Neuroendovascular Management of Carotid Cavernous Fistulae

Carotid-cavernous fistulas (CCFs) represent a group of rare, abnormal arteriovenous communications between the carotid arterial system and the cavernous sinuses (CS). CCFs often produce ophthalmologic symptoms related to increased CS pressures and retrograde venous drainage of the eye. Although endovascular occlusion remains the preferred treatment for symptomatic or high-risk CCFs, most of the data for these lesions is limited to small, single-center series. As such, we performed a systematic review and meta-analysis evaluating endovascular occlusions of CCFs to determine any differences in clinical outcomes based on presentation, fistula type, and treatment paradigm.

A retrospective review of all studies discussing the endovascular treatment of CCFs published through March 2023 was conducted using PubMed, Scopus, Web of Science, and Embase databases. A total of 36 studies were included in the meta-analysis. Data from the selected articles were extracted and analyzed using Stata software version 14.

1494 patients were included. 55.08% were female and the mean age of the cohort was 48.10 years. A total number of 1516 fistulas underwent endovascular treatment, 48.05% of which were direct and 51.95% of which were indirect. 87.17% of CCFs were secondary to a known trauma while 10.18% were spontaneous. The most common presenting symptoms were 89% exophthalmos (95% CI: 78.0–100.0; I² = 75.7%), 84% chemosis (95% CI: 79.0–88.0; I² = 91.6%), 79% proptosis (95% CI: 72.0–86.0; I² = 91.8%), 75.0% bruits (95% CI: 67.0–82.0; I² = 90.7%), 56% diplopia (95% CI: 42.0–71.0; I² = 92.3%), 49% cranial nerve palsy (95% CI: 32.0–66.0; I² = 95.1%), 39% visual decline (95% CI: 32.0–45.0; I² = 71.4%), 32% tinnitus (95% CI: 6.0–58.0; I² = 96.7%), 29% elevated intraocular pain (95% CI: 22.0–36.0; I² = 0.0%), 31% orbital or pre-orbital pain (95% CI: 14.0–48.0; I² = 89.9%) and 24% headache (95% CI: 13.0–34.0; I² = 74.98%). Coils, balloons, and stents were the three most used embolization methods respectively. Immediate complete occlusion of the fistula was seen in 68% of cases and complete remission was seen in 82%. Recurrence of CCF occurred in only 35% of the patients. Cranial nerve paralysis after treatment was observed in 7% of the cases.

Exophthalmos, Chemosis, proptosis, bruits, cranial nerve palsy, diplopia, orbital and periorbital pain, tinnitus, elevated intraocular pressure, visual decline and headache are the most common clinical manifestations of CCFs. The majority of endovascular treatments involved coiling, balloons and onyx and a high percentage of CCF patients experienced complete remission with the improvement of their clinical symptoms.

Patients with indirect carotid cavernous fistulas (CCF) were reviewed to emphasize the importance of diagnosing patients even with trivial findings and to raise awareness. Eighteen patients diagnosed as CCF were included. Neuro-ophthalmological findings before and after treatment, diagnostic investigations, treatment, clinical course was noted. Twelve patients were female (67%), 6 were males (33%) and the mean age at presentation was 54 years (range: 29–70 years). Conjunctival hyperemia was present in all patients. Seventeen (94%) patients had proptosis and diplopia, nine (50%) had orbital pain and/or headache, four (22%) had blurred vision, one (5.5%) had ptosis. On examination, 17 patients (94%) had restricted eye movements, four (22%) had low visual acuity and five patients had (28%) increased intraocular pressure (IOP). One patient had been diagnosed as myasthenia gravis and two as thyroid orbitopathy and had been on treatment accordingly before CCF was diagnosed. In two patients, bilateral findings were present despite unilateral CCF on angiography. Barrow Type B fistula was found in 7 (38%), Barrow Type D in 11 (62%) patients. In three bilateral CCF was detected. All were treated by endovascular intervention. Residual deficits at the sixth month control were, eye movement deficits in seven (39%), decrease in visual acuity in one (5.5%) and elevated IOP in one (5.5%) patient. Indirect CCF patients generally present with mild symptoms and the diagnosis may be overlooked. Mild progressive ophthalmoparesis with conjunctival hyperemia must be warning. Though rare bilateral CCF can be detected as well as unilateral CCF with bilateral findings.

A direct carotid-cavernous fistula (CCF) is a rare complication that typically occurs in closed head injuries. The balloon-assisted occlusion is the most used treatment. However, this technique requires multiple inflation/deflations, which could be problematic in a vessel with acute vessel injury.

In this study we reported a case of 72-year-old male patient with posttraumatic CCF, treated endovascularly with a transarterial Comaneci-assisted coiling embolization, highlighting the advantages and pitfalls of this innovative endovascular treatment.

The Comaneci-assisted coiling technique has proven to be advantageous both in the microcatheterization of the CCF and during coiling with remodeling technique. In addition, this device does not interrupt the vascular flow compared to balloon-assisted coiling and does not require long-term antiaggregation therapy with respect to the stent placement.

Carotid cavernous aneurysm (CCA) rupture is rare. However, it can result in various complications such as carotid cavernous fistula (CCF), epistaxis, spontaneous thrombosis, and subarachnoid hemorrhage.

We report a 65-year-old woman with a history of uncontrolled diabetes who was referred with complaints of acute headache, diplopia, proptosis, and chemosis. Ophthalmic examination revealed elevated intraocular pressure in the right eye, optic disk edema, and retinal venous congestion. Canthotomy was performed based on a diagnosis of orbital compartment syndrome (OCS). Further imaging revealed a dilated superior ophthalmic vein and cavernous sinus, as well as swelling of the extraocular muscles in the right eye. Digital subtraction angiography revealed the fistulous connection between the cavernous part of the internal carotid and cavernous sinus (direct CCF) due to the large ruptured CCA, resulting in retrograde flow through the superior and inferior ophthalmic veins. Successful endovascular coiling of the aneurysm resulted in complete occlusion of the fistula. Postintervention ophthalmic examination demonstrated progressive improvement of ophthalmic signs and symptoms; however, the patient's right eye remained sightless.

In patients with clinical manifestation of OCS with no history of any predisposing risk factors, diagnosis of ruptured cavernous sinus aneurysm and resulting direct CCF should be considered. In such cases, emergent imaging along with early endovascular intervention can resolve OCS and prevent permanent ocular injury and vision loss.

The cavernous sinuses are a pair of intradural venous compartments that lies between the periosteal and meningeal layers of the dura. They are located centrally within the skull in the anteromedial portion of the middle cranial fossa where they are bordered medially by the pituitary gland and the body of the sphenoid bone. These structures are related to important cranial nerves and importantly, the internal carotid artery. Surgery near the sella turcica can encounter this venous structure.

This chapter will cover the diagnosis and management of the causes of double vision and ocular misalignment. Although the bulk of the discussion will concentrate on third, fourth, and sixth nerve palsies, other supranuclear, nuclear, neuromuscular junction, myopathic, and restrictive disorders will be detailed or alluded to. The relevant anatomy of the ocular motor system will be discussed first, followed by signs and symptoms in patients with diplopia, then the diagnosis and management. The differential diagnosis of the major entities that cause diplopia will then be reviewed.