Introduction: Anterior ischemic optic neuropathy (AION) is an ischemic disorder of the optic nerve and a common cause of acute, painless, permanent vision loss. It is divided into two types: arteritic AION (AAION) and non-arteritic anterior ischemic optic neuropathy (NAION). Although subretinal fluid associated with optic disc edema has been reported in cases of NAION, it is rarely described in AAION. Case Presentation: An 86-year-old female with a history of polymyalgia rheumatica presented with sudden vision loss in the left eye. Initial examination revealed left pallid optic disc edema with peripapillary hemorrhages. Optical coherence tomography (OCT) of the left macula showed intraretinal and submacular fluid. The patient was started on 50 mg of oral prednisone daily. The diagnosis of giant cell arteritis (GCA) was later confirmed with a positive temporal artery biopsy. Three weeks after presentation, an OCT was completed which demonstrated complete resolution of the intraretinal and submacular fluid. Although the presence of both intraretinal and subretinal fluid has been previously documented in cases of NAION, it is rarely reported in a patient with GCA. Conclusion: This is a newly described case of subretinal and intraretinal fluid in a patient with AAION. We postulate that the pathophysiology behind this is mediated by associated choroidal ischemia leading to altered permeability. OCT is an important imaging modality allowing for signs of GCA to be better characterized.

Anterior ischemic optic neuropathy (AION) is an ischemic disorder of the optic nerve and a common cause of acute, painless, permanent vision loss. Etiologically and pathogenetically, it is divided into two types: arteritic AION (AAION) and non-arteritic AION (NAION). While the pathophysiology of NAION is still a subject of debate, the pathophysiology of AAION is a result of endothelial cell inflammation, subsequent thrombosis and occlusion of the arteries that supply blood to the optic nerve head (ONH) [1].

Giant cell arteritis (GCA), also known as temporal arteritis, is an inflammatory vasculitis of the medium and large vessels. The most common ophthalmic complication of GCA is AAION. Although subretinal fluid associated with optic disc edema has been reported in cases of NAION, it is rarely described in AAION. We report a case of an 86-year-old patient who had submacular and intraretinal fluid associated with AAION.

An 86-year-old woman was referred urgently to ophthalmology for sudden left eye vision loss. She had a past medical history of polymyalgia rheumatica diagnosed 3 years prior and treated with 2.5 years of Prednisone, starting at 20 mg, and tapered to 10 mg. She also had osteoporosis and a previous hip replacement. Her medications included aspirin 81 mg daily and denosumab injections.

She reported a 2-week history of initially inferior vision loss in the left eye that progressively involved her entire visual field. She initially had a mild headache around the time of vision loss, but this did not persist. She denied jaw claudication, scalp tenderness, fevers, myalgias, and neck pain. She presented to the emergency room and had a CT of the head and orbits with contrast that was reported as normal. Bloodwork was performed and showed a hemoglobin of 113 g/L, platelets 247 × 109/L, erythrocyte sedimentation rate 48 mm/h, and C-reactive protein was 12.4 mg/L (normal less than 8). Ophthalmological examination showed a visual acuity of 20/20 OD and light perception OS. There was a left RAPD and anterior segment examination was normal. Dilated fundus examination showed a normal appearing right optic nerve with a cup-to-disc ratio of 0.5 and there was left pallid optic disc edema with peripapillary hemorrhages (shown in online suppl. Fig. 1a, b; for all online suppl. material, see https://doi.org/10.1159/000539147). Optical coherence tomography (OCT) of the retinal nerve fiber layer was normal in the right eye with an average thickness of 86 μm OD and was elevated in the left eye with an average thickness of 200 μm (shown in online suppl. Fig. 2). OCT of the macula showed intraretinal and submacular fluid OS (shown in Fig. 1). A diagnosis of GCA was suspected due to the severity of vision loss, the appearance of the pallid optic disc edema and her elevated CRP. She was started on Prednisone 1 mg/kg (50 mg PO daily). Six days later, a temporal artery biopsy was performed and was positive for GCA. OCT at this time demonstrated resolving submacular fluid (shown in Fig. 2a, online suppl. Fig. 3). Three weeks after initial presentation, the patient returned for subsequent follow-up. An additional OCT was completed, which showed complete resolution of the intraretinal and submacular fluid (shown in Fig. 2b, online suppl. Fig. 4). The patient had a final follow-up 3 months after initial presentation. At that time, the visual acuity was 20/20 OD and hand motions OS, there was a left RAPD and optic atrophy in the left eye. The macula was flat and there was no residual subretinal fluid. The CARE Checklist has been completed by the authors for this case reported, attached as online supplementary material.

