Ocular Ischaemic Syndrome

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Disease

  • Chronic ocular hypoperfusion
  • Cause: marked ipsilateral carotid stenosis (>90%), mostly internal carotid artery, typically due to atherosclerosis, rarely dissection, or giant cell arteritis

Clinical Presentation

  • Vision loss over several weeks to months, occasionally sudden or transient (amaurosis fugax)
  • Ocular and periocular pain
  • Diffuse episcleral injection
  • Corneal edema
  • Anterior chamber flare with few or no cells (“ischaemic pseudoiritis”)
  • Iris atrophy
  • Rubeosis iridis
  • Retina: venous dilation, narrowing of arterioles, haemorrhages, microaneurysms, papilledema, cotton wool spots, neovascularisation of the disc / elsewhere
  • IOP often not elevated due to decreased ciliary body function

Differential Diagnoses

Work-up

  • Fluorescein angiography: delayed filling of the choroid and retina, prolonged arteriovenous passage time, diffuse leakage
  • Carotid duplex ultrasound: stenosis of the internal carotid artery

Treatment

  • Consider reducing intraocular pressure -> to increase perfusion pressure
  • Panretinal photocoagulation -> evaluate if rubeosis iridis or retinal neovascularisation is present
  • In case of neovascular glaucoma: medical or surgical therapy
  • Carotid endarterectomy (not possible with 100% stenosis) -> postoperatively the IOP might be elevated due to increased ciliary body perfusion
  • Treatment of cardiovascular risk factors

Sources

  • EyeWiki Ocular Ischemic Syndrome
  • The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7th Edition (2016)
  • Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8th Edition (2015)