Embolization techniques of spontaneous direct carotid-cavernous fistulae: a single-center experience.

PURPOSE: Spontaneous direct carotid-cavernous fistula (CCF) are usually caused by a ruptured carotid cavernous aneurysm. We studied treatment of spontaneous direct CCFs in a single-center cohort of a high-volume tertiary referral center, reporting anatomical details, technical approaches of treatment, and outcomes. METHODS: Adult patients with a spontaneous direct CCF treated between 2010-2022 with follow-up MRI and/or DSA imaging available were retrospectively analyzed. We studied age, sex, clinical presentation, angiographic findings, treatment techniques, outcomes, and complications. RESULTS: Out of 80 patients with CCFs, twelve patients were treated for a non-traumatic direct CCF (15%) in 13 sessions. Median age was 65 years. Two patients had an underlying connective tissue disorder. In 10 cases, the direct CCF was caused by a ruptured cavernous carotid aneurysm. The direct CCFs were treated by endovascular transarterial embolization (10 cases), transvenous embolization (1 case), or surgery (1 case). Selective closure of the shunt was possible in 10 patients. Two patients were treated with parent vessel occlusion (PVO; one endovascular; one surgical, with bypass). Complications occurred in 2 / 12 patients (17%), with permanent morbidity in two patients (17%): trigeminal neuralgia after PVO and new infarct after surgical PVO and bypass. Selective closure of CCF resulted in no morbidity. There was no mortality in our series. CONCLUSION: Spontaneous direct CCFs are caused by rupture of a cavernous carotid aneurysm in most cases. Selective closure of the shunt, usually feasible transarterially with coils, achieves good results. Reconstructive endovascular techniques are preferred to minimize treatment related neurological complications.

Inferior Ophthalmic Vein-Dominant Dural Cavernous Fistula Embolization via Combined Orbitotomy and Direct Puncture of Inferior Ophthalmic Vein: A Case Report and Literature Review.

Dural carotid-cavernous fistulas (DCF) typically drain into the superior ophthalmic vein. Predominant involvement of the inferior ophthalmic vein (IOV) is rare, with only 4 documented cases in the literature. Here, the authors describe a case of a 51-year-old man who presented with acute left-sided proptosis, dysmotility, and vision loss and was found to have an IOV-dominant type D dural carotid-cavernous fistulas. The fistula could not be embolized by transfemoral endovascular access or orbitotomy alone and was ultimately managed with combined orbitotomy and direct IOV puncture. All previous reports of IOV-dominant dural carotid-cavernous fistulas in the literature were similarly inaccessible via the transfemoral approach. This case highlights the challenges of IOV cutdown and proposes an alternative management strategy. When IOV cutdown is precluded by the fragile, collapsed, or deep nature of the vessel, conversion to percutaneous IOV puncture may offer a safe and effective approach and mitigate the risks of direct puncture alone.

Extraocular muscle enlargement and proptosis in carotid cavernous fistulas.

PURPOSE: To evaluate the prevalence and pattern of extraocular muscle enlargement and proptosis in patients with carotid cavernous fistulas (CCF). METHODS: We conducted a retrospective study on patients with digital subtraction angiography (DSA) confirmed CCFs with neuroimaging (computed tomography or magnetic resonance imaging) performed prior to the DSA. The maximum extraocular muscle diameters were recorded. Extraocular muscles were considered enlarged if they were greater than two standard deviations above the normal muscle diameters. Proptosis was defined as the distance between the interzygomatic line to the anterior globe of ≥2 mm compared to the contralateral orbit or ≥21 mm. RESULTS: Forty orbits from 20 patients were included. The mean age of participants was 65 ± 15 years and 13 (65%) were female. Thirteen (65%) fistulas were indirect and seven (35%) were direct. There was enlargement of at least one muscle in 11 (27.5%) orbits, and this was not correlated with the type of fistula (direct/indirect). The inferior rectus was most commonly enlarged in seven orbits (17.5%), followed by the medial rectus in five orbits (12.5%). Proptosis was found in 17 (43%) orbits and was more common ipsilateral to the fistula (58% ipsilateral group vs 19% contralateral group, p < .01). CONCLUSION: Extraocular muscle enlargement was observed in over one-fourth of CCFs. When enlarged, the inferior and medial rectus muscles are most commonly involved. These findings may help clinicians and radiologists when evaluating the CT or MRI scans of patients with suspected CCFs.

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.

Intermittent Manual Carotid Compression as a Treatment Option for Indirect Low Flow Carotid Cavernous Fistula.

