This slide deck uses a case-based format to explain the presentation, diagnosis, and treatment of dural cavernous fistulae as well as the management of spontaneous choroidal detachments.

1. Dural Cavernous Fistula with
Spontaneous Choroidal Detachments
Prepared and presented by
Steve Christiansen, MD
August 5, 2014

2.  80+ year old female referred for 4 month history of red,
irritated eyes and headaches
 Headaches bi-frontal, not relieved with hydrocodone or fentanyl
 Eyes chronically red, not improved with artificial tears
 “When doing puzzles things jump around” and “feels like
eyeballs are bulging”
 Denies diplopia, pulsatile tinnitus, vision loss

3.  Prior ocular history
 Dry AMD OU
 Cataract surgery OU 5 mo prior – vision not improved since surgery
 PMH
 CVA x 2
 A-fib – warfarin
 Hypothyroid – levothyroxine
 Hypertension –metoprolol, sotalol
 CHF – furosemide
 COPD – inhaled meds
 PSH
 60 pack year smoking history
 Lives in long-term care facility

4. Note the “corkscrew-like” appearance to the episcleral vessels

5.  VA 20/60 OD and 20/50 OS sc, PH 20/40 OU
 Tonometry 12 OD, 14 OS by applanation
 Pupils equal, reactive, no RAPD
 Full motility and confrontation VF
 Hertel’s base 101, 22 mm OU
 Critical flicker fusion 15.6/15.8 Hz (lower limit normal is 27 Hz)

6.  Vascular
▪ Carotid-cavernous fistula
▪ Arterio-venous malformation (AVM)
▪ Cavernous sinus thrombosis
 Autoimmune
▪ Thyroid eye disease
 Inflammatory
▪ Scleritis
▪ Episcleritis
 Other
▪ Sturge-Weber Syndrome
▪ Chronic conjunctivitis

7. Vascular Diagnostic
Studies
A.
B.
C.
D.
Disease-
Characterizing
Studies
A.
B.
C.
D.
Additional Relevant
Exam Techniques
A.
B.
C.
D.

8. Additional Relevant Exam Techniques
A.
B.
C.
D.
Pulse-synchronous orbital pulsation
Orbital auscultation
Goldmann Tonometry
Gonioscopy

9. Note the presence of pulsatile proptosis, which may occur in NF-1 due to absence of
the sphenoid wing as well as in carotid-cavernous fistulae, orbital roof fractures, and
arteriovenous malformations.
Neurology.org

10. • Bruit, from the french word for noise, can be subjective or objective
• May appear as a buzzing, swishing, or roaring and represents a classical
symptom of high-flow CCFs
• Found in 25% of patients with DCSFs. Bruit develops due to arteriovenous
turbulences within the CS, which may reach inner ear organ via sound
transmission through the skull
• May decrease or disappear completely following thrombosis, spontaneous
occlusion of fistula feeder, or during manual compression of carotid artery in
the neck
• Also found in arteriosclerotic stenosis of carotid artery, subclavian artery, or
vascular tumors, as well as due to a hypoplasia/aplasia of sphenoid bone via
transmissions of brain pulsations to the orbit

11. Look for wide to-and-fro fluctuations of the mires on Goldmann tonometry and
measure the magnitude of difference in to-and-fro motion of the mires.
YouTube.com– M D Singh

12. Note the blood in Schlemm’s Canal due to raised episcleral venous
pressure, which can also appear in Sturge-Weber Syndrome, Graves
Disease, and Superior Vena Cava Syndrome
Gonioscopy.org

13. Vascular Diagnostic Studies
A.
B.
C.
D.
MRA
CTA
Orbital ultrasound with color doppler
Duplex carotid ultrasound with color doppler

24. “Abnormal flow within the left cavernous sinus and
within the vessel seen posterior to the dorsum sella.”

31. “There is slightly more conspicuous flow seen in the left
ophthalmic vein.”

32. Impression
1. Chronic small vessel ischemic disease
2. Early venous draining and arterialized flow into a
retroclival vessel and the left cavernous sinus
without significant superior ophthalmic vein dilation
compatible with indirect cavernous carotid fistula

33. Disease-characterizing studies
A.
B.
C.
D.
B-scan ultrasound
Perimetry
OCT
Fundus photography

34. “Very enlarged superior
ophthalmic veins with very high
flow (right greater than left) as
well as fluid under peripheral
retina in right eye.”

35. OD: small island of I2e isopter
centrally with otherwise normal
field
OS: loss of the I2e isopter, with
mild superior constriction

36. “Normal macula, cup to disc of 0.6 OU, no evidence of fluid on exam or
by OCT of macula and nerve head”

37.  80+ year old female, hx of smoking, HTN, HLD
 4 mo headaches, “bouncing images”, dilated, tortuous episcleral
vessels and poor vision s/p CE and PCIOL
 Blood in Schlemm’s canal on gonioscopy
 B-scan with enlarged, high-flow superior ophthalmic vein
 MRA with arterialized flow into cavernous sinus
 Diagnosis? Indirect (dural) carotid cavernous sinus fistula

40. C-C fistulas
Direct
“Carotid-Cavernous
fistula”
A
Indirect
“Dural-cavernous
fistula”
B C D

41. • Single tear in wall of cavernous ICA, producing direct (fistula) between ICA and
veins that drain into cavernous sinus (e.g. ophthalmic vein)  arterialization
• High flow
• Etiology
• Head Trauma (MVA, fights, falls) often due to rupture of pre-existing cavernous ICA aneurysm
• May become symptomatic directly after injury, or not until days to weeks later

42. Dural ICA branches
and
cavernous sinus
Extradural ECA branches
and
cavernous sinus
Extradural ECA branches,
dural ICA branches,
and cavernous sinus
• Most often low-flow
• Most commonly in middle-aged or elderly women
• Increased risk with systemic hypertension, atherosclerosis, vascular disease
• Typically occur spontaneously

44. Pulse-synchronous
orbital pulsation
Elevated IOP and
pulsating mires on
applanation tonometry
Blood visible in
Schlemm’s Canal

45. Ptosis OS, proptosisOD,
exposure keratopathyOD
Chemosis, motility
restriction due to venous
stasis, EOM edema
Cranial nerve palsy
(most often CN VI)

46. Unilateral dilation of retinal veins on the side of a direct carotid-cavernous sinus fistula. The
patient was a 25-year-old woman with a traumatic, left carotid-cavernous sinus fistula. A,
The ophthalmoscopic appearance of the right posterior pole is normal. The retinal vessels
are of normal caliber. B, Ophthalmoscopic view of the left posterior pole shows moderate
dilation of retinal veins unassociated with retinal hemorrhage.

47. Patient A Patient B
Numerous flame-shaped
(superficial) and punctate (deep)
intraretinal hemorrhages
Numerous punctate (deep)
intraretinal hemorrhages

48. Optic disc swelling, retinal
hemorrhages, dilated retinal
veins due to CRVO
Conjunctival chemosis

49. Additional mystery
manifestation…
stay tuned

50.  Direct CC fistula
• Fistula-closing procedures
• Ligation, non-detachable balloon tip of Fogarty Catheter
• Occlusion of ICA may lead to neurologic ischemic damage in
addition to worsening of ocular disease
• Surgical techniques
• Endovascular coils or detachable balloons
• Direct surgical repair of fistula

51. • Indirect Dural Cavernous Fistula
• No difference in mortality compared to normal population
• Decision to treat based on severity of associated ocular disease
• 20-50% close spontaneously
• Recommendation is to follow patients with mild ocular manifestations to
see if fistula will close spontaneously
• Treat associated ocular disease
• Surgical treatment options include endovascular coils, balloon occlusion

52. • Patient’s nurse reports
• Periorbital edema mildly worse, eyes still red, headaches
• Now with FBS, dryness OU
• Our recommendations
• Artificial tears
• Check IOP locally
• If OK, return for scheduled follow-up in 3 weeks

53. • “Gritty, sawdust-like feeling in eyes,” and “eyelid swelling”
• VA unchanged
• Tonometry 12 OD, 12 OS by applanation, no pulsating mires
• Confrontational VF somewhat constricted
• 3+ PEE OU, 360 degrees of pannusOS, ectropionOD > OS

54. “360 degree choroidal effusions OD”

55. “360 degree choroidal effusions with punctate intraretinal hemorrhages OS”

56. Bilateral choroidal detachments

58. Choroidal detachment/effusion
• Fluid collection between choroid and sclera
• Causes include
• hypotony following glaucoma surgery, inflammation, neoplasm, trauma,
venous congestion
• Hemorrhagic - blood accumulation due to rupture of choroidal vessels
• Serous – Increased serous transudation through choroid capillary walls or
from drop in IOP caused by increased uveoscleral outflow of aqueous or
elevated hydrostatic venous pressure
• Treatment options include management of IOP, surgical drainage of effusion,
or watchful waiting

59. Choroidal detachment/effusion and dural cavernous fistula
• 250 cases of carotid or dural cavernous fistula, 10 patients (2.5%) with
vision loss secondary to choroidal effusion or retinopathy.
• 9 patients with severe thrombosis of ophthalmic vein, 7 with
thrombosis of cavernous sinus
• Syndrome of Paradoxical Worsening
• All studies opted for conservative management with spontaneous
resolution in majority of cases
• Consulted neurosurgery and scheduled appt for 7/15/14 in case of no
improvement/worsening

60.  External exam largely unchanged
 Dilated fundus exam revealed…

61. Complete resolution of choroidal detachments

62. Steve Christiansen, MD
August 12, 2014

63. References
1. Miller, NR. Carotid Cavernous Sinus Fistulas. Chapter 42.
2. TateshimaS, Akiyama M, Hasegawa Y, Abe T. [Paradoxical worsening of cavernous sinus dural
arteriovenous fistula: case report with serial angiograms]. No Shinkei Geka. 2005 Sep;33(9):911-7.
Japanese. PubMed PMID: 16164188.
3. Neurology.org. Resident videos. Pulsatile proptosis.
4. YouTube.com. Singh MD. Applanation.
5. Gonioscopy.org. Elevated EpiscleralVenous Pressure.
6. Yamada M, Ikeda N, Mimura O, Maeda Y. [Choroidal detachment associated with spontaneous carotid
cavernous fistula]. Nihon GankaGakkai Zasshi. 1991 Jul;95(7):704-9. Japanese. PubMed PMID: 1927752.
7. MazzeoV, Galli G, Signori D, Perri P. Spontaneous choroidal detachment and 'red-eyed shunt syndrome':
two clinical entities with the same cause? Int Ophthalmol. 1985 Sep;8(3):129-38. PubMed PMID: 3905659.
8. 8. Klein R, Meyers SM, Smith JL, Myers FL, Roth H, Becker B. Abnormal chloroidal circulation: association
with arteriovenous fistula in the cavernous sinus area. Arch Ophthalmol. 1978 Aug;96(8):1370-3. PubMed
PMID: 678174.
9. Wang ML, Seiff SR, HalbachVV, Christenbury JD, Jaben SL. Total choroidal detachment complicating dural
arteriovenous sinus fistula. Ophthalmic Surg. 1993 Dec;24(12):856-7. PubMed PMID: 8115106.
10. Preechawat P, Narmkerd P, Jiarakongmun P, PoonyathalangA, PongpechSM. Dural carotid cavernous
sinus fistula: ocular characteristics, endovascular management and clinical outcome. J Med Assoc Thai.
2008 Jun;91(6):852-8. PubMed PMID: 18697384.
Steve Christiansen, MD
August 12, 2014