Bilateral Low-Flow Type-D Dural Carotid-Cavernous Fistula: Diagnosis and Treatment with 3D Time-of-Flight Magnetic Resonance Angiography.

BACKGROUND Carotid-cavernous fistula (CCF) is a rare, atypical vascular shunt between the carotid arterial system and the venous channels of the cavernous sinus, classified according to the shunt's anatomy, by etiology (resulting from trauma or occurring spontaneously), or by hemodynamic characteristics (such as low- or high-flow fistulas). CASE REPORT A 62-year-old female patient with poorly controlled arterial hypertension presented with bilateral periorbital edema, conjunctival chemosis, ophthalmoplegia, diplopia, and diminished visual acuity. On magnetic resonance angiography (MRA), abnormal arterial flow along the cavernous sinuses was noted, suggestive of bilateral CCF. The diagnosis of indirect dural low-flow CCF (Barrow Type D) was later confirmed by digital subtraction angiography, with feeding arteries from intracavernous internal carotid artery branches, and meningeal branches of the external carotid artery, draining bilaterally to ophthalmic veins, the intracavernous sinus, and the inferior petrosal sinus. The patient was successfully treated with endovascular embolization. At 7-month follow-up, no residual arteriovenous shunting was detected. This case highlights the importance of non-invasive radiological methods for CCF, and presents rarely published radiological findings of bilateral Type-D dural CCFs on 3-dimensional time-of-flight MRA with post-treatment MRA follow-up. CONCLUSIONS Regardless of the patient's history of possible trauma, a patient presenting with bilateral periorbital edema, conjunctival chemosis, ophthalmoplegia, diplopia, and diminished visual acuity should have a spontaneous bilateral CCF investigated to prevent delayed treatment. Experienced neuroradiologists are needed to accurately detect indirect CCF, since this condition often does not demonstrate classic symptoms.

Off-label use of large diameter Concerto fibered coils through a 0.017 inch microcatheter for transvenous embolization of indirect carotid-cavernous fistulas: two case reports.

BACKGROUND: A carotid-cavernous fistula is an abnormal communication between the arteries and veins within the cavernous sinus. While conservative management may be prudent in low risk cases, many patients require intervention and endovascular embolization has evolved as the preferred method of treatment. Embolization can be performed via either the transarterial or transvenous approach. One major challenge of the transvenous approach is the complex and variable compartmentation of the cavernous sinus, which often requires the use of low profile microcatheters to navigate and reach the fistulous point. Fibered coils are also preferred when performing transvenous embolization of carotid-cavernous fistula, as they are of higher thrombogenicity and allow for faster occlusion of the fistula. However, most low profile (0.017-inch) microcatheters are not able to deploy fibered coils based on the manufacturer's instructions. CASE PRESENTATION: We present two successful cases of off-label use of Medtronic Concerto fibered coils via a 0.017-inch microcatheter during transvenous embolization of carotid-cavernous fistula in a 60-year-old and an 80-year-old Chinese female, respectively. CONCLUSION: Our case series highlight the possibility of deploying large diameter (up to 10 mm) Concerto fibered coils through a low profile (0.017-inch) microcatheter in an off-label manner for transvenous embolization of indirect carotid-cavernous fistula.

[Carotid-cavernous fistulas]. / Sinus-cavernosus-Fisteln.

BACKGROUND: Carotid cavernous fistulas (CCFs) are rare but clinically significant vascular anomalies characterized by abnormal connections between the cavernous sinus and arteries. This overview presents a comprehensive analysis of anatomy, classification, clinical presentation, diagnosis, imaging, and therapy of CCFs. The cavernous sinus, a central venous structure in the brain, is of critical importance for understanding CCFs due to its proximity to key structures such as the internal carotid artery and cranial nerves. CLASSIFICATION: CCFs are classified into direct and dural types, with direct fistulas typically being high-flow and dural fistulas being low-flow. The symptomatology varies greatly and can range from noises in the head, diplopia, red eye, tearing, to blurred vision and headaches. The diagnostic assessment requires a combination of detailed medical history, neurological and ophthalmological examination, and the use of imaging techniques. METHODS: In imaging, computed tomography (CT) and magnetic resonance imaging (MRI) are crucial for depicting the anatomical structures and blood vessels, while digital subtraction angiography (DSA) is considered the gold standard for accurate representation of the fistula. The treatment of CCFs is complex and depends on the type of fistula, location, and clinical condition of the patient. CONCLUSION: This overview emphasizes the importance of precise diagnosis and individualized therapy to achieve optimal results and avoid complications. Ongoing developments in medical imaging and treatment techniques will continuously improve the treatment outcomes of patients with CCFs.

