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Slide Notes

Anisocoria is a not uncommon finding in the emergency room. The following is a listing of 9 cases presenting in the ED with the chief complaint of "funny pupils". Though the complaint is the same, the diagnosis and treatment vary greatly.
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20/20: "One is the Loneliest Number"

Published on Sep 18, 2016

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PRESENTATION OUTLINE

"ONE IS THE LONELIEST NUMBER"

ANISOCORIA IN THE ED
Anisocoria is a not uncommon finding in the emergency room. The following is a listing of 9 cases presenting in the ED with the chief complaint of "funny pupils". Though the complaint is the same, the diagnosis and treatment vary greatly.
Photo by Demonsub

Case 1

  • 14yo M, asthma
  • Comes in with asthma attack
  • Receives oxygen + nebulized Albuterol via BiPAP
  • Recovering well when his right pupil became fixed and dilated, and "just looks funny" to mom

14yo male comes into the ED with an asthma attack. Treatment received without complication but while recoving, his mother became anxious about her sons right pupil which was growing larger than the left, and "just looks funny".

leaky albuterol inhaler

Leaky albuterol inhaler!

A misplace nebulizer mask can create a breeze of beta-agonist flowing directly to the eye. This can results in sympathetic stimulation and enlargement of one pupil vs. the other.

Dx - "clinical gestault"
Tx - fix the mask; self-resolving


Nebulizer-Associated Anisocoria
Neil Iosson, M.R.C.P., M.B., B.Chir.
N Engl J Med 2006; 354:e8March 2, 2006DOI: 10.1056/NEJMicm050851


Picture:
Carcinoide bronquial como causa de asma refractario a tratamiento... http://elsurl.com/lbbXBImI

case 2

  • 32yo F p/w acute painless enlarged right pupil, mild blurry vision
  • Effected pupil constricts poorly to light
  • Said she doesn't recall when it started but friend recently pointed out that it "looked funny"
  • Effected pupil constricted 30 min after given dilute pilocarpine (0.1%) solution
32yo female comes into the ED with acute painless enlargement of the right pupil and mild blurry vision. She's not sure how long she's had this problem but her friend recently noticed how "funny" her pupils looked.

On exam you noted no reaction to light in the effected pupil but response in contralateral eye when light shined in either eye. There was also a slow constrictive response to prolonged near effort
(accommodation).

A dilute pilocarpine (0.1%) solution was applied, resulting in pupil constriction.

Adie's pupil

"Little Old Adie's"
Adie's Pupil (aka, tonic pupil, "little old Adie's")

Tonically dilated pupil with no reaction to light, slow constrictive response to prolonged near effort
(accommodation), and slow and tonic radiation after constriction.
80% unilateral, F>M (3:1); Average - 25-45yo; Prevalence - 2/1,000 people

Physiology (Loewenfeld Hypothesis): Destruction of nerve innervation to ciliary muscle (accommodation) and constrictor papillae muscle most likely due to viral infection (linked to syphillis, tumor, trauma, giant cell arteritis).

Aberrant regeneration - cells that supply ciliary muscle regenerate and innervate both ciliary muscle and
pupillary sphincter muscle. (near response in a tonic pupil is present but slow because elicited by nerve cells designed for accommodation, a slower movement than pupillary constriction.

Dx - Pilocarpine challenge - constricts in response to dilute pilocarpine (0.05 – 0.1%) - vs. normal/atropine pupil

Tx - glasses, tx if symptomatic





Adie WJ. Tonic pupils and absent tendon reflexes: a benign disorder sui generis; its complete and incomplete forms. Brain 1932;55:98–113.

Image and case: http:/​/​dx.​doi.​org/​10.​1212/​WNL.​0b013e3182698cc5

Adie WJ. PSEUDO-ARGYLL ROBERTSON PUPILS WITH ABSENT TENDON REFLEXES: A BENIGN DISORDER SIMULATING TABES DORSALIS. Br Med J 1931; 1:928.

Bremner FD, Smith SE. Bilateral tonic pupils: Holmes-Adie syndrome or generalized neuropathy? Br J Ophthalmol. 2007;92:1620-1623. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2095539/

Chakravarty A, Mukherjee A, Roy D. Ross syndrome--a case documentation. Acta Neurol Scand 2003; 107:72.

