examination, while subsequent clinical and angiographic changes suggested progression of optic nerve head ischemia. This clinical course highlights a practical diagnostic problem: optic nerve involvement in CLRAO may not be fully characterized by a single baseline examination. Serial fundus assessment combined with OCT, FFA, and visual function monitoring may provide additional value over isolated fundus examination by helping correlate retinal ischemia, optic disc leakage, structural changes, and functional impairment. The didactic purpose of this case is to illustrate three practical points: optic nerve involvement should be suspected when optic disc edema, pallor, or persistent visual dysfunction accompanies retinal arterial ischemia; repeated optic disc assessment is needed when disc edema progresses after initial presentation; and multimodal imaging, especially OCT and FFA, helps correlate retinal ischemia, optic disc leakage, and visual function changes.

Case Presentation:

A 62-year-old woman presented to the ophthalmology outpatient department with a 2-day history of painless visual loss in the right eye. The visual impairment occurred after she woke up from a nap. Her medical history included type 2 diabetes mellitus for 10 years and a gastric ulcer for 5 years. She had been treated with metformin sustained-release tablets, but glycemic control was unsatisfactory. No relevant family history or social history was reported. On initial ophthalmic examination, best-corrected visual acuity (BCVA) was hand motion at 10 cm (HM/10 cm) in the right eye and 10/40 in the left eye. A relative afferent pupillary defect was present in the right eye. Fundus examination showed a pale and edematous optic disc, grayish-white retinal edema extending from the optic disc toward the macular region with macular involvement, and attenuation of the retinal artery.

Diagnosis, Assessment, and Plan:

Based on the acute painless monocular visual loss, retinal whitening in the cilioretinal artery territory, arterial attenuation, and optic disc edema, the patient was admitted for emergency evaluation of retinal arterial occlusion with suspected optic nerve head involvement. The assessment focused on confirming the retinal arterial occlusion pattern, evaluating the extent of optic nerve involvement, excluding intracranial or inflammatory causes of acute visual loss, and identifying systemic vascular or embolic risk factors. Humphrey visual field testing showed severe visual field loss in the right eye. Optical coherence tomography demonstrated edema and thickening of the inner retinal layer with loss of the foveal contour, as well as marked optic disc edema. Visual evoked potential testing showed a preserved P2 peak at 1 Hz with reduced amplitude in the right eye. Blood pressure was 148/85 mmHg, and fasting blood glucose was 8.23 mmol/L. Head and orbital magnetic resonance imaging showed no intracranial mass lesion, inflammatory change, or cerebrovascular abnormality. Carotid artery ultrasonography revealed uneven bilateral carotid intima and plaque formation at the bifurcation of the right common carotid artery, with less than 25% vascular stenosis. Renal function, coagulation testing, and electrocardiography showed no major abnormalities. Neurological consultation did not indicate acute stroke or the need for emergency endovascular intervention. The patient had no history of smoking or alcohol consumption. She had a 3-year history of coronary heart disease but was not receiving regular medication. No previous antiplatelet or anticoagulant therapy was reported. HbA1c, lipid profile, echocardiography, and cardiac rhythm monitoring were not available, which limited the systemic vascular and embolic-source evaluation. Erythrocyte sedimentation rate and C-reactive protein were within the normal range, reducing clinical suspicion for arteritic ischemic optic neuropathy.

The differential diagnosis included arteritic AION, inflammatory optic neuropathy, compressive optic neuropathy, diabetic papillopathy, and embolic retinal arterial occlusion. Arteritic AION/GCA was considered clinically less likely because the patient had no headache, temporal tenderness, scalp tenderness, jaw claudication, fever, fatigue, or weight loss, and ESR and CRP were within the normal range. Inflammatory or compressive optic neuropathy was considered less likely because cranial and orbital MRI showed no inflammatory lesion, orbital mass, or intracranial compressive lesion. Diabetic papillopathy was considered but was less favored because of the acute severe visual loss, relative afferent pupillary defect, retinal whitening in the cilioretinal artery territory, and FFA evidence of retinal arterial filling delay and optic disc leakage. Embolic retinal arterial occlusion remained a consideration; however, carotid ultrasonography showed less than 25% stenosis, electrocardiography showed no major abnormality, and neurological consultation did not indicate acute stroke.

