Carotid-Cavernous Fistule

Case series
Preface

Definition • abnormal communication between the carotid arterial system

and the cavernous sinus.

Etiology • Mostly due to trauma involving basal skull fracture

Sign & • Mostly present with some ocular manifestation : conjunctival

chemosis, proptosis, pulsating exophthalmos, diplopia,

symptom
ophthalmoplegia, congestion of the retinal veins, secondary
glaucoma,orbital pain, audible bruits and blindness.
Case report

• 55 years old of female admitted to our neuroophthalmology clinic

M. Djamil Hospital Padang in April 17 th 2018.

• Chief complaint : Sudden bilateral redness eyes over the past 2

weeks.
Case report
history of illness

• Sudden bilateral redness over past 2 weeks

Recent • Followed by bilateral protruding & exhibited visual disturbance gradually
• Limited movement of eyeball to temporal side

• No history of trauma
Past • No preciding history of vascular disease
• Never performed blood glucose and lipid profile test

Family • No history of inherited medical disease

Case report
physical examination

Vital sign
• BP: 110/70mmHg
• HR 72x/minute
• RR 18x/minute
• T 37 C
Case report
physical examination (con’t)
Case report
clinical figures
Case report
supportive examination

Metabolic panel

• Fasting glucose test: 160 mg/dl

• Two-hours post-prandial glucose
test: 370 mg/dl
• Cholesterol: 239 mg/dl
• Triglyceride: 291 mg/dl
Case report
supportive examination (con’t)

Brain CT :

Bilateral enlargement
of superior
ophthalmic veins
 Case report
diagnosis, planning and treatment

Diagnosis Planning Treatment

• Suspect bilateral carotid- • Angiography CT scan • Timolol ed 0,5 % twice a day ODS
cavernous fistule
• Consult to internal departement • Artificial tears ed 4-times a day
ODS

• External manual compression of

cervical carotid artery

• Internal diagnosis : diabetes melitus type 2 + dislipidemia

• Internal planning : regulation of dietery intake + metformin 500 mg /q8h
Follow up
week 2

Anamnesis ophtalmology examination

• Symptom of swollen dan

redness reduce
Follow up
week 2 (con’t)

opthalmology examination Ancilary test

Random blood glucose : 160 mg/dl

Follow up
week 2 (con’t)

Brain MRI :

Bilateral dilatation
of superior
opthtalmic vein
and medial rectus
muscle
Follow up
week 2 (con’t)

Diagnosis Treatment

• Bilateral carotid-cavernouse fistule • Timolol ed 0,5 % twice a day ODS

• Artificial tears ed 4-times a day ODS

• Metformin 3x500 mg

• External manual compression of

cervical carotid artery

• Control glucose and lipid dietary intake

Follow up
week 6

Anamnesis Ophtlamology examination

• The redness on both eyes

gradually reduced.
• There were no swollen and
pain.
Follow up
week 6 (con’t)

Ophtalmology examination Ancilary test

Metabolic panel

• Fasting glucose test: 126 mg/dl

• 2-hours pos-prandial glucose test: 143 mg/dl
• Cholesterol: 181 mg/dl
• Triglyceride: 207 mg/dl
Follow up
week 6 (con’t)

CT scan angiography
• bilateral widening of superior
ophthalmic vein

• dilatation of cavernous sinus and

abnormal vascular connection
between cavernous

• sinus and internal carotid artery

 Follow up
week 6 (con’t)

Diagnosis treatment

• Bilateral carotid-cavernous fistule • Artificial tears ed 4-times a day

ODS

• Metformin 3x500 mg

• External manual compression of

cervical carotid arter

• Control glucose and lipid dietary

intake
 Follow up
week 10

Anamnesis Ophtlamology examination

Redness and swollen were

totally disapperead
 Follow up
week 10 (con’t)

Opthlamology examination Ancilary test

• Random glucose test: 160 mg/dl

 Follow up
week 10 (con’t)

Diagnosis Treatment

• Bilateral carotid cavernous fistule • Artificial tears ed 4-times a day ODS

• Metformin 3x500 mg

• External manual compression of

cervical carotid artery

• Control glucose and lipid dietary

intake
 Discussion
anatomy of cavernous

• Cavernous sinus is unexpansible extradular

venous plexus which relate to intra
cavernous ICA and meningeal branches of ICA Meningeal
and ECA branches
of ICA &
ECA

