9/22/2015

The : A Window on the

Steven A. Newman, M.D. University of Virginia Charlottesville, VA

Financial Disclosure

• Are you kidding? • I’m a Neuro-ophthalmologist.

The Problem • What the look like when seen by the surgeon (68yo referred for , presenting with decreased vision):

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Pupil – A Little History

• Galen (2 nd Century AD): did not cause • Covering one eye resulted in slight pupillary dilatation • Covering a blind eye would not produce dilatation • Based on Galen: if the opposite pupil did not dilate when covering an eye couching for cataract was not likely to improve vision

Pupil – A Little History

• 1855 von Graefe: “warned ophthalmologists not to be in such a hurry with dilating drops that they missed important pupillary signs.”

The Free Zone

• What do you call the hole in a donut?

• ?George

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Pupil - Introduction

• Window on the eye and visual system • End organ to the IIIrd and sympathetics • Anterior portion of the uveal tract

Pupil - Introduction

• One objective sign in neuro-ophthalmology – Very useful in functional or non-cooperative patients – Sort from and media • Separate afferent from efferent pathology – Fixed pupil/anisocoria: efferent – Afferent pupillary defect: afferent

Pupil – Final Pathway

• Sphincter: – 1mm wide – Posterior – 70 separate segments – Parasympathetic

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Pupil – Final Pathway

• Dilator: – Radial – Stop .3mm short of margin – Sympathetic innervation – Pigmented myoepithelial cells

Pupil – 4 Neuron Arc

• Retina – optic nerve – tract – pretectal nuclei • Pretectal nuclei – posterior commissure – Edinger-Westphal complex • Edinger-Wesphal nucleus – IIIrd nerve (inferior division) – • Ciliary ganglion – short ciliary nerves – pupillary sphincter

Pupil – Anatomy - Parasympathetics

• Leave Edinger-Westphal nucleus • Travel w/ fascicular III • Peripheral in subarachnoid III • Inferior division III • Inferior oblique branch • Ciliary ganglion (synapse) • Short posterior ciliary nerves • Innervate sphincter and

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Pupil – Anatomy - Sympathetics • Hypothalamus (supraoptic nucleus) • Intermediolateral column • Ciliospinal nucleus of Budge-Waller • Ventral ramus • Paraspinal sympathetics • Pericarotid complex • Superior cervical ganglion • Pericarotid – cavernous – VI • Nasociliary branch V – ciliary ganglion

PERRLA

Is It Regular?

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Pupil Irregularity

• Congenital – Coloboma • Acquired – Traumatic – Inflammatory – Ischemia – Degenerative

Pupil Size

• Balance tone – Sympathetic – Parasympathetic • Pharmacologic • Neurologic • Local effects

Pupil – Size - Background

• Ambient light • Retinal adaptation • Sleep: loss sympathetic • Arousal: increase sympathetic • Lid closure: increase parasympathetic • Ciliospinal reflex: increase sympathetic

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Pupil – Size - Miscellaneous

• Age: increasing • Iris color: light iris larger • Severe miosis – Pontine hemorrhage – Narcotics – Phospholine iodide

Mydriasis

• Trauma • Pharmacologic • IIIrd nerve dysfunction

Are They Equal?

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Anisocoria

• Noticeable in >20% population – Increase with age – 5% >1mm – >50% >.2mm • Afferent system pathology will not cause anisocoria

Anisocoria

• Goldilocks question – Small pupil too small – Large pupil too large • Record pupil size – Pupil gauge – Photographs • Examine in dim light/bright light • Fellow travelers – Lid position – Motility

Anisocoria - Essential

• Difference equal in light and dark • May be variable • Occasionally reverse

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Anisocoria – Small Pupil

• Anisocoria greater in the dark • Local pathology – Inflammation – Posterior synechiae • Pharmacologic (miotics) • Horner’s syndrome

Horner’s Syndrome

• Loss of sympathetic innervation • Clinical signs – Miosis – – Anhidrosis – ? Increase • Congenital – Heterochromia

Horner’s - Diagnosis • Exclude local pathology • Pharmacologic – Cocaine test – Apraclonidine test

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Horner’s - Diagnosis • Exclude local pathology • Pharmacologic – Cocaine test – Apraclonidine test

Baseline Horner’s in Dark

Post-apraclonidine OU

Horner’s - Location

• First order – Long tract signs – Brainstem pathology • Second order – Neck – Lung apex • Third order – Carotid – Cavernous sinus

Horner’s - Localization • Fellow travelers • Pharmacologic testing – Paredrine test (release norepi): dilate (3 rd intact)

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Horner’s - Localization • Fellow travelers • Pharmacologic testing – Paredrine test (release norepi): no dilate (3 rd involved)

Horner’s - Workup

• Indications – All first/second order – Non-isolated third order • MRI – Brainstem – Neck/lung apex • MRA – R/o dissection

