Spontaneous Carotid-cavernous Fistula Research Articles

Percutaneous transjugular approach without arterial monitoring for the treatment of a direct carotid-cavernous fistula with vascular Ehlers-Danlos syndrome: illustrative case.

Vascular Ehlers-Danlos syndrome (vEDS) because of COL3A1 mutations is a rare inherited collagen vascular disease associated with spontaneous arterial dissections, aneurysms, vessel rupture, and organ rupture. A direct carotid-cavernous fistula (CCF) is the most common central nervous system vascular anomaly in vEDS; however, its treatment is challenging due to extremely fragile arteries and veins. A 22-year-old woman presented with pulsatile tinnitus and mild diplopia. CCF formation without trauma, cervical dissecting aneurysms, thin skin, and multiple ligament tears, as well as a genetic analysis, led to a diagnosis of vEDS. To minimize the risk of vascular injury in the thoracoperitoneal cavity, the internal jugular vein was directly punctured and the CCF was embolized transvenously using the triple-overlay road-mapping technique without arterial monitoring. The CCF was completely occluded, and the patient showed an excellent clinical course without neurological or vascular complications. Physicians and neurosurgeons should consider vEDS when treating younger patients with spontaneous CCF without trauma and investigate the possibility of genetic abnormalities and systemic vascular pathology. Transvenous embolization of a CCF through the transjugular route using the triple-overlay road-mapping technique can minimize the risk of vascular injury in a patient with vEDS.

Direct spontaneous high-flow carotid-cavernous fistula in a 9-month-old child after nasolacrimal duct repair

Successful treatment of direct spontaneous carotid-cavernous fistula in a 9-month-old child and review of the literature devoted to this issue are reported in the manuscript. Spontaneous high-flow carotid-cavernous fistula is extremely rare in children. Detailed diagnosis of the fistulae includes CT-angiography and cerebral subtraction angiography. Previous surgeries (nasolacrimal canal repair in this case) may be considered as an etiological factor of carotid-cavernous fistula. Transarterial transfemoral endovascular embolization of the fistula with microcoils ensured closure of abnormal communication and complete regression of clinical manifestations in our case.

Central retinal vein occlusion complicating spontaneous dural carotid-cavernous fistula after phacoemulsification.

In this report, we are presenting a case of a 49-year-old female complaining of defective vision in the left eye. The main complaints were: pain, redness, mild proptosis, and high intraocular pressure. She had a history of uneventful phacoemulsification surgery 3 months prior to presenting to us. Investigations revealed a macular edema caused by central retinal vein occlusion and computed tomography angiography showed an early opacified left cavernous sinus with a dilated superior ophthalmic vein along with a fistula between the meningeal branches of the carotid arteries and the cavernous sinus. Improvement of ocular symptoms was achieved after endovascular treatment by transarterial and transvenous embolization.

Superior ophthalmic vein and ophthalmic artery in immediate evaluation after endovascular treatment of carotid-cavernous fistulas.

PurposeTo validate superior ophthalmic vein (SOV) and ophthalmic artery (OA) usefulness in immediate evaluation of new endovascular approaches to treat carotid-cavernous fistulas (CCFs).Material and methodsA retrospective review of 597 intracerebral malformation embolisations yielded 40 embolisations of CCF in the treatment of 18 patients. Two interventional radiologists performed detailed radiological angiographic assessments.ResultsMean age at initial admission was 58.9 years (SD 18.5 years, range 24-85 years). Patients presented with: chemosis (50%), ocular bruit (50%), exophthalmos (61%), diminished visual acuity (77.8%), headache (16.7%), and intracerebral haemorrhage (5.55%), and 5.55% were asymptomatic. Unilateral fistulas (10-55.5%) showed more diversified venous drainage pattern than bilateral ones (8-44.4%). There were statistically significant differences in post-traumatic and spontaneous CCF regarding age (p = 0.036), type of fistula (p = 0.0008), and presence of pseudoaneurysm (p = 0.036). 77.8% of patients had increased ipsilateral SOV diameter. SOV enlargement was not associated with type of fistula, history of trauma, or degree of exophthalmos. Ipsilateral ophthalmic artery was visible in all patients on both pre- and postprocedural angiography on lateral projection. Pre- and post-procedural SOV diameter was significantly different. Internal carotid artery patency was 100%, while the overall final angiographic or clinical success was 85.7%. We had three cases of peri-procedural complications.ConclusionsWe reported changeable dynamics of SOV and OA after endovascular treatment of CCFs and proved the feasibility of coils and Onyx-18 in the treatment thereof.