PURPOSE: To evaluate the efficacy of intermittent manual carotid compression (IMCC) in the management of low-flow carotid cavernous fistulae (CCF). MATERIALS AND METHODS: Patients diagnosed with low-flow CCF and treated with IMCC over a period of 13 years were retrospectively analyzed. Data analyzed included demographic details, clinical features, type of CCF, and response to therapy. Outcomes were categorized as complete cure, partial cure, and no cure or worsening. Patients with complete and partial cure were grouped as good outcome whereas those with no cure or worsening as poor outcome. RESULTS: A total of 44 patients were advised IMCC for low-flow CCF, of whom five were lost to follow-up. Results of the remaining 39 patients were analyzed, of whom 21 (53.8%) were males. The mean age at presentation was 54.38 ± 14.54 years. The median duration between the onset of symptoms and presentation was 5.0 ± 4.10 months. The common presenting features were episcleral congestion and proptosis (89.7%), extraocular motility restriction (66.7%), and diplopia (48.7%). Most common CCF type was type D (34, 87.2%). All patients were advised to undergo IMCC. Good outcome was seen in 35 patients (89.7%, 95% CI: 80.2% to 99.2%), whereas poor outcome was seen in four (10.3%) patients. The mean duration of follow-up was 15.91 ± 21.87 months. CONCLUSION: The present study found a good outcome with IMCC in 89.7% cases of indirect low-flow CCF with no major complications. It should be considered the management of choice in patients who are systemically unfit or cannot afford endovascular embolization.

Flow diverter stents for pediatric traumatic carotid cavernous fistula: a case report and literature review.

A 10-year-old, male patient with a head injury caused by a fall presented with chemosis, exophthalmos, right orbital bruit, and intracranial venous reflux, based on which posttraumatic carotid cavernous fistula (CCF) was diagnosed. Coil embolization was semi-urgently performed for the dangerous venous drainage. After the treatment, right abducens nerve palsy newly appeared. To treat the neurological symptoms and preserve the parent artery, curative endovascular treatment using a pipeline embolization device (PED) with coil embolization was performed after starting dual antiplatelet therapy (DAPT). The CCF and abducens nerve palsy finally resolved, and the internal carotid artery (ICA) was remodeled. Use of the PED with adjunctive coil embolization was effective and safe in the present case of pediatric traumatic direct CCF.

The Possibility of Internal Carotid-Cavernous Fistula After Maxillary Fracture.

The authors first reported a 42-year-old female patient with carotid-cavernous fistula (CCF) that occurred after maxillary fractures. She developed unilateral exophthalmos, bulbar conjunctival hyperemia, and complained of tinnitus after the operation, and was diagnosed as internal CCF through computerized tomography and Digital Substraction Angiography. After the diagnosis, the internal carotid artery was embolized with a coil under general anesthesia. The treatment effect was satisfactory but the vision of the affected side was eventually lost due to the long course of congestion. Although patients with maxillary fractures can safely undergo surgery, some unexpected complications may occur in trauma, such as internal CCF. Therefore, each patient must be subjected to accurate posttraumatic examinations and inquiries to discover possible clinical complications. Patients with exophthalmos, tinnitus, and conjunctival hyperemia may be accompanied by internal CCF. Once diagnosed, appropriate treatment such as embolization should be carried out in time to eliminate further serious sequelae.

Flow diversion in direct carotid-cavernous fistula refractory to multiple coil embolizations: case report and review of the literature.

OBJECTIVES: Direct carotid-cavernous fistulas are high-flow abnormal connections between the carotid artery and cavernous sinus, which are associated with significant morbidity and mortality if left untreated. In addition to endovascular coil embolization, there is an evolving role for alternative stand-alone or adjunctive treatment approaches. We describe a case of flow diversion as an adjunctive treatment approach in a treatment-resistant direct carotid-cavernous fistula and review the literature on the use of flow diversion in this clinical context. MATERIALS AND METHODS: We describe the clinical course, imaging findings, and outcome of a patient who developed a traumatic carotid-cavernous fistula requiring multiple interventions. We also performed an updated literature review of all published cases of flow diversion use in the treatment of direct CCFs. RESULTS: Our patient achieved angiographic cure and significant improvement in symptoms at 6-month follow-up. A total of 53 cases of flow diversion use in direct carotid-cavernous fistulas were identified from a literature search. In one third of cases, flow diversion was used as a stand-alone treatment modality. Angiographic resolution was achieved in 90% of cases at a median follow-up duration of 6 months. One half of reported cases had complete resolution of symptoms and a further third noted clinical improvement in at least some of their symptoms. CONCLUSIONS: There is an emerging role for flow diversion as a safe and effective stand-alone or adjunctive treatment option for direct carotid-cavernous fistulas. Our case further supports a role for flow diversion in treatment-resistant fistulas with high-risk features. As treatment approaches continue to evolve, prospective, randomized data will be needed to establish the role of flow divertors in the hierarchy of available treatments for carotid-cavernous fistulas.

