OP28 Ophthalmology Toronto Notes 2020

Pupils

Pupils
• pupil size is determined by the balance between the sphincter muscle and the dilator muscle
• sphincter muscle is innervated by the parasympathetic nervous system carried by CN III
5 Targets of Retinal Signals
• dilator muscle is innervated by the sympathetic nervous system (SNS) • Pretectal nucleus (pupillary reflex/eye
■ first-order neuron = hypothalamus → brainstem → spinal cord movements)
■ second-order/preganglionic neuron = spinal cord → sympathetic trunk via internal carotid artery → • Lateral geniculate body of thalamus
superior cervical ganglion in neck • Superior colliculus (eye movements)
■ third-order/postganglionic fibres originate in the superior cervical ganglion, neurotransmitter is • Suprachiasmatic nucleus (optokinetic)
• Accessory optic system (circadian
norepinephrine rhythm)
◆ as a diagnostic test, 4-10% cocaine prevents the reuptake of norepinephrine, and will cause
dilation of normal pupil, but not one with loss of sympathetic innervation (Horner’s Syndrome)
• see Neurology, Figure 8, N8

Pupillary Light Reflex

• light shone directly into eye travels along optic nerve (CN II, afferent limb) → optic tracts → bilateral
midbrain
• impulses enter bilaterally in midbrain via pretectal area and Edinger-Westphal nuclei
• nerve impulses then travel down CN III (efferent limb) bilaterally to reach the ciliary ganglia, and finally
to the iris sphincter muscle, which results in the direct and consensual light reflexes

α1 – Pupillary dilator muscle contraction (mydriasis)

β2 – Ciliary muscle relaxation (non-accommodation); increased aqueous humour production
M3 – Pupillary sphincter contraction (miosis); increased ciliary muscle contraction (Accommodation)

Pupil Abnormalities
Denervation Hypersensitivity
• when postganglionic fibres are damaged, the under-stimulated end-organ attempts to compensate by
developing an increase of neuroreceptors and becomes hypersensitive
• postganglionic parasympathetic lesions (i.e. Adie’s pupil)
■ pupil will constrict with 0.125% pilocarpine (cholinergic agonist), normal pupil will not
• postganglionic sympathetic lesions (this test is used to differentiate between pre- and post-ganglionic
lesions in Horner’s syndrome)
■ pupil will dilate with 0.125% epinephrine, normal pupil will not

Local Disorders of Iris

• posterior synechiae (adhesions between iris and lens) due to iritis can present as an abnormally shaped
pupil
• ischemic damage (e.g. post-acute angle-closure glaucoma) usually occurs at 3 and 9 o’clock positions
resulting in a vertically oval pupil that reacts poorly to light
• trauma (e.g. post-intraocular surgery)

Anisocoria
• unequal pupil size
• idiopathic/physiologic anisocoria
■ 20% of population
■ round, regular, <1 mm difference
■ pupils reactive to light and accommodation
■ responds normally to mydiatrics/miotics
■ post eye surgery, or extensive retinal laser treatment
• see Table 9 for other causes of anisocoria
OP29 Ophthalmology Toronto Notes 2020
Pupils

Patient with Anisocoria

Relevant history and examination with specific attention to:

• History of ocular trauma
• Check old photographs (ptosis, ocular deviation, long standing anisocoria)
• Use of topical medications
• Exposure to toxins and drugs
• Associated ocular and neurologic symptoms/signs

Which pupil is abnormal?

Examine pupils in light and dark

Anisocoria accentuated Anisocoria accentuated

Anisocoria equal in
by darkness by light
light and dark
(small pupil abnormal) (large pupil is abnormal)

Dilation lag Brisk reaction Isolated Ptosis/Ophthalmoplegia

Ptosis to light Sluggish to light
Light near dissociation

Test with 10% cocaine Use of 0.1% pilocarpine Third nerve palsy

Small pupil does Both pupils dilate Large pupil Large pupil does
not dilate symmetrically constricts not constrict

Horner’s syndrome Adie’s tonic pupil Use of 0.1% pilocarpine

Minimal/no constriction

Physiologic anisocoria Pharmocologic anisocoria

Patient Must Fixate on Distant Target

Figure 21. Approach to anisocoria
Reproduced with permission from: Kedar S, Biousse V, Newman NJ. Approach to the patient with anisocoria. In: UpToDate, Rose, BD (editor), UpToDate, Waltham, MA,
2011. Copyright 2011 UpToDate, Inc. For more information visit www.uptodate.com.

