CAROTID-CAVERNOUS

FISTULA (CCF)
OVERVIEW
• Relevant anatomy
• Introduction
• Symptoms
• Signs
• Investigations
• Grading
• Management
• Follow-up
ANATOMY
• Cavern means cave in Latin
• Paired dural sinuses located in middle
fossa lateral to the body of sphenoid
bone
• Cavernous sinus is defined as dural
envelope in which the cavernous
segment of ICA courses
ANATOMY
• Extend from medial part of SOF
anteriorly, to the area between dorsum
sellae medially and upper part of
meckel’s cave
• Boat-like shaped with the narrow keel at
the SOF and posterior broader bow wall
lateral to the DS
• Medial wall is osteal dural layer
• Lateral wall is meningeal layer
 ANATOMY

• Boat-like shaped with the narrow keel at

the SOF and posterior broader bow wall
lateral to the DS
• Medial wall is osteal dural layer
• Lateral wall is meningeal layer
ANATOMY
• ICA - cavernous segment
• Between petrolingual ligament
and proximal dural ring
• CN
• III, IV, V1, V2, VI
ANATOMY
• INflow
• Superior & Inferior opthlamic veins
• Sphenoparietal sinus
• Middle meningeal vein
• OUTflow
• Superior & Inferior petrosal sinus
• Pterygoid plexus
• Basilar plexus
• COMMUNICATION
• Anterior & Posterior
intercavernous sinus
INTRODUCTION
• Communication between the carotid arterial system and cavernous
sinus, either
• Direct – between the cavernous segment of ICA and cavernous sinus or
• Indirect – between one or more dural branches of ICA, ECA or both and
cavernous sinus
• Also classified according to;
• Flow – low vs high
• Etiology – spontaneous vs traumatic
EPIDEMIOLOGY
• Incidence about 0.2% in patients with TBI
• Up to 4% in patients with base of skull fracture
• Direct/high flow CCF due to trauma accounts for 70% of all CCF
• Commonly present in young male
• Indirect/low flow/spontaneous CCF accounts for 30% of all CCF
• Commonly seen in older/post menopausal women
ETIOLOGY
• Traumatic
• More commonly associated with direct fistula
• TBI
• Iatrogenic – surgical, endovascular
• Spontaneous
• Indirect fistula
• Cavernous ICA aneurysm
• Atherosclerotic vessel
• Hypertension
• Connective tissue disease e.g. Ehlers-Danlos syndrome, FMD
SYMPTOMS
• Ocular
• Anterior draining fistula
• Inferior or superior ophthalmic vein (IOV/SOV)
• Non-ocular
• Posterior or inferior draining fistula
• Basilar venous plexus, inferior and superior petrosal sinus & sphenoparietal
• Direct fistula – severe rapid progressive symptoms (days-weeks)
• Indirect – mild-moderate slow progressive symptoms (weeks-months)
NON-OCULAR
• Uncommon
• Epistaxis
• Venous infarction
• Intracerebral bleed
• Cranial neuropathies
• Trigeminal, facial and ocular paresis
• Usually only one cranial nerve involvement – most common oculomotor
nerve
OCULAR SYMPTOMS
• Due to antegrade blood flow into IOV and SOV, stasis of both venous
and arterial circulation within eye and orbit, increase in episcleral and
orbital venous pressure and impaired arterial flow to the cranial
nerves within cavernous sinus
• Usually unilateral to the fistula, but can be bilateral or sometimes
contralateral
• Classic Dandy’s triad
• Pulsating exophthalmos
• Chemosis
• Orbital bruit
OCULAR SYMPTOMS
• Ocular pulsation + proptosis =
pulsating exophthalmos
• Proptosis – swelling and protrusion
of the eyeball
• Ocular pulsation due to
transmission of high wave pulsation
from ICA into the ophthalmic veins
• However, most of patients are not
conscious of increased ocular
pulsation
OCULAR SYMPTOMS
• Chemosis
• Red eye
• Because the tarsus of upper eyelid
is thicker and firmer, chemosis is
usually limited to inferior bulbar
and palpebral conjunctiva
• In more severe cases, the palpebral
conjunctiva may prolapse
OCULAR SYMPTOMS
• Orbital bruit
• Buzzing/swishing/roaring sound that synchronous with the heartbeat
• Usually disturbing the patients from working, relaxing or even sleep
• Subjective bruit – only heard by the patient
• Objective bruit – heard by both the patient and the examiner
• Palpable bruit = thrill
OCULAR SYMPTOMS
• Other ocular symptoms:
• Double vision
• Reduced visual acuity
• Orbital/ocular pain
• Facial pain/reduced sensation
• Eye lids swelling
CLINICAL FINDINGS
• Higher Mental Function
• Normal
• Unless in severe cases with posterior draining fistula causing cortical venous
infarct
CLINICAL FINDINGS
• CN II
• Chemosis with conjunctival/episcleral veins arterialization (“cork screw vessel”)
• Proptosis
• Eyelid oedema/swelling
• Observe for visible ocular pulsation (palpate the radial artery)
• Palpate for ocular thrill (palpate the radial artery)
• Auscultation
• VA – impaired
• Positive RAPD
• Fundoscopy – dilated/occlusion of retinal veins, optic disc swelling, retinal
hemorrhage, vitreous hemorrhage, retinal detachment
 Chemosis with radial
cork-screw vessels

