Clinical Presentations, Venous Drainage Patterns, and Treatment Outcomes in

Carotid Cavernous Fistula

Yamin Shwe, MD, David Altschul, MD

Santiago Ortega-Gutierrez, MD, MSc

Johanna T. Fifi, MD

Background • Carotid cavernous fistulas (CCFs) are abnormal communication between

the carotid arteries and cavernous sinus

• Clinical symptoms can be mild to severe based on size, flow rate and shunt location

• Some are reversible with early endovascular treatment

• Indication for treatment – cortical reflux, vision loss, hemorrhage

• Types of CCFs (Barrow’s classification) – type A, B, C, D

Figure - Ellis et al 2012

Hypotheses

• Venous drainage pattern correlates with clinical symptoms

• Endovascular treatment improves outcome

Method

• Retrospective review of 46 adults and one infant (5mo-82yrs)

• Clinical presentations - ocular and neurological symptoms from Jan 2004-June 2014

• Complete ophthalmological, neurological exam and DSA prior to treatment and at follow up

• Clinical symptoms and venous drainages were recorded

Method

• Clinical symptoms – Orbital

• Chemosis, proptosis

– Cavernous • Ptosis, diplopia, ophthalmoplegia, cranial nerves palsies

– Ocular • Increased intraocular pressure, decreased vision, eye

pain

– Cortical • Headache, tinnitus, ataxia

Method

• Venous drainage – Anterior

• Superior ophthalmic, inferior ophthalmic veins

– Posterior • Superior petrosal, inferior petrosal, sphenoparietal

sinuses

– Inferior • Pterygoid plexus

– Superior • Superficial middle cerebral vein

Method

• Primary outcome - association between venous drainage pattern and clinical symptoms

• Secondary outcome - clinical symptom improvement at follow up

• Variables were analyzed using χ2 and Fisher exact test

Signs and symptoms No. of cases

Symptoms

Proptosis 31

Decreased vision 13

Diplopia 12

Headache 12

Tinnitus 10

Eye pain 5

Face pain 3

Ataxia 3

Signs

Chemosis 27

6th nerve palsy 18

3rd nerve palsy 6

Increased ocular pressure 4

Vision loss 4

Results

Clinical Symptoms vs. Venous Drainage

Anterior Posterior Inferior Superior

Orbital (n=36) (%) (%) (%) (%)

Yes 30 (83) 6 (16) 12 (33) 5 (13)

No 6 (16) 30 (83) 24 (66) 31 (86)

p value 1.00 0.508 0.018* 0.097

Cavernous (n=26)

Yes 21 (80) 8 (30) 14 (53) 8 (30)

No 5 (19) 18 (69) 12 (46) 18 (69)

p value 0.986 0.274 0.501 0.274

Ocular (n=16)

Yes 13 (81) 2 (12) 4 (25) 3 (18)

No 3 (18) 14 (87) 12 (75) 13 (81)

p value 0.999 0.747 0.190 0.997

Cortical (n=16)

Yes 14 (87) 3 (18) 10 (62) 3 (18)

No 2 (12) 13 (81) 6 (37) 13 (81)

p value 0.960 0.997 0.276 0.997

Venous Drainage vs. Barrow Classification

A B C D P

Superior ophthalmic vein 8 6 10 12 1.000

Inferior ophthalmic vein 1 2 0 0 0.693

Superior petrosal sinus 1 0 0 6 0.264

Inferior petrosal sinus 8 1 2 6 0.037*

Sphenoparietal sinus 0 0 0 2 0.986

Pterygoid plexus 5 0 1 0 0.007*

Superficial middle cerebral vein 3 2 1 3 1.000

Factors Related to Symptom Recovery at Follow Up

Total (n=47)

Status at follow up P value

Improved No change Worsened

Age (yrs) 57±20 54±20 64±27 81 0.344

Sex Male 14 11 3 0 0.53

Female 33 20 12 1

Venous Drainage Anterior 39 26 12 1 1.000

Posterior 10 9 1 0 0.657

Superior 10 8 1 1 0.226

Inferior 21 15 6 0 0.986

Symptoms Orbital 36 24 11 1 0.999

Cavernous 26 19 6 1 0.703

Ocular 16 9 7 0 0.854

Cortical 16 10 6 0 0.988

Barrow Type A 9 7 2 0 0.682

B 9 7 5 0

C 12 7 5 0

D 17 12 5 0

Management Embolization 40 30 9 1 0.006*

Conservative 7 1 6 0

0

5

10

15

20

25

30

35

Improved Same Worsened

Embolization

Conservative

No

. p

ati

en

ts

Symptoms at follow up

*

Improved Same Worse

Discussion

• Our study showed that majority had anterior venous drainage and orbital symptoms but they did not reach statistical significance.

• Venous drainage patterns have been previously described to correlate with clinical symptoms. However, in our small study group, we did not find a consistent pattern of association between the two.

• Nevertheless, patients undergoing endovascular treatment were much more likely to experience symptom improvement at follow up.

Conclusion

• Clinical symptoms did not reliably predict venous drainage pattern among patients with CCFs in our small study group.

• DSA remains the gold standard when evaluating patients with suspected CCFs.

• Future studies with larger sample sizes are needed to better correlate clinical and angiographic findings and to quantify the effect of treatment based on the time to intervention.

Acknowledgement

Mentors

David Altschul, MD

Santiago Ortega-Gutierrez, MD, MSc

Johanna T. Fifi, MD

Others

Srinivasan Paramasivam, MD

Alejandro Berenstein, MD

References

• Ellis, J.A., et al., Carotid-cavernous fistulas. Neurosurg Focus, 2012. 32(5): p. E9

• Larsen D, et al., Treatment of carotid-cavernous sinus fistulae. Interv Neurorad: Strategies and Practical Techniques. Philadelphia: WB Saunders; 1999:215–26

• Barrow, D.L., et al., Classification and treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg, 1985. 62(2): p. 248-56.

• Neil, M.R., Carotid-cavernous Fistulas. Walsh and Hoyt’s Clinical Neuro Ophthalmology. 6th ed. Chapter 42. Lippincott Williams&Wilkins. (2005).

• Jung, K.H., et al., Clinical and angiographic factors related to the prognosis of cavernous sinus dural arteriovenous fistula. Neuroradiology, 2011. 53(12): p. 983-92.