• It also relate to cranial nerves and other Determine

nerves : the cause
Intra
cavernous and
ICA management
• Lateral wall of cavernous sinus : N.III,
N.IV, first branch of N.V of CCF
• Lateral of ICA : N.VI
Cavernous
• Course in the surface of artery :
sinus
sympatic nerves

Ellis JA, Goldstein H, Connoly S. Carotid-Cavernous Fistulas. Neurosurg Focus 2012; 32 (5):E9.
Discussion
anatomy of cavernous sinus
Discussion
disease classification

Classification of CCF could classified as mentioned below :

• Direct
Anatomical • Indirect
High-flow vs low flow Patient in this report
was considered as :

• Trarumatic event indirect CCF probably

Etiology • Non traumatic event precipitated by
atherosclerosis event
(dyslipidemia and
• Hypertension, atherosclerosis
Hemodynamic event
DMT2)

Skuta GL, Cantor BL, Weiss JS. Carotid Cavernous Fistulas. In: Orbit,Eyelid and Lacrimal System. San
Fransisco : American Academy of Ophthalmology Section 7; 2016-2017. Pp 67-69.
 Discussion
classification (con’t)

Other classification of CCF was introduced by barrow (1985) based on

angiogrpahy

Type B
Type A
Communication of dural
Communication of ICA
ICA branches and
and cavernous sinus
cavernous sinus

Type C Type D
Communication of dural Communication of Dural
external carotid branches of ICA +
branches and cavernous external carotid artery
sinus with cavernous sinus

Preechawat P, Narmkerd P, Jiarakongmun P, Poonyathalang A, Pongpech SM. Dural Carotid Cavernous Sinus Fistula: Ocular Characteristics, Endovascular Management and
Clinical Outcome. J Med Assoc Thai 2008; 91:852-8.
 Discussion
ocular manifestation

Ocular manifestation Type of manifestation

mechanism
• Ocullar manifestation is due to bloodflow • Proptosis
throught CCF and its impact adjacent tissue • Eyelid & facial changes
(cranial nerve)
• Conjuctival chemosis
• reduced arterial flow to the cranial nerve within • Arterialization of conjunctiva and episcleral vein
the cavernous sinus • Ocullar pulsation
• the stasis of both venous and arterial circulation • Bruit
in the eye and orbit
• Diplopia
• increase in episcleral and orbital venous pressure • Opthtalmoscopic finding
• Trigerminal nerve dysfunction
• The site of fistule could be ipsilateral • glaucoma
(mostly),bilateral or even contralateral

Gupta S, Thakur AS, Bhardwaj N, Singh H. Carotid Cavernous Sinus Fistula Presenting with Pulsating Exophthalmos and Secondary Glaucoma. J Indian Med
Assoc. 2008; 106:312.
 Discussion
ocular manifestation

Proptosis Eyelid and facial changes

• Most common sign  could occurred
simultaneously / gradually
• Mostly occurs in ipsilateral side, but
could also occur in contralateral side
• Eyelid could be swollen in CCF
• if the superior ophthalmic vein
widens, the medial part of the upper
eyelid may be stretched and swollen

• Significant proptosis could be

followed by cranial bruit / pulsation

In this patient :
• Proptosis occur gradually in both eye
• Eyelide of patient also be swolen
 Discussion
ocular manifestation

Conjunctival chemosis Arterialization of conjunctiva

and episcleral vein
• Occur before proptosis occurred • Conjuctiva & episeclral vessel become more
more prominent when its happen prominent due to arterial blood flow throught
it  mimic conjunctivitis

• In severe case, Conjuctival could

prolapse become necrotic and • Arterialization could be widespread or limited
infected to only some vessel

In this patient :
• Conjunctival chemosis occurred mostly in temporal of conjunctiva bulbi
• Arterialization of episcleral patient also occurred this patient
 Discussion
ocular manifestation

Ocullar pulsation Corneal damage

• Due to transmitting pulse from ICA to • Keratopathy mostly occurred in direct
ophthalmic vein CCF

• The pulsation could also transmit to • Keratopathy is due to severe proptosis or

temporal fossa, eyelid and orbit neural paresistrigerminal neuropathy

• Pulsation mostly occur due to trauma

 direct CCF

In this patient :
• There are no pulsation detected in this patient indirect CCF
• There was no keratopathy, severe proptosis and trigerminal neuropathy in this patient
 Discussion
ocular manifestation

Ocullar pulsation Corneal damage

• Due to transmitting pulse from ICA to • Keratopathy mostly occurred in direct
ophthalmic vein CCF

