Carotid-Cavernous Fistula A Case Report.
Carotid-Cavernous Fistula :A Case Report
1
2
I Komang Oka Krishna , I Wayan Niryana , Putu Patriawan
3
1
Departement of General Surgery, School of Medicine, University of Udayana, Bali
Department of Neurosurgery, Neurovascular Division, School of Medicine, University of Udayana, Bali
3
Departement of Radiology, Interventional Radiology Division, School of Medicine, University of Udayana,
Bali
2
Abstract
Introduction. We present a case of direct carotid-cavernous fistula (CCF). Direct carotid-cavernous fistula
are high-flow shunts with a direct connection between the internal carotid artery and the cavernous sinus.
The goals of treatment are to eliminate the fistula and preserve carotid artery patency
Presentation of Case: A 19-year-old female presented with left eye discomfort, increasingly edema of
tarsal conjunctiva, pulsatile tinnitus and progressive visual loss on the left eye.She had a recent history of
moderate head injury and maxillofacial fracture. Examination showed proptosis, chemosis and orbital
bruit. Digital subtraction angiography confirmed CCF, which was managed endovascularly. The patient
recovered after treatment with visual loss on her left eye.
Discussion. CCF has a variety of presenting clinical signs, imaging manifestations, and treatment options
available. Early diagnosis and prompt treatment to prevent loss of visual function in patients with CCF.
Conclusion. CCF is a rare and dangerous neurological disorder that should be promptly diagnosed and
treated.
Keyword :carotid-cavernous fistula, endovascular treatment, visual loss
Corresponding author :
I Komang Oka Krishna
Address :Departement of Surgery, General Sanglah Hospital
JalanDiponegoro Denpasar Bali ( 80114 )
Ph0361 227911
Mobile : 081338158989
Email : alinekrishna@gmail.com
Introduction
fistula (CCF) are high-flow shunts with a direct connec
onnection between the
Direct carotid-cavernous fistul
avernous sinus. CCF is a rare sight and lifethreatening
tening disorder, can be
internal carotid artery and the caver
s. spontaneous), velocity of blood flow (high vs. low flow)
flo
and anatomy
classified by etiology (traumatic vs.
s. external carotid vs. both). Some fistulas are charac
acterized by a direct
(direct vs. dural; internal carotid vs.
us ssegment of the internal carotid artery and the cavern
ernous sinus. These
connection between the cavernous
w ttype. Most often caused by a single, traumatic tear in
i the arterial wall,
fistulas usually are of the high flow
ernous sinus fistulas. Other CCFs are dural. Many of these lesions are
they are called direct carotid-cavernous
ous m
malformations that develop spontaneously, often
ten in the setting of
actually congenital arteriovenous
tension, connective tissue disease, and during or after childbirth. Dural
atherosclerosis, systemic hypertens
consist of a communication between the cavernous
nous sinus and one or
carotid-cavernous sinus fistulas cons
internal carotid artery, the external carotid artery, or both.
bo These fistulas
more meningeal branches of the inter
blood flow. The goals of treatment are to eliminate
nate the fistula and
usually have low rates of arteriall bl
preserve carotid artery patency.
Presentation of Case
ate head injury and
A 19-year-old female, was a motorcycle accident victim with moderate
one week stay in hospital. Reconstruction surgery and
an has performed
maxillofacial fracture that required on
y swollen
s
left eyelid,
with miniplate. In the interval of one month, she felt left eye discomfort ,increasingly
visual loss in the left eye.
pulsatile tinnitus and progressive vis
e sshowed proptosis, chemosis and orbital bruit.(Fig.1)
g.1) Visual acuity was
Examination of the left eye
ye and there were no other neurological findings. Head CT scan than
no light perception on her left eye
superior ophthalmic vein. (Fig.2) Due to the suspici
picion of a CCF, the
performed, showed a dilated leftt super
giography to diagnose and to perform embolization.
patient underwent an cerebral angiogr
graphy confirmed the presence of a high flow fistulous
fis
connection
Digital subtraction angiograph
d ar
artery and the cavernous sinus by demonstrating
ng rapid
r
filling of the
between the left internal carotid
nal carotid arterial injection (Fig 3)and than performed
perf
ballooning
cavernous sinus following interna
ful on the first procedure. (Fig.4) After three months
onths the patient had no
embolization which was successful
isual loss on her left eye (Fig.5)
further complications except for visua
Figure 1
ows showed chemosis, edema of tarsal conjunctiva
This figure show
andproptosis of the left eye.