Balloon-Assisted Roadmap Technique to Enable Flow Diversion of a High-Flow Direct Carotid-Cavernous Fistula.

BACKGROUND: The use of flow diverters as a first-line treatment for direct carotid cavernous fistula (CCF) is a relatively new approach in the neurointerventional field which allows obliteration of the fistula with less mass effect from coils in the cavernous sinus. Safe and successful deployment of a flow diverter requires adequate imaging of the parent vessel, which may be challenging in the setting of high-flow CCF without antegrade flow. OBJECTIVE: To facilitate adequate parent vessel imaging in the setting of high-flow CCF to enable the safe development of a flow diverter device. METHODS: Here we present the case of a patient with delayed presentation of post-traumatic direct CCF after a motor vehicle accident, with no antegrade flow past the fistulous connection. We used temporary balloon occlusion of the fistulous connection to enable road-map imaging of the parent vessel and flow-diverter placement. "Drag and drop" device opening in the middle cerebral artery facilitated better deployment of the flow-diverter against retrograde cavernous flow through the fistula. RESULTS: Temporary balloon occlusion of the fistulous connection was used to acquire a roadmap to facilitate safe deployment of a flow diverter and subsequent treatment of the CCF with transvenous coil embolization, with complete resolution of symptoms. CONCLUSION: Balloon-assisted roadmap use is a novel means of visualizing the parent vessel in direct CCF to facilitate safe flow diverter deployment.

TRANSVENOUS APPROACH FOR INDIRECT CAROTID-CAVERNOUS FISTULA USING DETACHABLE COILS: A CASE REPORT AND REVIEW OF TREATMENT OPTIONS.

Carotid-cavernous fistula (CCF) is a relatively rare pathology with a low incidence compared with other vascular pathologies. They can be classified based on hemodynamics as low- or high-flow fistulas, and anatomically as direct or indirect fistulas. Anatomy of the shunt somewhat dictates the selection of endovascular treatment, meaning the venous or arterial approach and selection of embolizing materials. Although there is general agreement as to when to access CCF transvenously or transarterialy, which depends on the shunt being direct or indirect, there is no uniform agreement on which occlusion method should be used. Herein, we report a case of an 80-year-old woman treated for indirect CCF using detachable coils. We also provide a brief review of the literature, including recent advances in treatment of said entities. In conclusion, selection of both the approach and material used depends on the operator's experience and preference.

Delayed-Onset Cranial Nerve Palsy After Transvenous Embolization of Indirect Carotid Cavernous Fistulas.

BACKGROUND: Carotid cavernous fistulas (CCF) often present with diplopia secondary to cranial nerve palsy (CNP). Immediate development of postoperative CNP has been described in the literature. This study described delayed-onset of CNP after complete and reconfirmed obliteration of the CCF and resolution of initial CNP. METHODS: A retrospective analysis was performed on patients with indirect CCF between 1987 and 2006 at 4 academic endovascular centers. Details of the endovascular procedures, embolic agents used, and complications were studied. Partial or complete obliteration was determined. Immediate and delayed cranial nerve palsies were independently assessed. RESULTS: A total of 267 patients with symptomatic indirect CCF underwent transvenous endovascular treatment. Four patients (1.5%) developed delayed abducens nerve (VI) palsy after complete resolution of presenting symptoms after embolization. Delayed presentation ranged between 3 and 13 months after complete resolution of initial double vision and cranial nerve palsies. Transvenous coil embolization through the inferior petrosal sinus was performed in all 4 affected patients. All had follow-up angiography confirming durable closure of their CCF. MRI did not show new mass lesions or abnormal soft tissue enhancement. In all 4 patients, their abducens nerve (VI) palsy remained. CONCLUSIONS: Delayed CNP can develop despite complete endovascular obliteration of the CCF. The cause of delayed CNP is not yet determined, but may represent fibrosis and ischemia. Long-term follow-up is needed even after complete neurological and radiological recovery is attained in the immediate perioperative period.

Gaining Access to the Superior Ophthalmic Vein for Endovascular Embolization of Indirect Carotid-Cavernous Fistulas.