These findings supported cilioretinal artery occlusion with progressive optic nerve head ischemic involvement. Because the therapeutic window for intravenous thrombolysis or emergency endovascular intervention had passed, conservative management for retinal arterial occlusion was initiated, including supportive oxygen therapy, intraocular pressure reduction, ocular microcirculation therapy, neurotrophic treatment, antiplatelet therapy, lipid-lowering therapy, and glucose control. After progression of optic disc edema and evidence of optic nerve head ischemia, parabulbar triamcinolone was considered as a local anti-edema approach after weighing systemic steroid-related risks instead of systemic corticosteroid therapy because of the patient’s poorly controlled diabetes, older age, and history of gastric ulcer.

Protocol

The study was reviewed and approved by the Medical Ethics Committee of Xiangyang Central Hospital on May 8, 2026 (approval number: 2026-IIT-067). Written informed consent for publication of the anonymized clinical details and clinical images was obtained from the patient.

1. Initial evaluation of acute painless monocular visual loss

1. The time of symptom onset, mode of onset, affected eye, pain status, and associated neurologic or systemic symptoms were recorded. Specific inquiry was made regarding headache, temporal tenderness, scalp tenderness, jaw claudication, fever, fatigue, weight loss, transient ischemic attack, stroke, atrial fibrillation, coronary heart disease, smoking, alcohol consumption, diabetes, hypertension, and current antiplatelet or anticoagulant therapy.
2. Relevant ocular history and systemic comorbidities were documented, including diabetes control, cardiovascular disease, gastric ulcer, medication adherence, and contraindications to systemic corticosteroid therapy.
3. Best-corrected visual acuity (BCVA) was measured in both eyes using a standard notation system. For non-chart visual acuity, the testing distance was recorded, such as hand motion at 10 cm (HM/10 cm), hand motion at 40 cm (HM/40 cm), or finger counting at 10 cm (FC/10 cm).
4. Pupillary examination was performed, and the presence of a relative afferent pupillary defect was documented.
5. Anterior segment examination and intraocular pressure measurement were performed in both eyes before treatment. Baseline intraocular pressure was recorded for later safety comparison.
6. Dilated fundus examination and fundus photography were performed. The optic disc, retinal vessels, macular region, peripapillary retina, and retinal whitening pattern were assessed.
7. Cilioretinal artery occlusion was suspected when retinal whitening involved the cilioretinal artery territory, especially when the lesion extended from the optic disc toward the macular region and was accompanied by attenuation or delayed filling of the corresponding artery.

2. Multimodal ophthalmic imaging and functional testing

1. Macular optical coherence tomography (OCT) was performed to evaluate inner retinal edema, retinal thickening, and loss of the normal foveal contour. In this case, OCT was performed using a Spectral-domain OCT system.
2. Optic disc OCT or retinal nerve fiber layer (RNFL) scanning was performed to evaluate optic nerve head edema. Available RNFL or optic disc parameters were recorded when they were available from the device report.
3. Humphrey visual field testing was performed to assess visual field loss. In this case, testing was performed using an automated visual field analyzer. The testing protocol, reliability indices, and main abnormal findings, including severe generalized depression, altitudinal defect, or other field-loss patterns, were recorded when available.
4. Visual evoked potential (VEP) testing was performed to evaluate visual pathway function. In this case, VEP was performed using a visual evoked potential recording system. The stimulation protocol, P-wave latency, amplitude, and interocular asymmetry were recorded when available.
5. Fundus fluorescein angiography (FFA) was performed when retinal arterial occlusion, delayed perfusion, embolic disease, or optic disc leakage required further evaluation. In this case, FFA was performed using a fundus fluorescein angiography imaging system. Arterial, venous, and late phases were acquired and interpreted. The affected vessel, arterial filling delay, segmental filling defect, venous filling delay, capillary nonperfusion, and optic disc leakage were recorded.
6. Multimodal findings were used to define diagnostic checkpoints. Cilioretinal artery occlusion was diagnosed when retinal whitening was located in the cilioretinal artery perfusion territory, and FFA showed delayed or segmental filling of the corresponding artery. Concurrent or progressive optic nerve head ischemic involvement was suspected when optic disc edema, optic disc pallor, relative afferent pupillary defect, visual field loss, VEP abnormality, OCT evidence of disc edema, or FFA optic disc leakage was present.

