DR. P. JOHN PAUL

CAROTID – CAVERNOUS SINUS FISTULA

20 year old gentleman a manual laborer by occupation admitted with c/o buzzing sound in the left ear for past 4 months c/o protrusion of left eye ball for past 3 months

History of presenting illness : Patient was apparently normal before 8 months when he met with an RTA H/o loss of consciousness + H/o Vomiting + H/o left ear bleed and nasal bleed + There was no history of seizures

Patient was initially treated at nearby General Hospital & referred to our higher referral Centre At our institute patient was treated conservatively and discharged .,

At that time of discharge , Patient GCS 15/15 Bilateral Pupils Equal & Reacting to Light External Ocular Movements full No facial lag Patient was ambulant Vitals stable

4 months before patient first noticed a buzzing sound in the left temporal region .

Initially it was mild in character and now patient experiences the sound even during night which disturbs his sleep sometimes

The intensity of sound increases after any heavy work

The buzzing sound is present intermittently

The buzzing sound is not associated with headache or vomiting

No H/o spinning sensation of self or surroundings

Patient noticed gradual protrusion of left eye ball for past 3 months

which increased in size

No H/o deviation of left eye ball to one side

Protrusion of left eye was not associated with pain

No H/o pulsation of the eye ball

H/o blurring of vision of left eye for past 6 months

Blurring of vision is more for distant objects

Able to perceive colors normally

No H/o diplopia

Patient noticed swelling of left eye lid for past 4 months which

initially started in a small size and gradually increased in size

H/o of dilated vessels seen over left upper eye lid for past 3 months

H/o redness present in left eye for past one week .

No H/o pain in the left eye

No H/o watering of left eye or itching

No H/o similar c/o in the right eye

No H/o fever or any neck swellings

No H/o loss of weight in the presence of increased appetite

No H/o evening rise of temperature

No H/o swelling or patches anywhere in the body

No H/o epistaxis

No H/o neck stiffness

Able to perceive smell normally in both nostrils

No H/o benumbed sensation over face

No H/o difficulty in chewing

No H/o deviation of angle of mouth or drooling of saliva

No H/o taste disturbances

H/o blocked sensation in the left ear for past 7 months

No H/o of ringing noise in the ears

No H/o spinning sensation of head or surroundings

No H/o nasal regurgitation / Difficulty in swallowing

No H/o difficulty in shrugging the shoulders or turning head

side to side

No H/o difficulty in marshaling food

No h/o weakness of limbs No H/o sensory disturbancesNo H/o palpitation or abnormal sweating No H/o bladder or bowel disturbancesNo H/o swaying or dysarthriaNo H/o suggestive of higher mental function disturbances

Past History : Not a Known DM / HT / TB pt No H/o previous surgeries Personal History : Known smoker and alcoholic

Family History : No H/o similar illness among family members

EXAMINATION Moderately built Moderately nourished Not pale / icteric No neck nodes palpable No swelling or patches anywhere in the body PR : 100 /min BP : 130 /80 mm Hg RR : 20 /min

Patient is Conscious Oriented Speech , language and memory normal Right handed individual MMSE 30/30

Cranial Nerves : Cranial Nerves Right Left

I Normal Normal

II VA

20/20 20/70

VF

Normal Normal

CV

Normal Normal

Fundus No disc edema No disc edema

No optic atrophy No optic atrophy

No new retinal vessels

No new retinal vessels

III , IV ,VI Cranial Nerves : Eye ball is in mid position External Ocular Movements full Pupils equal and reacting to light normally No nystagmus Mechanical ptosis Distant between upper eyelid margin to pupillary margin 5mm on right side , 2 mm on left side Local Examination left eyelid : Diffuse swelling of left upper eyelid Dilated tortuous vessels seen True proptosis + , Nafziger’s sign +ve Proptosis is pulsatile in nature Redness is present in the conjunctiva , redness extends beyond upper eyelid margin

On palpation , the swelling is warm , not tender swelling is soft and compressible palpable thrill + orbital margins normal audible bruit + V , VII cranial nerves normal VIII cranial nerve : Weber – lateralised to left ear Rinnie – BC > AC on left side IX , X , XI , XII normal on both sides Spinomotor System : Bulk , Tone , Power normal in all 4 limbs Reflexes : Superficial reflexes – normal Deep tendon reflexes – normal Gait normal

No sensory disturbances No cerebellar signs No signs of meningeal irritation Spine and cranium appear normal

Other system examination : CVS : S1 S2 + No added sounds RS : NVBS + Abdomen : Soft , No organomegaly

CT BRAIN PLAIN IMAGES WITH BONE WINDOW

MRI IMAGES

CT ANGIO IMAGES

64 SLICE CT CEREBRAL ANGIOGRAM

Evidence of enlargement of left cavernous sinus and dilated

tortuous para cavernous veins with several multidirectional

draining veins

All veins showed opacification in arterial phase itself

Anteriorly – enlarged dilated , tortuous left superior

ophthalmic vein draining into cavernous sinus

Evidence of dilated tortuous supraorbital , supratrochlear ,

angular vein, retromandibular vein in the left side draining

through the external jugular vein .

Posteriorly- enlarged left inferior petrosal sinus and IJV

Laterally – enlarged left sphenoparietal sinus

Contralaterally inter cavernous veins causing dilatation of right

cavernous sinus and right ophthalmic vein

Evidence of thickened extraocular muscles

Evidence of mild thinning of left optic nerve noted at the

orbital apex on left side

ANATOMY OF CAVERNOUS SINUS

•Cavernous Sinus is an extradural space contained between the

two layers of dura laterally and superiorly and

periosteum covering the lateral portion of the sphenoid sinus

and the sphenoid bone inferiorly and medially.

