Orbital Infarction Syndrome

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 by Shannon S. Joseph, M.D. on April 30, 2023.

Disease Entity


Orbital infarction syndrome has been described as ischemia of all intraorbital and intraocular structures. Due to the vast anastomotic vascular supply of the orbit, it is a rare occurrence.


There are many described etiologies in the literature that are listed below.

  • Common carotid artery occlusion—most common
  • Carotid artery dissection
  • GCA
  • Mucormycosis
  • Myelofibrosis
  • Ruptured anterior choroidal artery aneurysm
  • Surgical complications
  • Mechanical thrombectomy 2/2 emboli s/p acute ischemic stroke
  • Status post sclerotherapy for facial vascular malformations
  • Trauma
  • Compression—S/p bifrontal craniotomy, prolonged compression from unconsciousness 2/2 drug use (cocaine/heroin) 
  • Sickle Cell Disease


Orbital infarction syndrome develops secondary to hypoperfusion of the ophthalmic artery and its branches, which includes the internal carotid artery, ophthalmic artery, ocular and orbital branches of the ophthalmic artery including the central retinal artery and ciliary arteries. There are anastomoses with collateral circulation between the ophthalmic artery and branches of the external carotid. Ophthalmic artery occlusion alone will likely not cause these symptoms. For orbital infarction to occur, it requires collateral blood flow compromise in addition to develop ophthalmic artery compromise. The presentations can vary. In addition, there is retinal infarction (central retinal artery compromise), anterior ischemic optic neuropathy, and choroidal ischemia (compromised posterior ciliary arteries), extraocular muscle or cranial nerve  impairment, and anterior segment ischemia (long ciliary artery compromise).  The central retinal artery and short posterior ciliary arteries are the only terminal branches of the ophthalmic artery, so only direct hypoperfusion of all its branches will lead to orbital ischemia.



It is described as acute onset with symptoms of eye pain and acute blindness. Diplopia is not a common symptom. There may be a history of acute perfusion failure, systemic vasculitis, or orbital cellulitis with vasculitis which could lead to these findings. A history of being found supine or face down for unknown reasons or due to drug abuse/intoxication is also possible.


  • No light perception (NLP) vision
  • Total ophthalmoplegia
  • +/- proptosis
  • Anterior segment ischemia: cell and flare, corneal epithelial edema , Descemet’s folds ,irregular size and function of pupil, hypotony (depending on mechanism)
  • Posterior segment ischemia: RAPD, optic disc edema, optic disc pallor, severe attenuation of arteries and veins, diffuse retinal opacification
  • +/- Elevated IOP (dependent on mechanism)

Clinical diagnosis

History and physical exam

Diagnostic Imaging

These tests will help rule out other potential causes of similar presenting diseases. 

  • CT orbits with/without contrast
  • MRI orbit with/without contrast
  • MRI brain with/without contrast

Laboratory test

There are mainly to help rule out other potential etiologies in the differential. 

  • BMP 
  • CBC with platelets 
  • HbA1C 
  • ACE 
  • ESR 
  • CRP 
  • TSI 
  • Blood cultures 

Differential diagnosis


General treatment

Therapy is dependent on the etiology of the disease. This could be expectant management vs medical vs surgical treatment. 


In most cases, there was total permanent blindness with retinal and optic nerve damage. The anterior ischemia and ophthalmoplegia will resolve with time.


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  8. Helen, Onakpoyaoluwatoyin, et al. “Bilateral Orbital Infarction and Retinal Detachment in a Previously Undiagnosed Sickle Cell Hemoglobinopathy African Child.” Nigerian Medical Journal, vol. 54, no. 3, 2013, p. 200., doi:10.4103/0300-1652.114571.
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