Spontaneous Direct Carotid-cavernous Fistula Research Articles

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

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. 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. Out of 80 patients with CCFs, twelve patients were treated for a non-traumatic direct CCF (15%) in 13 sessions. Median age was 65years. 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. 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.

Identification of a de novo Case of COL3A1-Related Ehlers-Danlos Syndrome in a Young Woman Presenting With Spontaneous Direct Carotid-Cavernous Fistula.

Identification of a de novo Case of COL3A1-Related Ehlers-Danlos Syndrome in a Young Woman Presenting With Spontaneous Direct Carotid-Cavernous Fistula.

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.

Diagnosis and endovascular treatment of spontaneous direct carotid-cavernous fistula

Spontaneous direct carotid-cavernous fistula (CCF) is relatively rare and few reports have been found in the literature. The aim of this paper was to report the clinical characteristics, imaging findings and curative effect of endovascular treatment for patients with spontaneous direct CCF. We retrospectively analyzed the clinical data of nine patients with spontaneous direct CCF admitted between May 2003 and November 2007 and the outcomesof endovascular treatment. Sudden neuro-ophthalmological symptoms were the most common clinical presentation at diagnosis (n=8). No patients had a history of head trauma. Cerebral digital subtraction angiography (DSA) was performed on all cases under local anesthesia and seven cases received endovascular treatment. In eight patients, internal carotid angiograms recorded during the early arterial phase revealed aneurysms located in the cavernous sinuses, and in one patient, a dilated internal carotid artery (ICA) was seen. Among the nine cases, seven received endovascular treatment via a transarterial approach and complete occlusion of the fistula was obtained with no technique-related complications, one died suddenly before treatment and one gave up treatment. A detachable balloon was used as the embolic material in two cases, a detachable balloon and detachable coil as the embolic material in two cases, balloon-assisted coil embolization in two cases and covered stents were successfully placed in the parent vessel to exclude the aneurysm and fistula from circulation in one case. During a follow-up period of 3-48 months, all treated patients remained asymptomatic except for one patient who suffered from ipsilateral decreased vision. Most spontaneous direct CCF may be caused by a ruptured intracavernous aneurysm with direct shunting into the cavernous sinus. Endovascular treatment seems to be a safe and effective method for treating spontaneous direct CCF.

Three cases of spontaneous direct carotid cavernous fistulas associated with Ehlers-Danlos syndrome type IV

We are reporting three cases of spontaneous direct carotid cavernous fistulas (CCFs) associated with Ehlers-Danlos syndrome (EDS) out of a series of 147 direct CCFs. The internal carotid artery could be preserved in only one case. Two patients had severe bilateral irregularities of caliber of both internal carotid arteries. Two patients had recurrence of their symptoms after a first balloon was detached in the cavernous sinus (CS) necessitating a second treatment. Two patients died several months later from complications associated with their disease. The treatment of direct CCFs associated with Ehlers-Danlos syndrome is more difficult and more risky than most direct CCFs. The permanent occlusion of the ICA, which may be difficult to avoid, increases the risks of development of aneurysm on the contralateral side.

Treatment of Carotid-Cavernous Fistulas Associated with Ehlers-Danlos Syndrome

Abstract Four patients with Ehlers-Danlos syndrome presented spontaneously with symptomatic direct carotid-cavernous fistulas. Two had previously (2 and 15 years, respectively) had contralateral carotid-cavernous fistulas that were treated with carotid occlusion procedures. The patients' ages at presentation ranged from 19 to 49 years, with a mean of 32 years. All four patients underwent attempted embolization procedures. Two patients had transarterial embolization, one with balloons and liquid adhesives, one with platinum coils and a balloon; the carotid artery was preserved in one and sacrificed in one other. In one patient, who underwent direct puncture of the carotid artery, a massive hematoma causing airway compromise required intubation and emergent surgical repair, The fistula spontaneously closed during this event. In the fourth patient, two transvenous embolizations resulted in markedly decreased flow, but diversion of residual flow to cortical veins produced a fatal pontine hemorrhage. The vascular fragility associated with Ehlers-Danlos syndrome can produce spontaneous direct carotid-cavernous fistulas and makes both diagnostic and therapeutic procedures more difficult.

Treatment of carotid-cavernous fistulas associated with Ehlers-Danlos syndrome

Four patients with Ehlers-Danlos syndrome presented spontaneously with symptomatic direct carotid-cavernous fistulas. Two had previously (2 and 15 years, respectively) had contralateral carotid-cavernous fistulas that were treated with carotid occlusion procedures. The patients' ages at presentation ranged from 19 to 49 years, with a mean of 32 years. All four patients underwent attempted embolization procedures. Two patients had transarterial embolization, one with balloons and liquid adhesives, one with platinum coils and a balloon; the carotid artery was preserved in one and sacrificed in one other. In one patient, who underwent direct puncture of the carotid artery, a massive hematoma causing airway compromise required intubation and emergent surgical repair. The fistula spontaneously closed during this event. In the fourth patient, two transvenous embolizations resulted in markedly decreased flow, but diversion of residual flow to cortical veins produced a fatal pontine hemorrhage. The vascular fragility associated with Ehlers-Danlos syndrome can produce spontaneous direct carotid-cavernous fistulas and makes both diagnostic and therapeutic procedures more difficult.