Figure 2
Head CT scan showing dilated left superior ophthalmic vein.
Figure 3
High flow fistulous connection between the left internal carotid artery and the cavernous sinus by
demonstrating rapid filling of the cavernous sinus following internal carotid arterial injection
Figure 4
Post ballooning embolization showing complete obliteration of the CCF
Figure 5
This figure shows the status of the patient three month after ballooning embolization with great
improvement
Discussion
The complexity of carotid cavernous fistulas (CCF) is a result of their location within the unique
anatomical structure of the body where an artery crosses a venous structure. Through the superior and
inferior ophthalmic veins the cavernous sinus receives venous blood that is drained via the
sphenoparietal sinus, superior petrosal sinus, basilar plexus and pterygoid plexus. When passing through
the foramen lacerum the internal carotid enters into the cavernous sinus and in the posterior wall it is
divided into three segments: 1) posterior ascending segment, 2) horizontal segment, the largest segment
1,2
of the intracavernous carotid, 3) superior ascendant segment.
Abnormal communications between the cavernous sinus and the carotid artery can be classified
by etiology (traumatic; spontaneous; iatrogenic), by flow dynamics (low flow; high flow) and by anatomy
(direct versus dural; ICA versus external carotid artery (ECA) versus both). In direct CCFs there is a direct
communication between the ICA and the cavernous sinus. An indirect CCF is defined as an
arteriovenousfistula between the meningeal branches in the dura and the cavernous sinus. A clinically
useful classification is the anatomical-angiographic classification by Barrow et al. and is based on the
communication between the carotid artery, its branches and the cavernous sinus in which fistulas are
2,3
divided in four types.
Up to 76% of CCFs are traumatic caused by severe injuries such as vehicle accidents or
2,3,4
penetrating traumas. CCFs are seen in 0.2-0.3 % of craniofacial trauma
Direct fistulas are the most
common and are caused by trauma in 70 to 90% of the time, mainly basilar skull fracture. They occur
more often in young adult men because of their higher involvement in accidents .They can also be
iatrogenic (rhinoseptoplasties, endoscopic sinonasal surgeries, transsphenoidalhypophysectomy) or
spontaneous by aneurysm rupture in the cavernous portion of the carotid or congenital malformation that
break spontaneously due to vascular disease linked to collagen (e.g. Ehlers-Danlos syndrome,
atheromatous disease or hypertension). Spontaneous fistulas have a higher incidence in postmenopausal women, with no predominance in a particular When compared to dural fistula, the size of
direct lacerations direct fistula is proportionally greater and determines the difference of the endovascular
3,4.
treatment between them
Direct CCFs have a different hemodynamic change compared to the dural fistulas. After the
establishment of the arteriovenous fistula the blood pressure and flow are transmitted to the cavernous
sinus. This causes reverse flow and venous dilation of the veins that drain into the sinus, as well as an
increased flow in their usual draining veins. When they reverse their venous flow the ophthalmic veins
cause engorgement and orbital congestion that prevents drainage of the aqueous humor and increases
intraocular pressure and secondary glaucoma. The elevated intraocular pressure may compromise retina
perfusion and result in decreased visual acuity, a surgical emergency. Eye oedema resulting from the
impairment of venous drainage may lead to mechanical limitations of the extraocular muscles which may
3,5,6
already have their function compromised by compression on the cranial nerves
The intercavernous sinuses can transmit the same hemodynamic changes, to the other side
resulting in a similar clinical picture. The result of blood flow reversal and increased venous pressure in
the ophthalmic veins can result in exophthalmos, ophthalmic fremitus, chemosis, proptosis, and diplopia.
Loss of vision occurs in about 90% of direct CCFs and 20 to 30% of indirect CCFs.Glaucoma and retinal
perforation are symptoms of direct CCF and may appear days or weeks after the TBI. Ischemia involving
cranial nerves III, IV, V and VI may be premature and evident due to mass effect on the cavernous sinus.
