Transcript Grand Rounds

Grand Rounds Conference
Jinghua Chen, MD, PhD
University of Louisville
Department of Ophthalmology and Visual Sciences
May 1, 2015
History
CC: Double vision for two days.
HPI:
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12 year old boy presented with diplopia for 2 days. About 2 days
ago he noticed double vision and he had problem looking to his
right with his right eye.
He denied any vision change.
Past Medical History
POH:
PMH:
Allergy:
FH:
Eye Medication:
Myopia (-3.50 D OD and -2.25 D OS)
Asthma
Chocolate
None contributory
None
Exam
OD
OS
BCVA:
20/20
20/20
Pupils:
43
43
No RAPD OU
IOP:
13
11
EOM
Limited abduction OD
CVF:
Full OU
Anterior segment:
Normal OU
DFE:
Normal OU
Eye Movement Exam in ED
Lab Workup in ED
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Hematology: Normal
ICP: 26 (<25 mm Hg)
CSF:
Appearance: clear
 Color: colorless
 RBC: 10 (0/ µL)
 WBC: 138, 94% lymph (0-8/ µL)
 Glucose: 40 (50-80 mg/dL)
 Total protein: 47 (15–45 mg/dL)
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MRI without and with Contrast
Normal MRI T2 weighted images
Assessment
12
year old boy presented with diplopia for 2 days. LP shows elevated ICP, mild
increased WBC, normal MRI
Other
history:
One week ago patient developed high fever with some neck stiffness.
He also complained eye pain and headache.
Impression:
Differential
Right 6th nerve palsy due to Meningitis
Diagnosis:
Infection, viral illness
Brain tumor: lesion of cerebellopontine angle
Nucleus aplasia: Duane’s syndrome
Ischemic mononeuropathy: most common in adults
Trauma
Inflammation: petrous bone, facial pain, Gradenigo syndrome
Migraine headache
Elevated pressure inside the brain
Management
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Patient received one time dose of iv Rocephin
inside hospital.
Follow up office visit 10 days later.
Patient states doing better.
 No diplopia, crossing or drifting.
 No blurred vision.
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The sixth nerve has the longest subarachnoid
course of all cranial nerves
Ophthalmology 2nd . 2004: 1324
Pediatric Sixth Nerve Palsies
The Rochester Epidemiology Project - Olmsted County residents
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The annual combined incidence of third, fourth, and sixth nerve palsies was 7.6
per 100,000 (95% confidence interval, 5.1 to 10.1).
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The fourth (36%), followed by the sixth (33%), the third (22%), and multiple
nerve palsies (9%).
Am J Ophthalmol. 1999 Apr;127(4):388-92.
American Academy of Ophthalmology; 2006:118
Etiologies of Acquired 6th Nerve Palsy
*6% to 30% attributed to a miscellaneous group of causes that includes leukemia, migraine,
pseudotumor cerebri, multiple sclerosis; the miscellaneous group of etiologies reflects the
poor localizing value of sixth nerve paresis.
**6% to 29%, etiology undetermined, reflecting vulnerability of the nerve to conditions which
are transient, benign and unrecognizable.
J Ophthalmic Vis Res. 2013 Apr; 8(2): 160–171.
Isolated Sixth Nerve Palsy
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The 6th cranial nerve is the most frequently
affected nerve in an isolated ocular motor palsy.
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Diabetes mellitus, hypertension or history of a
recent viral infection.
Syndromes of the Sixth Nerve PalsyLocalizing Signs
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1. Brainstem
2. Subarachnoid space
3. Petrous apex
4. Cavernous sinus/superior orbital fissure
5. Orbit
Brainstem Syndrome
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A lesion in the posterior fossa may be compressive,
ischemic, inflammatory (multiple sclerosis in young
adults) or degenerative and may involve the fifth,
seventh and eighth cranial nerves
J Ophthalmic Vis Res. 2013 Apr; 8(2): 160–171.
Subarachnoid Space - Elevated
Intracranial Pressure Syndrome
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Downward displacement of the brainstem causing stretching of the sixth nerve as its
exits the pons and inside Dorello’s canal.
30% of patients with pseudotumor cerebri have sixth nerve paresis as the only
neurologic deficit
Other pathologies in the subarachnoid space include hemorrhage, meningeal infections
(viral, bacterial, fungal), inflammation (sarcoidosis) or infiltrations (lymphoma,
leukemia, carcinoma).
