Neuro-Ophthalmology Case #1

Authors: Paige Campbell (1), Dr. Imran Jivraj (1), Dr. Alex Kaplan (2)

Affiliations: (1) University of Alberta (2) University of Toronto

ID: 72M; sudden vision loss OS with 1 month history of jaw pain.

Past Ocular History:

  • Pseudophakic OU

Ocular gtts: None

Relevant Medical History:

  • Hypertension

  • Normal CTA head and neck with minimal carotid disease 3 weeks prior

  • ESR 110, CRP 98

Medications: Amlodipine

  • IVFA at 22 seconds 

    Choroidal Phase.

    Key features:

    • Patchy, delayed filling of the choroid with prominent nasal ischemia.

  • IVFA at 26 seconds

    Arterial phase

    • Continued and more prominent patchy, incomplete — mottled background choroidal fluorescence with nasal choroidal ischemia (wedge like pattern)

    • Delayed and incomplete optic nerve head perfusion

    • Prolonged ateriovenous transit time (incomplete arterial filling at 26 seconds)

    • There is only one hyperfluoresent vessel. This is showing filling of a cilioretinal artery. It represents an anatomical variant of the posterior ciliary circulation, only present in 15-30% of the population. The artery fills early/first because it usually arises from the posterior ciliary arteries (choroidal circulation), rather than the central retinal artery.

  • IVFA at 33 seconds

    Early Arterio-Venous Phase with Laminar Flow

    • Continued mottled background of choroidal fluorescence with nasal choroidal ischemia (wedge like pattern)

    • Prolonged ateriovenous transit time (33 seconds with incomplete arterio-venous filling)

  • IVFA at 2 minutes

    Late Arteriovenous Phase

    • Although there was evidence of choroidal ischemia, retinal arterial filling is complete, but remarkably slow.

    • No obvious emboli, plaque or arterio-venous obstruction.

    • Slight hyperfluoresence around the disc (physiologic versus inflammatory)

  • The diagnosis is arteritic anterior ischemic optic neuropathy (AAION) secondary to giant cell arteritis (GCA). IVFA shows delayed choroidal filling with prolonged arteriovenous filling - a hallmark of posterior ciliary artery ischemia in GCA. These findings, combined with sudden vision loss, proceeding jaw pain, elevated inflammatory serological markers, a hyper-reflective OCT and subtle cherry red spot on clinical examination all point towards AAION related to giant cell arteritis.

  • The differential diagnosis for AAION with delayed choroidal filling on IVFA includes:

    • Severe Central Retinal Artery Occlusion (CRAO) — may rarely show delayed choroidal filling if ciliary circulation is affected

    • Ophthalmic Artery Occlusion — leads to both retinal and choroidal nonperfusion.

    • Hypotensive Ischemic Optic Neuropathy — peri-operative or shock-related, usually bilateral

    • Embolic Posterior Ciliary Artery Occlusion — less common, often sectoral

    • Confirm choroidal hypoperfusion, a hallmark of arteritic anterior ischemic optic neuropathy (AAION)

    • Assess for other causes of vision loss, such as central retinal artery occlusion or retinal vasculitis

    • Support the clinical suspicion of GCA in a patient with acute vision loss and systemic symptoms, aiding prompt and time sensitive treatment decision with intravenous steroids.

    1. Hayreh SS. Giant cell arteritis: Its ophthalmic manifestations. Indian J Ophthalmol. 2021 Feb;69(2):227-235. doi:10.4103/ijo.IJO_1681_2012.

    2. Vodopivec I, Rizzo JF. Ophthalmic manifestations of giant cell arteritis. Rheumatology (Oxford). 2018 Feb 1;57(suppl_2):ii63-ii72. doi:10.1093/rheumatology/kex428345.

    3. Yu E, Chang JR. Giant Cell Arteritis: Updates and Controversies. Front Ophthalmol. 2022 Mar 17;2:848861. doi:10.3389/fopht.2022.848861‌