Fletcher WA, Sharpe JA. Tonic pupils in neurosyphilis. Neurology 1986; 36:188.

Jacobson DM, Vierkant RA. Comparison of cholinergic supersensitivity in third nerve palsy and Adie’s syndrome. J Neuroophthalmol. 1998;18(3):171-75. http://www.ncbi.nlm.nih.gov/pubmed/9736199

case 3

  • 16yo M comes in after fistfight with best friend over girl
  • Feels fine, but blurry vision and "funny looking pupil"
  • L pupil does not react to light or accommodation
  • Patient pupil remains permanently dilated despite multiple surgies
  • Patient goes on to record 26 albums and receive induction into the Rock n Roll Hall of Fame
16yo male comes in after fistfight with best friend over girl. Patient says he feels fine - just a little sore with some blurry vision - but on physical examination, the left pupil does not react to light or accommodation.

Full workup is done without noting any intracranial abnormalities, hemorrhage.

Patient is refered to ENT, where he is treated yet pupil remains permanently dilated despite multiple surgies.

Still goes on to record 26 albums and receive induction into the Rock n Roll Hall of Fame despite ocular deficit.

traumatic mydriasis

Dx. Traumatic Mydriasis (aka, "David Bowie Syndrome")

Blunt injury to the globe may result in traumatic mydriasis or, less commonly, miosis. Traumatic mydriasis is often associated with iris sphincter tears that can permanently alter the shape of the pupil. Miosis tends to be associated with anterior chamber inflammation

Dx - CT, MRI - head
Tx - rest +/- surgery


http://www.aao.org/bcscsnippetdetail.aspx?id=4276f81f-5d7f-48b4-ba87-ad3f4d...

Facts: http://fortune.com/2016/01/11/david-bowie-career-sales/

case 4

  • 35yo caucasian F p/w ocular pain, blurred vision, dilated R pupil that "doesn't look normal'
  • ROS - hx of stumbling gait and tendency to fall over last 4 years
  • Sensations of electricity running down their spine; aggravated by flexing cervical spine
  • Sx's worsened last week after running local half marathon
Case: 35yo caucasian F prevents with ocular pain, blurred vision, and dilated R pupil. On review of systems she notes to have had a history of stumbling gait and tendency to fall over last 4 years. Has also had sensations of electricity running down their spine; aggravated by flexing cervical spine.
She also notes that the symptoms worsened last week after running local half marathon.

multiple sclerosis

Multiple sclerosis

Multiple sclerosis (MS) is most common immune-mediated inflammatory demyelinating disease of the central nervous system.


Dx:
1) MRI of brain, eye orbit, and s.cord - shows demyelination in both space and time - need 2+ lesions to make diagnosis of MS
2) LP - incr protein, oligoclonal bands (IgG on electrophoresis) - 90% of patients

Tx:
- There is no cure. Goal of tx is to prevent relapses and relieve sx's of acute exaccerbations.

- Acute - high-dose IV glucocorticoids (Methylpred - 500-1000mg/day x 5d) - note - oral not as efficacious (steroids have no effect on risk of future attacks or change in natural history of disease; just speed concurrent attack recovery)

- Maintenance:
- IFN-B ( use especially in patients with acute-onset optic neuritis)
- Glatiramer
- Natalizumab
- Dimethyl fumarate (oral)
- Teriflunomide (oral)

- Vitamin D (decreased susceptibility to attacks)

Calabresi PA, Kieseier BC, Arnold DL, Balcer LJ, Boyko A, Pelletier J, et al. Pegylated interferon ß-1a for relapsing-remitting multiple sclerosis (ADVANCE): a randomised, phase 3, double-blind study. Lancet Neurol. 2014 Jul. 13(7):657-65.


Polman CH, Reingold SC, Edan G, et al. Diagnostic criteria for multiple sclerosis: 2005 revisions to the "McDonald Criteria". Ann Neurol. 2005 Dec. 58(6):840-6.

US Food and Drug Administration. FDA approves new multiple sclerosis treatment: Tecfidera. March 27, 2013. Available at http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm345528.htm. Accessed: April 2, 2013.

case 5

  • 75yo F p/w painful, red eye, sudden vision loss, halos around lights
  • First noticed when left matinee movie while walking to his car
  • PE - rock-hard, dilated pupil, decreased visual acuity
75yo female comes into the ED with the sudden onset of a painful, red eye, sudden vision loss, halos around lights. She said she first noticed when left matinee movie while walking to his car.

