This document discusses carotid cavernous fistulas (CCFs), abnormal connections between the carotid artery and cavernous sinus. CCFs can be direct, with high blood flow from the internal carotid artery, or indirect, with lower flow from dural branches. Presentation depends on type and cause but often includes proptosis, chemosis, and bruit. Diagnosis involves CT, MRI and cerebral angiography. Treatment options include observation, compression therapy, endovascular coiling/plugging, covered stents, parent artery occlusion, transvenous embolization, surgery and radiosurgery. The goal is to occlude direct CCFs while preserving carotid patency.

1. CAROTID CAVERNOUS FISTULA
Dr. Rabail Akbar Qazi
Neurosurgery Resident

2. ANATOMY

17. INTRODUCTION
 CCFs are abnormal acquired arterioveous shunts
between the carotid artery and the cavernous sinus
 May occur spontaneously or result from trauma or
surgical or endovascular manipulation
 Trauma accounts for 75% of all cases of CCF
 Risk of SAH is low. Major risk is to vision.

18.  TYPES:
 Direct (Type A)
 Indirect (Types B –D)
 Direct are high flow shunts between the ICA and
cavernous sinus
 Indirect are low flow shunts from dural arteries which are
branches of ECA (except type B)

20. TYPE A CCF
 TRAUMATIC
 0.2 % of patients with craniocerebral trauma
 3.8% of all patients experiencing a skull base fracture
 Direct tear of ICA , or less frequently, one of its branches
 SPONTANEOUS
 Ruptured cavernous sinus ICA aneurysm
 Connective tissue disorders

22. IATROGENIC CCFs
 Following surgical manipulation close to or within the
cavernous sinus
 Procedures such as:
 Transphenoidal pituitary surgery
 Sinus surgery
 Rhinoplasty
 Carotid endarterectomy with fogarty maneuvre
 Percutaneous rectogasserian procedures
 Cavernous ICA endovascular balloon angioplasty and
stent deployment

23. PRESENTATION
 CLASSIC TRIAD:
 Chemosis
 Pulsatile proptosis
 Ocular bruit
 Presentation is dependent on the type and etiology
of the CCF
 Two typical groups:
 Young men with traumatic CCFs
 Elderly women with spontaneous CCFs

24. DIRECT CCFs
 Acute onset and rapid progression
 Most commonly:
 Exophthalmos
 Chemosis
 Orbital bruits
 Diplopia
 Ophthalmoplegia
 Visual disturbances
 Headaches
 Cerebellar and brainstem signs
 Cranial nerve disturbances

25. INDIRECT CCFs
 Progression may be slow
 Most common signs:
 Arterialization of conjunctival veins
 Chemosis
 Exophthalmos
 Diplopia
 Cranial bruit
 Retro-orbital headache
 Decreased visual acuity

30. INVESTIGATIONS

31. COMPUTED TOMOGRAPHY
 The hallmark of CT is the visualization of bony
fractures and fresh blood.
 Dilated ophthalmic veins and enlarged ocular
muscles maybe detected.
 The key finding in both direct and spontaneous
CCFs is the dilated superior ophthalmic vein

35. Magnetic Resonance Imaging
 More sensitive for initial diagnosis as it visualizes the
intracranial and intraorbital soft tissues and vascular
structures in more detail
 Enlarged intraorbital veins and muscles with
exophthalmos, dilated intracranial veins and
enlarged CS
 MRA able to detect further vascular pathologies

40. CEREBRAL ANGIOGRAPHY
 Gold standard for definitive diagnosis of CCF
 Examination of the ICA and ECA of both sides as
well as vertebrobasilar system is necessary
 Shunting of blood from ICA into the cavernous sinus
 Enlarged draining veins
 Retrograde flow from CS

41.  Huber Maneuver:
 Lateral view
 Inject vertebral artery and manually compress affected
carotid
 Helps identify upper extent of fistula, multiple fistulous
openings and complete transection of ICA.
 Mehringer Hieshima Maneuver:
 Inject contrast 2-3ml/s into affected carotid while
compressing the carotid in the neck
 Controls flow to help demonstrate the fistula

44. MANAGEMENT
 Indications for treatment:
 Proptosis
 Visual loss
 Cranial nerve VI palsy
 Intractable bruit
 Severely elevated intraocular pressure
 Increased filling of cortical veins on angiography

45.  OBSERVE:
 20-50% of low flow CCF spontaneously thrombose ,
observe as long as visual acuity is stable and intraocular
pressure is < 25
 Symptomatic, high flow CCF needs urgent treatment

47. CAROTID COMPRESSION THERAPY
 Contralateral hand: 10s : 4-6 /hr. Reduces AV
shunting + Increases outlet venous pressures - >
Thrombosis
 Most useful in the treatment of indirect fistulas
resulting in spontaneous closure in upto 30% of the
patients

48. ENDOVASCULAR TREATMENT
 Options include:
 Electrolytically detachable coils
 Amplatzer Vascular plug
 The goal of treatment in direct CCFs is to occlude
the tear between the ICA and the cavernous sinus
while preserving the patency of the ICA.

49. DETACHABLE BALLOON OCCLUSION
 The small-diameter vessels that often make up dural
fistulas usually do not allow the introduction of a
balloon.
 However, the large carotid defect commonly present
in type A CCFs frequently permits transarterial
balloon occlusion of the fistula with preservation of
the ICA
 The advantage of balloon occlusion of a CCF is the
ability to occlude the fistula rapidly with preservation
of the ICA.

51. COIL AND MATERIAL EMBOLIZATION
 Transarterial embolization with coils or other embolic
material now is the mainstay of endovascular
treatment for high-flow direct CCFs
 Embolization can be achieved with detachable
platinum coils, silk and liquid embolic agents such
as n-butyl cyanoacrylate (n-BCA), and ethylene-vinyl
alcohol copolymer (EVOH)

53. COVERED STENT GRAFT PLACEMENT
 extremely useful for the immediate obliteration of a
direct CCF, while preserving ICA patency

54.  PARENT ARTERY OCCLUSION
 TRANSVENOUS EMBOLIZATION
 With indirect fistulas, it is mandatory to place coils on
the venous side, otherwise new feeders will be
recruited.

55. SURGICAL TREATMENT
 Indications for surgical repair include compromised
proximal arterial access that prevents endovascular
repair or causes it to fail.
 Surgical management remains a consideration for
salvage of failed endovascular treatments
 Trapping between surgically placed clips

56. RADIOSURGERY
 Gamma knife radiosurgery can be used either alone
or as an adjunct therapy before/after endovascular
intervention
 the 22-mo average lag between treatment and
complete symptom relief is a significant drawback
 inability to manage emergencies and traumatic
fistulae inhibit the usage of radiosurgery as a first
line treatment