Table 9. Summary of Conditions Causing Anisocoria

Features Site of Lesion Light and Anisocoria Mydriatics/ Effect of
Accommodation Miotics Pilocarpine
ABNORMAL MIOTIC PUPIL (impaired pupillary dilation)
Argyll- Irregular, usually Midbrain Poor in light; Dilates/
Robertson bilateral better to Constricts
Pupil accommodation
Horner’s Round, unilateral, Sympathetic Both brisk Greater in Dilates/
Syndrome ptosis, anhydrosis, system dark Constricts
pseudoenophthalmos
ABNORMAL MYDRIATIC PUPIL (impaired pupillary constriction)
Adie’s Tonic Irregular, larger in Ciliary ganglion Poor in light, Greater in Dilates/ Constricts
Pupil bright light better to light Constricts (hypersensitivity
accommodation to dilute
pilocarpine)
CN III Palsy Round Superficial ± fixed (acutely) Greater in Dilates/ Constricts
CN III at 7-9 mm light Constricts
Mydriatic Round, uni- or Iris sphincter Fixed at 7-8 mm Greater in No effect Will not constrict
Pupil bilateral light
OP30 Ophthalmology Toronto Notes 2020
Pupils

Dilated Pupil (Mydriasis)

Sympathetic Stimulation
• fight or flight response CN III palsy with pupillary involvement
• mydriatic drugs: epinephrine, dipivefrin (Propine®), phenylephrine may be associated with a posterior
communicating artery aneurysm
Parasympathetic Under-stimulation
• cycloplegics/mydriatics: atropine, tropicamide, cyclopentolate (parasympatholytic)
• CN III palsy
■ eye deviated down and out with ptosis present
■ etiology includes stroke, neoplasm, aneurysm, acute rise in ICP, DM (may spare pupil), trauma
■ both mydriatics and CN III palsy cause pupil dilation; however, pupils in CN III palsy will constrict Midbrain
briskly to pilocarpine, while pupils dilated from mydriatics will not

Acute Angle-Closure Glaucoma

• fixed, mid-dilated pupil Basilar
Posterior artery
communicating
Adie’s Tonic Pupil artery CN III
• 80% unilateral, F>M
• pupil is tonic or reacts poorly to light (both direct and consensual) but constricts with accommodation Normal
• caused by benign lesion in ciliary ganglion; results in denervation hypersensitivity of Post. comm. artery
parasympathetically innervated constrictor muscle
■ dilute (0.125%) solution of pilocarpine will constrict tonic pupil but have no effect on normal pupil
• long-standing Adie’s pupils are smaller than unaffected eye CN III
Parasymp.
Trauma Somatic motor
• damage to iris sphincter from blunt or penetrating trauma
• iris transillumination defects may be apparent using ophthalmoscope or slit-lamp Externally Compressive CN III Lesion
• pupil may be dilated (traumatic mydriasis) or irregularly shaped from tiny sphincter ruptures

Constricted Pupil (Miosis) Down and out

mydriasis
Senile Miosis Post. comm.
• decreased sympathetic stimulation with age artery
aneurysm

