Carotid Cavernous Fistula after Mandibular Fracture : A Case Report

ABSTRACTIntroduction Carotid Cavernous Fistula (CCF) is a condition in which an abnormal communication exists between carotid artery system and cavernous sinus. This is a rare condition that occur in 0.17-0.27% patients after craniomaxilofacial trauma especially skull base fracture. Direct CCF usually developed within hours until few weeks after trauma. Only limited cases of delayed CCF after madibular fracture had been reported.Purpose To report a case of CCF developed 8 weeks after mandibular fracture in Cicendo Eye Hospital.Case A 18-year-old male with proptosis, lagophtalmos, episcleral injection, limited eye movement to superior, lateral, and inferior came to Cicendo Eye Hospital. His visual acuity, contrast, and color sensitivity were within normal limit. He had motorcycle accident 8 weeks before symptoms appeared. He had only mandibular fracture with no any other fracture. His CT-Scan revealed CCF. The patient planned to do digital substraction angiography by Neuro Surgery Department. He did carotid massage while waiting for definitive therapy. After 1 week his eye movement was slightly improved. Conclusion Carotid cavernous fistula could develop after mandibular fracture. Clinicians should monitor patient with head trauma until several weeks after trauma because the risk of delayed symptoms of CCF. Carotid massage could be advised while waiting for definitive therapy. Keyword carotid cavernous fistula, mandibular fracture, direct, trauma, delayed symptoms

INTRODUCTION

Carotid cavernos fistula (CCF) is a condition in which anomalous connection exist

between carotid artery system and cavernous sinus. Carotid cavernous fistula after

trauma occur in 0.17-0.27% patients. Post traumatic CCF is a rare condition that

could develops within hours until a few weeks after craniomaxillofacial trauma

especially skull base fracture. Therefore, many clinicians are not familiar with their

clinical presentations. Ophthalmologist may be the first physician to encounter a

patient with clinical manifestations of CCF.1–3

The untreated CCF may cause intracranial hemorrhage, visual loss, secondary

glaucoma, worsening pulsatile proptosis, exposure keratitis, cranial nerve deficits and

fatal epistaxis. All clinicians treating trauma to the maxillofacial region should be

aware of the possibility of such serious complications even though it is rarely

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happened. Only limited cases of delayed CCF after mandibular fracture had been

reported. 1,2,4–6 This is a case report of a patient who develop CCF 8 weeks after

mandibular fracture.

CASE REPORT

A 18-year-old male came to Neuro-ophtalmology unit of Cicendo Eye Hospital

with eyeball protrusion, limited eye movement, horizontal double vision, eye redness

on the left eye and whoosing sound since 1 month ago. He had motorcycle accident 3

months ago. He was felt from motorcycle and his head crushed the road. He wore

helmet but it slip off from his head. He was hospitalized for one day in RSUD

Ciamis. He was diagnosed with left mandibular fracture and planned to referral but he

refused to go at that time.

Figure 1 Limited eye movement on the left eye Source : Cicendo Eye Hospital

On physical examination in Cicendo Eye Hospital, he was fully allert and vital

sign was within normal limit. He had swelling and slight deformities on the left jaw.

The patient was ortotropia. Right eye movement was within normal limit. Left eye

movement was limited -2 to superonasal and superotemporal, -1 to nasal, and -3 to

temporal as seen on Figure 1. Ocular bruit was positive on the left eye. Visual acuity

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on both eyes are 1.0 with Snellen Chart. Intraocular pressure (IOP) was 17 mmHg on

the right eye and 23 on the left eye. He had left eye proptosis and 2 mm of

lagophtalmos. No ptosis on the left eye. Hertel examination on right eye was 15 mm

and left eye was 19 mm.

Anterior segment examination on the right eye was within normal limit. He had

corckscrew turtous episcleral vessels, mild corneal abration, and relative afferent

pupillary defect grade I on the left eye as seen on Figure 2A. Funduscopy findings on

right eye was round disc, defined border, cup to disc ratio (CDR) 0.6 and disc

cupping. Funduscopy findings on left eye was round disc, defined border, cup to disc

ratio (CDR) 0.6, disc cupping, and blood vessels turtousity as seen on Figure 2B.

Amsler grid, Ishihara color plate, and contrast sensitivity test result on both eyes were

within normal limit. Cranial nerve V1 examination showed numbness on the left side,

V2, and V3 within normal limit. Other cranial nerve were within normal limit.

A. B.

Figure 2 A.Episcleral Vessels Injection B. Blood Vessels Turtousity on Fundus Source : Cicendo Eye Hospital

Ocular Computed Tomography of optic disc showed bilateral enlargement of CDR

and disc cupping. No visual field defect found in this patient based on Humphrey

30.2 test. First CT-Scan that was done right after the accident revealed only left

mandibular fracture. No sign of basilar skull fracture, mid facial fracture, or any other

fracture. First CT-Scan also showed no sign of dilated cavernous sinus as seen on

Figure 3.