On physical exam you notice rock-hard, dilated pupil, decreased visual acuity.

Acute angle glaucoma

Acute angle closure

Defined as at least 2 of the following symptoms:
ocular pain, nausea/vomiting, and a history of intermittent blurring of vision with halos; and at least 3 of the following signs:
IOP greater than 21 mm Hg, conjunctival injection, corneal epithelial edema, mid-dilated nonreactive pupil, and shallower chamber in the presence of occlusion.

This is an EM emergency!

Dx - Tonometry, Gonioscopy
*DO NOT dilate pupils!*

Tx - Immediate optho referral -> laser iridotomy

"TAP" it (1 drop q1 min): 0.5% timolol, 1.0% apraclonidine, 2.0% Pilocarpine

Berkoff DJ, Sanchez LD. An uncommon presentation of acute angle closure glaucoma. J Emerg Med. 2005 Jul. 29(1):43-4.

Tarongoy P, Ho CL, Walton DS. Angle-closure glaucoma: the role of the lens in the pathogenesis, prevention, and treatment. Surv Ophthalmol. 2009 Mar-Apr. 54(2):211-25.

case 6

  • 16yo F p/w painless "funny-looking eye' and concern for infection
  • Was visiting grama when she got some dust in her R eye
  • Used grama's eye drops to relieve irritation
  • Drops relieved eye, but soon after her sister noticed that the center of one eye looked bigger than the other.
16yo femal comes in with painless "funny-looking eye and concern for infection. She was visiting grama when she got some dust in her right eye. Fortunately she was able to use grama's eye drops to relieve irritation. However, soon after her sister noticed that the center of one eye looked bigger than the other.

pharmacologic pupil

(Atropine Drops for grama's glaucoma)
Atropine-induced mydriasis.

Pharmacologic anisocoria can present as mydriasis or miosis following administration of agents that act on the pupillary dilator or sphincter muscles. Anticholinergics such as atropine, homatropine, tropicamide, scopolamine and cyclopentolate lead to mydriasis and cycloplegia by inhibiting parasympathetic M3 receptors of the pupillary sphincter and ciliary muscles.

Dx - clinical picture and careful history
Tx - rinse eye; resolves with cessation of the offending agent

http://eyewiki.aao.org/Anisocoria

case 7

  • 45yo M p/w progressive headaches, worse in morning
  • Recently noted blurriness in L eye while reading
  • Also noted he woke this morning with L side facial numbness that hasn't subsided
  • PE - dilated pupil, decreased visual acuity, pupil not reactive to light or accommodation
45yo males comes in complaining of progressively worsening headaches over the last 2 months, worse in morning. Has notice blurriness in left eye while reading and also mentioned he woke this morning with left side facial numbness that hasn't subsided.

On physical exam you note a dilated pupil, decreased visual acuity, pupil not reactive to light or accommodation

Brain tumor

(Cavernous Sinus Meningioma)
Brain Tumor

Brain tumors can produce symptoms and signs by local brain invasion, compression of adjacent structures, and increased intracranial pressure (ICP). In addition to the histology of the tumor, the clinical manifestations are determined by the function of the involved areas of the brain:

- Increased intracranial pressure (ICP)
- Direct compression of essential gray or white matter
- Shifting of intracranial contents
- Secondary cerebral ischemia

Symptoms may be nonspecific and include any of the following:
- Headache
- Altered mental status
- Ataxia
- Nausea
- Vomiting
- Weakness
- Gait disturbance

Facial paralysis due to CN7 palsy can be misconstrued as facial "numbness" due to inability to move face.

Dx - CT, MRI-brain
Tx - dependent on tumor type

DeAngelis LM. Brain tumors. N Engl J Med. 2001 Jan 11. 344(2):114-23.

Forsyth PA, Posner JB. Headaches in patients with brain tumors: a study of 111 patients. Neurology 1993; 43:1678.

