A Case of Painful Right Ophthalmoplegia Omar AlMasri, MS VI

1
A Case of Painful Right Ophthalmoplegia

• Omar AlMasri, MS VI
• VMS at the Department of Neurosurgery, BIDMC

2
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.

3
Presentation

• 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.

4
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
5
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

6
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

7
Workup (Labs) 6/7/09

• CBC NL
• Coagulation profile NL
• Blood chemistry/ KFT NL
• Glucose 119 to 156 (Consistently elevated)
• U/A NL
• CSF (LP)
• WBC 1/microL
• RBC 385/microL (tub 4)
• TotProt 64mg/dL
• Glucose 84mg/dL

8
Workup (Labs) 6/7/09

• HbSAg Negative
• HbSAb Borderline positive
• HbCAg Negative
• HCVAb Negative
• VDRL Negative
• TB-PCR Not detected
• Lyme Disease Ab Screen Negative

9
Workup (Labs) 6/7/09

• 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)

10
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.

11
MRI T1 Post CN
12
MRI T2 MRV
13
MRI FLAIR
14
Arterial phase
Right Common Carotid Left Common Carotid
15
Venous phase Road Map
16
Arterial phase
17
Arterial phase
18
Outcome

• IOP
• Visual acuity

19
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

20
Caroticocavernous Fistulas

• Two major types
• Direct high flow (A)
• Indirect (Dural) low flow (B, C, D)

• Etiologies of the direct type
• Acquired
• Trauma (most common)
• Rupture of an intracavernous ICA aneurysm
• Iatrogenic
• Fibromuscular dysplasia
• Collagen vascular diseases
• Spontaneous (25)

Etiologies of the dural type Spontaneuous Acquire
d Trauma Thrombophlebitis Iatrogenic Dural venous
thrombosis Possible hormonal association
21
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)

22
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

23
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

24
References

• Alessandra Biondi, Dan Milea, Christophe Cognard,
  Giuseppe K. Ricciardi, Fabrice Bonneville, Re?my
  van Effenterre Cavernous Sinus Dural Fistulae
  Treated by Transvenous Approach through the
  Facial Vein Report of Seven Cases and Review of
  the Literature. AJNR 2412401246, June/July 2003
• Galen F. H. Chun, Thomas A. Tomsick Transvenous
  Embolization of aDirect Carotid Cavernous Fistula
  through the Pterygoid Plexus. AJNR, 231156-1159,
  August 2002. Neil R. Miller, MDDiagnosis and
  management of dural carotidcavernous sinus
  fistulas. Neurosurg. Focus, 23(5)E13, 2007.
• Jaime Badilla, MD Charles Haw, MD, FRCSC Jack
  Rootman, MD, FRCSC Superior Ophthalmic Vein
  Cannulation through a Lateral Orbitotomy for
  Embolization of a Cavernous Dural Fistula. Arch
  Ophthalmol.,125(12)1700-1702, 2007.
• M. S. Greenberg. Carotid-cavernous fistula.
  Handbook of Neurosusrgery 6th Ed., 28.6845-846,
  2006.
• Perry P. Ng, M.D., Randall T. Higashida, M.D.,
  Sean Cullen, M.D., Reza Malek, M.D., Van V.
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  Endovascular strategies for carotid cavernous and
  intracerebral dural arteriovenous fistulas.
  Neurosurg Focus 15 (4)Clinical Pearl 1, 2003.
• T. J. K. Leonard, I. F. Moseley, M. D. Sanders
  Ophthalmoplegia in carotid cavernous sinus
  fistula. British Journal of Ophthalmology,
  68128-134, 1984.
• YU Jia-sheng, LEI Ting, CHEN Jin-cao, HE Yue,
  CHEN Jian and LI Ling Diagnosis and endovascular
  treatment of spontaneous direct carotid-cavernous
  fistula. Chin Med J, 121(16)1558-1562, 2008
• Luca Remonda, Susanne Beatrice Frigerio, Robert
  Bu?hler, and Gerhard Schroth Transvenous Coil
  Treatment of a Type A Carotid Cavernous Fistula
  in Association with Transarterial Trispan Coil
  Protection. AJNR 25611613, April 2004