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.