Purdy RA, Kirby S. Headaches and brain tumors. Neurol Clin. 2004 Feb. 22(1):39-53.

case 8

  • 50yo M with PMH diabetes
  • P/W sudden right facial swelling, proptosis and complete ophthalmoplegia, fever
  • Besides the pain, he's anxious because one pupil looks different than the other and won't go away
  • PMH - tooth extraction 4d ago
50yo male with hx of uncontrolled diabetes comes in with sudden onset right facial swelling, proptosis and complete ophthalmoplegia. Besides the pain, he's anxious because one pupil looks different than the other and won't go away. Of note, he had a tooth extraction 4d ago and the pain has been intermittent.

cavernous sinus thrombosis

Cavernous sinus thrombosis

Usually a late complication of an infection of the central face or paranasal sinuses. Other causes include bacteremia, trauma, and infections of the ear or maxillary teeth. Cavernous sinus thrombosis is generally a fulminant process with high rates of morbidity and mortality.

The cavernous sinuses receive venous blood from the facial veins (via the superior and inferior ophthalmic veins) as well as the sphenoid and middle cerebral veins. They, in turn, empty into the inferior petrosal sinuses, then into the internal jugular veins and the sigmoid sinuses via the superior petrosal sinuses. This complex web of veins contains no valves; blood can flow in any direction depending on the prevailing pressure gradients. Since the cavernous sinuses receive blood via this distribution, infections of the face including the nose, tonsils, and orbits can spread easily by this route.

Dx - CT, MRI, blood cultures
Tx - Broad-spectrum IV antibiotics

Andrews CM, Hawk HE, Holmstedt CA. Case Report: Septic cavernous sinus thrombosis. Neurol Clin Neurosci. 2014. 2:117-118.

Seow VK, Chong CF, Wang TL, Lin CM, Lin IY. Cavernous sinus thrombophlebitis masquerading as ischaemic stroke: a catastrophic pitfall in any emergency department. Emerg Med J. 2007 Jun. 24(6):440.

Tang Y, Booth T, Steward M, Solbach T, Wilhelm T. The imaging of conditions affecting the cavernous sinus. Clin Radiol. 2010 Nov. 65(11):937-45.


Image and case courtesy of: http://www.radpod.org/2007/11/01/cavernous-sinus-thrombosis/

case 9

  • 67yo F with PMH of HTN and a 35 pack-year smoking hx p/w sudden onset h/a and large R pupil that she noticed coincided with new-onset h/a
  • H/A worst at onset, 9/10 in severity
  • Patient also notes neck pain, nausea but no vomiting, blurred vision
67yo F with PMH of HTN and a 35 pack-year smoking history presents with sudden onset of headache, 9/10 in severity, and large right pupil that she noticed coincided with headache. Patient also notes neck pain, nausea but no vomiting, blurred vision. Has never had anything like this before.

Vital signs - BP - 189/114, HR - 42, RR - 14, Sat - 97% RA

cerebral aneurysm

Cerebral Aneurysm/ SAH

The clinical presentation of cerebral aneurysms includes symptoms associated with major aneurysmal rupture (eg, SAH), minor aneurysmal hemorrhage (eg, warning leak or sentinel bleed), non-hemorrhagic manifestations (eg, mass effects or cerebral ischemia), and asymptomatic scenarios (eg, incidental aneurysm detection or identification through screening)

MC location - anterior communicating artery

Dx:
- CT, CTA
- LP - Shows xanthochromia (blood in CSF)
- MR or angiography to identify site of bleed

Tx:
- Hyperventilate
- Elevate head of bed
- d/c anticoagulants
- Maintain SBP- Definitive tx - surgical metal coil
*Craniectomy to drain hematoma if present

Vernooij MW, Ikram MA, Tanghe HL, Vincent AJ, Hofman A, Krestin GP, et al. Incidental findings on brain MRI in the general population. N Engl J Med. 2007 Nov 1. 357(18):1821-8.

Image courtesy of http://www.neuroophthalmology.ca/textbook/disorders-of-eye-movements/iv-neu...

SUMMary

  • Keep the nebulizer tight!
  • "Little old Adie's" loves dilute pilocarpine
  • Knockout punch can knock out pupil
  • Separation of space and time... think MS
  • If old, acute, painful eye.... think acute angle glaucoma
  • Tumors and aneurysms can compress.... get imaging
  • Recent infection/sinusitis/tooth pulling.... watch the danger zone!
  • Sudden onset, WHOML, max at onset.... you've got a bleeder!