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Carotid-cavernous fistula (CCF) associated with persistent trigeminal artery (PTA) is a rare but important clinical entity. We present a case treated by microcoil embolization with preservation of internal carotid, PTA, and hasilar artery flow following embolization. A 62-year-old female developed pulsatile tinnitus followed by left eye proptosis and diplopia. Examination revealed a cranial nerve VI palsy and an objective bruit over the left orbit. Angiographic evaluation revealed a carotid cavernous fistula originating from a persistent trigeminal artery. Placement of a detachable balloon across the fistula site while preserving the PTA proved impossible, and the fistula was treated with microcoils following placement of a microcatheter across the fistula into the cavernous sinus. Complete closure of the fistula was followed by resolution of the patient's symptoms. Preservation of all major vessels including the PTA was accomplished through the use of coil embolization. Careful evaluation of the angiogram is necessary to identify PTA associated with a CCF. Previous reports have described treatment of CCF with PTA by surgical or balloon ocolusion, some involving sacrifice of the PTA. Examination of the relevant embryology and anatomy reveals, however, that occlusion of the PTA must be approached with caution due to potential supply to the posterior circulation.

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Summary

A 33-year-old woman was evaluated for a right carotid-cavernous fistula revealed by a proptosis and chemosis of the right eye. The initial angiogram showed a left persistent pharyngo-stapedial artery (Ph-SA). A temporal bone CT suggested bilateral pharyngo-stapedial artery persistence. The right Ph-SA was not opacified in the first angiogram because of the high degree of shunting in the fistula. Four months later the patient was admitted for treatment of the carotid-cavernous fistula. In the meantime, the fistula had altered, with spontaneous thrombosis of the ophthalmic vein, and decrease of the vascular steal, explaining that the right Ph-SA was clearly visible on the angiogram performed during the procedure.

The carotid-cavernous fistula was completely occluded with five detachable coils. The follow-up included 3 Tesla MR angiography that showed complete closure of the fistula with preservation of the right ICA and bilateral persistent pharyngo-stapedial arteries.

Summary

Aneurysmal rupture of the intra-cavernous carotid artery may cause idiopathic carotid-cavernous fistula (CCF), and the treatment choice for occluding shunting fistula in this type of CCF is an endovascular approach using detachable balloons. However, little has been reported on treating such lesions with the intra-aneurysmal embolization using Guglielmi detachable coils (GDCs). To our knowledge, ours is the first reported case of successful treatment by selective intra-fistula and intra-aneurysmal embolization with GDCs. A 74-year-old woman exhibited proptosis and chemosis of her left eye over a period of one month. Symptoms of double vision in conversion and pulsatile murmur in her left eye were also noted. Angiography revealed an intra-cavernous aneurysm of the left internal carotid artery (ICA) with a shunting fistula, which drained into the dilated cavernous sinus, superior orbital vein (SOV), superior petrosal sinus, inferior petrosal sinus, and pterygoid plexus. We thought the fistula would occlude by intra-aneurysmal embolization, but we had no confidence of tight packing of the aneurysm since the aneurismal neck was relatively wide. So, we embolized the venous side of the shunting fistula and then the dome of the aneurysm with GDCs. Immediately after the operation, her symptoms and signs were ameliorated, and complete occlusion of the CCF was observed on long-term follow-up. We suggest selective intrafistula and intra-aneurysmal embolization with GDCs as an alternative method of treatment of idiopathic CCF originating from aneurysmal rupture of the intra-cavernous carotid artery.

Introduction

A case of communicating carotid-cavernous sinus fistula (CCF) after minor closed head injury is presented.

Case presentation

A 45-year-old Caucasian male presented to the emergency department of a tertiary care hospital with the chief complaint of blurred vision and facial numbness. The patient had experienced a minor head injury 1 month ago with loss of consciousness. After a 2-week symptom-free period, he developed scalp and facial numbness, along with headache and vision problems. His vital signs were within normal limits, but on examination the patient was noted to have orbital and carotid bruits with several concerning neurological findings. CT and MRI confirmed the suspicion of carotid-cavernous sinus fistula, which was managed by cerebral angiography with coil embolization of this fistula. The patient was symptom free at the 8-month follow-up.