Pathophysiology of carotid-cavernous fistulas in vascular Ehlers-Danlos syndrome: a retrospective cohort and comprehensive review

BackgroundVascular Ehlers-Danlos syndrome (vEDS) is a rare condition characterized by connective tissue fragility. Direct spontaneous carotid-cavernous fistula (sCCF) is reportedly pathognomonic of vEDS. We conducted this study to understand the possible mechanisms of occurrence of sCCF in this subset of patients.MethodsWe conducted a retrospective analysis of a monocentric vEDS cohort along with a literature review regarding sCCF in this condition.ResultsOf 133 patients regularly followed in our centre between 2000 and 2017, 13 (9.8%) had a diagnosis of direct sCCF (92.3% female, median age 33.0 years, interquartile range (IQR) [26.0–39.5]). There were 7 Glycine missense and 6 splice-site variants but no variant leading to haploinsufficiency. The literature search identified 97 vEDS patients with direct sCCF (79.4% female, 7.2% sex not reported, median age 31.0 years, IQR [24.0–39.0]). Increased carotid circumferential wall stress, higher carotid distensibility and lower carotid intima-media thickness could contribute to a higher risk for direct sCCF in vEDS. There is no predictive factor for the occurrence of sCCF apart from female sex in vEDS.ConclusionsIn vEDS, anatomical and pathophysiological features of the intra-cavernous internal carotid artery make it prone to shunting in the cavernous sinus, due either to a spontaneous rupture or to a spontaneous dissection with pseudoaneurysm formation. Direct sCCF in seemingly healthy young individuals should be highly suggestive of vEDS and prompt further investigation.

A red eye induced by a spontaneous carotid cavernous fistula

A red eye induced by a spontaneous carotid cavernous fistula

Bilateral carotid-cavernous fistula presented with unilateral symptomatology

Introduction. Carotid-cavernous fistula presents an abnormal communication between carotid arterial system and the venous cavernous sinus. Due to anatomical characteristics of the sinus, the clinical picture is first manifested as ophthalmic problem. These fistulas are either of spontaneous or traumatic origin with different hemodynamics. They are usually on one side. However, bilateral carotidcavernous fistulas are rare and deserve special interest. Case report. We presented a 76-years-old female patient, who complained on diplopia and right eye protrusion and esotropia. Ophthalmological findings aroused suspicion of a right-side spontaneous carotid-cavernous fistula. Further non-invasive and invasive investigations confirmed our initial diagnosis. Unexpectedly, digital subtraction angiography revealed another fistula, on her left side. Both fistulas were of low flow and did not need therapy. Conclusion. Carotid- cavernous fistula represent certain diagnostic and therapeutic challenge. Although they are not indicated in a clinical picture, sometimes bilateral fistulas can be found during appropriate diagnostic procedures.

Epileptic Seizures Induced by a Spontaneous Carotid Cavernous Fistula.

A 79-year-old woman was admitted to our emergency department with complaints of fainting and loss of consciousness three times during the past month. She was diagnosed with epilepsy and started to be treated with antiepileptic drug. Physical examination showed, in the left eye, chemosis, limited eye movements in all directions, and minimal exophthalmos as unexisting symptoms on admission developed on the sixth day. Orbital magnetic resonance imaging (MRI) and digital subtraction angiography (DSA) imaging revealed a carotid cavernous fistula (CCF). Epileptic attacks and ophthalmic findings previously present but diagnosed during our examinations were determined to ameliorate completely after performing the coil embolization. Based on literature, we present the first case with nontraumatic CCF manifesting with epileptic seizures and intermittent eye symptoms in the present report.

