Carotid-cavernous fistula

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Carotid-cavernous fistula
Other namesCCF
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Oblique section through the cavernous sinus.
Specialty Neurology, cardiology   OOjs UI icon edit-ltr-progressive.svg

A carotid-cavernous fistula results from an abnormal communication between the arterial and venous systems within the cavernous sinus in the skull. It is a type of arteriovenous fistula. As arterial blood under high pressure enters the cavernous sinus, the normal venous return to the cavernous sinus is impeded and this causes engorgement of the draining veins, manifesting most dramatically as a sudden engorgement and redness of the eye of the same side.

Contents

Presentation

dilated blood vessels in the eye in CCF Corkscrew blood vessels in left eye.jpg
dilated blood vessels in the eye in CCF

CCF symptoms include bruit (a humming sound within the skull due to high blood flow through the arteriovenous fistula), progressive visual loss, and pulsatile proptosis or progressive bulging of the eye due to dilatation of the veins draining the eye. Pain is the symptom that patients often find the most difficult to tolerate.[ citation needed ]

Patients usually present with sudden or insidious onset of redness in one eye, associated with progressive proptosis or bulging. They may have a history of similar episodes in the past.[ citation needed ]

Causes

Carotid cavernous fistulae may form following closed or penetrating head trauma, surgical damage, rupture of an intracavernous aneurysm, or in association with connective tissue disorders, vascular diseases and dural fistulas.[ citation needed ]

Diagnosis

Selective angiography of the external carotid artery showing an indirect type D right carotid cavernous fistula, filling of the cavernous sinus (arrow) and retrograde drainage into the right superior ophthalmic vein (arrowhead) 1471-2415-12-28-1Cerebral angiogram.jpg
Selective angiography of the external carotid artery showing an indirect type D right carotid cavernous fistula, filling of the cavernous sinus (arrow) and retrograde drainage into the right superior ophthalmic vein (arrowhead)

This is based on MRI scan, magnetic resonance angiography and CT scan. A cerebral digital subtraction angiography (DSA) enhances visualization of the fistula.[ citation needed ]

Classification

Various classifications have been proposed for CCF. They may be divided into low-flow or high-flow, traumatic or spontaneous and direct or indirect. The traumatic CCF typically occurs after a basal skull fracture. The spontaneous dural cavernous fistula which is more common usually results from a degenerative process in older patients with systemic hypertension and atherosclerosis. Direct fistulas occur when the Internal Carotid artery (ICA) itself fistulizes into the Cavernous sinus whereas indirect is when a branch of the ICA or External Carotid artery (ECA) communicates with the cavernous sinus.[ citation needed ]

A popular classification divides CCF into four varieties depending on the type of arterial supply.[ citation needed ]

TypeDescription
AFistulous supply from the internal carotid artery
BSupply from the dural branches of internal carotid artery
Cfrom meningeal branches of ext carotid artery
Dcombined ICA+ECA

Treatment

The mainstay of treatment for CCF is endovascular therapy. [2] This may be transarterial (mostly in the case of direct CCF) or transvenous (most commonly in indirect CCF). Occasionally, more direct approaches, such as direct transorbital puncture of the cavernous sinus or cannulation of the draining superior orbital vein are used when conventional approaches are not possible. Spontaneous resolution of indirect fistulae has been reported but is uncommon. Staged manual compression of the ipsilateral carotid has been reported to assist with spontaneous closure in selected cases.[ citation needed ]

Direct CCF may be treated by occlusion of the affected cavernous sinus (coils, balloon, liquid agents), or by reconstruction of the damaged internal carotid artery (stent, coils or liquid agents).[ citation needed ]

Indirect CCF may be treated by occlusion of the affected cavernous sinus with coils, liquid agents or a combination of both. [3] [4] [5]

Related Research Articles

<span class="mw-page-title-main">Vein</span> Blood vessels that carry blood towards the heart

Veins are blood vessels in the circulatory system of humans and most other animals that carry blood toward the heart. Most veins carry deoxygenated blood from the tissues back to the heart; exceptions are those of the pulmonary and fetal circulations which carry oxygenated blood to the heart. In the systemic circulation, arteries carry oxygenated blood away from the heart, and veins return deoxygenated blood to the heart, in the deep veins.

<span class="mw-page-title-main">Fistula</span> Abnormal connection between two epithelialized surfaces, often organs

In anatomy, a fistula is an abnormal connection joining two hollow spaces, such as blood vessels, intestines, or other hollow organs to each other, often resulting in an abnormal flow of fluid from one space to the other. An anal fistula connects the anal canal to the perianal skin. An anovaginal or rectovaginal fistula is a hole joining the anus or rectum to the vagina. A colovaginal fistula joins the space in the colon to that in the vagina. A urinary tract fistula is an abnormal opening in the urinary tract or an abnormal connection between the urinary tract and another organ. An abnormal communication between the bladder and the uterus is called a vesicouterine fistula, while if it is between the bladder and the vagina it is known as a vesicovaginal fistula, and if between the urethra and the vagina: a urethrovaginal fistula. When occurring between two parts of the intestine, it is known as an enteroenteral fistula, between the small intestine and the skin as an enterocutaneous fistula, and between the colon and the skin as a colocutaneous fistula.

