Delayed Traumatic Caroticocavernous fistulaCase Report

Dr.A.Kowsalya, Dr.S.Mahesh Kumar, Dr. Tushar Grover, Aravind Eye Hospital, Madurai

Case ReortCaroticocavernous fistula is an abnormal communication between the Cavernous Sinus and the Carotid Arterial system. CCF can be classified based on 1.Etiology (Traumatic/ Spontaneous), 2. Velocity of blood flow (High or low) and 3.Anatomy (Direct vs. Dural, Internal Carotid vs. External Carotid vs. both).1 Direct CCFs are caused by a single traumatic tear in the arterial wall following Head Trauma, Penetrating Orbitofacial injuries, causing direct connection between the Cavernous segment of Internal Carotid Artery and Cavernous Sinus. These fistulas are of high flow type. Here we describe a young patient who presented as a case of Traumatic third nervepalsy, developed Direct Carotico cavernous Fistula a month later, well managed and recovered. Although TCCFs usually manifest symptoms early after trauma, in this case, the patient presented clinical signs 6 weeks post-injury, which was also the longest time reported in previous literature.2

A 32 yrs old male presented to us in August 2012, with complaints of Double vision for 15 days which was binocular on looking towards the right side. He gave history of head injury in a Road Traffic accident 1month back with loss of consciousness for 30 minutes and nasal bleed. Diplopia was more on looking towards right side and it was binocular. He had pain on left gaze.

On examination, his general condition was good. His best corrected visual acuity in both eyes was 6/6. Left eye was exotropic and hypertropic with mild ptosis and mid dilated non-reacting pupil. There was restriction of elevation, depression, and adduction in the left eye. Fundus of right eye was normal and left eye showed resolved Berlin’s edema. Colour vision

and Central fields were normal in both eyes. Hess, Diplopia charting showed incomplete third nerve palsy in the left eye. His higher functions, sensory and motor system were normal. All other Cranial nerves were normal. There were no cerebellar signs or gait disturbance. CT Brain showed fracture of left frontal bone extending to frontal sinus and orbit with subarachnoid hemorrhage. With the above findings a diagnosis of Traumatic Incomplete, pupil involving Third nerve palsy (Left eye) was made and given assurance and advised review after a month. 15 days later, he presented with history of sudden protrusion of eyeball, redness, blurred vision in the left eye for 3 days with no diplopia. He also complained of an abnormal blowing sound (pulsatile tinnitus) in the left ear for 3 days. On examination, left eye showed proptosis, severe circumcorneal congestion, corkscrew shaped vessels, chemosed forniceal conjunctival vessels inferiorly (Fig 1,

2). Anterior chamber showed exfoliative material and the pupil was 6mm dilated and fixed and adduction, abduction, depression were completely restricted with minimal restriction of elevation. (Fig 3). Intraocular pressure was 30 mmHg in the left eye and the visual acuity had dropped to 6/60. A clinical diagnosis of Posttraumatic Carotico Cavernous fistula (left side) was made.

Fig 1 : Carotico cavernous fistula – cork screw vessels

Fig 2 : Carotico cavernous fistula Proptosis, conjunctival chemosis

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MRI/MRA Brain was done, which showed Dilated Superior Ophthalmic vein and Cavernous sinus on left side suggestive of CCF (Fig 4, 5). The patient was referred to a higher institute of Neurosciences.

Internal Carotid Artery (Fig 6). Hence DSA Intervention with Balloon embolisation and complete obliteration of fistula was done (Fig

Digital Subtraction Angiography (DSA) revealed a rent measuring 3.5mm in the posteroinferior segment of the horizontal segment of Cavernous

7, 8). Patient came for review with us 9 days after the procedure with significant symptomatic improvement (Fig 9). One month later his best-corrected visual acuity was 6/6 in the left eye and had regained all movements except abduction and 6 months later he had recovered completely (Fig 10).

