Orbital Infarction Syndrome

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 by Michael T Yen, MD on August 25, 2020.


Disease Entity

Disease

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.

Etiology

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


Pathophysiology

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.

Diagnosis

History

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.


Signs

  • 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

  • MRV/CTV

  • MRA/CTA

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

  • Orbital cellulitis (bacterial, viral, fungal)


  • GCA


  • Cavernous sinus thrombosis


  • Carotid-cavernous fistula


  • Orbital inflammatory syndrome

  • Brainstem infarction



Management

General treatment

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

Prognosis

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


References

1) Borruat, Frangois-Xavier, et al. “Orbital Infarction Syndrome.” Ophthalmology, vol. 100, no. 4, 1993, pp. 562–568., doi:10.1016/s0161-6420(93)31606-4.


2) Yang, S W, et al. “Two Cases of Orbital Infarction Syndrome.” Korean Journal of Ophthalmology, vol. 14, no. 2, 2000, p. 107., doi:10.3341/kjo.2000.14.2.107.


3) Wirotenurairueng, T, and N Poungvarin. “Orbital Infarction Syndrome in Nephrotic Syndrome Patient with Extensive Carotid Arteries Occlusion.” J Med Assoc Thai, vol. 90, no. 11, Nov. 2007, pp. 2499–2505.


4) Zimmerman CF, Patten PDV, Golnik KC, Kopitnik Jr. TA, Anand R. Orbital infarction syndrome after surgery for intracranial aneurysms. Ophthalmology. 1995;102:594-598.


5) Campo, Davinia Larrosa, et al. “Global Orbital Infarction Syndrome After a Carotid Artery Dissection.” JAMA Neurology, vol. 76, no. 1, Jan. 2019, p. 111., doi:10.1001/jamaneurol.2018.2880.


6) Maier, Philip. “Bilateral Orbital Infarction Syndrome After Bifrontal Craniotomy.” Archives of Ophthalmology, vol. 125, no. 3, Jan. 2007, p. 422., doi:10.1001/archopht.125.3.422.


7) Brinjikji, Waleed, et al. “Orbital Infarction Syndrome Following Mechanical Thrombectomy Secondary to Embolization in New Territory.” World Neurosurgery, vol. 117, 2018, pp. 326–329., doi:10.1016/j.wneu.2018.06.082.


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.


9) Bogousslavsky J, Pedrazzi PI, Borruat FX, Regli F. Isolated complete orbital infarction: a common carotid artery occlusion syndrome. Eur Neurol. 1991; 31:72-76.


10) Pfister, Roswell R. “The Intraocular Changes of Anterior Segment Necrosis.” Eye, vol. 5, no. 2, 1991, pp. 214–221., doi:10.1038/eye.1991.36.Orbital infarction after cocaine use.

11) Stavern, G. P. Van, and M. Gorman. “Orbital Infarction after Cocaine Use.” Neurology, vol. 59, no. 4, 2002, pp. 642–642., doi:10.1212/wnl.59.4.642.


12) Liang, I-Chia, et al. “Orbital Infarction Syndrome after Multiple Percutaneous Sclerotherapy Sessions for Facial Low-Flow Vascular Malformation: A Case Report and Literature Review.” Indian Journal of Ophthalmology, vol. 64, no. 8, 2016, p. 595., doi:10.4103/0301-4738.191508.


13) Mcbride, Cameron L., et al. “Orbital Infarction Due to Sickle Cell Disease without Orbital Pain.” Case Reports in Ophthalmological Medicine, vol. 2016, 2016, pp. 1–4., doi:10.1155/2016/5867850.