Carotid-cavernous fistula (CCF) is the most common arteriovenous

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Carotid-cavernous fistula (CCF) is the most common arteriovenous Powered By Docstoc


Dr Mazhar Ishaq
FRCS(Ed), FRCOphth (UK),
Professor of Ophthalmology, Army Medical College Rawalpindi,
Classified Eye Specialist, Military Hospital Rawalpindi.

Dr Muhammad Aamir Arain,
Registrar Ophthalmology, Military Hospital Rawalpindi.

Dr Saadullah Ahmed,
Registrar Ophthalmology, Military Hospital Rawalpindi.

Dr Muhammad Khizar Niazi,
FCPS, Registrar Vitreo-Retina Department
Classified Eye Specialist, Military Hospital Rawalpindi.

The study was self sponsored. We were thankful for the efforts of Dr
Lubna Adeeb, Abrar CT and MRI Rawalpindi for her cooperation and
efforts in making early diagnosis.

      Proptosis due to carotid cavernous fistula is rare sequelae of head
injury. We report a case of post traumatic, direct high flow cavernous
fistula that resolved spontaneously 06 weeks after carotid angiography. It
however resulted in loss of vision due to delay in early treatment. In all
cases of post traumatic proptosis, carotid cavernous fistula should always
be kept in mind.

      Carotid–cavernous sinus fistulas (CCF) can result from head injury
or may arise spontaneously. Post-traumatic cases account for about 75%
of direct fistulas between the internal carotid artery and the cavernous
sinus. These are often associated with fracture base of skull. [1-3]
Spontaneous closure of CCFs by thrombosis of the cavernous sinus is
uncommon, especially in the traumatic high flow type. [4] Cases of
spontaneous occlusion of a traumatic CCF after orbital angiography have
been reported. [5] We present the case of a patient who sustained a blunt
head injury resulting in direct type carotid–cavernous sinus fistula which
closes spontaneously without any intervention.


      Patient aged 37, presented on 7th May 2008 with history of head
injury three weeks prior to presentation followed by bulging of her left eye,
whooshing noises in her head and double vision. There was also history
of ptosis of her left eye, swelling of both upper and lower lids on left side,
left sided temporal headache and bleeding from left ear. There was no
previous history of any ocular and systemic diseases.
      Her general physical examination and systemic examination was
unremarkable.    Ophthalmic     examination    on    presentation   revealed
corrected visual acuity of 20/30 in right and 20/40 in left. Her pupils were
round but unequal in reaction in left eye as compared to right eye; rests of
her optic nerve functions were unremarkable. There was severe ptosis in
left eye.   Adnexa showed swelling of both upper and lower lids with
resistance to retropulsion. Conjunctiva showed chemosis and dilated
episcleral blood vessels.
       Her ocular movements were painful and limited in all directions of
gaze. Forced duction test revealed restriction of all extra ocular muscles.
She was having significant proptosis of 24mm with difference of 6mm
from other eye. Fundus showed dilated veins with no haemorrhages or
disc edema. Intraocular pressure was 38 mm of Hg with pulsating mires.
Gonioscopy revealed open angle with no neovascularization. Ocular and
cephalic bruits were audible. There was no increase in proptosis after
Valsalva maneuver showing absence of venous anomalies in orbit.
       On investigations there was fracture of petrous part of temporal
bone on X Ray Skull lateral view.      CT scan orbit axial view showed
prominent superior ophthalmic vein and engorged extra ocular muscles
on left side. Her Doppler ultrasound also revealed prominent superior
ophthalmic vein on left side. Carotid arteriography revealed dilated
cavernous sinus and arterialization of superior ophthalmic vein with
retrograde flow. The contrast medium from internal carotid artery was
seen filling the cavernous sinus through fistula and then flowing
retrograde into the superior ophthalmic vein. Her CT Angiography also
revealed dilated superior ophthalmic vein and enlarged cavernous sinus
on left side (Figure-1 A-C).
       On the basis of history, clinical examination and investigations a
diagnosis of left traumatic direct high flow carotid cavernous fistula was
made. She was treated with intraocular pressure lowering eye drops
(Alphagan eye drops BD, Co-Dorzol eye drops BD), artificial tears,
painkillers and intravenous antibiotics (injection Augmentin I/V 1.2 gm
      A treatment plan of endovascular approach for embolization of
carotid cavernous fistula with balloon and coil was made. Due to financial
constrains the patient managed the amount needed for intervention in
about 06 weeks time. The condition of patient rapidly deteriorated before
surgical intervention was made and she started losing her vision in left
eye. Her proptosis had markedly increased. Her conjunctival congestion
worsened. Her fundus with merely dilated veins developed marked
hemorrhages and edema (Figure-2 A & C).
      A few days before surgery it was noted that her chemosis started
decreasing, proptosis reduced, intraocular pressure decreased, and
ocular bruit stopped; whooshing noises were no more audible (Figure-2
B). A repeat CT angiogram also confirmed spontaneous closure (Figure-1
D). Her ocular movements started to regain and it was concluded that the
fistula was closed spontaneously. But unfortunately she failed to regain
her vision in left eye due to optic atrophy and vascular occlusion (Figure-2
D). She was advised not to take aspirin, warfarin, heparin or any other
thrombolytic therapy lifelong by cardiologist.