This is different from the indirect CCF that has slow evolution and usually manifests with ocular
hyperaemia and tortuous arterializations of the conjunctiva, without proptosis. After angiography 20 to
7
50% of indirect fistulas close spontaneously
The patient’s history and the clinical examination often point to the diagnosis: a direct CCF is
suspected in every patient with a painful red eye, chemosis , exophthalmia and orbital bruit, especially
2,3,4
after a previous head trauma. The gold standard complementary diagnostic method is the selective
digital arteriography of the carotid artery. This exam shows the size and estimates the damage of the
fistula. The tests that have a complementary role in diagnosis are magnetic resonance, computed
7,8.
tomography angiography and Transcranial Doppler
The differential diagnosis should include posterior scleritis, endocrine exophthalmia, idiopathic
inflammation of the orbit, retrobulbar bleeding, cavernous sinus thrombosis, malignancyof the orbit and
8,9,10
arteriovenous malformations of the orbit/cavernous sinus.
To ensure proper diagnosis we emphasize
the importance of multidisciplinary examination of the eye function and periorbital condition.
Treatment of a direct CCF is mandatory for immediate orbital symptom relief and to prevent the
development of intracranial venous hypertension. Most direct CCFs can be treated electively,urgent
treatment is necessary only in patients with progressive visual loss, corneal exposure, severe proptosis
with pain and high intraocular pressure, intolerable bruit, epistaxis, sphenoid sinus aneurysm, severe
1,2,4,11
retro-orbital pain, cortical venous drainage or coma
Carotid Cavernous Fistula treatment has
become more effective, safer and less invasive with the advent of new neuroimaging techniques When
4
the fistula is small and asymptomatic, further treatment is not indicated.
The goal of treatment is the occlusion of the fistula while maintaining carotid artery patency,
which is mandatory when the collateral blood flow via the contralateral ICA is insufficient. The treatment
1,2,4,5,6
of choice for direct CCFs is transarterial embolization with detachable balloons
A latex or silicone
balloon is mounted on a microcatheter and introduced in the cavernous carotid artery via the
percutaneous approach of the femoral artery. Direct puncture of the carotid artery can also be used. The
procedure is generally performed under general intubation analgesia. The high-flow shunt carries the
device to the venous site of the fistula, where the balloon is then inflated and detached in situ. A complete
obliteration can be achieved in 80-90% of all direct CCFs. The carotid artery blood flow can be preserved
4,11,12
in up to 75% of patients
The size of the cavernous sinus and of the fistula may affect the success
rate of the procedure. When the size of the fistula is too large or incompatible with the size of the balloon,
the use of electrodetachable coils decreases the size of the hole allowing for the occlusion of the
13
remaining fistula. . Radiological examination of the skull is necessary after embolization to control the
location of the balloon. In select cases, open surgery, radiosurgery, or conservative management are also
treatment options. Symptom resolution with low rates of recurrence can be expected in most cases after
1,2
appropriate therapy.
Liang found significant differences between the time of the first symptoms and embolization by
3
comparing groups with and without complications . Collaborating that with early detection and immediate
8,11,12
intervention it is possible to accomplish full visual recovery with proper treatment
. Intravenous
treatment has been recommended for indirect CCF. This is a safer treatment with less arterial thrombotic
events, lower rate of treatment failure and easier implementation. The most common complications
observed in the treatment of CCF by embolization are the migration of the balloon, formation of a false
aneurysm, on the internal carotid (which usually recedes spontaneously), perforation of the superior
ophthalmic vein, orbital haemorrhage and injury of the abducens and trochlear nerves. Post treatment
10.
recurrences and deaths have been reported
After successful occlusion of the CCF, the orbital symptoms gradually disappear, and in the
presence of elevated ocular pressure, resolution to normal intraocular pressure within 72 hours can be
expected. In some cases intraocular pressure normalization needs several months. The orbital
congestion resolves usually after several weeks, reconstitution of cranial nerve function may lag several
weeks (19). Diploplia almost always improves but in some cases, operative shortening of the lateral
8,10
rectus muscle may be necessary
When the CCF is left untreated, vision is eventually lost in 89% of
patients. In 1981, before the use of intra-arterial procedures, reported progressive visual loss in 28% of
14
patients, even with carotid artery surgery. More recently, visual acuity was preserved or improved in 94%
of cases after treatment with detachable balloons in patients with preoperative visual loss. There are only
10,11
some case reports of reversal of blindness after endovascular treatment.
