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A Case of Painful Right
Ophthalmoplegia
Omar AlMasri, MS VI
VMS at the Department of Neurosurgery,
BIDMC
Patient Profile
• LV is a 66 year-old RH lady works in the
dining hall at a local school
• Previous history of hypertension, migraine
and dyslipidemia
• Transferred from Mount Auburn Hospital after
having a CT showing a possible CC fistula.
Presentation
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The patient was transferred to BIDMC with painful right eye
ophthalmoplegia.
She was doing well until 11 days PTA, when she developed redness of
her eye after an episode of diplopia and blurred vision, and was
diagnosed with conjunctivitis by her PCP and given antibiotics.
Her condition remained stable with symptoms improving until 6/6 when
she developed dull pain with swelling around her right eye and
forehead. This pain was constant with associated nausea, vomiting and
photophobia.
When walking she felt off balance. She attributed this to double vision
when she looks down.
No fever, chills, rash, or stiff neck
No documented history of head trauma
Seen at Mount Auburn Hospital on 6/6 and was noted to have right
periorbital edema, chemosis, and painful ophthalmoplegia.
CT at Mount Auburn suggested a carotid-cavernous fistula.
History
Meds:
Lisinopril 10mg
Fiorcet (acetaminophen, butalbital,
caffeine) PRN for headache (took 2 to 3
only)
Allergies:
Atorvastatin/ other unknown lipid lowering
agents
Influenza virus vaccine
Past medical and surgical history:
Fallopian tube ligation 33 years PTA
Thyroidectomy 20 years PTA
Left breast multiple cystectomies 4 years
PTA
Hypertension diagnosed 1 year PTA
Hypercholesterolemia
Family history:
Negative for recent infections or a
similar condition, non-contributory
otherwise
Social history:
Shares apartment with, daughter lives
in apartment above
No history of recent travel
Non-smoker, occasional drinker (very
rarely)
Owns a dog
Physical exam
• Vital signs were stable, and the patient was
afebrile
• Right periorbital edema
• Right eye ptosis / no bruit but continuous hum
• Diplopia
• Proptosis of right eye
• Chemosis of right eye
• Full visual fields to confrontation
• Mildly decreased visual acuity compared to
the left
Physical exam
• Larger pupil on the right (5mm) compared to
the left (3mm) and both are briskly reactive
• Limited ROM of right eye in all directions
(esp. laterally)
• End-gaze nystagmus with increased effort
• IOP: Right eye (45mmHg), Left eye
(18mmHg)
• Limited abduction bilaterally
• Neurological examination including CN V and
cerebellar exam is non-localizing
• The rest of the examination is unremarkable
Workup (Labs) 6/7/09
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CBC NL
Coagulation profile NL
Blood chemistry/ KFT NL
Glucose 119 to 156 (Consistently elevated)
U/A NL
CSF (LP)
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WBC 1/microL
RBC 385/microL (tub #4)
TotProt 64mg/dL
Glucose 84mg/dL
Workup (Labs) 6/7/09
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HbSAg Negative
HbSAb Borderline positive
HbCAg Negative
HCVAb Negative
VDRL Negative
TB-PCR Not detected
Lyme Disease Ab Screen Negative
Workup (Labs) 6/7/09
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ESR 8mm/hr (0-20)
Anticardiolipin Antibody IgG 2.7 GPL 0 - 15
Anticardiolipin Antibody IgM 24.1 MPL 0 - 12.5
Lupus anticoagulant Negative
ANA Negative
ANCA Negative
Protein electrophoresis NAD
Rheumatoid factor 4IU/mL (0 - 14)
CRP 4.7 mg/dL (0 - 5.0)
C3 111mg/dL (90 - 180) C4 33mg/dL (10 - 40)
Workup (Imaging)
6/7 Underwent an MRI/ MRV study which showed
6/8 Underwent a contrast angiography study with an
attempt to embolize the fistula
6/9 She underwent another angiographic study, with a
facial cut-down to cannulate the right facial vein.
MRI T1
Post CN
MRI T2
MRV
MRI FLAIR
Arterial phase
Right Common Carotid
Left Common Carotid
Venous phase
Road Map
Arterial phase
Arterial phase
Outcome
• IOP
• Visual acuity
Date
Right eye
(mmHg)
Left eye
(mmHg)
6/7
45
18
6/8
32
6/9
36
25
6/10 (postop)
6
9
Date
Right eye
Left eye
6/7
20/200
20/100
6/9
20/70
20/60
6/10
20/40
20/30
Outcome (6/10) Follow up
• Improving right eye edema, chemosis,
proptosis, blurred vision and double vision,
but states that she sees better with one eye
closed.
• Improving left eye chemosis
• Left eye esotropia
• Pupils equal at 3mm and reactive bilaterally
• No eye pain
• Persistent bilateral Abducent nerve palsy
Caroticocavernous Fistulas
• Two major types:
– Direct; high flow (A)
– Indirect (Dural); low flow (B, C, D)
• Etiologies of the direct
Etiologies of the dural
type:
type:
Spontaneuous
Acquired
Trauma
Thrombophlebitis
Iatrogenic
Dural venous thrombosis
Possible hormonal
association
– Acquired
• Trauma (most common)
• Rupture of an
intracavernous ICA
aneurysm
• Iatrogenic
• Fibromuscular dysplasia
• Collagen vascular diseases
– Spontaneous (25%)
Clinical presentation and
the mechanisms behind it
• Irritation/ taruma of traversing nerves as a result of trauma.
– CN III, IV, V, VI palsies (Diplopia, ophthalmoplegia)
• Retrograde flow of arterialized blood through the superior and
inferior ophthalmic veins into the orbit.
– Proptosis (pulsating), chemosis, pain, and reduced visual
acuity, ocular/ cranial bruit.
– Retinal perfusion pressure compromise leading to
permanent blindness
• “Steal” phenomenon
– Hemispherical hypoperfusion if the circle of Willis collateral
structures are inadequate
• High-flow fistula, damage to venous wall.
– SAH (Rarely)
Treatment
• Mandatory in cases of involvement of the visual functions, and
in the presence of a cortical venous drainage seen in 26-31% of
cases (High risk for hemorrhage)
• Allowing time for vein to arterialize
• Advocated Acetazolamide therapy to decrease IOP
• Ipsilateral/ contralateral carotid compression
• Arterial approach
– Balloon embolization
– Stenting
• Venous approaches
– Coiling
• Surgical resection
QuickTime™ and a
decompressor
are needed to see this picture.
Complications
• Acute thrombosis
• Occlusion of the vein w/o occluding the fistula
• In cases of SOV exposure include difficulty in
identifying the vein, and injury to the supraorbital
nerve and levator muscle, + others
• Damage or perforation of vein esp. the SOV near
the trochlea
• Infection
• Dislodgement
• ICA sacrificing and retrograde flow
References
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