• The pulsation could also transmit to • Keratopathy is due to severe proptosis or

temporal fossa, eyelid and orbit neural paresistrigerminal neuropathy

• Pulsation mostly occur due to trauma

 direct CCF

In this patient :
• There are no pulsation detected in this patient indirect CCF
• There was no keratopathy, severe proptosis and trigerminal neuropathy in this patient
 Discussion
ocular manifestation

Bruit Diplopia

• Mostly occurred in direct CCF • Occurred in 60-70% case CCF, probably

due to asymmetric orbital movement 
optalmoparesis / ophtalmoplegia
• Bruit represent the heartbeat which
transmitted to ICA systolic phase
• Mostly affect abducen nerves located
within cavernous sinus
• Could be Subjective / objective bruit

In this patient :
• There are no bruit detected in this patient indirect CCF
• There was limitation in abduction of right eye
 Discussion
ocular manifestation

Ophtalmoscopic finding Trigerminal nerve dysfunction

• Facial pain mostly occurred in
• Widening retinal veins trigerminal area in patient with CCF
• Swolen optical disc & retinal
bleeding
Direct • Sensoric sensing alteration (hypo / hyper
• Subhyaloid & vitreous bleeding esthesia) also could be found in
CCF
• non-rhegmatogenous ablation retina trigerminal area of CCF
or choroid effusion (rare)

In this patient :
• Funducopic finding in this patient was considered in normal limit
• There is no sensoric sensing complain in trigerminal area of this patient
 Discussion
ocular manifestation

Glaucoma
• Occurred in 30-50% direct CCF cases

• Mostly due to direct orbital congestion  in association with severe proptosis & chemosis

• Intraocular pressure in most cases is usually somewhat elevated, but some patients experience
intraocular pressure of 50-60mm Hg

In this patient :

• When the patient admitted to our clinic at the first time, the IOP of left eye was
26mmHg but the right eye was still normal limit.

• after 2-weeks following topical antiglaucoma (timolol twice a day), the IOP was
reduced to be 16mmHg.
Discussion
imaging examination

Cerebral
angiography

Modality
Computed
tomogrpahy of MRI

imaging

Orbital
doppler
Rucker JC, Biousse V, Newman NJ. Magnetic Resonance
ultra Angiography Source Images in Carotid Cavernous
sonography
Fistulas. Br J Ophthalmol 2004; 88:311.
Discussion
orbital doppler ultrasonography

• Color doppler
• Marked by the presence of
flow reversal or thrombosis in
the superior ophthalmic vein
(SOV) at color Doppler

• Spectral doppler
• arterializations with low
resistance flow in SOV

Leibovitch I, Modjtahedi S, Duckwiler GR, Goldberg RA. Lessons Learned from Difficult or Unsuccessful Cannulations of the
Superior Ophthalmic Veinin the Treatment of Cavernous Sinus Dural Fistulas. Ophthalmology 2006; 113:1220-6.
 Discussion
computed tomography

CCF features on CT :

• proptosis of the affected globe

CT in this patient
• dilatation of the cavernous sinus and shows :
superior ophthalmic vein
• bilateral
• enlargement of the extraocular muscles enlargement of
ophthalmic vein
• Periorbital fat edema
• There’s no widening
• Other features which might be affect of cavernous sinus
the cavernous sinus (skull fracture,
intracranial haemorage)

Skuta GL, Cantor BL, Weiss JS. Carotid Cavernous Fistulas. In: Orbit,Eyelid and Lacrimal System. San Fransisco :
American Academy of Ophthalmology Section 7; 2016-2017. Pp 67-69.
 Discussion
magnetic resonance imaging

• More superior than CT

• Could detect minimum superior
ophthalmic vein dilation, subtle
proptosis and small extraocular
muscle

• Sensitivity could be enhanced

by using paramagnetic
contrast, T2-weighted and SWI
MRI in this patient shows :
sequences.
bilateral enlargement of superior ophthalmic veins
Santos D, Monsignore LM, Nakiri GS. Imaging Diagnosis of Dural and medialrectus muscle, and cavernous sinus without any
Direct Cavernous Carotid Fistulae. Radiol Bras 2014; 47(4):251-255.
unilateral proptosis
 Discussion
cerebral angiography

• Cerebral angiography is gold • abnormal connection

standard method for between cavernous sinus
diagnosing CCF and internal carotid artery

• Bilateral dilatation and

• Role of angiography tuortosity of superior
ophthalmic veins in sella
tursica
• Indirect CCF : detects the
fistula and identifies the
supplying meningeal branches