Conclusion
CCF is a rare and dangerous neurological disorder that should be promptly diagnosed and
treated. Although rare, CCF is a serious complication that should be considered in the differential
diagnosis of ocular proptosis and visual loss associated with maxillofacial trauma. Cerebral angiography
is the gold standard imaging modality used in the diagnosis and classification of CCF. Although
historically difficult to treat, these lesions are now routinely managed with low rates of morbidity and
mortality. Endovascular intervention with a goal of complete fistula occlusion while preserving normal
blood flow through the internal carotid artery has emerged as the treatment of choice. In select cases,
open surgery, radiosurgery, or conservative management are also treatment options. Symptom resolution
with low rates of recurrence can be expected in most cases after appropriate therapy.
.
References
1. Ellis JA, Goldstein H, Connoly ES, Meyers PM. Carotid-cavernous fistulas.Neurosurg
Focus,2012, 32(5)
2. Miller NR. Dural Carotid-Cavernous Fistulas: Epidemiology, Clinical Presentation, and
Management.Neurosurgclin N Am.2012,23 :179-192
3. Liang W, Xiaofeng Y, Weiguo L, Wusi Q, Gang S, Xuesheng Z. Traumatic carotid cavernous
fistula accompanying basilar skull fracture: a study on the incidence of traumatic carotid
cavernous fistula in the patients with basilar skull fracture and the prognostic analysis about
traumatic carotid cavernous fistula. J Trauma. 2007, 63 (5): 1014-1020.
4. Naesens R, Mestdagh C, Breemersch M, Defreyne L. Direct carotid-cavernous fistula: a case
report and review of the literature. Bulletin de la SocieteBelgedOphtalmologie 2006:43–54.5.
5. Martin S, TeoM, Bhattacharya JAlakandy L.Carotico-cavernous fistula: An educational case
International Journal of Surgery Case Reports, 2013 (4): 858– 860
6. Guimares AC, de Carvalho GM, Chone CT, Pfeilsticker LN. Carotid cavernous fistula: A rare
complication of maxillofacial trauma. Head Neck Oncol, 2014 10;6(3):23.
7. Cheng JW, Wei RL, Cai JP, Zhu H, Li Y. Imaging diagnosis of carotid-cavernousfistula. Zhonghua
Yan KeZaZhi 2007;43:36–94
8. Charlin RE, Pacheco PB, Villarroel FW, Urbina FA. Carotid cavernous fistula: the importance of
its opportune diagnosis and treatment to prevent blindness. Rev Med Chil. 2004, 132(10):12211226.
9. Karaman E, Isildak H, Haciyev Y, Kaytaz A, Enver O. Carotid-cavernous fistula after functional
endoscopic sinus surgery. J Craniofac Surg. 2009, 20(2):556-558.
10. Yang ZJ, Li HW, Wu LG, Zheng JN, Zhang JD, Shi XW, Chu GR. Prognostic analysis and
complications of traumatic carotid cavernous fistulas after treatment with detachable balloon
and/or coil embolization. Chin J Traumatol. 2004, 7(5):286-288.
11. Tjoumakaris SI, Jabbour PM, Rosenwasser RH. Neuroendovascular management of carotid
cavernous fistulae. NeurosurgClin N Am 2009;20:447–52.