•Contents:

the ICA with its typical S shape

III, IV, VI Cranial nerves with V1, V2 divisions

CORONAL SECTION THROUGH C.S.

COMMUNICATIONS OF CAVERNOUS SINUS

ANTERIORLY :• superior and inferior ophthalmic veins • sphenoparietal sinus. •

THE TWO CSs COMMUNICATE THROUGH • the anterior and posterior intercavernous sinus forming the circular

sinus.

POSTERIOR DRAINAGE • basilar plexus • superior and inferior petrosal sinuses.

INFEROLATERALLY • dural veins draining into the pterygoid plexus.

Sl.No

TRIANGLE BOUNDARIES

MEDIAL LATERAL BASE CONTENTS

1 Anteromedial Cr.N II Cr.N III Antr petroclinoid fold

Distal horizontal ICA

2 Paramedial Cr.N III Cr.N IV Antr petroclinoid fold

Horizontal ICA

3 Parkinson Triangle Cr.N IV V1 Antr petroclinoid fold

Distal Horizontal ICA

4 Anterolateral ( Mullen’s ) V 1 V 2 SOF to Foramen rotundum

Horizontal ICA

5 Lateral V 2 V 3 F.rotundum to F.ovale

Lateral loop of ICA

6 Posterolateral ( Glassock’s ) GSPN Arcuate eminence to F.spinosum

V3 lateral margin

Postr & Lateral loop of ICA

7 Posteromedial ( Kawase’s ) V3 – Gasserian ganglion

GSPN Petrous apex Postr surface of medial loop of ICA

CAROTID – CAVERNOUS SINUS FISTULA

- abnormal communication between the carotid artery ( internal or external ) or its branches and the cavernous sinus

Classification :

1. Spontaneous or traumatic

2. High or low flow

3. Direct or indirect – according to their arterial supply

BARROW’S CLASSIFICATION Type A – Direct shunt between ICA to cavernous sinus

- high flow fistulas

- usually caused by trauma , base of skull #

- rupture of intracavernous ICA

- Young males are affected most commonly

may occur in Ehlers Danlos syndrome

- these fistulas don’t close spontaneously and hence

intervention is needed

TYPE A FISTULA

Type B , C, D fistulas are dural or indirect fistulas

- they are low flow fistulas

- occur in middle aged females

TYPE B :

Communication between meningeal branches of ICA and cavernous sinus

TYPE C :

Communication between meningeal branches of ECA and cavernous

sinus

TYPE D :

Communication between meningeal branches of both ICA and ECA and

cavernous sinus

These are the commonest indirect fistulae

TYPE B CCF

TYPE C CCF

TYPE D CCF

CLINICAL PRESENTATION - Orbital or retro orbital pain

- chemosis ( 55 % )

- pulsatile proptosis ( 72 % ., common in anteriorly placed CCF )

- ocular or cranial bruit ( 80 % )

- deterioration of visual acuity ( 18 % )

- diplopia –( causes )

ischemic dysfunction of cranial nerves,

mechanical compression of the nerves,

restricted movement secondary to venous engorgement of orbital contents.

- pupillary dilatation

- ophthalmoplegia ( initially unilateral & progresses bilaterally ( 24 % )

- exposure keratitis

- increased Intra Ocular Pressure - neo vascularization of iris or retina leading to retinal hemorrhages , CRVO or CRAO , cotton wool patches and retinal detachment - facial pain - Headache - Intracranial hemorrhages - raised ICP - rarely SAH - lower cranial nerve palsies ( 49 % ) - pulse synchronous tinnitus - epistaxis ( rare – but fatal ) - glaucoma

DIFFERNTIAL DIAGNOSIS

• AVM • Cavernous sinus thrombosis • cavernous sinus tumors • orbital tumors • skull base tumors • mucocele • thyroid eye disease • orbital pseudo tumor • orbital cellulitis

INVESTIGATIONS CT and MRI : usually demonstrate proptosis serpiginous and enlarged intraocular vessels including superior ophthalmic vein ( best seen in T2 Coronal ) convexity of lateral wall of cavernous sinus CATHETER BASED ANGIOGRAPHY : ( GOLD STANDARD )

rapid opacification of petrosal sinus or ophthalmic vein seen 1. Huber maneuver : Lateral view , inject VA and manually compress affected carotid , helps to identify upper extent of fistula 2. Mehringer – Heishima maneuver : inject contrast@ 2-3 ml /s into affected carotid while compressing the carotid in the neck below the catheter tip – will demonstrate the fistula

INDICATIONS FOR TREATMENT 1. Proptosis 2. Visual loss 3. Cranial nerve VI palsy 4. Intractable bruit 5 . Severely elevated IOP 6 . Increased filling of cortical veins on angiography

CHOICE OF TREATMENTDIRECT FISTULAS :• endovascular repair•detachable balloons •detachable coils•onyx (N-butyl-2-cyanoacrylate (NBCA))• covered stents

INDIRECT :•onyx• radiosurgery

ENDOVASCULAR TREATMENT Transarterial route : through ICA – direct type through ECA for dural fistulas

Transvenous route : through petrosal sinus through superior ophthalmic vein Open micro surgical Methods : 1. Anterior approach 2. Lateral appraoch

Stereotactic radiosurgery .

THANK YOU