PUPIL
Dr.Sandra Mosses
JR1M3
 aperture located in the centre of the iris that
allows light to enter the retina
 control the amount of light entering the eye
 ensure optimal vision for the lighting conditions
 pupils should be equal in size, round, regular,
centered in the iris and should exhibit specific
reflex responses PERRLA
Size
 normal –2-6 mm in diameter
 ordinary ambient light ---3-4 mm in diameter
Ambient
illuminatio
n
Para
Sympatheti
c
supply
Sympatheti
c
supply
PUPIL GAUGE
Miosis <2 mm
 Old age
 Hyperopia
 Alcohol abuse
 Neurosyphilis
 Diabetes
 Levodopa therapy
 Horner's syndrome
 Pontine hematoma
Spastic or irritative miosis-- spasm of
the pupillary sphincter
 Corneal/intraocular fbs
 Miotic drops
 Spasm of the near reflex
 Chronic anterior segment
ischemia
 Adie's pupil
Scarring miosis
 Iridocyclitis-- scarring bind the
pupil down to the cornea (anterior
synechia) or lens (posterior
synechia) and cause miosis and
pupillary irregularity
Paralytic miosis-- paralysis of the
Acquired miosis
ophthal
disorders
Mydriasis >6 mm
 anxiety, fear, pain
 myopia
 bilateral lesions of the retina /anterior visual
pathways
 midbrain lesions
 cardiac arrest
 cerebral anoxia
 terminal condition
 Large pupils were
once considered a
sign of youth and
beauty, and the
anticholinergic
belladonna (Ital. “fair
lady”) alkaloids were
named for their
ability to produce
this effect
 Persons with light
irises have larger
pupils than those
Effects of Drugs on the Pupil
 Atropine
 Homatropine
 Scopolamine
 Epinephrine/norepinephr
ine
 Phenylephrine
 Hydroxyamphetamine
 Cocaine
Anticholinergics/sympatho
 Pilocarpine
 Methacholine
 Muscarine
 Physostigmine/neostigmi
ne
 Opiates
 Ergot derivatives
Cholinomimetics/cholineste
MYDRIASIS MIOSIS
Shape
 round, with a smooth, regular outline
Abnormalities –
 iritis
 Synechia
 congenital coloboma (a gap in the iris)
 prior trauma
 iridectomy
Equality
 reactivity of the normal eye and the consensual light
reflex will ensure pupil size remains equal
 difference of 0.25 mm in pupil size is noticeable
 difference of 2 mm is considered significant
Physiologic anisocoria
 1 mm difference --15% to 20%
 degree of inequality remains about the same in light
and dark
 pupils react normally to all stimuli and to instilled
drugs
ANISOCORIA
TOURNAY ‘S PUPILLARY
PHENOMENON
eccentric pupil
spontaneous, cyclic
displacement of the pupil
from the centre of iris
• local eye disease
• Severe midbrain
disorders
CORECTOPIA IRIDIS (ectopia
pupillae, Wilson's sign)
MICROCORIA ---
MEGALOCORIA
POLYCORIA--multiple
pupils
Double pupil
Light
Reflex
 Always have the patient fix at a distance
 Normal pupillary light reflex -- brisk constriction
followed by slight dilatation back to an
intermediate state (pupillary escape)
 Escape may occur because of adaptation of the
visual system to the level of illumination
 Responses --prompt, sluggish, or absent, graded
from 0 to 4+
Accommodation Reflex
near response, near reflex, accommodation-
convergence synkinesis, near synkinetic triad
THICKENIN
G OF LENS
CONVER
GENCE
OF EYES
MIOSIS
 primary stimulus for accommodation is
blurring
 Without the near response, attempting to
focus on a close object would result in
blurred vision or frank diplopia
 Accommodation --contraction of the ciliary
muscle relaxes the zonular fibers,
permitting the lens to become more
convex because of its inherent elasticity---
Ciliospinal reflex
 consists of dilation of the pupil on painful
stimulation of the skin of I/L neck
 Local cutaneous stimulation (e.g., scratching
the neck)--activates sympathetics through
connections with the ciliospinal center at C8-
T2 --sympathetic nervous system—dilator
pupillae--I/L pupil dilate
 intact ciliospinal reflex is evidence of
brainstem integrity when evaluating a
comatose patient
 Oculosensory /oculopupillary reflex
consists of either constriction of the pupil or dilation
followed by constriction in response to painful
stimulation of the eye or its adnexa
 Piltz-Westphal reaction
pupils normally constrict on attempted lid closure