3. Systemic vascular, inflammatory, and embolic-source evaluation

1. Blood pressure and blood glucose were measured at presentation. Blood tests were obtained according to local emergency and vascular-occlusion protocols, including inflammatory markers and vascular risk assessment.
2. Arteritic AION/GCA risk was assessed by documenting headache, temporal tenderness, scalp tenderness, jaw claudication, fever, fatigue, weight loss, polymyalgia rheumatica symptoms, ESR, and CRP. Temporal artery ultrasound or biopsy was arranged when clinical suspicion for GCA remained significant.
3. Cranial and orbital MRI was performed to evaluate compressive, inflammatory, ischemic, or intracranial causes of acute visual loss.
4. Carotid artery ultrasonography was performed to assess carotid plaque and stenosis.
5. Electrocardiography was performed and neurological consultation was requested when retinal arterial occlusion or embolic disease was suspected. Echocardiography and cardiac rhythm monitoring were considered when embolic-source evaluation was clinically indicated or available.

4. Acute management of suspected retinal arterial occlusion

1. It was determined whether the patient presented within the local therapeutic window for thrombolysis or emergency endovascular intervention. Neurological or stroke-team evaluation was requested when appropriate.
2. If the patient presented outside the treatment window or was not eligible for reperfusion therapy, conservative treatment was initiated according to local emergency protocols for retinal arterial occlusion.
3. Oxygen therapy was administered according to institutional practice. In this case, a gas mixture of 95% oxygen and 5% carbon dioxide was inhaled for 10–15 min per session, repeated once per hour for 2–4 h.
4. Intraocular pressure-lowering therapy was administered when it was used to support retinal perfusion. In this case, brinzolamide eye drops were applied to the affected right eye, one drop three times daily.
5. Ocular microcirculation therapy was administered when clinically selected. In this case, compound anisodine hydrobromide injection (2 mL) was administered by right temporal peribulbar injection once daily.
6. Neurotrophic, antiplatelet, lipid-lowering, and glucose-lowering therapies were administered according to the patient’s systemic risk profile and local practice. In this case, mecobalamin tablets 0.5 mg were given orally three times daily, atorvastatin calcium 20 mg was given orally once nightly, aspirin enteric-coated tablets 100 mg were given orally once daily, and metformin 0.5 g was given orally twice daily.

5. Local corticosteroid administration and safety monitoring

1. The risk-benefit profile was assessed before corticosteroid treatment. Diabetes control, gastric ulcer history, age, intraocular pressure, infection risk, and the uncertain benefit of steroid therapy for NAION were considered.
2. The patient was prepared in the supine position. The periocular skin was disinfected with povidone-iodine solution before injection.
3. Parabulbar triamcinolone was administered to the affected eye after periocular antisepsis. In this case, triamcinolone acetonide 40 mg was administered once by right parabulbar injection.
4. Visual acuity, fundus findings, intraocular pressure, blood glucose, ocular pain, infection signs, and other injection-related complications were monitored after injection.
5. Whether post-injection intraocular pressure elevation, marked hyperglycemia, infection, or other complications occurred was documented.