Endovascular Modalities for the Treatment of Cavernous Sinus Arteriovenous Fistulas: A Single-Center Experience

Cavernous sinus (CS) fistulas are classified into traumatic and spontaneous. Traumatic carotid-cavernous fistulas (CCFs) are usually direct internal carotid artery (ICA) high-flow fistulas; whereas spontaneous CCFs are usually dural, low-flow fistulas and generally possess less severe symptoms than direct carotid-cavernous fistulas. This study involved 34 patients who were classified into 2 groups: Group A included 26 patients with direct carotid-cavernous fistula; and Group B included 8 patients with indirect dural cavernous fistula. All patients had ocular manifestations. One patient had subarachnoid hemorrhage. Coils were used alone in 19 cases of direct fistula and in 1 case of dural fistulas. Coils and Onyx (Covidien, Mansfield, MA, USA) were used in 7 cases of direct fistula and in 2 cases of dural fistulas. Onyx alone was used to treat 5 cases with dural fistulas but none of the cases with direct fistulas. Covered stents and coils were used in 2 cases of direct fistulas. All patients in both groups showed full recovery of their clinical signs and symptoms. Only 1 procedure-related complication was observed (3%) in which a patient had an embolic event and trigeminal dysesthesia as a result of Onyx reflux through external carotid artery-ICA anastomosis. Coils are superior solid embolic agents used for the treatment of direct high-flow fistulas, while Onyx is more valuable in dural low-flow CCF. Onyx shortens the procedure time and decreases procedure cost. Onyx injection inside the CS proper through the transarterial or transvenous route may be safer than Onyx injected inside dural arteries supplying the CS. However, more cases are needed to determine this.

Intractable Posterior Epistaxis due to a Spontaneous Low-Flow Carotid-Cavernous Sinus Fistula: A Case Report and a Review of the Literature

We report a case of a 90-year-old patient with intractable posterior epistaxis presenting as the only symptom of a nontraumatic low-flow carotid-cavernous sinus fistula. Purpose of this case report is to introduce low-flow carotid-cavernous sinus fistula in the differential diagnosis of intractable posterior epistaxis. We provide a literature review for the sequence of actions for the confrontation of posterior epistaxis. We also emphasize the significance of the radiological diagnostic and therapeutic procedures in the management of posterior epistaxis due to pathology of the cavernous sinus. The gold-standard diagnostic procedure of carotid-cavernous sinus fistula is digital subtraction angiography (DSA). DSA with coils is also the state-of-the-art therapy. By failure of DSA, neurosurgery or stereotactic radiosurgery (SRS) may be used as alternatives. SRS may also be used as enhancement procedure of the DSA. Considering the prognosis of a successfully closed carotid-cavernous sinus fistula, recanalization occurs only in a minority of patients. Close follow-up is advised.

Venous drainage patterns in carotid cavernous fistulas.

Purpose. The carotid-cavernous fistula (CCF) is an abnormal arteriovenous communication and its drainage pathways may affect the clinic presentation and change treatment approach. We evaluated drainage patterns of CCFs by digital subtraction angiography (DSA) and categorized drainage pathways according to their types and etiology. Materials and Methods. Venous drainage patterns of 13 CCFs from 10 subjects were studied and categorized as anterior, posterior, superior, inferior, and contralateral on DSA. Drainage patterns were correlated to types and etiology of CCFs. Diagnosis of CCFs was first made by noninvasive imaging techniques. Results. On DSA, traumatic CCFs were usually high flow, direct type while spontaneous CCFs were usually slow flow, indirect type. Bilaterality and mixed types were observed among the indirect spontaneous CCFs. In all CCFs, anterior and inferior drainages were the most common. Contrary to the literature, posterior and superior drainages were noted only in high flow and long standing direct fistulas. Contralateral drainage was not observed in all, supporting plausible compartmentalization of cavernous sinuses. Conclusion. Types, etiology, and duration of the CCFs may affect their drainage patterns. DSA is valuable for categorization of CCFs and verification of drainage patterns. Drainage pathways may affect the clinic presentation and also change treatment approach.