<span class="mw-page-title-main">Internal carotid artery</span> Artery of the human brain

The internal carotid artery is an artery in the neck which supplies the anterior circulation of the brain.

<span class="mw-page-title-main">Vascular surgery</span> Medical specialty, operative procedures for the treatment of vascular disorders

Vascular surgery is a surgical subspecialty in which vascular diseases involving the arteries, veins, or lymphatic vessels, are managed by medical therapy, minimally-invasive catheter procedures and surgical reconstruction. The specialty evolved from general and cardiovascular surgery where it refined the management of just the vessels, no longer treating the heart or other organs. Modern vascular surgery includes open surgery techniques, endovascular techniques and medical management of vascular diseases - unlike the parent specialities. The vascular surgeon is trained in the diagnosis and management of diseases affecting all parts of the vascular system excluding the coronaries and intracranial vasculature. Vascular surgeons also are called to assist other physicians to carry out surgery near vessels, or to salvage vascular injuries that include hemorrhage control, dissection, occlusion or simply for safe exposure of vascular structures.

<span class="mw-page-title-main">Cerebral circulation</span> Brain blood supply

Cerebral circulation is the movement of blood through a network of cerebral arteries and veins supplying the brain. The rate of cerebral blood flow in an adult human is typically 750 milliliters per minute, or about 15% of cardiac output. Arteries deliver oxygenated blood, glucose and other nutrients to the brain. Veins carry "used or spent" blood back to the heart, to remove carbon dioxide, lactic acid, and other metabolic products. The neurovascular unit regulates cerebral blood flow so that activated neurons can be supplied with energy in the right amount and at the right time. Because the brain would quickly suffer damage from any stoppage in blood supply, the cerebral circulatory system has safeguards including autoregulation of the blood vessels. The failure of these safeguards may result in a stroke. The volume of blood in circulation is called the cerebral blood flow. Sudden intense accelerations change the gravitational forces perceived by bodies and can severely impair cerebral circulation and normal functions to the point of becoming serious life-threatening conditions.

<span class="mw-page-title-main">Intracranial hemorrhage</span> Hemorrhage, or bleeding, within the skull

Intracranial hemorrhage (ICH), also known as intracranial bleed, is bleeding within the skull. Subtypes are intracerebral bleeds, subarachnoid bleeds, epidural bleeds, and subdural bleeds.

<span class="mw-page-title-main">Cerebral angiography</span> Angiography that produces images of blood vessels in and around the brain

Cerebral angiography is a form of angiography which provides images of blood vessels in and around the brain, thereby allowing detection of abnormalities such as arteriovenous malformations and aneurysms. It was pioneered in 1927 by the Portuguese neurologist Egas Moniz at the University of Lisbon, who also helped develop thorotrast for use in the procedure.

<span class="mw-page-title-main">Moyamoya disease</span> Disease characterized by constriction of brain arteries

Moyamoya disease is a disease in which certain arteries in the brain are constricted. Blood flow is blocked by constriction and blood clots (thrombosis). A collateral circulation develops around the blocked vessels to compensate for the blockage, but the collateral vessels are small, weak, and prone to bleeding, aneurysm and thrombosis. On conventional angiography, these collateral vessels have the appearance of a "puff of smoke".

<span class="mw-page-title-main">Arteriovenous fistula</span> Medical condition

An arteriovenous fistula is an abnormal connection or passageway between an artery and a vein. It may be congenital, surgically created for hemodialysis treatments, or acquired due to pathologic process, such as trauma or erosion of an arterial aneurysm.

<span class="mw-page-title-main">Cavernous sinus</span> Sinus in the human head

The cavernous sinus within the human head is one of the dural venous sinuses creating a cavity called the lateral sellar compartment bordered by the temporal bone of the skull and the sphenoid bone, lateral to the sella turcica.

<span class="mw-page-title-main">Confluence of sinuses</span> Venous sinus in the skull

The confluence of sinuses, torcular Herophili, or torcula is the connecting point of the superior sagittal sinus, straight sinus, and occipital sinus. It is below the internal occipital protuberance of the skull. It drains venous blood from the brain into the transverse sinuses. It may be affected by arteriovenous fistulas, a thrombus, major trauma, or surgical damage, and may be imaged with many radiology techniques.

<span class="mw-page-title-main">Superior petrosal sinus</span> One of the dural venous sinuses located beneath the brain

The superior petrosal sinus is one of the dural venous sinuses located beneath the brain. It receives blood from the cavernous sinus and passes backward and laterally to drain into the transverse sinus. The sinus receives superior petrosal veins, some cerebellar veins, some inferior cerebral veins, and veins from the tympanic cavity. They may be affected by arteriovenous malformation or arteriovenous fistula, usually treated with surgery.

<span class="mw-page-title-main">Superior ophthalmic vein</span> Vein of the orbit around the eye

The superior ophthalmic vein is a vein of the orbit that drains venous blood from structures of the upper orbit. It is formed by the union of the angular vein, and supraorbital vein. It passes backwards within the orbit alongside the ophthalmic artery, then exits the orbit through the superior orbital fissure to drain into the cavernous sinus.