12. Lo D, Vallee JN, Bitar A, Guillevin R, Lejean L, Van Effenterre R, Chiras J. Endovascular
management of carotid-cavernous fistula combined with ipsilateral internal carotid artery
occlusion due to gunshot: contra-lateral arterial approach. ActaNeurochir. 2004, 146:403-406
13. Sawlani V, Phadke R, Kumar S, Gujral RB. Gugliemi detachable coils in the treatment of carotidcavernous fistula. Clinradiol. 2004, 59:86-90
14. Palestine G, Younge B, Piepgras D. Visual prognosis in carotid-cavernous fistula. Arch
Ophthalmol 1981; 99 : 1600-1603
1
2
I Komang Oka Krishna , I Wayan Niryana , Putu Patriawan
3
1
Departement of General Surgery, School of Medicine, University of Udayana, Bali
Department of Neurosurgery, Neurovascular Division, School of Medicine, University of Udayana, Bali
3
Departement of Radiology, Interventional Radiology Division, School of Medicine, University of Udayana,
Bali
2
Abstract
Introduction. We present a case of direct carotid-cavernous fistula (CCF). Direct carotid-cavernous fistula
are high-flow shunts with a direct connection between the internal carotid artery and the cavernous sinus.
The goals of treatment are to eliminate the fistula and preserve carotid artery patency
Presentation of Case: A 19-year-old female presented with left eye discomfort, increasingly edema of
tarsal conjunctiva, pulsatile tinnitus and progressive visual loss on the left eye.She had a recent history of
moderate head injury and maxillofacial fracture. Examination showed proptosis, chemosis and orbital
bruit. Digital subtraction angiography confirmed CCF, which was managed endovascularly. The patient
recovered after treatment with visual loss on her left eye.
Discussion. CCF has a variety of presenting clinical signs, imaging manifestations, and treatment options
available. Early diagnosis and prompt treatment to prevent loss of visual function in patients with CCF.
Conclusion. CCF is a rare and dangerous neurological disorder that should be promptly diagnosed and
treated.
Keyword :carotid-cavernous fistula, endovascular treatment, visual loss
Corresponding author :
I Komang Oka Krishna
Address :Departement of Surgery, General Sanglah Hospital
JalanDiponegoro Denpasar Bali ( 80114 )
Ph0361 227911
Mobile : 081338158989
Email : alinekrishna@gmail.com
Introduction
fistula (CCF) are high-flow shunts with a direct connec
onnection between the
Direct carotid-cavernous fistul
avernous sinus. CCF is a rare sight and lifethreatening
tening disorder, can be
internal carotid artery and the caver
s. spontaneous), velocity of blood flow (high vs. low flow)
flo
and anatomy
classified by etiology (traumatic vs.
s. external carotid vs. both). Some fistulas are charac
acterized by a direct
(direct vs. dural; internal carotid vs.
us ssegment of the internal carotid artery and the cavern
ernous sinus. These
connection between the cavernous
w ttype. Most often caused by a single, traumatic tear in
i the arterial wall,
fistulas usually are of the high flow
ernous sinus fistulas. Other CCFs are dural. Many of these lesions are
they are called direct carotid-cavernous
ous m
malformations that develop spontaneously, often
ten in the setting of
actually congenital arteriovenous
tension, connective tissue disease, and during or after childbirth. Dural
atherosclerosis, systemic hypertens
consist of a communication between the cavernous
nous sinus and one or
carotid-cavernous sinus fistulas cons
internal carotid artery, the external carotid artery, or both.
bo These fistulas
more meningeal branches of the inter
blood flow. The goals of treatment are to eliminate
nate the fistula and
usually have low rates of arteriall bl
preserve carotid artery patency.
Presentation of Case
ate head injury and
A 19-year-old female, was a motorcycle accident victim with moderate
one week stay in hospital. Reconstruction surgery and
an has performed
maxillofacial fracture that required on
y swollen
s
left eyelid,
with miniplate. In the interval of one month, she felt left eye discomfort ,increasingly
visual loss in the left eye.
pulsatile tinnitus and progressive vis
e sshowed proptosis, chemosis and orbital bruit.(Fig.1)
g.1) Visual acuity was
Examination of the left eye
ye and there were no other neurological findings. Head CT scan than
no light perception on her left eye
superior ophthalmic vein. (Fig.2) Due to the suspici
picion of a CCF, the
performed, showed a dilated leftt super
giography to diagnose and to perform embolization.
patient underwent an cerebral angiogr
graphy confirmed the presence of a high flow fistulous
fis
connection
Digital subtraction angiograph
d ar
artery and the cavernous sinus by demonstrating
ng rapid
r
filling of the
between the left internal carotid
nal carotid arterial injection (Fig 3)and than performed
perf
ballooning
cavernous sinus following interna
ful on the first procedure. (Fig.4) After three months
onths the patient had no
embolization which was successful
isual loss on her left eye (Fig.5)
further complications except for visua
Figure 1
ows showed chemosis, edema of tarsal conjunctiva
This figure show
andproptosis of the left eye.
