Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2024 Jul 31;16(7):e65821.
doi: 10.7759/cureus.65821. eCollection 2024 Jul.

Neuro-Ophthalmic Manifestations of Carotid Cavernous Fistulas: A Systematic Review and Meta-Analysis

Affiliations
Review

Neuro-Ophthalmic Manifestations of Carotid Cavernous Fistulas: A Systematic Review and Meta-Analysis

Ali Al-Shalchy et al. Cureus. .

Abstract

Carotid-cavernous fistulas (CCFs) are pathologic, arteriovenous communications between the carotid artery and cavernous sinus. They cause various complex neuro-ophthalmic symptoms by shunting the flow of arterial blood into the venous system. In this study, a systematic review is conducted on the neuro-ophthalmic presentations associated with CCFs. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 guidelines were followed during the systematic review. We searched PubMed, Scopus, and Web of Science from inception to December 31, 2023. Articles written in English on patients with confirmed CCFs reporting clinical features, diagnostic modalities, treatment approaches, and outcomes were included. Abstracted data included demography, clinical presentations, venous flow dynamics, trauma history, investigative methodology, approaches to treatment, and outcomes. Overall, 33 studies with a total number of 403 patients were included. The mean age at presentation was 42.99 years for patients with direct CCFs and 55.88 years for those with indirect CCFs. Preponderance was observed in male patients with direct CCFs, constituting 51.56%, while females predominated in those with indirect CCFs, at 56.44%. The clinical symptoms in all patients with CCFs were proptosis in 58 cases (14.39%), conjunctival congestion in 29 patients (7.20%), diplopia in nine patients (2.23%), vision blurring in four patients (0.99%), eyelid swelling in five patients (1.24%), pain in the eye in three patients (0.74%), and an upper lid mass in one patient (0.25%). Endovascular treatments, including coil and Onyx embolization, have been effective in relieving clinical symptoms and arresting the progression of these symptoms. In conclusion, the common clinical features in CCFs usually underline proptosis, congestion, and diplopia, necessitating a comprehensive neuro-ophthalmological review. Prompt identification of the symptoms of blurred vision is crucial to avoid permanent damage. Lid swelling, ocular pain, and an upper lid mass are less common but equally essential presentations for comprehensive evaluation. The recognition of these variable presentations is essential not only for timely intervention but also for the improvement in patient outcomes, thus emphasizing the role of clinician awareness in managing CCF cases.

Keywords: carotid cavernous fistula; cranial nerve pathology; neuro-ophthalmic presentations; optic nerve; venous flow dynamics.

PubMed Disclaimer

Conflict of interest statement

Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. PRISMA Flow Diagram of the Included Articles.
PRISMA: Preferred Reporting Items for Systematic Reviews and Meta-Analyses
Figure 2
Figure 2. Anatomical Illustration of the Carotid Cavernous Fistula (CCF).
ICA: Internal carotid artery Image Credit: Sura H. Talib

References

    1. Targeted compartmental embolization of cavernous sinus dural arteriovenous fistulae using transfemoral medial and lateral facial vein approaches. Agid R, Willinsky RA, Haw C, Souza MP, Vanek IJ, terBrugge KG. Neuroradiology. 2004;46:156–160. - PubMed
    1. Carotid-cavernous fistula: current concepts in aetiology, investigation, and management. Henderson AD, Miller NR. Eye (Lond) 2018;32:164–172. - PMC - PubMed
    1. Carotid-cavernous fistulas. Ellis JA, Goldstein H, Connolly ES Jr, Meyers PM. Neurosurg Focus. 2012;32:0. - PubMed
    1. Endovascular techniques for treatment of carotid-cavernous fistula. Gemmete JJ, Ansari SA, Gandhi DM. J Neuroophthalmol. 2009;29:62–71. - PubMed
    1. Management of 100 consecutive direct carotid-cavernous fistulas: results of treatment with detachable balloons. Lewis AI, Tomsick TA, Tew JM Jr. Neurosurgery. 1995;36:239–244. - PubMed

LinkOut - more resources