Parasympathetic Stimulation Central Vascular CN III Lesion

• local or systemic medications such as:
■ cholinergic agents: pilocarpine, carbachol
■ cholinesterase inhibitor: phospholine iodide
■ opiates, barbiturates Down and out
pupil spared
Horner’s Syndrome
• lesion in sympathetic pathway Central infarct
• difference in pupil size greater in dim light, due to decreased innervation of adrenergics to iris dilator © Andreea Margineanu 2012
muscle
• associated with ptosis and anhydrosis of ipsilateral face/neck Figure 22. CN III lesions with and without
• application of cocaine 4-10% (blocks reuptake of norepinephrine) to eye does not result in pupil mydriasis
dilation (vs. physiologic anisocoria), therefore confirms diagnosis
• hydroxyamphetamine 1% (stimulates norepinephrine release) will dilate pupil if central or
preganglionic lesion, not postganglionic lesion
Horner’s MAP
• now more commonly, topical apraclonidine 0.5% Miosis
• postganglionic lesions result in denervation hypersensitivity, which will cause pupil to dilate with Anhydrosis
0.125% epinephrine, whereas normal pupil will not Ptosis
• causes: carotid or subclavian aneurysm, brainstem infarct, demyelinating disease, cervical or
mediastinal tumour, Pancoast tumour, goitre, cervical lymphadenopathy, surgical sympathectomy,
Lyme disease, cervical ribs, tabes dorsalis, cervical vertebral fractures

Iritis
• miotic pupil initially
• can become irregularly shaped pupil due to posterior synechiae
• later stages non-reactive to light

Argyll-Robertson Pupil
• both pupils irregular and <3 mm in diameter, ± ptosis
• does not respond to light stimulation
• responds to accommodation (light-near dissociation)
• suggestive of neurosyphilis or other conditions (DM, encephalitis, MS, chronic alcoholism, CNS
degenerative diseases)

Other Causes
• optic neuritis, retinal lesions
OP31 Ophthalmology Toronto Notes 2020
Malignancies

Relative Afferent Pupillary Defect

Normal Pupillary Response 1. Swinging Light Test

Direct response Consensual response Normal eye Pathological eye

Constriction of Constriction of Constriction of both pupils normal

stimulated eye unstimulated eye

Aqueduct 2. Swinging Light Test

Pretectal nucleus Normal eye Pathological eye
Edinger-Westphal
nucleus (III)
Constriction of both pupils normal

Oculomotor nerves (III)

© Sam Holmes 2016 after Merry Shiyu Wang 2012

Optic chiasm
Optic nerve (II) 3. Swinging Light Test
Normal eye Pathological eye
Cilliary
ganglion
Pupils appear to dilate – positive RAPD

Constrictor muscles Rapidly swing light to pathological eye

of pupil

Figure 23. Relative afferent pupillary defect

• also known as Marcus Gunn pupil

Cataracts never produce a RAPD
• impairment of direct pupillary response to light caused by a lesion in visual afferent (sensory) pathway,
anterior to optic chiasm
• differential diagnosis: large RD, BRAO, CRAO, CRVO, advanced glaucoma, optic nerve compression,
optic neuritis (most common)
It is possible to have RAPD and normal
• does not occur with media opacity (e.g. corneal edema, cataracts) vision at the same time, e.g. in damaged
• pupil reacts poorly to light and better to accommodation superior colliculus caused by thalamic
• test: swinging flashlight hemorrhage
■ if light is shone in the affected eye, direct and consensual response to light is decreased
■ if light is shone in the unaffected eye, direct and consensual response to light is normal
■ if the light is moved quickly from the unaffected eye to the affected eye, “paradoxical” dilation of Differentiate RAPD from physiologic
both pupils occurs pupillary athetosis (“hippus”), which is
■ observe red reflex, especially in patients with dark irides rapid, rhythmic fluctuations of the pupil,
with equal amplitude in both eyes
• if the defect is bilateral there is no RAPD, as dilation is measured relative to the other eye

Malignancies
• uncommon site for 1° malignancies
• see Retinoblastoma, OP38

Lid Carcinoma
Etiology
• basal cell carcinoma (rodent ulcer) (90%)
■ spread via local invasion, rarely metastasizes
■ ulcerated centre, indurated base with pearly rolled edges, telangiectasia
• squamous cell carcinoma (<5%)
■ spread via local invasion, may also spread to nodes and metastasize
■ ulceration, keratosis of lesion
• sebaceous cell carcinoma (1-5%)
■ often masquerades as chronic blepharitis or recurrent chalazion
■ highly invasive, metastasizes
• other: Kaposi’s sarcoma, malignant melanoma, Merkel cell tumour, metastatic tumour