Recovery From Ophthalmoplegia and Proptosis After Repair of Bilateral Carotid-Cavernous Sinus Fistulas

A carotid-cavernous sinus fistula is an anomalous communication of dural branches from the intracerebral artery or external carotid artery to the cavernous sinus. Spontaneous carotidcavernous sinus fistulas have been associated with the presence of ruptured carotid aneurysms, atherosclerosis, diabetes mellitus, and genetic disorders such as Ehlers-Danlos syndrome or fibromuscular dysplasia. However, bilateral spontaneous carotidcavernous sinus fistulas are still considered uncommon.1,2 A 36-year-old man presented with 5 months of progressive headaches, diplopia, and progressive blurry vision. He had no history of head trauma, and both the headaches and the blurry vision were exacerbated by lying down in the supine position. Examination revealed significant proptosis, conjunctival injection, and abducens nerve palsies bilaterally (Figure, A and B). His visual field examination was within normal limits; however, his visual acuity was impaired, and tonometry revealed markedly elevated intraocular pressure (37 mm Hg in the left eye and 34 mm Hg in the right; reference range, 10-21 mm Hg). No retro-orbital bruit or upper motor neuron signs were noted. Magnetic resonance imaging of the brain showed an anterior displacement of the pituitary gland by a vascular structure, but no parenchymal changes were seen. An abnormal vascular structure was redemonstrated by magnetic resonance angiography, but the diagnosis of a dural fistula could not be confirmed. Conventional digital subtraction angiography revealed an indirect low-flow dural shunt between meningeal branches of both the internal carotid artery and the external carotid artery with the cavernous sinus bilaterally (Barrow type D; Figure, C). Endovascular embolization of the lesion (Figure, C and D) was performed using a combined anterior and posterior transvenous approach, which led to the immediate lowering of intraocular pressure and an improvement in visual acuity. At 1-month follow-up, the patient had a near complete resolution of his bilateral cranial nerve VI palsies (Figure, E and F; Video).

Fistule carotido-caverneuse : confrontation des aspects cliniques et neuroradiologiques pré- et post-embolisation

We report the case of an 85-year-old patient, referred by her ophthalmologist for neuro-ophthalmologic consultation at the Clermont-Ferrand University Medical Center, for conjunctival hyperemia and ocular hypertension in the left eye for 12 months. The patient's only past medical history was treated, controlled arterial hypertension and there was no mention of falling or head trauma. MRI (magnetic resonance imaging) and angiography identified a type D carotid-cavernous fistula. Intravenous embolization was performed under general anesthesia by retrograde selective catheterization facial vein and ophthalmic vein and placement of coils into the cavernous sinus, resulting in regression of physical and functional signs immediately after the procedure. This case illustrates the symptoms of spontaneous carotid-cavernous fistula and immediate efficacy of embolization. We report the epidemiology, characteristics and various presentations of the condition.

Balloon-Assisted Coiling of the Cavernous Sinus to Treat Direct Carotid Cavernous Fistula

This study evaluated clinical and neuroradiological results in 13 consecutive patients with spontaneous and traumatic direct carotid cavernous fistulas treated at our center between January 2006 and September 2012. All patients were treated by coiling of the cavernous sinus. Coiling was always performed while a semi-compliant non-detachable balloon was temporarily inflated in the internal carotid artery. This technique (balloon-assisted coiling) permitted a clear visualization of the fistula, facilitated coil positioning and protected the patency of the artery. All patients' clinical data and radiological examinations were reviewed; nine patients underwent radiological and clinical follow-up, with a mean duration of 3.8 years (range: six months-six years). Overall results at discharge showed a complete occlusion of the fistula in seven patients (7/13, 54%) and a resolution of symptoms in eight patients (8/12, 67%). Radiological follow-up showed complete occlusion of the fistula in all patients (9/9, 100%) and clinical follow-up showed a resolution of symptoms in eight patients (8/9, 89%) and persistent symptoms in one (1/9, 11%). No procedure-related complications occurred. Balloon-assisted coiling of the cavernous sinus for the treatment of direct carotid cavernous fistulas proved an effective and safe technique, both in angiographic and clinical terms, and may be considered a technical improvement.