Discussion

Carotid-cavernous sinus fistula is a rare condition that is usually caused by blunt or penetrating trauma to the head, but can develop spontaneously in about one fourth of patients with CCF. The connection between the carotid artery and cavernous sinus leads to increased pressure in the cavernous sinus and compression of its contents, and thereby produces the clinical symptoms and signs seen. Diagnosis depends on clinical examination and neuroimaging techniques. The aim of management is to reduce the pressure within the cavernous sinus, which results in gradual resolution of symptoms.

Summary

The venous sinuses commonly found in the margins of the diaphragm and sella are venous interconnections between the bilateral cavernous dural sinuses and are termed intercavernous communications or intercavernous sinuses. They form a venous ring, a single “circular sinus” that extends throughout the skull base. We report the first case to our knowledge of an intercavernous sinus fistula. We emphasize the importance of thorough knowledge of lesion characteristics before considering any interventional procedure.

An 84-year-old woman presented with alarming progressive orbital symptoms for one month affecting her left eye. A cerebral angiogram showed an intercavernous sinus fistula supplied by internal and external carotid arterial branches.

Transvenous embolization through retrograde catheterization of the right inferior petrosal sinus allowed complete coil occlusion of the lesion. Cerebral angiography confirmed the absence of residual blood flow through the fistula.

This report represents the first case of an intercavernous sinus dural arteriovenous fistula successfully treated with transvenous embolization. A detailed awareness of the regional anatomy is essential for treatment approach and favorable outcomes.

After the spontaneous relief of initial symptoms by traumatic carotid-cavernous fistula (CCF), paradoxical worsening of patient's condition can be followed. We present a case of a 60-yr-old man whose audible bruit from a traumatic CCF had completely disappeared. A few days later, however, the patient had spontaneous intracerebral hematoma with cortical venous drainage. Complete obliteration of the fistula was achieved after embolization. When initial audible bruit in traumatic CCF disappears suddenly, cerebral angiography should be performed to differentiate venous hypertension by the hemodynamic changes of the cavernous sinus channels from spontaneous resolution of CCF.

Background

Carotid 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 Report

A 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.

Conclusions

To 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.

Summary

We report our experience with transarterial embolization of traumatic carotid-cavernous fistulae (TCCFs) by using Gugliemi detachable coil (GDC). From 2000 to 2007 at our institution, 11 patients with 12 TCCFs underwent transarterial GDC embolization because of failure to occlude fistulae by detachable balloon with preservation of the parent artery.

The cause of the failure to occlude the fistula by detachable balloon was small fistula tract (n=9) and/or tortuous parent artery (n=3) or repeated balloon puncture by bony fragment (n=1). All TCCFs were successfully occluded by a single session transarterial GDC embolization. The average number of coils were eight (range, two-16) with an average length of 104 cm (range, 12-283 cm). No statistically significant procedure-related neurological complication or recurrent TCCF was observed in any of the patients.

Transarterial GDC embolization is a useful method in the treatment of TCCFs, particularly in those TCCFs with small fistula tract or small CS.

Summary

This study was designed to elucidate the generating mechanism, diagnosis and treatment of traumatic carotid cavernous fistula (tCCF) concomitant with pseudoaneurysm in the sphenoid sinus. Six cases of tCCF concomitant with pseudoaneurysm in the sphenoid sinus were analyzed in this study. Clinical history, neurological examination, CT and MRI scans, pre- and postembolization cerebral angiograms and follow-up data were included.

All patients presented with massive epistaxis and symptoms of tCCF. The pseudoaneurysms and fistulas were occluded with detachable balloons, and preservation of the parent artery in two cases. One patient also had indirect carotid cavernous fistula (CCF) on the contralateral side embolized by transfacial vein approach with microcoils. Complete symptom resolution was achieved in all cases, without procedure related complications. During the follow-up period all patients returned to work.