J Ophthalmic Vis Res. 2013 Apr; 8(2): 160–171.
Petrous Apex Syndrome
Gradenigo's Syndrome
1904 the syndrome was introduced by Giuseppe
Gradenigo
A complication of otitis media and mastoiditis
Triad of diplopia, facial pain and otorrhea
http://pedemmorsels.com/gradenigos-syndrome-and-otitis-media/
Cavernous Sinus Syndrome
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Third, fourth, fifth, sixth and sympaththetic fibers.
Internal carotid artery aneurysm
J Ophthalmic Vis Res. 2013 Apr; 8(2): 160–171.
Orbital Syndrome
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Proptosis
Congestion of conjunctival vessels and chemosis
Optic atrophy or papilledema.
Horner syndrome may be present
Trigeminal signs
Tumors of the orbit, orbital pseudotumor, thyroid eye
disease, orbital cellulitis or myositis.
The Six Mimickers of Sixth Nerve Palsy
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Thyroid eye diseases
Myasthenia gravis
Duane’s syndrome
Spasm of the near reflex
Delayed break in fusion
Old blowout fracture of the orbit
Management of Pediatric Patients
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Identify and treat the cause of the condition, and to relieve the symptoms.
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Neoplasms, especially of the posterior fossa account for 39%. (Robertson DM, Arch Ophthalmol
1970;83:574-579.)
Trauma accounts for 54.4%. (Abbas Bagheri, J Ophthalmic Vis Res 2010;5:32-37.)
Maintain binocular vision:
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Fresnel prisms
Injection of botulinum toxin into the ipsilateral medial rectus.
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Development of an abducens nerve palsy following minimal head trauma should raise the
suspicion of a compressive lesion such as a tumor.
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Spontaneous recovery of an abducens nerve palsy may occur even with skull base tumors or
leukemia, perhaps from axonal regeneration, resorption of hemorrhage in tumors or immune
response to the tumor.
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Surgery:
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Vertical muscle transposition procedures such as Jensen's, Hummelheim's or whole muscle transposition.
Operation on both the lateral and medial rectii of the affected eye.
References
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BSCS 2014-2015 Book 5, Neuro-Ophthalmology: 220-221
Azarmina M, Azarmina H. The Six Syndromes of the Sixth Cranial Nerve J Ophthalmic Vis Res. 2013 Apr; 8(2): 160–171.
Shrader EC, Schlezinger NS. Neuro- ophthalmologic evaluation of abducens nerve paralysis. Arch Ophthalmol. 1960;63:84–91.
Rucker CW. The causes of paralysis of the third, forth, and sixth cranial nerves. Am J Ophthalmol. 1966;61:1293–1298.
Johnston AC. Etiology and treatment of abducens paralysis. Trans Pac Coast Otoophthalmol Soc Annu Meet. 1968;49:259–277.
Robertson DM, Hines JD, Rucker CW. Acquired sixth-nerve paresis in children. Arch Ophthalmol. 1970;83:574–579.
Rush JA, Younge BR. Paralysis of cranial nerves III, IV, and VI. Cause and prognosis in 1000 cases. Arch Ophthalmol. 1981;99:76–
79.
Patel SV, Mutyala S, Leske DA, Hodge DO, Holmes JM. Incidence, associations, and evaluation of sixth nerve palsy using a
population-based method. Ophthalmology. 2004;111:369–375.
Bagheri A, Babsharif B, Abrishami M, Salour H, Aletaha M. Outcomes of surgical and non-surgical treatment for sixth nerve
palsy. J Ophthalmic Vis Res. 2010;5:32–37.
Quah BL, Ling YL, Cheong PY, et al. A review of 5 years' experience in the use of botulinium toxin A in the treatment of sixth
cranial nerve palsy at the Singapore National Eye Centre. Singapore Med J.1999 Jun;40(6):405-9. Boger WP 3rd, Puliafito CA,
Magoon EH. Recurrent isolated sixth nerve palsy in children. Ann Ophthalmol. 1984 Mar;16(3):237-8, 240-4.
Holmes JM, Mutyala S, Maus TL. Pediatric third, fourth, and sixth nerve palsies: a population-based study. Am J
Ophthalmol. 1999 Apr;127(4):388-92.
Repka MX, Lam GC, Morrison NA. The efficacy of botulinum neurotoxin A for the treatment of complete and partially
recovered chronic sixth nerve palsy. J Pediatr Ophthalmol Strabismus 1994;31:79-83.