CAROTID CAVERNOUS SINUS FISTULA FINDINGS IN OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY BEFORE AND AFTER PERCUTANEOUS EMBOLIZATION.

BACKGROUND/PURPOSE: Optical coherence tomography angiography has been used to evaluate the posterior segment in a wide variety of pathologies because it is a noninvasive image technique, but its role in the evaluation of the retina in a case of carotid cavernous sinus fistula has not been described yet. METHODS RESULTS: We present a patient who consulted with spontaneous left superior eyelid hematoma and was diagnosed with left indirect carotid cavernous sinus fistula. In this study, optical coherence tomography angiography was used to evaluate the different macular capillary plexuses in a patient with carotid cavernous sinus fistula and a clinical situation of secondary local venous stasis before and after percutaneous embolization. Augmented vessel density was seen in superficial and deep capillary plexuses and in choriocapillaris before the percutaneous embolization, and a decrease of the parameters was seen after the treatment. CONCLUSION: All macular capillary plexuses presented with augmented vessel density levels that normalized after treatment. These findings were previously undescribed, and they suggest that optical coherence tomography angiography may be useful to initially evaluate patients with carotid cavernous sinus fistula who are planned to undergo embolization and to follow them up until normalization of the vascular structures is reported.

Patch Technique for Primary Treatment of Type A Carotid Cavernous Fistula: A Case Series and Technical Notes.

BACKGROUND: The utilization of flow diverters (FDs) in the treatment of high-flow Type A carotid cavernous fistulas (CCFs) has been described before mainly as an adjunct to the traditional endovascular techniques and rarely as a stand-alone treatment. In this study, we retrospectively evaluated our experience with FDs as the solo nonadjunctive treatment of Type A CCF with severe cortical venous reflux (CVR). METHODS: A retrospective review was performed of patients with Type A CCFs who were treated using FDs' patch technique (PT). Patients' demographics, clinical data, and preoperative and postoperative ocular examination were recorded. The procedure technique, pipeline embolization device (PED) diameters, and immediate and late procedure outcomes were described. RESULTS: Three patients were included in this case series. All patients had history of trauma and presented with decreased visual acuity, cranial nerve II deficit, limited extraocular muscles' movement, and increased intraocular pressure (IOP). Diagnostic angiography was performed, which confirmed high-flow Type A CCF. Endovascular treatment was performed through distal radial access in 2 patients and femoral access in 1 patient by deploying 4 sequentially enlarging PEDs with immediate resolution of the ocular symptoms. Follow-up angiography confirmed complete resolution of CCF in 2 patients. One patient was lost to follow-up; however, angiogram at 4 months demonstrated residual small CCF with significant improvement from postprocedure angiogram. CONCLUSIONS: The patch technique using sequentially enlarging FDs is a reasonable alternative solo technique for the treatment of direct CCF symptoms and results in immediate resolution of CVR while preserving the cavernous sinus anatomy.

Neovascular glaucoma with combined retinal vascular occlusion in carotid cavernous fistula.

Carotid cavernous fistulas (CCFs) can present with varied ophthalmic manifestations. The most important vision-threatening complications of CCF include glaucoma and retinal vascular occlusions. We report a case of a man in his early 30s who developed a post-traumatic direct CCF. The patient denied undergoing embolisation therapy. This resulted in aggravation of his condition with onset of combined retinal venous and artery occlusion leading to neovascular glaucoma and severe vision loss. He was treated with medical management followed by diode laser photocoagulation to control intraocular pressure. Diagnostic cerebral angiography done 3 months later showed complete closure of the fistula; hence, no further intervention was advocated. Combined vascular occlusion is a rare vision-threatening occurrence in cases of CCF. Timely intervention with closure of the fistula can prevent the development of vision-threatening complications.

