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Persistent Red Eye Unresponsive to Topical Treatment

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 68-year-old woman presented to the emergency eye clinic with a 3-week history of progressive left eye redness and discomfort unresponsive to topical antibiotics or lubricants, as prescribed by her optometrist. The intraocular pressure (IOP) was raised (26 mm Hg) and was treated with a topical β-blocker. Otherwise, her condition was managed conservatively for presumed viral conjunctivitis.

She self-presented 6 days later with intermittent visual blurring, mild eyelid swelling, and conjunctival chemosis. The IOP remained raised (24 mm Hg). An inflammatory or allergic etiology was suspected; therefore, topical corticosteroids and a prostaglandin analogue were also prescribed.

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Carotid-cavernous fistula

C. Refer the patient for urgent neuroimaging

Clinically, the most likely diagnosis is carotid-cavernous fistula (CCF)—an abnormal communication between the carotid artery (or its branches) and the cavernous sinus, causing high-pressure arterial blood flow within the low-pressure veins, sinuses, and cavernous sinus.1,2 Arterialized (“corkscrew”) conjunctival blood vessels are shown in Figure 1. Other features of this case consistent with CCF included raised IOP (due to increased episcleral venous pressure), conjunctival chemosis, and visual blurring. Significant visual loss may occur secondary to traumatic, ischemic, or glaucomatous optic neuropathy.

In 75% of CCF cases there is a direct connection between the intracavernous segment of the internal carotid artery (ICA) and the cavernous sinus, usually due to arterial wall trauma after head injury.3 Acute high-flow symptoms of direct CCFs (commonly used terms) include visual blurring, motility disorders, proptosis, chemosis, and orbital bruits.

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Article Information

Corresponding Author: Aaron Jamison, MBChB, Tennent Institute of Ophthalmology, Gartnavel General Hospital, 1053 Great Western Rd, Glasgow G12 0YN, United Kingdom (aaronjamison@gmail.com).

Published Online: November 19, 2020. doi:10.1001/jamaophthalmol.2020.4062

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Henderson  AD , Miller  NR .  Carotid-cavernous fistula: current concepts in aetiology, investigation, and management.   Eye (Lond). 2018;32(2):164-172. doi:10.1038/eye.2017.240 PubMedGoogle ScholarCrossref
2.
Ellis  JA , Goldstein  H , Connolly  ES  Jr , Meyers  PM .  Carotid-cavernous fistulas.   Neurosurg Focus. 2012;32(5):E9. doi:10.3171/2012.2.FOCUS1223 PubMedGoogle Scholar
3.
Debrun  GM , Viñuela  F , Fox  AJ , Davis  KR , Ahn  HS .  Indications for treatment and classification of 132 carotid-cavernous fistulas.   Neurosurgery. 1988;22(2):285-289. doi:10.1227/00006123-198802000-00001 PubMedGoogle ScholarCrossref
4.
Lewis  AI , Tomsick  TA , Tew  JM  Jr .  Management of 100 consecutive direct carotid-cavernous fistulas: results of treatment with detachable balloons.   Neurosurgery. 1995;36(2):239-244. doi:10.1227/00006123-199502000-00001 PubMedGoogle ScholarCrossref
5.
Meyers  PM , Halbach  VV , Dowd  CF ,  et al.  Dural carotid cavernous fistula: definitive endovascular management and long-term follow-up.   Am J Ophthalmol. 2002;134(1):85-92. doi:10.1016/S0002-9394(02)01515-5 PubMedGoogle ScholarCrossref
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