 Cochleopupillary reflex/Vestibulopupillary reflex
constriction followed by dilation occurs in response to a
loud noise/stimulation of the labyrinthine system
 Psychic reflex
dilate in response to fear, anxiety, mental concentration
because of sympathetic nervous system activity
Large Pupils
 pupillary parasympathetics occupy a position on the
dorsomedial periphery of the nerve as it exits the
brainstem
 compressive lesions -- affect the pupil (aneurysm)
 Ischemic lesions-- spare pupil (diabetic third nerve
palsies) because the periphery of the nerve has a better
vascular supply
 This rule is not absolute: pupil-sparing third nerve
palsies have been reported with aneurysms (in up to
10% of cases), as have diabetic palsies involving the
III rd CN palsy
Adie's tonic
pupil
PUPIL RULE ---
BARTON
Complete pupil sparing with
otherwise complete and
isolated palsy of CN III is
never due to an aneurysm
HUTCHINSON’S PUPIL
 Pupil is involved early
and prominently with
third nerve compression
due to uncal herniation
Cavernous sinus
lesions
 ocular sympathetics are involved
along with CN III
 pupil may be midposition
 compression of both CN III and the
pericarotid sympathetics, leaving the
pupil mid-size but unreactive
 should not be mistaken for pupil
sparing
Adie's (Holmes-Adie) tonic pupil
 Asymptomatic Young woman suddenly noticing
Unilaterally enlarged pupil
 Pupillary reaction to light absent
 Reaction to near slow but preserved
 Ciliary ganglion or short ciliary nerves or both
 Depressed or absent deep tendon reflexes,
particularly in the lower extremities
 Old adie's pupil --unilateral miosis
Small Pupils
HORNER'S
SYNDROME
ARGYLL
ROBERTSON PUPIL
HORNER’S SYNDROME [J. F.
Horner]
 Sympathetic dysfunction
Ptosi
s
Miosis Anhidrosis
Apparent
enophthalmo
s
Loss of the
ciliospinal reflex
Ocular
hypotony
Increased
amplitude
of
accommoda
tion
vasodilatio
n in
affected
distributio
n
 Small pupil dilates poorly in the dark
 Pupillary asymmetry greater in the
dark than in the light generally means
Horner's syndrome
 The pupil in Horner's syndrome not
only dilates less fully, it dilates less
 Ptosis of the upper lid due to denervation of Müller's
muscle is only 1 mm to 3 mm
 The lower lid is frequently elevated 1 mm to 2 mm
because of loss of the action of the lower lid accessory
retractor that holds the lid down (inverse ptosis)
 Resulting narrowing of the palpebral fissure causes
apparent enophthalmos
 Fibers mediating facial sweating travel up the external
carotid--lesions distal to the carotid bifurcation produce
no facial anhidrosis except for perhaps a small area of
medial forehead that is innervated by sympathetic fibers
traveling with the internal carotid
FIRST ORDER HORNER'S SYNDROME
 Interruption of the sympathetic pathways between
the hypothalamus and the spinal cord (Wallenberg's
syndrome)
SECOND ORDER HORNER'S SYNDROME
 2nd order neuron lies in the ciliospinal center at C8-
T2
 Lesion involving this portion of the pathway (e.g.,
syringomyelia, C8 root lesion)
THIRD ORDER HORNER'S SYNDROME
 third order neuron lies in the superior sympathetic
CAUSES OF HORNERS
SYNDROME
 Brainstem lesions (especially of the lateral
medulla)
 Internal carotid artery thrombosis or
dissection
 Cavernous sinus disease
 Apical lung tumors
 Neck trauma
 Cluster headache
 Isolated manifestation of syringomyelia
Behaviour of unequal pupils in light
& dark
Reverse Horner's syndrome
(Porfour du Petit syndrome)
 Unilateral mydriasis, facial
flushing and hyperhidrosis
 Due to transient sympathetic
overactivity in the early stages of
a lesion involving the sympathetic
pathways to one eye
Pharmacologic testing to assess
level of lesion
 Cocaine drops instilled into the eye can confirm
the presence of Horner's syndrome
 Cocaine blocks the reuptake of norepinephrine
from the nerve terminals, increasing its effect
 With Horner's syndrome of any type, there is
less norepinephrine being released, less
accumulates at the pupillodilator, and cocaine
will fail to dilate the affected pupil