Endoscopic endonasal transsphenoidal Onyx embolization of a paracavernous dural arteriovenous fistula.

Paracavernous dural arteriovenous fistulas (pdAVFs) are extremely rare and can mimic carotid cavernous fistulas (CCFs) in both clinical presentation and imaging characteristics. Access to the venous pouch often presents the greatest challenge in the treatment of pdAVFs. Here we present a novel access technique utilizing an endoscopic endonasal transsphenoidal approach, where we directly puncture the venous pouch under both stereotactic guidance and endoscopic visualization, thereby completely embolizing a pdAVF with no alternate access (video 1). neurintsurg;15/10/1055/V1F1V1Video 1Technical video demonstrates the complete embolization of a pdAVF using an endoscopic endonasal transsphenoidal approach.

Efficiency of endovascular management with a combination of Onyx and coils for direct and indirect carotid cavernous fistula treatment: Experience of a single center.

BACKGROUND: To evaluate the safety and efficacy of endovascular treatment with a combination of Onyx and coils for carotid cavernous fistulas (CCFs), and to characterize the factors associated with clinical and angiographic outcomes for direct and indirect CCFs. METHODS: This retrospective study included 31 patients with CCF treated with an endovascular procedure between December 2017 and March 2022. RESULTS: Direct and indirect CCFs were found in 14 (45.2%) and 17 (54.8%) cases, respectively. Direct CCFs included eleven traumatic carotid cavernous fistulas. The most common symptom on admission was chemosis, which was seen in 17 (54.8%) patients. Eight (25.7%) cases were treated by the transarterial approach. Fourteen (45.2%) cases were treated using the femoral vein-inferior petrosal sinus approach. Seven (22.6%) were treated by direct puncture of the superior ophthalmic vein. Two (6.5%) were treated by the femoral vein-facial vein approach. Immediate complete occlusion and follow-up rates were 93.5% and 96.7%, respectively. Twenty-nine (96.7%) patients experienced an improvement in their symptoms at clinical follow-up. Chemosis was significantly improved or resolved in 15 patients. Ophthalmoplegia was improved or resolved in 10 patients. Visual impairment was improved in 6 patients. Proptosis was improved or resolved in 5 patients. One case (3.2%) experienced procedure-related complication presented with transient oculomotor nerve palsy. In univariate subgroup analysis, use of balloon, treatment approach, and history of head trauma were significantly different between the direct and indirect CCF groups. CONCLUSIONS: Endovascular treatment with a combination of Onyx and coils is a safe and effective therapy for CCFs. In this study, the transarterial approach was a favorable option for embolization of direct CCFs. In contrast, the transvenous approach may be the first choice of treatment for indirect CCFs.

High index of suspicion: diagnosing a carotid-cavernous fistula.

A woman in her 70s presented with acute bilateral retro-orbital headache, diplopia, chemosis and eye swelling. Ophthalmology and neurology were consulted after detailed physical examination and diagnostic workup including laboratory analysis, imaging and lumbar puncture. The patient was diagnosed with non-specific orbital inflammation and was started on methylprednisolone and dorzolamide-timolol for intraocular hypertension. The patient's condition improved slightly, but a week later, she developed subconjunctival haemorrhage in the right eye, which prompted investigation for a low-flow carotid-cavernous fistula. Digital subtraction angiography showed bilateral indirect carotid-cavernous fistula (Barrow type D). The patient underwent bilateral carotid-cavernous fistula embolisation. Her swelling improved considerably on day 1 after the procedure and her diplopia improved over the following weeks.

Transfacial venous embolisation of a spontaneous indirect paediatric caroticocavernous fistula.

We present a rare case of a spontaneous indirect caroticocavernous fistula (CCF) in an early adolescent, who exhibited a 1-month history of redness, decrease in vision and proptosis of the right eye without a history of trauma or connective tissue disorder. An MRI of the brain and orbit and a digital subtraction angiography confirmed the diagnosis of Barrow type D CCF. The same was then treated with coiling and a liquid embolising agent using the transfacial venous route, resulting in complete obliteration. Reduction in conjunctival congestion with mild improvement in visual acuity was noted postprocedure. Complete recovery was noted at the 6-month follow-up visit. CCF, though rare in the paediatric population, should be highly considered in the differential diagnosis when the above-mentioned complaints are present. Early diagnosis and intervention have favourable outcomes.