Falling from a high speed motorcycle without wearing a helmet may be one of the main causes of this disease. The site of impact during the accident mostly localizes in the frontal and lateral of the orbit. Intracavernous sinus hypertension of tCCF combining with fracture of the lateral wall of the sphenoid may lead to the formation of a pseudoaneurysm in the sphenoid sinus. MRI scan is very helpful in the diagnosis of this disease before the patient receives angiography. Detachable balloon occlusion of the pseudoaneurysm and fistula is a safe and efficient treatment.

Summary

A 26-year-old man presented with symptoms of progressive bilateral exophthalmos and swelling of the eyelids after a severe head injury. Angiography confirmed a direct carotid-superior hypophyseal arterial (SHA) cavernous fistula with petrosal sinus and intracavernous sinus drainage. Successful transarterial coil embolization of the fistula was performed with resolution of the patient's symptoms. To our knowledge, post-traumatic arteriovenous fistula between SHA and the cavernous sinus has not been previously reported. We hereby demonstrate an effective, minimally invasive method of occluding a rare fistula by transarterial embolization.

Background:

We report herein a case of cavernous sinus (CS)-dural arteriovenous fistula (DAVF) with brainstem venous congestion that was successfully treated by transarterial embolization, followed by radiotherapy.

Case Description:

An 80-year-old woman presented with right eye chemosis and left hemiparesis. T2-weighted magnetic resonance imaging showed hyperintensity of the pons. Diagnostic cerebral angiography demonstrated CS-DAVF draining into the right superior orbital vein and petrosal vein, and fed by bilateral internal and external carotid arteries. Transarterial embolization was performed and followed by radiotherapy, resulting in resolution of the pontine lesion and neurological and ophthalmological symptoms within 5 months.

Conclusions:

We also review the literature regarding therapy for CS-DAVF with brainstem venous congestion. Once CS-DAVF with venous congestion of the brainstem has been definitively diagnosed, immediate therapy is warranted. Treatment with transarterial embolization followed by radiation may be an important option for elderly patients when transvenous or transarterial embolization is not an option.

This article describes a 22-year-old man who presented to the Howard University Hospital emergency room with acute onset of swelling, proptosis, and decreased vision in the right eye preceded by 24 hours of nausea and vomiting. The patient's visual acuity was count fingers in the involved eye with marked proptosis and limitation of ocular motility. There was no history given of any ocular or head trauma. A computed tomography scan of the orbits showed diffuse symmetric enlargement of the extraocular muscles of the right eye, felt to be consistent with an orbital inflammatory pseudotumor. The patient was treated with intravenous steroids initially, then placed on oral prednisone. After minimal improvement on the steroids, a selective external carotid angiogram showed a moderate-sized dural cavernous sinus fistula. The patient underwent selective embolization of the fistula with rapid resolution of periorbital edema and proptosis. Visual acuity was stabilized at 20/200 in the right eye. The differential diagnosis and pathogenesis of carotid cavernous sinus fistulas and the likely pathogenesis of the fistula in this case are discussed.

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Carotid cavernous fistula (CCF) is an abnormal communication between the cavernous sinus and the carotid arterial system. A CCF can be due to a direct connection between the cavernous segment of the internal carotid artery and the cavernous sinus, or a communication between the cavernous sinus, and one or more meningeal branches of the internal carotid artery, external carotid artery or both. These fistulas may be divided into spontaneous or traumatic in relation to cause and direct or dural in relation to angiographic findings. The dural fistulas usually have low rates of arterial blood flow and may be difficult to diagnose without angiography. Patients with CCF may initially present to an ophthalmologist with decreased vision, conjunctival chemosis, external ophthalmoplegia and proptosis. Patients with CCF may have predisposing causes, which need to be elicited. Radiological features may be helpful in confirming the diagnosis and determining possible intervention. Patients with any associated visual impairment or ocular conditions, such as glaucoma, need to be identified and treated. Based on patient's signs and symptoms, timely intervention is mandatory to prevent morbidity or mortality. The conventional treatments include carotid ligation and embolization, with minimal significant morbidity or mortality. Ophthalmologist may be the first physician to encounter a patient with clinical manifestations of CCF, and this review article should help in understanding the clinical features of CCF, current diagnostic approach, usefulness of the available imaging modalities, possible modes of treatment and expected outcome.