Figure 2
Head CT scan showing dilated left superior ophthalmic vein.
Figure 3
High flow fistulous connection between the left internal carotid artery and the cavernous sinus by
demonstrating rapid filling of the cavernous sinus following internal carotid arterial injection
Figure 4
Post ballooning embolization showing complete obliteration of the CCF
Figure 5
This figure shows the status of the patient three month after ballooning embolization with great
improvement
Discussion
The complexity of carotid cavernous fistulas (CCF) is a result of their location within the unique
anatomical structure of the body where an artery crosses a venous structure. Through the superior and
inferior ophthalmic veins the cavernous sinus receives venous blood that is drained via the
sphenoparietal sinus, superior petrosal sinus, basilar plexus and pterygoid plexus. When passing through
the foramen lacerum the internal carotid enters into the cavernous sinus and in the posterior wall it is
divided into three segments: 1) posterior ascending segment, 2) horizontal segment, the largest segment
1,2
of the intracavernous carotid, 3) superior ascendant segment.
Abnormal communications between the cavernous sinus and the carotid artery can be classified
by etiology (traumatic; spontaneous; iatrogenic), by flow dynamics (low flow; high flow) and by anatomy
(direct versus dural; ICA versus external carotid artery (ECA) versus both). In direct CCFs there is a direct
communication between the ICA and the cavernous sinus. An indirect CCF is defined as an
arteriovenousfistula between the meningeal branches in the dura and the cavernous sinus. A clinically
useful classification is the anatomical-angiographic classification by Barrow et al. and is based on the
communication between the carotid artery, its branches and the cavernous sinus in which fistulas are
2,3
divided in four types.
Up to 76% of CCFs are traumatic caused by severe injuries such as vehicle accidents or
2,3,4
penetrating traumas. CCFs are seen in 0.2-0.3 % of craniofacial trauma
Direct fistulas are the most
common and are caused by trauma in 70 to 90% of the time, mainly basilar skull fracture. They occur
more often in young adult men because of their higher involvement in accidents .They can also be
iatrogenic (rhinoseptoplasties, endoscopic sinonasal surgeries, transsphenoidalhypophysectomy) or
spontaneous by aneurysm rupture in the cavernous portion of the carotid or congenital malformation that
break spontaneously due to vascular disease linked to collagen (e.g. Ehlers-Danlos syndrome,
atheromatous disease or hypertension). Spontaneous fistulas have a higher incidence in postmenopausal women, with no predominance in a particular When compared to dural fistula, the size of
direct lacerations direct fistula is proportionally greater and determines the difference of the endovascular
3,4.
treatment between them
Direct CCFs have a different hemodynamic change compared to the dural fistulas. After the
establishment of the arteriovenous fistula the blood pressure and flow are transmitted to the cavernous
sinus. This causes reverse flow and venous dilation of the veins that drain into the sinus, as well as an
increased flow in their usual draining veins. When they reverse their venous flow the ophthalmic veins
cause engorgement and orbital congestion that prevents drainage of the aqueous humor and increases
intraocular pressure and secondary glaucoma. The elevated intraocular pressure may compromise retina
perfusion and result in decreased visual acuity, a surgical emergency. Eye oedema resulting from the
impairment of venous drainage may lead to mechanical limitations of the extraocular muscles which may
3,5,6
already have their function compromised by compression on the cranial nerves
The intercavernous sinuses can transmit the same hemodynamic changes, to the other side
resulting in a similar clinical picture. The result of blood flow reversal and increased venous pressure in
the ophthalmic veins can result in exophthalmos, ophthalmic fremitus, chemosis, proptosis, and diplopia.
Loss of vision occurs in about 90% of direct CCFs and 20 to 30% of indirect CCFs.Glaucoma and retinal
perforation are symptoms of direct CCF and may appear days or weeks after the TBI. Ischemia involving
cranial nerves III, IV, V and VI may be premature and evident due to mass effect on the cavernous sinus.