Incomplete Third Nerve Palsy Due to a Carotid-Cavernous Fistula.

INTRODUCTION: The approach to oculomotor nerve palsies involves the exclusion of compressive, infective, inflammatory, or ischemic lesions. Dural arteriovenous fistulae, including carotid-cavernous fistulae (CCF), are known causes of third nerve palsy. However, diagnosis can sometimes be delayed or missed due to the various clinical presentations. CCF remains a rare but treatable cause of third nerve palsy. CASE REPORT: A 63-year-old Chinese female presented to our unit with gradual onset horizontal diplopia. Clinical examination revealed a partial left oculomotor nerve palsy without congestive eye symptoms. A cerebral angiogram revealed a left indirect CCF supplied by the inferolateral trunk of the left internal carotid artery. She underwent successful transvenous embolization. She improved postprocedure and was discharged well on day 10 of her admission. She was reviewed at 2 months follow-up and had recovered completely. CONCLUSION: Our case is a classic description of a CCF with posterior drainage into the inferior petrosal sinus. We hope this case and a review of the literature can serve as a reminder to clinicians of the varied presentations of CCF. We believe this case adds value to the clinicians in contributing to their diagnostic process and to our interventional colleagues in highlighting a case with a successful postembolization outcome.

Venous outflow-targeted coil embolization of direct carotid-cavernous fistulas.

BACKGROUND: Coil embolization is the mainstay treatment for carotid-cavernous fistulas (CCFs). However, few studies have reported entire occlusion of engorged veins to interrupt venous outflow. We report our experience with venous outflow-targeted coil embolization of direct CCFs. METHODS: We retrospectively reviewed all the patients diagnosed with direct CCFs treated with venous outflow-targeted coil embolization between November 2013 and February 2020. Venous outflow-targeted coil embolization of the CCFs was performed as follows. First, transarterial stent-assisted coil embolization of CCFs was performed. If the venous outflow to the engorged veins persisted after transarterial stent-assisted coil embolization, entire occlusion of the engorged veins and additional coil packing within the cavernous sinus were performed to interrupt the venous outflow. RESULTS: Ten patients had undergone venous outflow-targeted coil embolization, 6 women (60%) and 4 men (40%). Transfemoral cerebral angiography showed high-flow, direct CCFs in all the patients. Venous outflow occurred through the superior ophthalmic vein (SOV) in all the patients and was completely interrupted by the entire occlusion of the engorged veins with fibered coils. Three patients (30%) had undergone additional treatment in a supplementary manner because of recurrent symptoms (chemosis in 1 patient, faint tinnitus in 2 patients) in the early postprocedural period (1 to 4 weeks). All the symptoms were resolved on follow-up. No additional recurrence was found during follow-up (1-75 months). No peri-procedural complications were encountered. CONCLUSIONS: Venous outflow-targeted coil embolization of CCFs would be a safe and effective treatment method.

Onyx Embolization of an Indirect Carotid-Cavernous Fistula with Cortical Venous Reflux: Technical Note.

BACKGROUND: Indirect carotid-cavernous fistulas (iCCFs) are shunts between meningeal branches of the internal carotid and/or the external carotid arteries and the cavernous sinus. They account for 83% of all carotid-cavernous fistulas (CCFs). Symptomatic iCCFs and those with increased risk of hemorrhage should be treated. Transvenous endovascular treatment is the preferred treatment modality. However, in complex cases, a combination of transarterial and transvenous approaches (multimodal treatment) is required. METHODS: A middle-aged woman presented with signs of increased intraocular pressure, blurry vision, diplopia, left proptosis, chemosis, conjunctival injection, ptosis, and cranial nerve VI palsy. Imaging confirmed the presence of a Barrow type D and Thomas type 4 iCCF with cortical venous reflux (CVR). RESULTS: The patient underwent transarterial and transvenous onyx embolization of the shunt, achieving a complete obliteration of the fistula. No complications occurred and the patient had a satisfactory postprocedural evolution. CONCLUSION: Multimodal onyx embolization is an effective option for the treatment of a complex symptomatic iCCF. If CVR is identified, these lesions should be promptly treated to prevent hemorrhage secondary to rupture.