46. Matas の手技による特発性頸動脈海綿静脈洞瘻の自然治癒促進例

46. Matas の手技による特発性頸動脈海綿静脈洞瘻の自然治癒促進例

Carotid‐cavernous fistula in term pregnancy due to spontaneous rupture of carotid‐cavernous aneurysm

Carotid-cavernous aneurysm accounts for 2-9% of all intracranial aneurysms. The rupture of carotid-cavernous aneurysm is usually caused by a trauma. Nevertheless, spontaneous rupture may rarely be encountered. Here, we report a term pregnant woman who was diagnosed to have a spontaneous carotid-cavernous fistula due to carotid-cavernous aneurysm rupture and was managed with detachable balloon and coils immediately after cesarean section.

Proptosis, Congestion, and Secondary Glaucoma Due to Carotid-Cavernous Fistula After Embolization

Carotid-cavernous fistulas (CCFs) are traumatic or spontaneously occurring communications between the carotid artery and the cavernous sinus. Carotid-cavernous fistulas can be due to a direct connection or indirect connections between the carotid artery system and the cavernous sinus. According to the etiologic classification, they may be of traumatic or spontaneous origin, and according to the angiography classification, they may be of direct or dural. Most CCFs are of spontaneous origin, and these are reported as frequently self-healing lesions. Spontaneous CCFs are mostly secondary to arteriosclerotic changes, which explains the increased ratio of elderly patients. Traumatic CCFs are usually of high-flow type and need intervention. The symptoms are various usually correlated to the size and type of venous drainage. The most frequent symptoms on presentation are proptosis, conjunctivitis, and chemosis; however, this picture may be complicated by optic nerve edema, cranial nerve palsies, and intracranial hemorrhage. We report a case with right low-flow dural CCF, which has worsened after angiography and recovered totally soon after endovascular embolization process.

Transvenous embolization in spontaneous direct carotid-cavernous fistula in childhood

Carotid cavernous fistula (CCF) is an abnormal arteriovenous communication in the cavernous sinus. Direct CCF results from a tear in the intracavernous carotid artery. Typically, it has a high flow and usually presents with oculo-orbital venous congestive features such as exophthalmos, chemosis, and sometimes oculomotor or abducens cranial nerve palsy. Indirect CCF generally occurs spontaneously with subtle signs. We report a rare case of spontaneous direct CCF in childhood who did not have the usual history of craniofacial trauma or connective tissue disorder but presented with progressive chemosis and exophthalmos of the right eye. This report aims also to describe the safety and success of transvenous embolization with coils of the superior ophthalmic vein and cavernous sinus through the inferior petrosal sinus.

Multimodal endovascular therapy of traumatic and spontaneous carotid cavernous fistula using coils, n-BCA, Onyx and stent graft

Background and purposeCarotid cavernous fistula (CCF) can be classified as either direct or indirect according to the arterial feeder source. The current standard treatment for CCF is endovascular embolization. In...

Spontaneous Carotid Cavernous Fistula in a Case with Protein S Deficiency that Newly Developed Ophthalmoplegia after Embolization

BackgroundCarotid cavernous fistula (CCF) is an abnormal communication between the carotid artery and the cavernous sinus. The pathogenesis of spontaneous CCF remains unclear, although sinus thrombosis is known to be a predisposing factor for dural arteriovenous fistula. Because spontaneous CCFs are mainly of the dural type, we considered that thrombogenic conditions, such as, protein S deficiency might be associated with CCF.Case ReportA 42-year-old woman complained of conjunctival injection and retro-orbital pain that first appeared 1-month before visiting our hospital. She had no history of head trauma or intracranial surgery. Exophthalmos and chemosis were observed in her left eye, which also had lower visual acuity and higher intraocular pressure than the right eye. Magnetic resonance images and cerebral angiography revealed a left dural CCF. Her protein S was low, at 41% (normal range: 70-140%), but other hematologic values related to coagulation were normal. Her symptoms were relieved after initial transvenous coil embolization. However, a newly developed sixth-nerve palsy was detected 4 days after initial embolization. Follow-up angiography revealed a minimal shunt, and thus transvenous coil embolization was repeated. Two days later, the ophthalmoplegia started reducing, and 1-month later it had almost disappeared.ConclusionsTo the best of our knowledge, this is the first report of spontaneous dural CCF in a Korean patient with concurrent protein S deficiency. Interestingly, transient sixth-nerve palsy developed after transvenous coil embolization in this patient. This additional symptom caused by the residual fistula was relieved after additional transarterial embolization.