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Figure 3 First CT-Scan Revealed Only Mandibular Fracture Source : RSUD Ciamis

After visited Cicendo Eye Hospital, patient then underwent second CT-Scan

examination with contrast. The result of second CT-Scan revealed enlargement of

cavernous sinus and dilated superior ophtalmic vein. The CT-Scan also showed

proptosis on left eye as seen on Figure 4.

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Figure 4 Second CT-Scan Revealed Dilated Cavernous Sinus with Aneursysm of Left Carotid Artery and Left Ophtalmic Artery Source : Cicendo Eye Hospital

The patient diagnosed with Carotid Cavernous Fistula sinistra et causa Trauma,

keratitis exposure, secondary glaucoma OS, compressive optic neuropathy OS, and

mandibular fracture sinistra. Patient was consulted to Glaucoma unit and Neuro-

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surgery Department. The patient was treated with Citicholine tablet 1x1000mg,

Ofloxacin eye drop 6x1 OS, artificial tears 6x1 OS, Timol 0.5% 2x1 OS, and advised

to do carotid massage while waiting definitive therapy. Neuro-surgery department

planned to do Digital Substraction Angiography (DSA) to this patient.

Patient then came to follow up 1 week later. His IOP was 15 mmHg on the right

eye and 16 mmHg on the left eye. After 1 week of carotid massage, the patient’s

condition was slightly improved. His left eye movement was limited only -3 to

temporal as seen on Figure 5. Prognosis in this patient quo ad vitam dubia ad bonam,

quo ad functionam dubia ad bonam, quo ad sanactionam ad bonam.

Figure 5 The Eye Movement Slightly Improved after 1 Week Carotid Massage Source : Cicendo Eye Hospital

DISCUSSION

Anatomical knowledge of the cavernous sinus is essential for understanding the

clinical symptoms and treatment options for CCF. The cavernous sinus is a venous

channels network with extravascular space through which the Internal Carotid Artery

(ICA) and cranial nerve (CN) VI course as seen on Figure 6. CN VI is often the first

cranial nerve to be involved because it lies medially and not protected. The CNs III,

IV, and V (first and second divisions) run within the dura of the lateral wall of the

cavernous sinus. The orbit is the first structure to manifest the symptoms because

ophthalmic veins drain to cavernous sinuses. Carotid Cavernous Fistula will

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compromise CN III, IV, V, VI which was seen in this patient. Compromised CN III,

IV, VI cause limitation of eye movement in this patient. Compromised CN V caused

numbness on area around affecting eye.4,5,7

Figure 6 Coronal drawing of Cavernous Sinus Source : Williams4

In 1985, Barrow D classified CCF into 4 types including A, B, C and D as seen

on Figure 7. Type A is direct high-flow shunts between the ICA and the cavernous

sinus. Type A is usually caused by head trauma which is called traumatic direct CCF

like what was occured in this patient. High flow, direct fistulas will caused a bruit.

Type B is dural shunts between meningeal branches of the internal carotid artery and

the cavernous sinus. Type C is dural shunts between meningeal branches of the

external carotid artery (ECA) and cavernous sinus. Type D is dural shunts between

meningeal branches from both internal and external carotid arteries and the cavernous

sinus.3,4,8

Figure 7 Burrow Classification of CCF Source : Williams et al4

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Direct CCFs may occur in the setting of traumatic tear in the wall of the cavernous

segment of the ICA or spontaneously by cavernous ICA aneurysm rupture. The

cavernous segment of the ICA is especially susceptible to damage with severe

craniofacial trauma in the setting of basilar skull fractures particularly middle fossa

fractures. This fracture may damage the carotid canal. In this case report, patient

develop CCF with mandibular fracture. No fracture was found on other sites. Only

limited literature had been reported CCF with mandibular fracture. Paza et al

reported CCF 2 days after madibular fracture. In case of mandibular fracture, direct

CCF may develop due to acceleration-deceleration forces applied across the

cavernous segment of the ICA, which is relatively fixed in the carotid canal and at the

dural ring near the anterior clinoid. This indirect trauma to the cavernous portion of

the internal carotid artery resulted in tearing of the muscular wall of the artery and

bleeding into the sinus.2,4,9

Carotid-cavernous fistula causes increased pressure inside cavernous sinus. This

leading to retrograde blood flow, especially through the ophthalmic veins. Dilation

and arterialization of superior ophthalmic vein and its branches happen afterward.

