A Case of Painful Right Ophthalmoplegia

Omar AlMasri, MS VIVMS 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• 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.

HistoryMeds:

Lisinopril 10mgFiorcet (acetaminophen, butalbital, caffeine) PRN for headache (took 2 to 3 only)

Allergies:Atorvastatin/ other unknown lipid lowering agentsInfluenza virus vaccine

Past medical and surgical history:Fallopian tube ligation 33 years PTAThyroidectomy 20 years PTALeft breast multiple cystectomies 4 years PTAHypertension diagnosed 1 year PTAHypercholesterolemia

Family history:Negative for recent infections or a similar condition, non-contributory otherwise

Social history:Shares apartment with, daughter lives in apartment aboveNo history of recent travelNon-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• 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

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

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)

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 (post-op)

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 type:– Acquired

• Trauma (most common)• Rupture of an intracavernous ICA aneurysm

• Iatrogenic• Fibromuscular dysplasia• Collagen vascular diseases

– Spontaneous (25%)

Etiologies of the dural type:

SpontaneuousAcquired

TraumaThrombophlebitisIatrogenicDural venous thrombosis

Possible hormonal association

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

References1. Alessandra Biondi, Dan Milea, Christophe Cognard, Giuseppe K. Ricciardi, Fabrice

Bonneville, Re ｴ 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 24:1240–1246, June/July 2003

2. Galen F. H. Chun, Thomas A. Tomsick: Transvenous Embolization of aDirect Carotid Cavernous Fistula through the Pterygoid Plexus. AJNR, 23:1156-1159, August 2002. Neil R. Miller, MD:Diagnosis and management of dural carotid–cavernous sinus fistulas. Neurosurg. Focus, 23(5):E13, 2007.

3. 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.

4. M. S. Greenberg.: Carotid-cavernous fistula. Handbook of Neurosusrgery 6th Ed., 28.6:845-846, 2006.

5. Perry P. Ng, M.D., Randall T. Higashida, M.D., Sean Cullen, M.D., Reza Malek, M.D., Van V. Halbach, M.D., Christopher F. Dowd, M.D.: Endovascular strategies for carotid cavernous and intracerebral dural arteriovenous fistulas. Neurosurg Focus 15 (4):Clinical Pearl 1, 2003.

6. T. J. K. Leonard, I. F. Moseley, M. D. Sanders: Ophthalmoplegia in carotid cavernous sinus fistula. British Journal of Ophthalmology, 68:128-134, 1984.

7. 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

8. Luca Remonda, Susanne Beatrice Frigerio, Robert Bu ｨ hler, and Gerhard Schroth: Transvenous Coil Treatment of a Type A Carotid Cavernous Fistula in Association with Transarterial Trispan Coil Protection. AJNR 25:611–613, April 2004