This is different from the indirect CCF that has slow evolution and usually manifests with ocular
hyperaemia and tortuous arterializations of the conjunctiva, without proptosis. After angiography 20 to
7
50% of indirect fistulas close spontaneously
The patient’s history and the clinical examination often point to the diagnosis: a direct CCF is
suspected in every patient with a painful red eye, chemosis , exophthalmia and orbital bruit, especially
2,3,4
after a previous head trauma. The gold standard complementary diagnostic method is the selective
digital arteriography of the carotid artery. This exam shows the size and estimates the damage of the
fistula. The tests that have a complementary role in diagnosis are magnetic resonance, computed
7,8.
tomography angiography and Transcranial Doppler
The differential diagnosis should include posterior scleritis, endocrine exophthalmia, idiopathic
inflammation of the orbit, retrobulbar bleeding, cavernous sinus thrombosis, malignancyof the orbit and
8,9,10
arteriovenous malformations of the orbit/cavernous sinus.
To ensure proper diagnosis we emphasize
the importance of multidisciplinary examination of the eye function and periorbital condition.
Treatment of a direct CCF is mandatory for immediate orbital symptom relief and to prevent the
development of intracranial venous hypertension. Most direct CCFs can be treated electively,urgent
treatment is necessary only in patients with progressive visual loss, corneal exposure, severe proptosis
with pain and high intraocular pressure, intolerable bruit, epistaxis, sphenoid sinus aneurysm, severe
1,2,4,11
retro-orbital pain, cortical venous drainage or coma
Carotid Cavernous Fistula treatment has
become more effective, safer and less invasive with the advent of new neuroimaging techniques When
4
the fistula is small and asymptomatic, further treatment is not indicated.
The goal of treatment is the occlusion of the fistula while maintaining carotid artery patency,
which is mandatory when the collateral blood flow via the contralateral ICA is insufficient. The treatment
1,2,4,5,6
of choice for direct CCFs is transarterial embolization with detachable balloons
A latex or silicone
balloon is mounted on a microcatheter and introduced in the cavernous carotid artery via the
percutaneous approach of the femoral artery. Direct puncture of the carotid artery can also be used. The
procedure is generally performed under general intubation analgesia. The high-flow shunt carries the
device to the venous site of the fistula, where the balloon is then inflated and detached in situ. A complete
obliteration can be achieved in 80-90% of all direct CCFs. The carotid artery blood flow can be preserved
4,11,12
in up to 75% of patients
The size of the cavernous sinus and of the fistula may affect the success
rate of the procedure. When the size of the fistula is too large or incompatible with the size of the balloon,
the use of electrodetachable coils decreases the size of the hole allowing for the occlusion of the
13
remaining fistula. . Radiological examination of the skull is necessary after embolization to control the
location of the balloon. In select cases, open surgery, radiosurgery, or conservative management are also
treatment options. Symptom resolution with low rates of recurrence can be expected in most cases after
1,2
appropriate therapy.
Liang found significant differences between the time of the first symptoms and embolization by
3
comparing groups with and without complications . Collaborating that with early detection and immediate
8,11,12
intervention it is possible to accomplish full visual recovery with proper treatment
. Intravenous
treatment has been recommended for indirect CCF. This is a safer treatment with less arterial thrombotic
events, lower rate of treatment failure and easier implementation. The most common complications
observed in the treatment of CCF by embolization are the migration of the balloon, formation of a false
aneurysm, on the internal carotid (which usually recedes spontaneously), perforation of the superior
ophthalmic vein, orbital haemorrhage and injury of the abducens and trochlear nerves. Post treatment
10.
recurrences and deaths have been reported
After successful occlusion of the CCF, the orbital symptoms gradually disappear, and in the
presence of elevated ocular pressure, resolution to normal intraocular pressure within 72 hours can be
expected. In some cases intraocular pressure normalization needs several months. The orbital
congestion resolves usually after several weeks, reconstitution of cranial nerve function may lag several
weeks (19). Diploplia almost always improves but in some cases, operative shortening of the lateral
8,10
rectus muscle may be necessary
When the CCF is left untreated, vision is eventually lost in 89% of
patients. In 1981, before the use of intra-arterial procedures, reported progressive visual loss in 28% of
14
patients, even with carotid artery surgery. More recently, visual acuity was preserved or improved in 94%
of cases after treatment with detachable balloons in patients with preoperative visual loss. There are only
10,11
some case reports of reversal of blindness after endovascular treatment.