Fig 3 : Total External Ophthalmoplegia

Fig 4 : MRA Brain - Carotico cavernous fistula

Fig 5 : MRI/MRA Brain – Dilated Superior ophthalmic vein with Carotico cavernous fistula

Fig 6 : DSA – Rent in the horizontal segment of Cavernous Internal Carotid Artery

Fig 7 : DSA – Post balloon embolization

Fig 8 : Post procedure - Complete obliteration of the fistula

34 AECS Illumination

Discussion: Barrow3 classifies CCF as Types A, B, C, D. Type A or Direct- Direct connection between the cavernous segment of the Internal Carotid artery and the cavernous sinus. Type B - Abnormal communication between the cavernous sinus and one or more meningeal branches of the Internal Carotid artery. Type C – Abnormal communication between the cavernous sinus and the meningeal branches of the external carotid artery. Type D - Abnormal communication between the cavernous sinus and meningeal branches of Internal carotid artery and External carotid artery. Classic symptoms of Carotico Cavernous Fistula include exophthalmos, orbital or cephalic bruit, ocular hypertension, severe orbital pain, headache, conjunctival and episcleral injection, chemosis, extraocular muscle palsies and visual deterioration. The signs and symptoms vary according to the size and location of the lesion as well as the predominant venous drainage.4 In high flow Direct CCF, there is usually rapid onset of redness, proptosis, and chemosis of one or both eyes often associated with ophthalmoplegia and bruit. Venous stasis retinopathy or ischemic central retinal vein occlusion may develop in few patients.5 Episceral and conjunctival vessels become dilated with arterial blood (Arterialisation) and have a corkscrew configuration.5

Orbital Ultrasound has been used in the diagnosis of fistulas.4,6 The key findings are Dilated Superior ophthalmic vein and congestion of the

orbital issue including mild thickening of all the extra ocular movements. The next method of assessing CCFs is Neuro Imaging- CT Doppler, MRI/MRA. Findings include dilated superior ophthalmic vein, asymmetric enlargement of the extraocular muscles, proptosis, pseudoaneurysm with bulging of cavernous sinus.4 Intravenous Digital subtraction Angiography has been used in evaluating CCF.6

Between 10 and 60% of spontaneous CCFs will close spontaneously.7 The closure is secondary to partial or complete thrombosis of the cavernous sinus or its tributaries.The goal of therapy is to cure cranial nerve palsies and provide symptomatic relief for the patients. The most important thing in considering treatment is to identify a center with a very low morbidity and mortality rate that has considerable experience with modern embolization techniques. Recently balloon/coil embolisation has been advocated primarily for treating traumatic fistulas. Balloon/coil positioning is done by an arterial or venous endovascular approach through the superior ophthalmic vein. Vinuela et al series showed a significant clinical improvement in most of the embolised dural fistulas.

CCF is a rarely life threatening problem. Loss of vision is the main risk of not treating. On the other hand the procedures used to treat the condition carry a small but definite mortality and a rather significant morbidity.

Fig 9 : 1 month post procedure - Significant recovery of proptosis and extraocular movements

Fig 10 : 6 moths review – complete recovery of proptosis and all extraocular movements

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References

1. Walsh and Hoyt's clinical neurophthalmology. (5th ed) vol 1:1220.

2. Nguyen T, Cho YH, Jang YJ, Park MC, Shin SJ.Long delayed traumatic carotid-cavernous sinus fistula. J Craniofac Surg. 2013 May;24(3):e237-9

3. Barrow DL, Spector RH, Braun IF, Landman JA, Tindall SC, Tindall GT. Classification and treatment of spontaneous carotid cavernous fistulas. J Neurosurg.1985;62:248–56

4. Vinuela F, Fox AJ, Debrun GM, et al. Spontaneous carotid – cavernous fistulas: clinical, radiological, and therapeutic considerations. J Neurosurg 1984; 60: 976-84

5. Yanoff M, Duker JS. Ophthalmology. 2nd ed. Spain: Mosby; 2004. pp. 1403–4.

6. Santhosh J, Sanjay S. Neurophthalmology. 3rd ed. Aravind Eye Hospital; 1995. Radiology in neurophthalmolgy: Endovascular interventions in ophthalmology; p. 275.

7. Debrun GM, Vinuela F, Fox AJ, et al. Indications for treatment and classification of 132 carotid – cavernous fistulas. Neurosurgery 1988 ; 22: 285-9

8. Usha Kim, Mahesh Kumar, et al. Atlas of imaging in Neuro ophthalmology and Orbit, 1st edition;2009IMAGES: