Grand Rounds August 25, 2006

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Grand RoundsAugust 25, 2006

• Jeffrey D. Colburn, M.D., PGY-2
• Vanderbilt Eye Institute

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The Case

• CC Right eye proptosis, vision loss
• 81 y.o. Cauc male
• Presented at outside facility for HA and neck
  pain
• Noted double vision, whooshing in Right ear
• s/p Neurosurgical procedure
• Poor vision right eye, stable
• Past Ocular Hx Reading Glasses. No
  injuries/surgeries.

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History

• Past Med/Surg Hx
• Hypothyroidism
• Hip fx with repair 1999
• Dyslipidemia
• Recent repair R fem pseudoaneurism
• Meds
• Gemfibrozil
• Levothyroxine
• ASA
• Allergies NKDA

• Social Hx
• No tobacco/ETOH
• ROS
• Negative except as per HPI

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Exam

• General Awake alert, NAD, mood/affect
  appropriate
• VA (near, 4.00) 20/400 OD, 20/40 OS1
• IOP 22,27 OD, 17 OS
• CVF grossly full
• Pupils 4 ? 2.5 OU, RAPD OD
• Motility -2 in all gaze positions OD, Full OS
• Hertel 19 OD, 16 OS (104mm)

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Exam

• External Proptosis, chemosis, conj injection,
  resistance to retropulsion OD
• Bruit heard on auscultation right orbit
• PLE
• LLL Crusted lashes, lagophthalmos 4mm OD
• Conj 3 chemosis, 3 injection OD
• K Clear Quiet OU
• A/C Formed Quiet OU
• Iris Intact OU
• Lens NSC OU
• Ant Vit Quiet OU

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Exam

• DFE
• Disks sharp margins, no papilledema or pallor
  OU
• C/D 0.7 OD, 0.5 OS
• Macula flat OU
• Periphery flat OU
• Vitreous clear OU
• Vascular wnl OU

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Differential Diagnosis
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Differential Diagnosis

• Vascular
• Carotid-cavernous fistula
• Arteriovenous malformation
• Cavernous sinus thrombosis
• Neoplastic
• Cavernous sinus tumors
• Orbital Tumors
• Skull base tumors
• Mucocele
• Trauma
• Retrobulbar hemorrhage
• Intraorbital foreign body

• Infectious
• Orbital cellulitis
• Mucormycosis
• Tuberculosis
• Neurologic
• Cranial nerve palsy
• Inflammatory/Infiltrative
• Thyroid eye disease
• Orbital pseudotumor
• Orbital vasculitis
• Wegeners granulomatosis
• Polyarteritis nodosa
• Intracranial sarcoidosis
• Tolosa-Hunt syndrome

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Further History

• 1/06 -- Onset double vision, whooshing in right
  ear
• Diagnosed with CC-Fistula
• 6/06/06 PCI with stent
• Complicated by massive intracranial hemorrhage
• Required ventriculostomy
• Decreased VA OD noted after awakening in ICU
• Extended hospital course, then rehab
• 7/21/06 Presents to outside hospital
• new HA, neck pain
• CT shows enlarged superior orbital vein
  consistent with CCF

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DiagnosisRecurrent Carotid Cavernous Sinus
Fistula
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Our part in management

• Exposure
• Lacrilube
• Increased IOP
• 2 hrs after initial eval, IOP up to 30,32 OD
• Cosopt started
• 3 hours later, IOP down to 22 OD
• Cosopt cont BID OD, followed IOP

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Carotid Cavernous Sinus Fistula

• Symptoms

• Signs

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Carotid Cavernous Sinus Fistula

• Symptoms
• Double vision
• eyelid droop
• facial pain/numbness

• Signs
• Proptosis
• chemosis
• ? IOP
• ocular pulse pressure
• orbital/temple bruit
• ptosis
• miosis
• ophthalmoplegia (most commonly CN VI)
• facial hypoesthesia
• optic disk swelling
• retinal venous dilatation
• intraretinal hemorrhage

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• Classic Sign
• Limbal injection with arterialized conjunctival
  episcleral vessels

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Anatomy
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Diagnostic Studies

• Orbital color doppler U/S
• Reversed, arterialized flow in S. Ophthal v.
• CT/MRI
• Enlarged S. Ophthal v.
• Enlarged EOM
• Proptosis
• Cerebral Angiography
• Gold Standard diagnosis
• View ICA, ECA, vertebral circulations

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Pathophysiology

• Retrograde venous drainage into orbit
• Venous HTN
• Enlarged EOMs
• Restriction ? Diplopia
• Proptosis ? Exposure keratopathy
• Chemosis Injection ? Red Eye
• Increased episcleral vortex venous pressure
• ? Increased IOP ? Secondary Glaucoma
• Venous Arterial Stasis
• Decreased ocular/retinal perfusion
• Decreased visual acuity
• Anterior Segment Ischemia
• Decreased perfusion to intra CS cranial nerves
• Ophthalmoplegias ? Diplopia

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Pathophysiology

• Also remember venous HTN in other directions as
  well. Important Example
• Retrograde cortical venous drainage
• 10-55 of CCF cases
• ? Severe HA
• ? Contralateral neuro deficits
• ? 30-40 risk of intracerebral hemorrhage
• ? May be fatal

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Secondary Glaucoma

• Neovascular ant segment ischemia
• Angle Closure choroidal effusions
• May see blood in Schlemms canal
• Unilateral
• Tx Medical

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Classification

• Direct CCF (High-flow)
• Most common (70-90)
• 75 traumatic (assoc. basal skull fx)
• Defect in intra-cavernous ICA
• Spontaneous mid-age, HTN, post-menopausal
  female
• Indirect CCF (Low-flow)
• Dural Shunt Meningeal arteries (ICA, ECA or
  combo)
• Can be spontaneous or traumatic also
• More insidious onset of symptoms

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Barrows Classification (1985)

• Type A Direct between ICA and CS
• Type B Dural ICA branches to CS (uncommon)
• Type C Dural ECA branches to CS
• Type D Dural ICA ECA branches to CS

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Prognosis

• Direct CCF
• Poor visual prognosis, 90 with severe vision
  loss
• Ocular optic nerve damage, exposure
  keratopathy, sec. glaucoma, ant segment ischemia,
  CRVO, ischemic ON
• Concern for intra-cerebral hemorrhage
• Indirect CCF
• Prognosis less severe
• Also concern for intra-cerebral hemorrhage
• Exacerbation remission is the hallmark
• May close spontaneously (10-60)

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Management

• Indications for Tx
• Lack of spontaneous closure, risk to eye/vision,
  intolerable symptoms, high-risk for stroke,
  venous thrombosis, mental status changes
• Surgical closure
• Rare in last 30 years
• Can be salvage option
• Interventional Radiology (balloon
  occlusion/embolization)
• Trans-arterial route directly through tear or
  embolization of feeding vessels
• Trans-venous through S. Ophthal. v. or Inferior
  Petrosal sinus

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Endovascular Management

• Meyers, et al. Am J Ophthalmology, 2002
• Retrospective interventional case series
• 133/135 consecutive cases had tx
• 121 (90) patients were cured clinically (mean
  f/u 56 mos)
• 4 patients with moderate/severe disability
• 6 with symptomatic complications
• Cerebral infarction, Decreased VA (2), Diabetes
  Insipidus, orbital eccymosis, retroperitoneal
  hematoma, DVTs (2)
• No operative mortality
• Conclusions
• High success rate
• Low complication/morbidity rate
• Patients ocular symptoms may be transiently
  worsened post-procedure

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Superior Ophthalmic Vein Approach

• First proposed by Hanneken, et al. in 1989.
• Direct access to cavernous sinus
• Potential complications puncture of S. ophthal
  v., orbital hemorrhage, infection, trochlea or
  other structure damage
• Goldberg, et al. Arch Ophthalmol, 1996.
• Retrospective clinical series
• 10 consecutive cases with dilated s. ophthal v.
• Anterior orbitotomy with cannulation
• 100 with resolution of s/s, halt of visual loss
• 90 achieved premorbid BCVA
• No significant complications
• Conclusions
• Especially effective with significant ICA
  contribution to CCF
• technically straightforward, safe, and effective
  treatment

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• Leibovitch, et al. Ophthalmology, 2006.
• Present difficult cases out of 25 consecutive
• Unable to isolate or cannulate 6
• Complicating factors
• Small or fragile vein (3)
• Large posterior varix rupture bleed (1)
• Clotted anterior SOV (2)
• SOV located inferiorly (1)
• Conclusions
• SOV important useful route
• Fragile or clotted veins, deep orbital
  dissections should be avoided

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Back to our patient

• 7/24/06 Endovascular Intervention (trans-art.)
• Angiogram shows previously place stent in R ICA,
  but persistent feeders
• Embolization of feeders from R Middle Meningeal
  Artery
• Multiple feeders from Right Internal Maxillary
  Artery were not embolized

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Course

• Hospital stay complicated by MRSA PICC line
  infection and UTI.
• D/C home 8/2/06
• Will f/u with Ophthalmology 9/5/06
• Scheduled for repeat cerebral angiogram and
  embolization of AVM on 9/25/06

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References

• Kanski, JJ. Clinical Ophthalmology A systematic
  Approach. 5th ed. Butterworth Heinemann, New
  York, 2003. p243-244, 574-575.
• Kunimoto, DY, et al. The Wills Eye Manual
  Office and emergency room diagnosis and treatment
  of eye disease. 4th ed. Lippincott Williams
  Wilkins, Baltimore, 2004. p126-128, 213-215.
• Ringer AJ, Salud L, Tomsick TA. Carotid cavernous
  fistulas Anatomy, classification, and treatment.
  Neurosurg Clin N Am 200516279-295.
• Meyers PM, et al. Dural carotid cavernous
  fistula Definitive endovascular management and
  long-term follow-up. Am J Ophthalmol
  200213485-92.
• Goldberg RA, et al. Management of cavernous
  sinus-dural fistulas Indications and techniques
  for primary embolization via the superior
  ophthalmic vein. Arch Ophthalmol
  1996114707-714.
• Leibovitch, et al. Lessons learned from difficult
  or unsuccessful cannulations of the superior
  ophthalmic vein in the treatment of cavernous
  sinus dural fistulas. Ophthalmology
  20061131220-1226.