• Direct CCF : detect internal • Enhancement of density

carotid artery wall laceration indicate widening of
cavernous sinus
Fifi JT, Meyers PM, Lavine SD, Cox V, Silverberg L, Mangla S,
et al. Complications of Modern Diagnostic Cerebral Angiography
in An Academic Medical Center. J Vasc Interv Radiol 2009;
20:442–447
Discussion
treatment modality

consevative

Treatment of
elevated IOP endovascular
CCF
treatment
modality

Radiosurgical Surgical
Barry RC, Wilkinson M, Ahmed RM, Lim CS, Parker GD,
McCluskey PJ, et al. Interventional Treatment of Carotid Cavernous
Fistula. J Clin Neurosci 2011; 18:1072–1079.
 Discusion
conservative treatment

• manual compression of the ipsilateral cervical carotid artery several times a day for 4–6 weeks

Cervical • effective for low-flow CCF

carotid • 1 year Recurrence free rate in indirect low-flow CCF 30%
artery
compression • Need close observation (funduscopic, IOP, visual acquity, chief complain)

• Failure of CCF closure after compression need other alternative therapy

In this patient :

• Has considered as indirect low flow CCF

• Plan : compression of the cervical carotid artery several time a day Ellis JA, Goldstein H, Connoly S. Carotid-Cavernous
• Result : complain resolve in 2-3 mo spontaneosly Fistulas. Neurosurg Focus 2012; 32 (5):E9.
Discusion
endovascular intervention

Modality : Transarterial or transvenous embolization

• first line therapy in most cases

• Caution for CCF from ICA branches  Increased risk for developing stroke
due to embolic reflux

• Effectivity for fistula occlusion is up to 90%

Morón FE, Klucznik RP, Mawad ME, Strother CM. Endovascular Treatment of High-Flow Carotid Cavernous Fistulas by Stent
Assisted Coil Placement. AJNR Am J Neuroradiol 2005; 26:1399-404.
Discusion
surgical intervention

• Waranted if endovascular intervention failure

• Procedure includes :
• suturing, clipping, or trapping the fistula
• packing the cavernous sinus to occlude the fistula
• sealing the fistula with fascia and glue
• ligating the internal carotid artery
• combination of all these procedures

• Overall success rates have been reported at between 31% and 79%

Preechawat P, Narmkerd P, Jiarakongmun P, Poonyathalang A, Pongpech SM. Dural Carotid Cavernous Sinus Fistula: Ocular Characteristics,
Endovascular Management and Clinical Outcome. J Med Assoc Thai 2008; 91:852-8.
Discusion
radiosurgical intervention

• Might be beneficial in indirect, low-flow CCF

• Succesfull rate up to 91.6 %

• In patient previously has direct CCF, succesfull rate by this modality only 2
from 5 patient (40%)

• Should not be used in emergency case  latency periode until

fistule closed need several month to years

Gemmete JJ, Chaudhary N, Pandey A, Ansari S. Treatment of Carotid Cavernous Fistulas. Curr Treat Options Neurol 2010; 12: 43–53.
 Discusion
treatment of elevated IOP

• Elevated IOP in CCF is caused by

elevation of episcleral venous in this patient :
preassure
 the patient had high IOP on her first admission
• Usualy respond to topical to our clinic After using timolol eyedrop twice
antiglaucoma medication a day for 2-weeks then the IOP was reduced from
26 mmHg to 16 mmHg on her left eye, the right
one was within normal IOP.
• Unresponsive by those therapy
indicate the need of closuring CCF  Timolol had been continued for 2-weeks and the
IOP was stable
• Iridotomy was indicate if Angle-
closure glaucoma was present  After 1 month of follow-up, timolol was stopped.
secondary to uveal congestion and
forward rotation of the ciliary body
Su Chang, Juan Pablo, Timo Krings. Low-Flow Direct Carotid Cavernous
Fistula Caused by Rupture of an Intracavernous Carotid Aneurysm.
Interventional Neuroradiology 20:476-481, 2014. Pp 476-481.
conclusion

• A carotid-cavernous fistula is an abnormal communication between the carotid arterial

system and the cavernous sinus which is most common caused by trauma (70-90%)

• Classification of CCF is based on anatomical, hemodynamic and etiological factors

• Diagnosis of CCF can be made by some ocular manifestations which was found in our patient.

• Imaging examination is useful in the diagnostic workup of CCF

• The goal of CCF treatment is to completely occlude the fistula while preserving the normal
flow of blood through the internal carotid artery

• conservative management is effective in the treatment of patient with indirect low-flow CCF.
Danke