Anisocoria – Large Pupil • Anisocoria greater in the light • Local pathology – Inflammation – Posterior synechiae • Pharmacologic (mydriatics) – Jimson weed • IIIrd nerve palsy • Adie’s syndrome

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Large Pupil – Local Pathology

• Sphincter damage following cataract surgery • Traumatic • Angle closure • Old inflammation – Posterior synecchiae – Pigment on capsule

Pupil - Unusual Syndromes

• Urrets-Zavalia syndrome: following PK (others), speculate about iris ischemia • Unrecognized : scopolamine patch

Anisocoria – IIIrd Nerve Palsy

• ALWAYS w/ motility disturbance • Associated ptosis • ? Reactivity

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Adie’s Syndrome

• Post-ganglionic denervation – Post viral – Traumatic • Sphincter paralysis • Vermiform movements • Regional corneal hypesthesia • Depressed deep tendon reflex

Adie’s Syndrome • Clinical findings – Dilated pupil (less with age) – Light/near dissociation – Vermiform movements • Pupillary drug testing – Hypersensitivity 1/10% pilocarpine

Adie’s syndrome

Following 1/10% pilo

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Pupillary Drug Testing

• Pilocarpine – 1/10% pilocarpine: hypersensitivity Adie’s – 1% pilocarpine: normal sensitivity (IIIrd) – 4% pilocarpine: confirm pharmacologic blockade • Cocaine – Failure dilate: 2nd & 3rd order Horner’s • Apraclonidine – Will dilate the suprasensitive smaller pupil • Paredrine – Failure dilate: 3rd order Horner’s

Does the pupil react?

If not does it react to near?

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Light Near Dissociation

• Midbrain – Argyll Robertson pupil – Dorsal midbrain syndrome • Ciliary ganglion – Adie’s syndrome • Long posterior ciliary nerve – Pan retinal photocoagulation • Bilateral

Argyll Robertson Pupil

• Midbrain microvascular pathology – – Diabetes • Small irregular pupil • Brisk reaction to near • Rapid redilate • Poor dilatation to atropine

Argyll Robertson Pupil - LN

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Argyll Robertson Pupil

• Other evidence of neuro-syphilis – – Charcot joint • Serology – VDRL – FTA-ABS

Light Near Dissociation

• Midbrain – Argyll Robertson pupil – Dorsal midbrain syndrome • Ciliary ganglion – Adie’s syndrome • Long posterior ciliary nerve – Pan retinal photocoagulation • Bilateral optic neuropathy

Dorsal Midbrain Syndrome

• Clinical – Absent up gaze – Retraction convergence – Collier’s sign • Pathology – Pineal tumor – Midbrain tumor – Dilated IIIrd ventricle • Associated –

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Light Near Dissociation

• Midbrain – Argyll Robertson pupil – Dorsal midbrain syndrome • Ciliary ganglion – Adie’s syndrome • Long posterior ciliary nerve – Pan retinal photocoagulation

Light Near Dissociation – Adie’s

If yes does it react equally?

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Hippus

• Pupil normally rebounds after constriction • Variable • Should be equal

Afferent Pupillary Defect

• Most important objective sign in ophthalmology • Indication of asymmetric optic nerve function • Correlates with visual field loss • Exceptions – Mild w/ – With large retinal pathology – Contralateral to tract

History

• Marcus Gunn (1904): described asymmetric response to covering each eye in the setting of optic nerve dysfunction • Kestenbaum (1946): popularized testing for an afferent pupillary defect and introduced the eponym "Marcus Gunn pupil" • Levatin: described the swinging flashlight test

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Regulate the Amount of Light Coming In

• Central "computer" to summate amount of light from each eye

Examination Techniques

• Every patient should be screened for an afferent pupillary defect • Fix accommodation – Visible distant Snellen letter – Dark room • May need to side light the eye to observe especially with dark iris

Examination Techniques

• Only one working pupil is necessary to observe for an APD

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Examination Techniques • Light source • Bright – Muscle light – Indirect • Not so bright as to produce an aversive reaction

Examination Techniques

• Equal time on each pupil • Rapid movement between eyes • Watch for the initial pupillary reaction • Secondarily – Observe the amount of contraction – Look for escape

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A Little More History • Fineberg & Thompson (1979): quantitate APD with neutral density filters

Quantitation APD

• Subjective gradation: 1-4+ scale • Patient subjective gradation: relative brightness • Neutral density filters – .3log steps (50% reduction in the amount of light) – Place over the better reacting pupil until equalized • Pupilometer (infrared)

Quantitation APD

• Filters in .3log steps • Additive

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Implications of APD

• Anterior segment pathology (/cataract) DOES NOT produce APD

Conclusions

• Three pupillary questions (remember PERRLA) – Regular? – Equal? – Reactive? • You don’t have to be a professor to learn a lot from a pupil

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