Cork-screw vessels due

to arterialization of the
episcleral/conjunctival
veins

Right eye proptosis

with chemosis and
eyelid swelling
PROPTOSIS
• Stand at bthe back of pt, view from above
• Compare the portrusion on eyeballs
PROPTOSIS
• Using exophthalmometer,
proptosis can be objectively
measured
• Hertel exophthalmometer is most
commonly used
• Measure distance between lateral
cantus with the corneal apex on
sagittal plane
• Normal range between 10-21mm
• Difference >2mm between eyes is
considered as proptosis
PROPTOSIS
• Alternatively, it can be measured
using a ruler
PROPTOSIS
• Touch test
• If the eyeball touch the ruler/pen,
then consider proptosis
• Poor man’s technique
AUSCULTATION
• Using bell (faint, low pitch)
• Point of auscultation
• Eye – direct
• Frontal - direct
• Temporal - STA
• Zygoma/Maxilla - STA,IMAX
• Mastoid – PAA
• Occipital - OA
• Palpate contralateral carotid artery
• Hold breath to increase the bruit intensity
• Fixed eye at one position to eliminate eyelid
tremor
AUSCULTATION
AUSCULTATION
FUNDOSCOPY
• Dilated retinal veins
• Optic disc swelling
• Retinal haemorrhages
• Retinal detachment
• Choroidal effusion
CLINICAL FINDINGS
• CN III, IV and VI
• Eye position at rest
• Head posture for compensatory
• Ptosis
• Pupil size and reaction to light
• Restricted eye movements

• CN V
• Reduced sensation in V1 and V2 distribution
• Reduced/absent corneal reflex
DDX
• CCF
• Orbital tumors – optic sheath meningioma
• Spheno-orbital meningioma
• Orbital cellulitis/conjunctivitis
• Cavernous sinus thrombosis
• Vasculitis – Tolosa-Hunt syndrome
INVESTIGATIONS
• Tonometry
• Measure IOP
• Pneumotonometry
• Measure difference in ocular pulse
amplitudes (defined as difference
between systolic and diastolic IOP) of
1.6mmHg between 2 eyes
• 100% sensitivity and 93% specificity
(Golnik et al)