Hydroxyamphetamine
 can distinguish a third order from other types of
Horner's syndrome
 release of norepinephrine, but only from intact nerve
endings
 If the third order neuron is intact, as with first or
second order Horner's syndrome, the pupil will dilate
in response to hydroxyamphetamine
 In a third order Horner's syndrome, there are no
surviving nerve endings in the eye to release
norepinephrine and the pupil will fail to dilate
Congenital Horner's syndrome
 may cause sympathetic heterochromia iridis
and other trophic changes of the head and
face
Pseudo-Horner's syndrome
 Thompson et al. described a group of patient's
with unilateral ptosis and miosis of unrelated
origin simulating oculosympathetic paresis,
 majority of patients had simple, physiologic
anisocoria accompanied by incidental ptosis
due to blepharochalasis
Argyll Robertson Pupil
 small (1 mm to 2 mm)
 irregular in outline
 light near dissociation --react poorly or not at all
to light, but very well to near
 Anterior visual pathway function must be
normal
 generally bilateral and symmetric
 lesion lies in the periaqueductal region,
pretectal area and rostral midbrain dorsal to the
Mechanism:
 Pupillary light reflex fibers enter the
dorsal brainstem
 Near response fibers ascend to the EW
nucleus from the ventral aspect
 Disorders that affect the dorsal rostral
brainstem may affect the light reaction but
leave the near reaction intact
CAUSES OF LIGHT NEAR
DISSOCIATION
 Neurosyphilis (mandate serological testing if present)
 Diabetic autonomic neuropathy (tabes diabetica)
 Myotonic muscular dystrophy
 Amyloidosis
 Adie's pupil
 Lyme disease
 Chronic alcoholism
 Chiasmal lesions
 Sarcoidosis
 Multiple sclerosis (MS)
 Aberrant regeneration of CN III
Afferent Pupillary Defect
 Key clinical technique in the evaluation
of suspected optic neuropathy
 detect a side-to-side difference even
when the lesion is mild and there is no
detectable difference in the direct light
reflex when testing each eye
individually
Swinging flashlight test
 OPTIC NERVE LESION--brain detects a relative diminution in light intensity
and the pupil may dilate a bit in response
 The pupil in the other eye dilates as well because the consensual reflex
constricting the pupil in the good eye is less active than its direct reflex
 On moving the light back to the good eye, the more active direct response
causes the pupil to constrict
 On moving back to the bad eye, the pupil dilates because the direct light
reflex is weaker than the consensual reflex that had been holding it down
 As the light passes back and forth, the pupil of the good eye constricts to
direct light stimulation and the pupil of the bad eye dilates to direct light
stimulation
 DYNAMIC ANISOCORIA
 weaker direct response or the paradoxical dilation of the light-stimulated
pupil is termed an afferent pupillary defect (APD), or Marcus Gunn pupil
Hippus / pupillary play/
athetosis
 Normal pupils may display constant, small amplitude
fluctuations in size under constant illumination
 no clinical significance, even when pronounced
 but can cause confusion in the evaluation of an
afferent pupillary defect
 Hippus is random; a true APD will be consistent over
multiple trials
 Pay attention to the first movement of the pupil--
consistently a dilation movement the patient has an
APD and not hippus
UNSUAL PUPILLARY
ABNORMALITIES
 Paradoxical pupil
 Constrict in darkness
 Congenital retinal and optic nerve disorders
 Mechanism is unknown
 Tadpole pupil
 Pupil intermittently and briefly becomes comma-
shaped
 Springing pupil
(benign, episodic pupillary dilation; mydriasis á bascule)
Intermittent, sometimes alternating, dilation of
one pupil lasting minutes to hours
Young, healthy women
Often followed by headache
 Periodic unilateral mydriasis migraine and as an
ictal phenomenon
 Scalloped pupils  familial amyloidosis
 Oval pupilsmajor intracranial pathology /may be a
transient phase in evolving injury to the third nerve
nuclear complex
Pupil

Pupil

  • 1.