6. Follow-up assessment and outcome documentation

1. Visual acuity and fundus findings were reassessed within the early post-treatment period when optic disc edema or retinal ischemia was progressing.
2. OCT was repeated to assess changes in macular edema, inner retinal structural change, optic disc edema, and RNFL parameters when available.
3. Visual field testing and VEP were repeated when the patient’s visual function permitted and when these tests were clinically available.
4. Objective endpoints were recorded, including BCVA, optic disc appearance, macular OCT findings, optic disc OCT/RNFL findings, visual field status, VEP findings, intraocular pressure, blood glucose stability, and adverse events.
5. Continue follow-up to document longer-term visual and structural outcomes.

Results

The clinical timeline was as follows: symptom onset occurred on June 18, 2025; the patient first presented and was admitted on June 20, 2025; initial fundus photography, macular OCT, optic disc OCT, ocular ultrasonography, cranial and orbital MRI, chest radiography, and electrocardiography were performed on June 20, 2025; visual field testing, laboratory testing, and carotid ultrasonography were performed on June 21, 2025; clinical worsening was noted on June 22, 2025; FFA, VEP testing, and parabulbar triamcinolone injec...

Discussion

CLRAO accompanied by optic nerve head ischemic involvement is a recognized but clinically important presentation. Previous reports have described overlap between retinal arterial occlusion and anterior ischemic optic neuropathy in different clinical contexts, including central retinal artery obstruction mimicking ischemic optic neuropathy and retinal arterial occlusion occurring with optic nerve ischemic disease in systemic or local vascular disorders^9,10,

Materials

List of materials used in this article

Name	Company	Catalog Number	Comments
Aspirin enteric-coated tablets	Bayer Healthcare Company Ltd.	Not available	Used for antiplatelet therapy
Atorvastatin calcium tablets	Qilu Pharmaceutical Co., Ltd.	Not available	Used for lipid-lowering therapy
Brinzolamide eye drops	Not available	Not available	Used for intraocular pressure reduction
Carotid ultrasound system	Mindray / GE	Mindray A20 / GE E11	Used for carotid vascular assessment
Compound anisodine hydrobromide injection	Shenyang Xingqi Pharmaceutical Co., Ltd.	Not available	Used for ocular microcirculation therapy
Cranial and orbital MRI system	Not available	Not available	Used for cranial and orbital imaging; specific model was not available from the clinical record
Cranial MRI system	United Imaging Healthcare	uMR 790	Used for cranial magnetic resonance imaging.
Fundus camera	Carl Zeiss Meditec	ZEISS CLARUS 500	Used for fundus photography
Fundus fluorescein angiography system	Heidelberg Engineering	Heidelberg Spectralis HRA	Used for fundus fluorescein angiography
Humphrey visual field analyzer	Carl Zeiss Meditec	ZEISS Humphrey 720i	Used for visual field testing
Mecobalamin tablets	North China Pharmaceutical Co., Ltd.	0.5 mg; catalog number not available	Used as neurotrophic therapy
Metformin tablets	Penglai Nuokang Pharmaceutical Co., Ltd.	0.5 g; catalog number not available	Used for glucose-lowering therapy
Optical coherence tomography system	Heidelberg Engineering	Heidelberg Spectralis OCT	Used for macular and optic disc OCT
Orbital MRI system	GE Healthcare	SIGNA Architect 3.0T	Used for orbital magnetic resonance imaging.
Povidone-iodine solution	Not available	Not available	Used for periocular antisepsis before parabulbar injection
Sterile syringe and needle for parabulbar injection	Not available	Not available	Used for parabulbar triamcinolone injection
Triamcinolone acetonide injection	Kunming Jida Pharmaceutical Co., Ltd.	40 mg; catalog number not available	Used for parabulbar injection
Visual evoked potential system	Roland Consult	RETI-port/scan 21	Used for visual evoked potential testing