Summary

Endovascular treatment of high-flow direct traumatic carotid cavernous fistula (CCF) carries many difficulties. One of them is that carotid dissection may be associated with pseudo-aneurysm formation even when the CCF can be successfully embolized by detachable balloons. This article details a unique technique of treating pseudo-aneurysm by obstructing the lumen with preservation of the parent artery.

The case presented here involves a 50-year-old man with a history of severe trauma. The angiography revealed that his bilateral high-flow carotid cavernous fistula was successfully embolized by detachable balloons and control digital subtraction angiography at the end of the procedure demonstrated the fistula closed and the internal carotid artery preserved. But two months later, a pseudo-aneurysm formed in the right internal carotid artery. Under endovascular treatment along with other techniques including coils, stent-assisted and covered stent, the pseudo-aneurysm was excluded with preserved ICA. Two years later, the fistula and pseudo-aneur-ysm both disappeared quietly without patent foreign body reaction in the parent arterial wall.

ABSTRACT

Direct carotid-cavernous fistula is an abnormal arteriovenous communication between the carotid artery and the cavernous sinus occurring spontaneously or following head trauma. The aim of this paper is to report our experience and a review of the literature regarding the curative effect of endovascular treatment for patients with post-traumatic direct CCF. We present five patients with direct post-traumatic CCF in whom endovascular treatment was applied and the outcomes of the endovascular treatment. Direct post-traumatic CCF may be completely occluded without technique-related complications using detachable balloons, coils as embolic material or by using covered stents placed in the parent vessel to exclude the fistula from circulation. Postembolization angiographies revealed that the fistula was successfully obliterated. Few days after the procedure the ophthalmic symptoms were much reduced, and completely resolved soon after.

In conclusion endovascular treatment represents an effective method for complete occlusion the direct CCF no matter of the technique chosen.

Summary

The study assessed the effectiveness and safety of endovascular covered stents in the management of intracranial pseudoaneurysms, fusiform aneurysms and direct carotid-cavernous fistulas.

Fourteen endovascular covered stents were used to repair three pseudoaneurysms, six fu-siform aneurysms and six direct carotid-cavernous fistulas. Aneurysms were in the carotid artery in seven cases, in the vertebral artery two cases. It was not possible to treat two additional cases transcutaneously for technical reasons
2/15.

Percutaneous closure of the lesions with an endovascular covered stent was successful in 13 of 15 cases. Initial follow-up showed good stent patency. No complications were observed after stent implantation. During follow-up, stent thromboses were detected in two of nine patients with follow-up digital subtracted angiography. One carotid-cavernous fistula of Barrow Type A transformed into Barrow Type D at nine month follow-up study was cured with a procudure of Onyx-18 injection.

Endovascular covered stents may be an option for percutaneous closure of intracranial pseudoaneurysms, fusiform aneurysms and direct carotid-cavernous fistulas. Endoluminal vascular repair with covered stents offers an alternative therapeutic approach to conventional modalities.

The aetiology of ophthalmoplegia in 15 patients with carotid-cavernous sinus fistula is discussed, and the clinical findings are correlated with angiographic and orbital CT appearances. After closure of the fistula the majority of patients with generalised ophthalmoplegia recovered full ocular movements rapidly, while patients with an isolated abduction weakness required much longer to return to normal. Orbital CT studies showed enlarged extraocular muscles in the patients with generalised ophthalmoplegia but muscles of normal size in those with abduction failure alone. After closure of the fistula repeat CT studies of patients with enlarged extraocular muscles showed a diminution in muscle size. We suggest that generalised ophthalmoplegia in carotid cavernous sinus fistula is due to hypoxic, congested extraocular muscles. Isolated abduction weakness is due to a sixth nerve palsy, which probably occurs either in the cavernous sinus or more posteriorly near the inferior petrosal sinus. A combination of these 2 mechanisms may be found in some patients.

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Summary

We describe a relatively unusual case of traumatic direct carotid-cavernous fistula in association with a giant intradural venous pouch and ipsilateral carotid dissection, related to carotid artery fistula located in the supraclinoid segment just below the origin of posterior communicating artery. Endovascular therapy could be accomplished by use of detachable coils transarterially. Awareness of an unusual intradural origin of a carotid-cavernous sinus fistula and the possibility of an embolization should be kept in mind.