Fig. 1.

Optical coherence topography of the macula OS prior to treatment with prednisone.

Fig. 1.

Optical coherence topography of the macula OS prior to treatment with prednisone.

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Fig. 2.

Optical coherence topography of the macula OS. a After a 6-day course of 50 mg oral prednisone daily. b After a 3-week course of 50 mg oral prednisone daily.

Fig. 2.

Optical coherence topography of the macula OS. a After a 6-day course of 50 mg oral prednisone daily. b After a 3-week course of 50 mg oral prednisone daily.

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All data generated or analyzed during this study are included in this article. The data are not publicly available on legal or ethical grounds; further, inquiries can be directed to the corresponding author.

We report a case of GCA with rare OCT findings of intraretinal and submacular fluid. This finding was made possible by the use of OCT as the fluid was not readily visible on fundus examination. The fluid was detected both in the subretinal and intraretinal compartments and responded rapidly to steroid treatment.

The presence of both intraretinal and subretinal fluid has been previously documented in cases of NAION. In a prospective multicenter study of 76 patients with NAION, macular OCT demonstrated that half of the patients presented with subretinal fluid, and 10% of the patients had fluid extending into the subfoveal space [2]. Hedges et al. [2] hypothesized that these findings of subretinal fluid accumulation in NAION are multifactorial. Decreased perfusion of the optic nerve may induce axoplasmic stasis, axonal swelling, and thus compromise the vascular supply to the ONH. The resulting ischemia may lead to a leakage of fluid from peripalliary vessels, preventing the retinal pigment epithelium from reabsorbing excess fluid and resulting in fluid accumulation in the subretinal space [2]. A more recent retrospective, single-center study by Chapelle et al. [3] of 32 patients with NAION discovered that 46.7% had subretinal fluid, and 20% had subfoveal fluid on OCT. This study theorized that venous hypertension caused by the optic disc edema induces transudation. They proposed that the volume of transudate generated at the optic disc is the critical factor, rather than dysfunction of retinal mechanisms, determining reabsorption [3].

To date, there is only been one documented case of subretinal fluid in a patient with GCA [4]. However, the patient in this report also presented with a nodular posterior scleritis. Posterior scleritis has previously been reported to cause subretinal fluid and this could not be ruled out as a cause of the subretinal fluid. For our case of isolated GCA, we hypothesize that the pathophysiology of the subretinal fluid is related to choroidal ischemia, which leads to reduced effectiveness of the retinal pigment epithelium. The hypoperfusion of the ONH and surrounding retina, as well as the spillover from the optic disc edema, may also lead to extravasation of fluid from the capillaries. Muller cells may become saturated and decrease the capacity for the retinal pigment epithelium to resorb excess fluid resulting in the development of subretinal fluid. There was a rapid improvement of the fluid with steroid treatment, indicating that it is likely not just a result of the adjacent optic disc edema.

In summary, this is a newly described case of subretinal and intraretinal fluid in a patient with AAION. We postulate that the pathophysiology behind this is mediated by associated choroidal ischemia leading to altered permeability. OCT is an important imaging modality allowing for the manifestations of GCA to be better characterized.

This research was conducted ethically in accordance with the World Medical Association Declaration of Helsinki. Ethical approval is not required for this study in accordance with local or national guidelines. Written and informed consent was obtained from the patient for publication of the details of their medical case and any accompanying images.

There are no financial or nonfinancial competing interests for all authors of this manuscript. The authors have no conflicts of interest to declare.

This study was not supported by any sponsor or funder.

Both authors (D.S. and J.M.) contributed to study conception based on a patient visit, analysis, and interpretation of the results and manuscript preparation.

All data generated or analyzed during this study are included in this article and its online supplementary material. The data are not publicly available on legal or ethical grounds. Further inquiries can be directed to the corresponding author.

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