<span class="mw-page-title-main">Dural arteriovenous fistula</span> Medical condition

A dural arteriovenous fistula (DAVF) or malformation is an abnormal direct connection (fistula) between a meningeal artery and a meningeal vein or dural venous sinus.

<span class="mw-page-title-main">Cavernous sinus thrombosis</span> Medical condition

Cavernous sinus thrombosis (CST) is the formation of a blood clot within the cavernous sinus, a cavity at the base of the brain which drains deoxygenated blood from the brain back to the heart. This is a rare disorder and can be of two types–septic cavernous thrombosis and aseptic cavernous thrombosis. The most common form is septic cavernous sinus thrombosis. The cause is usually from a spreading infection in the nose, sinuses, ears, or teeth. Staphylococcus aureus and Streptococcus are often the associated bacteria.

<span class="mw-page-title-main">Nasofrontal vein</span> Vein in the orbit around the eye

The nasofrontal vein is a vein in the orbit around the eye. It drains into the superior ophthalmic vein. It can be used for endovascular access to the cavernous sinus.

<span class="mw-page-title-main">Bonnet–Dechaume–Blanc syndrome</span> Medical condition

Bonnet–Dechaume–Blanc syndrome, also known as Wyburn-Mason syndrome, is a rare congenital disorder characterized by arteriovenous malformations of the brain, retina or facial nevi. The syndrome has a number of possible symptoms and can, more rarely, affect the skin, bones, kidneys, muscles, and gastrointestinal tract. When the syndrome affects the brain, people can experience severe headaches, seizures, acute stroke, meningism, and progressive neurological deficits due to acute or chronic ischaemia caused by arteriovenous shunting.

Sinus pericranii (SP) is a rare disorder characterized by a congenital epicranial venous malformation of the scalp. Sinus pericranii is an abnormal communication between the intracranial and extracranial venous drainage pathways. Treatment of this condition has mainly been recommended for aesthetic reasons and prevention of bleeding.

Interventional neuroradiology (INR) also known as neurointerventional surgery (NIS), endovascular therapy (EVT), endovascular neurosurgery, and interventional neurology is a medical subspecialty of neurosurgery, neuroradiology, intervention radiology and neurology specializing in minimally invasive image-based technologies and procedures used in diagnosis and treatment of diseases of the head, neck, and spine.

The venous plexus of hypoglossal canal is a small venous plexus surrounding the hypoglossal nerve as it passes through the hypoglossal canal. The plexus connects with the occipital sinus (intercranially), inferior petrosal sinus (intercranially), internal jugular vein (extracranially), condylar vein, and paravertebral venous plexus.

References

  1. 1 2 Thinda, S; Melson, MR; Kuchtey, RW (Jul 28, 2012). "Worsening angle closure glaucoma and choroidal detachments subsequent to closure of a carotid cavernous fistula". BMC Ophthalmology. 12: 28. doi: 10.1186/1471-2415-12-28 . PMC   3412712 . PMID   22839357.
  2. Al Saiegh, Fadi; Baldassari, Michael P.; Sweid, Ahmad; Bilyk, Jurij; Mouchtouris, Nikolaos; Hafazalla, Karim; Abendroth, Michael; Velagapudi, Lohit; Khanna, Omaditya; Chalouhi, Nohra; Sajja, Kalyan; Tjoumakaris, Stavropoula; Gooch, M Reid; Rosenwasser, Robert; Jabbour, Pascal (2020). "Onyx Embolization of Carotid-Cavernous Fistulas and Its Impact on Intraocular Pressure and Recurrence: A Case Series". Operative Neurosurgery. 20 (2): 174–182. doi:10.1093/ons/opaa308. PMID   33027818.
  3. Ong, CK; Wang, LL; Parkinson, RJ; Wenderoth, JD (2009). "Onyx embolisation of cavernous sinus dural arteriovenous fistula via direct percutaneous transorbital puncture". Journal of Medical Imaging and Radiation Oncology. 53 (3): 291–5. doi:10.1111/j.1754-9485.2009.02086.x. PMID   19624295. S2CID   25700375.
  4. Bhatia, Kartik D; Wang, Lily; Parkinson, Richard J; Wenderoth, Jason D (2009). "Successful Treatment of Six Cases of Indirect Carotid-Cavernous Fistula with Ethylene Vinyl Alcohol Copolymer (Onyx) Transvenous Embolization". Journal of Neuro-Ophthalmology. 29 (1): 3–8. doi: 10.1097/WNO.0b013e318199c85c . PMID   19458567. S2CID   23630211.
  5. Nadarajah, M.; Power, M.; Barry, B.; Wenderoth, J. (2011). "Treatment of a traumatic carotid-cavernous fistula by the sole use of a flow diverting stent". Journal of NeuroInterventional Surgery. 4 (3): e1. doi:10.1136/neurintsurg-2011-010000. PMID   21990483. S2CID   37417928.