Dilated veins increased in size, exerting pressure on bulbus and dislocating it. Venous

drainage of the orbit becomes progressively difficult leading to congestion

manifesting itself by proptosis, chemosis, and conjunctival arteriolarization.

Conjunctival arteriolarization is characterized by dilation and tortuosity of the

conjunctival and episcleral veins extending to the limbus. Diplopia occurs not only

due to dislocation of bulbus, but mainly due to affection of nerves III, IV, and VI.

Exposure keratopathy occurs as a result of proptosis. Increased episcleral pressure

and vortex venous pressure may result in elevated intraocular pressure (IOP) and

secondary glaucoma. Pulsatile tinnitus may develop as a result of turbulent arterial

blood flow through the venous system and an orbital bruit may be auscultated over

the orbit or cranium.1,9,10

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In 90% of cases, the symptoms appear within 2 to 3 weeks after injury. It is

important to remember that ocular signs of CCFs may develop with delayed

presentation in the setting of trauma. Shim et al reported direct CCF 7 month after

injury. In this patient, symptoms developed 8 weeks after injury.4–7

The gold standard in diagnosis of CCF is 4-vessel digital subtraction cerebral

angiography because it provides information on the location and size of the aneurysm

or fistula. CT with contrast is also quite useful. Revealing findings on CT Scan

include Cavernous Sinus enlargement, proptosis, extraocular muscle enlargement,

superior ophthalmic vein dilation, or dilation of cortical or leptomeningeal vessels.

However, the absence of these abnormalities on noninvasive imaging studies does not

exclude the diagnosis.While waiting DSA schedule, this patient underwent CT-

scan.1,7,9

Timely intervention is mandatory to prevent morbidity or mortality based on

patient's signs and symptoms. Patients with any associated visual impairment or

ocular conditions, such as glaucoma, need to be identified and treated. Secondary

glaucoma in this patient managed by medication. No visual impairment found in this

patient. First line treatment for direct CCF is endovascular intervention, which may

be accomplished using detachable balloons, coils, liquid embolic agents, or a

combination of these tools. Successful embolization of CCF will result in immediate

resolution of proptosis, chemosis, and bruits, but ophthalmoplegia and optic nerve

dysfunction may take 4 months to resolve.9–11

As dural CCFs often resolve spontaneously, low-risk cases may be managed

conservatively. Opposite to indirect CCFs (types B, C, and D), direct CCFs are less

likely to resolve spontaneously and usually require treatment in symptomatic patients.

In some large series, the rate of spontaneous closure of direct CCFs was reported to

be about 1.2–4%. The exact mechanism of these rare events remains a controversial

topic. Few documented cases of spontaneous resolution of type A fistulas are reported

in the literature, varying anywhere from a few days to 6 months. It is possible that the

initial breach in the wall of the carotid artery was very small, and spontaneously

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healed or endothelialized as with healing of an arterial dissection. Another

mechanism that has been postulated is the effect of iodinated contrast media on

vascular endothelium leading to thrombosis. 10,12,13

In another case report, carotid massage ipsilateral to the fistula resulted in

spontaneous closure 2 weeks after the patient’s catheter angiogram. Carotid

compression therapy has also been successful in closure of 17% of direct and 30% of

dural CCFs. While waiting definitive treatment, the patient was advised to compress

his left carotid artery manually about 1520 times a day as per tolerance . The patients

were instructed to compress the carotid artery and jugular vein with the contralateral

hand for 10 seconds. If the initial 10 seconds compression treatments were tolerated

without eliciting symptoms, their duration was gradually increased to a maximum of

several minutes per compression. The patient compressed the carotid artery and

jugular vein until pulsation was no longer palpable with the hand contralateral to the

lesion site. If weakness developed unbeknownst to the patient, the compressing hand

would fall and treatment automatically stop. The patients performed the external self-

treatment while sitting down; the initial sessions were monitored by a physician to

observe signs of neurological symptoms and bradycardia. Patients who reported

audible orbital bruit were instructed to continue the compression until the bruit

ceased.12–14

Eye movement in this patient slightly improved by carotid massage. Even though

limitation of eye movement in this patient decreased, the symptoms were not

completely resolved. Definitive therapy must still be done to remove the symptoms.

CONCLUSION

Carotid cavernous fistula could develop after mandibular fracture. Many patients

with CCF may initially present to an ophthalmologist, who should be able to make a

presumptive diagnosis in most cases. The ophthalmologist should be able to order

appropriate tests to help make a diagnosis and characterize the features of the disease.

In addition, the ophthalmologist should be able to monitor the course of the disease,

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including extraocular changes, fundus changes and IOP measurements. Clinicians

should monitor patient with head trauma until several weeks after trauma because the

risk of delayed symptoms of CCF. Carotid massage could be advised while waiting

for definitive therapy.

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