A 62-year-old woman has been suffered from cavernous sinus thrombophlebitis which was confirmed by four-vessel angiography, orbit magnetic resonance imaging, and blood culture. Three weeks after recovery of cavernous sinus thrombophlebitis, right eye proptosis and complete third, fourth, and sixth cranial nerve palsies developed. Best-corrected visual acuity decreased to 20/70 in the right eye. Repeat magnetic resonance imaging demonstrated a 1.5-cm-sized mass in the right cavernous sinus, suspicious for mycotic aneurysm. Amphotericin B supplementation was begun and was followed by successful transarterial Guglielmi detachable coil embolization. Four months later, extraocular movement was normalized, and visual acuity improved to 20/25 in the right eye.

We report a case of spontaneous right carotid-cavernous fistula (CCF) in a proximal segment of persistent primitive trigeminal artery (PPTA) and combined vascular anomalies such as left duplicated hypoplastic proximal posterior cerebral arteries and a variation of anterior choroidal artery supplying temporal and occipital lobe. A 45-year-old male presented with progressive right exophthalmos, diplopia, and ocular pain. With manual compression of the internal carotid artery, a cerebral angiography revealed a right CCF from a PPTA. Treatment involved the placement of detachable non-fibered and fibered coils, and use of a hyperglide balloon to protect against coil herniation into the internal carotid artery. A final angiograph revealed complete occlusion of PPTA resulted in no contrast filling of CCF.

Summary

A 62-year-old man with a traumatic high-flow right carotid-cavernous fistula was treated by transarterial balloon occlusion technique. However, because of the relatively small size of the fistula, the balloon could not enter into the cavernous sinus via the fistula. During the procedure, the shunt flow decreased significantly, and we stopped the procedure. Follow-up angiography performed 14 days after the procedure showed complete occlusion of the fistula with a small residual pseudoaneurysm. One year later, the pseudoaneurysm had decreased in size. Repeated transient decrease and stagnancy of blood flow at the fistula during the balloon procedure may have played an important role in the thrombosis in this patient.

Summary

The development of a high-flow carotid-cavernous fistula from the rupture of a large cavernous aneurysm successfully embolized by coils is rare. A 50-year-old male patient developed a high-flow carotid-cavernous fistula 48 hours after successful coiling of a large left cavernous aneurysm, presumably due to rupture of a focal dissection at or close to the neck of the aneurysm. He initially responded to daily self-compression of the left common carotid artery, but the fistula recurred. After failing to approach the fistula site via transvenous route, balloon trapping of the internal carotid artery was planned. Prior to its placement for functional occlusion test, the detachable balloon slipped into the fistula site and occluded it. It was thereafter detached in this position. The sequence of events, a large cavernous aneurysm spontaneous ruptured after coiling, suggested dissecting process or disease. We address in the report the complexity of the endovascular management of this rare association.

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.

Summary

Here, we present a 32-year-old male with proptosis and chemosis of the left eye following a close head injury. Digital subtraction angiography of the left internal carotid artery showed a left carotid-cavernous fistula (CCF) associated with a primitive trigeminal artery (PTA) variant. The patient was successfully treated with transvenous Guglielmi detachable coils embolization via the inferior petrosal sinus.

The PTA variant was preserved without cerebellar or brainstem infarct.

Purpose

To report a case of spontaneous direct carotid-cavernous fistula causing abrupt loss of vision.

Case Report

A 50-year-old woman with systemic hypertension but no history of ocular disease developed sudden proptosis, frozen eye, subconjunctival hemorrhage and loss of vision in her left eye over 2 hours. Imaging studies revealed a direct carotid-cavernous fistula. Management for high intraocular pressure was promptly initiated and the patient was referred to a neurosurgery service, but she refused any surgical intervention. Ultimately, she accepted to undergo manual carotid artery compression which resulted in significant reduction in the proptosis, but she lost all vision permanently.

Conclusion

Direct carotid-cavernous fistula can occur spontaneously and should be taken into account in patients with signs suggestive of direct carotid-cavernous sinus fistula even without history of trauma or connective tissue disorder.