ABSTRACT: Carotid-cavernous fistulas (CCFs) are abnormal connections between the carotid arterial system and the cavernous sinus. These acquired vascular malformations may result in severe orbital congestion and sight-threatening complications. The authors present their experience in gaining access to the superior ophthalmic vein to embolize indirect CCFs in three different patients. Surgical exposure and cannulation of the SOV were successful in all 3 cases. One patient developed an orbital compartment syndrome towards the end of the embolization process, after the irrigation cannula was inadvertently dislodged from the SOV. He required a lateral canthotomy and inferior cantholysis but did not suffer from any related sequelae. Signs and symptoms resolved gradually in all patients and cosmetic results were excellent. In our experience, the SOV offers a reliable access to indirect CCFs, but patients should be monitored closely during the embolization process to prevent ophthalmic complications.

Superior ophthalmic vein thrombosis post manual carotid compression for indirect carotid-cavernous fistula.

PURPOSE: To report 5 cases of superior ophthalmic vein thrombosis (SOVT) following intermittent manual carotid compression (IMCC) for indirect carotid-cavernous fistula (CCF) and to outline the management. METHODS: Retrospective observational case series of all patients who developed SOVT secondary to IMCC for indirect low flow CCF's at a tertiary care center. The demographic profile, clinical, imaging findings, treatment, and outcomes were studied. RESULTS: The mean age at presentation was 60.2 years (Range: 42-87 years). Four patients were male. All patients had a unilateral presentation. The mean time interval between starting IMCC and the development of SOVT was 1.18 months (Range: 0.25-3 months). Acute exacerbation of proptosis and chemosis associated with a decrease in vision was the presenting feature in all the patients. The mean visual acuity at presentation was 0.89 on the logMAR scale. The causes of reduced visual acuity were venous stasis retinopathy (n = 4) and compressive optic neuropathy (n = 1). Magnetic resonance imaging revealed enlarged superior ophthalmic vein with absent flow voids and post-contrast filling defects. Four patients received anticoagulation treatment with subcutaneous injection of enoxaparin 1 mg/kg twice daily for 5 days followed by oral warfarin 5 mg once daily along with oral steroids. Complete recovery of SOVT was noted in all patients at a mean duration of treatment of 0. 75 months (Range: 0-2 months). CONCLUSION: SOVT is a rare but possible complication in patients on IMCC for indirect CCF, and hence warrants close follow up. Early diagnosis and prompt management will help in preventing complications like permanent vision loss.

Transvenous embolization of bilateral indirect carotid-cavernous fistulas via a unilateral transorbital approach.

A 73-year-old man with a complex ophthalmologic history presented with several weeks of worsening diplopia, visual acuity, and proptosis bilaterally. Cerebral angiography demonstrated bilateral indirect Barrow type B carotid-cavernous fistulas (CCFs). Transarterial embolization was not attempted due to small arterial diameter and risk of stroke. Multiple attempts were made to access the fistula via a transfemoral venous approach and were unsuccessful. A transorbital puncture was performed, which allowed access to both cavernous sinuses via a unilateral approach. After embolization with Onyx, there was no residual fistula. The patient had a left-sided retrobulbar hematoma from the access. Right eye vision improved postoperatively.

Carotid Cavernous Fistula Mimicking Hemicrania Continua: A Case Report.

BACKGROUND: Hemicrania continua is a primary headache disorder characterized by a continuous, unilateral headache associated with ipsilateral cranial autonomic features that responds to indomethacin. By definition, the symptoms are not referable to an underlying structural pathology. However, several cases of secondary hemicrania continua related to underlying structural lesions have been reported. CASE: We present a case of a 53-year-old male with a prolonged, right-sided headache associated with intermittent right-sided ptosis, conjunctival injection, tearing, and nasal congestion, suggestive of hemicrania continua, who was found to have an indirect carotid-cavernous fistula, and who, after endovascular treatment of the fistula, had resolution of his symptoms. CONCLUSION: Alternative, and perhaps less common, causes of headache should be considered when the clinical presentation is atypical or does not clearly fulfill diagnostic criteria for primary headache disorders. Carotid cavernous fistulas should be included within this differential, and represent a potentially treatable and reversible cause of otherwise refractory headache.

Swollen and bloodshot eye following headache.