      Carotid-cavernous fistula (CCF) is the most common arteriovenous
malformation affecting the orbit. [6,7] Barrow et al. classified CCF into
four arteriographic types with respect to communication between internal
and external carotid arteries and their meningial branches with cavernous
sinus and its tributaries. [8]
      The cavernous sinuses are paired structures that lie within the
sphenoid bone and communicate with each other via the circular sinus.
The cavernous sinus contains a venous plexus that is part of the dural
venous system, receiving blood from the sphenoparietal sinuses and the
superior and inferior ophthalmic veins. There are a number of structures
that pass through the sinus including the internal carotid artery and
oculomotor, trochlear, trigeminal (ophthalmic and maxillary divisions) and
abducens cranial nerves. [1]

        Carotid-cavernous fistulas are broadly divided into two categories,
direct and indirect. Head injury following road-traffic accidents, fights and
falls account for approximately 75% of CCF. [7] The injuries may be
penetrating or non-penetrating and may be associated with basal or facial
skull fracture. [8] Instances of direct CCF following surgical procedures
(such    as   endoscopic    nasal   surgery,   vascular,   neurosurgery)   or
spontaneously from aneurysm ruptures have also been reported. [9]

        Endovascular approaches have been tried to correct high-flow post-
traumatic CCF. [10-12] Spontaneous closure of CCFs by thrombosis of
the cavernous sinus is uncommon, especially in the direct traumatic high
flow type fistulas though it has been previously described. [4] As a
mechanism of spontaneous occlusion, it is suggested that carotid
angiography played an important role in most of these cases. They also
suggested that stasis of the blood flow during venography may have
caused the formation of a thrombosis inside of the cavernous sinus, which
induced closure of the fistula.

        In the case we have reported, the patient managed the expenses
after about 06 weeks of diagnosis. Till then she lost vision in her left eye
and her proptosis had markedly increased, but before surgery her fistula
had closed spontaneously without any intervention. This is a quiet rarely
reported event. The fistula was probably closed due to the formation of
thrombus in cavernous sinus as reported by Alkhani et al [4] and it might
be the result of carotid angiography performed 06 weeks earlier.

     In the end we have concluded that though direct CCF is a rare
complication of head injury, it must be kept in mind in all cases of post
traumatic proptosis. Early diagnosis and prompt intervention will not only
save the eye but also the life of patient which is always at risk due to
intracerebral bleed secondary to rupture of dilated cavernous sinus.
                              A                                  B

                              C                                  D

FIGURE 1 A. CT scan orbit showing dilated superior ophthalmic vein on
left side B. Carotid arteriography showing retrograde flow in superior
ophthalmic vein. C. CT angiograph showing prominent superior
ophthalmic vein on left side. D. Post resolution CT Angiograph
                                 A                                  B

                                 C                              D

FIGURE 2 A. Proptosis on presentation B. 02 months after spontaneous
closure proptosis resolved. C. Fundus on presentation. D. Fundus 02
months post closure of CC fistula.

1. Skipworth J, Beary K, Gibbsons C, Carotid–Cavernous Sinus        Fistula:

Delayed Diagnosis Following Road Traffic Accident, Ann R Coll Surg

Engl. 2007 November; 89 (8): 807-810.

2.Fabian, TS; Woody, JD; Ciraulo, DL; Lett, ED; Phlegar, RF; Barker, DE,

et al. Posttraumatic carotid cavernous fistula: frequency analysis of signs,

symptoms and disability outcomes after angiographic embolisation. J

Trauma. 1999; 47:275–8.

3.Fattahi, TT; Brandt, MT; Jenkins, WS; Steinberg, B. Traumatic carotid–

cavernous fistula: pathophysiology and treatment. J Craniofac Surg.


4. Alkhani A, Willinsky R, TerBrugge K. Spontaneous resolution of

bilateral traumatic carotid cavernous fistulas and development of trans-

sellar intercarotid vascular communication: case report. Surg Neurol


5. Luo CB, Teng MM, Chang FC, Chang CY. Spontaneous thrombosis
and complete disappearance of traumatic carotid-cavernous fistulas after

angiography. J Chin Med Assoc. 2005 Oct;68 (10):487-90.

6. Das JK, Medhi J, Bhattacharya P, Borah N, Bhattacharjee K, Kuri GC,

Deka H, Goswami BJ. Clinical spectrum of spontaneous carotid-

cavernous fistula. Indian J Ophthalmol 2007;55:310-2

7. Barrow DL, Spector RH, Braun IF, Landman JA, Tindal SC, Tindal GT.

Classification and treatment of spontaneous carotid-cavernous sinus

fistulas. J Neurosurg 1985;62:248-56

8. Grove AS Jr. The dural shunt syndrome. Pathophysiology and clinical

course. Ophthalmology 1984;9:31-4

9. Kupersmith MJ, Berenstein A, Choi IS, Warren F, Flamm E.

Management of nontraumatic vascular shunts involving the cavernous

sinus. Ophthalmology 1988;95:121-30.
10. Debrun GM, Vinuela F, Fox AJ, Davis KR, Ahn HS. Indications for

treatment   and    classification   of   132   carotid-cavernous    fistulas.

Neurosurgery 1988;22:285-289

11. Fabian TS, Woody JD, Ciraulo DL, Lett ED, Phlegar RF, Barker DE,

et al. Posttraumatic carotid cavernous fistula: frequency analysis of signs,

symptoms, and disability outcomes after angiographic embolization. J

Trauma 1999;47:275-281

12. Guglielmi G, Vinuela F, Duckwiler G, Dion J, Stocker A. High-flow,

small-hole arteriovenous fistulas: treatment with electro detachable coils.

Am J Neuroradiol 1995;16:325-328

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