Conclusion
CCF is a rare and dangerous neurological disorder that should be promptly diagnosed and
treated. Although rare, CCF is a serious complication that should be considered in the differential
diagnosis of ocular proptosis and visual loss associated with maxillofacial trauma. Cerebral angiography
is the gold standard imaging modality used in the diagnosis and classification of CCF. Although
historically difficult to treat, these lesions are now routinely managed with low rates of morbidity and
mortality. Endovascular intervention with a goal of complete fistula occlusion while preserving normal
blood flow through the internal carotid artery has emerged as the treatment of choice. In select cases,
open surgery, radiosurgery, or conservative management are also treatment options. Symptom resolution
with low rates of recurrence can be expected in most cases after appropriate therapy.
.
References
1. Ellis JA, Goldstein H, Connoly ES, Meyers PM. Carotid-cavernous fistulas.Neurosurg
Focus,2012, 32(5)
2. Miller NR. Dural Carotid-Cavernous Fistulas: Epidemiology, Clinical Presentation, and
Management.Neurosurgclin N Am.2012,23 :179-192
3. Liang W, Xiaofeng Y, Weiguo L, Wusi Q, Gang S, Xuesheng Z. Traumatic carotid cavernous
fistula accompanying basilar skull fracture: a study on the incidence of traumatic carotid
cavernous fistula in the patients with basilar skull fracture and the prognostic analysis about
traumatic carotid cavernous fistula. J Trauma. 2007, 63 (5): 1014-1020.
4. Naesens R, Mestdagh C, Breemersch M, Defreyne L. Direct carotid-cavernous fistula: a case
report and review of the literature. Bulletin de la SocieteBelgedOphtalmologie 2006:43–54.5.
5. Martin S, TeoM, Bhattacharya JAlakandy L.Carotico-cavernous fistula: An educational case
International Journal of Surgery Case Reports, 2013 (4): 858– 860
6. Guimares AC, de Carvalho GM, Chone CT, Pfeilsticker LN. Carotid cavernous fistula: A rare
complication of maxillofacial trauma. Head Neck Oncol, 2014 10;6(3):23.
7. Cheng JW, Wei RL, Cai JP, Zhu H, Li Y. Imaging diagnosis of carotid-cavernousfistula. Zhonghua
Yan KeZaZhi 2007;43:36–94
8. Charlin RE, Pacheco PB, Villarroel FW, Urbina FA. Carotid cavernous fistula: the importance of
its opportune diagnosis and treatment to prevent blindness. Rev Med Chil. 2004, 132(10):12211226.
9. Karaman E, Isildak H, Haciyev Y, Kaytaz A, Enver O. Carotid-cavernous fistula after functional
endoscopic sinus surgery. J Craniofac Surg. 2009, 20(2):556-558.
10. Yang ZJ, Li HW, Wu LG, Zheng JN, Zhang JD, Shi XW, Chu GR. Prognostic analysis and
complications of traumatic carotid cavernous fistulas after treatment with detachable balloon
and/or coil embolization. Chin J Traumatol. 2004, 7(5):286-288.
11. Tjoumakaris SI, Jabbour PM, Rosenwasser RH. Neuroendovascular management of carotid
cavernous fistulae. NeurosurgClin N Am 2009;20:447–52.
12. Lo D, Vallee JN, Bitar A, Guillevin R, Lejean L, Van Effenterre R, Chiras J. Endovascular
management of carotid-cavernous fistula combined with ipsilateral internal carotid artery
occlusion due to gunshot: contra-lateral arterial approach. ActaNeurochir. 2004, 146:403-406
13. Sawlani V, Phadke R, Kumar S, Gujral RB. Gugliemi detachable coils in the treatment of carotidcavernous fistula. Clinradiol. 2004, 59:86-90
14. Palestine G, Younge B, Piepgras D. Visual prognosis in carotid-cavernous fistula. Arch
Ophthalmol 1981; 99 : 1600-1603