Right eye CCF showed ocular pulse

amplitude of 6mmHg, while left eye’s pulse
amplitude 2mmHg (4mmHg difference)
INVESTIGATIONS
• Orbital ultrasound
• Dilated SOV
• Congested and enlarged EOM
• Color doppler can evaluate flow
and direction of blood flow
• Presence of flow reversal in SOV
highly suggestive of CCF
INVESTIGATIONS
• CT/MRI
• Congested orbital with enlarged
EOM
• Dilated SOV
• Sensitivity for detection of fistula in
both in direct and indirect CCF
• CTA – 87%
• MRA – 80%
• DSA – 94.4%
• CT can be used to evaluate proptosis
PROPTOSIS ON CT (Park et al.)
• Hertel vs CT
• CT method 2 & 3 showed higher correlation in patient with proptosis
difference of more than 2mm between the eyes
• Pearson’s coefficient - 0.495, 0.634 & 0.635 respectively
INVESTIGATIONS
• DSA
• Gold standard
• Both diagnostic and therapeutic
• Grading – identify the feeding arteries in indirect CCF
• High-flow fistula usually show rapid opacification of cavernous sinus with
marked fistula drainage pattern (early opacification of SOV anteriorly or
petrosal sinus posteriorly)
• Low-flow show slower opacification of cavernous sinus with less marked
fistula drainage opacification
• Enlarged draining veins
• Retrograde flow, most commonly into the ophthalmic veins
Classification
BARROW CLASSIFICATION
TYPE A
TYPE B
TYPE C
TYPE D
TREATMENT
• Aim
• Obliterate the fistula communication and preserve the patency of the ICA
• Options
• Conservative Indications:
• Endovascular 1) Proptosis
• Surgery 2) Visual loss
• SRS 3) CN 6 palsy
4) Intractable bruit
5) Severe raised IOP > 25
6) Increased filling of cortical
veins on angiography
CONSERVATIVE
• Usually for indirect/low flow fistula
• Up to 70% of indirect CCFs close spontaneously
• ICA compression
• Contralateral hand
• Starting 4-6times per hour for 10s and titrating of the duration up to several
minutes
• Success rate ~ 35%
• Resolution between 2weeks to 7months
• Contradicted in patient with artherosclerotic disease
CONSERVATIVE
• SOV compression
• Using thumb over the superomedial orbital rim
• 10 mins
• 4-6 times daily
• Resolution in 4-6 weeks
• Lubricants for proptosis
• IOP-lowering agents
ENDOVASCULAR
• Preferred choice of treatment especially in direct fistula nowadays
• Indication for intervention
• Direct/high flow
• Uncontrollable IOP
• Unremitting diplopia
• Severe proptosis with corneal exposure
• Optic neuropathy
• Retinal ischemia
• Severe disturbing bruit
• Cortical venous drainage
• Failed conservative treatment
ENDOVASCULAR
• Transarterial via ICA
• Preferred route for direct
• Transvenous via IPS, SOV, IOV, facial vein
• IPS is preferred route
• Preferred route for indirect
• Material used
• Coils
• Detachable balloon
• Acrylic glue
• Ethylene vinyl alcohol polymer (onyx)
• +/- flow-diverter stent
SRS
• For indirect fistula
• 20-50Gy
• Delayed response
• Mild symptomatic patients
• Complete resolution with SRS in CCF range between 50-100%
SURGERY
• Rarely performed procedure for CCF nowadays
• Trapping,ligation or occlusion of the ICA, especially in direct fistula
• High morbidity from the ischaemic or embolic event
• May need to consider bypass surgery
• Surgical packing of the sinus with material promoting thrombosis
especially in indirect fistula
FOLLOW UP
• Intraoperative/postoperative DSA
• Check for the occlusion
• Recurrence especially in indirect types
• Resolution of the symptoms/signs
• Depends on the severity prior to intervention
• Days to month
• Pneumotonometer – reduce or equalization of ocular pulse amplitude
(only assess anteriorly draining fistula)
• Ophthalmology reassessment
REFERENCES
• Henderson AD, Miller NR. Carotid-cavernous fistula: current concepts in aetiology, investigation, and
management. Eye (Lond). 2018 Feb;32(2):164-172.
• Kurtz KJ. Bruits and Hums of the Head and Neck. In: Walker HK, Hall WD, Hurst JW, editors. Clinical
Methods: The History, Physical, and Laboratory Examinations. 3rd edition. Boston: Butterworths; 1990.
• Miller NR. Diagnosis and management of dural carotid-cavernous sinus fistulas. Neurosurg Focus.
2007;23(5):E13.
• Fernando Gallego, D., Rivas-Grajales, A. M., & Jose Gallego, C. (2015). Ocular Auscultation: A
Review. International Journal of Medical Students, 3(2), 102–106.
• Park NR, Moon JH, Lee JK. Hertel exophthalmometer versus computed tomography scan in proptosis
estimation in thyroid-associated orbitopathy. Clin Ophthalmol. 2019 Aug 2;13:1461-1467.
• Smith JH, Fugate JE, Claassen DO. Pearls & Oy-sters: the orbital bruit: a poor man's angiogram. Neurology. 2009
Oct 20;73(16):e81-2.
• Venturini M, Cristel G, Marzoli SB, Simionato F, Agostini G, Barboni P, De Cobelli F, Falini A, Bandello F, Del
Maschio A. Orbital color Doppler ultrasound as noninvasive tool in the diagnosis of anterior-draining carotid-
cavernous fistula. Radiol Med. 2016 Apr;121(4):301-7.
• Oxford Neurological Surgery