A ruptured cavernous carotid aneurysm (CCA) with carotid cavernous fistula can appear as a benign headache but progress to a swollen and bloodshot eye overnight. A 66-year-old woman visited emergency department with sudden onset of pain behind her left forehead and vomiting. She was treated for a migraine-like headache and discharged. She presented again on the next day with a persistent headache and a swollen left eye with blurred vision. An ophthalmologic examination revealed erythema of the left lid and chemosis at the temporal and lower bulbar conjunctiva. A cranial nonenhanced computed tomography (CT) scan had been performed at her previous visit. The scan exhibited a nodular mass lesion involving the left cavernous sinus. CT angiography was subsequently used to determine that the lesion was a giant aneurysm in the left cavernous internal carotid artery, causing enlargement of the left ophthalmic veins. The symptoms of her left eye rapidly progressed to severe chemosis, edematous change over periocular region, and limited movements after 8 h. The patient received emergent lateral canthotomy and inferior cantholysis to avoid acute orbital compartment syndrome and was subsequently treated with stent-assisted coil embolization. A ruptured CCA is an urgent condition that requires rapid assessment of both cranial vascular and ocular lesions. A history of sudden onset headache with a nonpainful acute unilateral red eye may serve as a clue to prompt additional diagnostic studies and ophthalmologist evaluation. Adequate radiological studies and early endovascular intervention can reduce the likelihood of permanent ocular injury and vision impairment.

CTA-guided outflow-targeted embolization of direct carotid-cavernous fistula.

This video presents a case of new-onset visual blurring, diplopia, and conjunctival injection after head injury. CTA of the brain revealed a direct carotid-cavernous fistula (dCCF) of the right side. Careful evaluation of CTA source images revealed that the fistula point was at the ventromedial aspect of the right cavernous internal carotid artery (ICA), about 3.6 × 3.6 mm2 in size, with 3 main outflow channels (2 intracranial and 1 extracranial) (CTA-guided concept). DSA of the brain also confirmed the diagnosis but was unable to locate the fistula point in a large-sized dCCF. Through a transfemoral artery approach, 3 microcatheters were navigated to each peripheral channel to initiate outflow-targeted embolization. Intracranial refluxes were blocked first to avoid cerebral hemorrhages, followed by the extracranial outflow. During embolization, accidental dislodge of one coil into the sphenoparietal vein occurred, but no attempt of coil retrieval was made. Complete obliteration of the dCCF was achieved, and the patient recovered well without new neurological deficits. 4D MRA at the 3-month follow-up showed no residual dCCF.The video can be found here: https://youtu.be/LH2lNVRZSPk.

Carotid-Cavernous Sinus Fistula caused by Persistent Primitive Trigeminal Artery Aneurysm rupture: A case report.

Carotid-Cavernous sinus fistula (CCF) caused by a ruptured aneurysm of persistent primitive trigeminal artery (PPTA) is exceptionally rare. Herein, we reported the case of a 64-year-old female who presented with headache, pulsatile tinnitus, and diplopia. Angiography showed a PPTA and CCF. Three-dimensional digital subtraction angiograms revealed a direct shunt between the PPTA aneurysm and the left cavernous sinus. The trigeminal carotid fistula was successfully treated with detachable coils and Onyx, and the PPTA was preserved. Hence, coil and Onyx occlusion of the cavernous sinus is a safe and effective treatment for trigeminal carotid fistula.

Endovascular treatment of carotid-cavernous sinus fistulas: ophthalmic and visual outcomes.

Purpose: The main objective was to report the epidemiology, clinical manifestations, angiography features, treatment modality and post-treatment outcomes in patients diagnosed with carotid cavernous fistulas (CCF). Methods: A retrospective review of the medical imaging database in conjunction with medical records from 2004 to 2017 at the Royal Brisbane and Women's Hospital (RBWH) was conducted.We identified 39 patients with CCF (16 direct, 23 indirect). A total of 37 diagnoses were confirmed by direct catheter angiography. The remaining two cases were diagnosed using magnetic resonance imaging/magnetic resonance angiography. Results: Coils were deployed in 100% of direct and 83% of treated indirect fistulas that were treated. Other embolic agents were deployed alone or in combination with coils. Successful angiographic closure was achieved in 93% of direct and 92% of indirect fistulas. Multiple treatments were required in 33% of direct and 16% of indirect fistulas. Visual acuity improved in patients with direct fistulae(p = 0.02) and was preserved in those with indirect fistulae. Post-treatment diplopia persisted in six patients with direct fistulas and three patients with indirect fistulas. Four patients with indirect fistulas experienced persistent ocular hypertension post-treatment compared to two patients with direct fistulas. Conclusions: Endovascular coils are the most commonly deployed treatment for CCF. Both indirect and direct fistulas achieved high rates of closure; however, indirect fistulas were less likely to require multiple treatments. Good post-procedural vision was achieved for both groups.