  • 2.
     aperture locatedin the centre of the iris that allows light to enter the retina  control the amount of light entering the eye  ensure optimal vision for the lighting conditions  pupils should be equal in size, round, regular, centered in the iris and should exhibit specific reflex responses PERRLA
  • 3.
    Size  normal –2-6mm in diameter  ordinary ambient light ---3-4 mm in diameter Ambient illuminatio n Para Sympatheti c supply Sympatheti c supply
  • 4.
  • 5.
    Miosis <2 mm Old age  Hyperopia  Alcohol abuse  Neurosyphilis  Diabetes  Levodopa therapy  Horner's syndrome  Pontine hematoma Spastic or irritative miosis-- spasm of the pupillary sphincter  Corneal/intraocular fbs  Miotic drops  Spasm of the near reflex  Chronic anterior segment ischemia  Adie's pupil Scarring miosis  Iridocyclitis-- scarring bind the pupil down to the cornea (anterior synechia) or lens (posterior synechia) and cause miosis and pupillary irregularity Paralytic miosis-- paralysis of the Acquired miosis ophthal disorders
  • 6.
    Mydriasis >6 mm anxiety, fear, pain  myopia  bilateral lesions of the retina /anterior visual pathways  midbrain lesions  cardiac arrest  cerebral anoxia  terminal condition
  • 7.
     Large pupilswere once considered a sign of youth and beauty, and the anticholinergic belladonna (Ital. “fair lady”) alkaloids were named for their ability to produce this effect  Persons with light irises have larger pupils than those
  • 8.
    Effects of Drugson the Pupil  Atropine  Homatropine  Scopolamine  Epinephrine/norepinephr ine  Phenylephrine  Hydroxyamphetamine  Cocaine Anticholinergics/sympatho  Pilocarpine  Methacholine  Muscarine  Physostigmine/neostigmi ne  Opiates  Ergot derivatives Cholinomimetics/cholineste MYDRIASIS MIOSIS
  • 9.
    Shape  round, witha smooth, regular outline Abnormalities –  iritis  Synechia  congenital coloboma (a gap in the iris)  prior trauma  iridectomy
  • 10.
    Equality  reactivity ofthe normal eye and the consensual light reflex will ensure pupil size remains equal  difference of 0.25 mm in pupil size is noticeable  difference of 2 mm is considered significant Physiologic anisocoria  1 mm difference --15% to 20%  degree of inequality remains about the same in light and dark  pupils react normally to all stimuli and to instilled drugs
  • 11.
  • 12.
  • 13.
    eccentric pupil spontaneous, cyclic displacementof the pupil from the centre of iris • local eye disease • Severe midbrain disorders CORECTOPIA IRIDIS (ectopia pupillae, Wilson's sign)
  • 14.
  • 15.
  • 16.
  • 17.
  • 19.
     Always havethe patient fix at a distance  Normal pupillary light reflex -- brisk constriction followed by slight dilatation back to an intermediate state (pupillary escape)  Escape may occur because of adaptation of the visual system to the level of illumination  Responses --prompt, sluggish, or absent, graded from 0 to 4+
  • 20.
    Accommodation Reflex near response,near reflex, accommodation- convergence synkinesis, near synkinetic triad THICKENIN G OF LENS CONVER GENCE OF EYES MIOSIS
  • 22.
     primary stimulusfor accommodation is blurring  Without the near response, attempting to focus on a close object would result in blurred vision or frank diplopia  Accommodation --contraction of the ciliary muscle relaxes the zonular fibers, permitting the lens to become more convex because of its inherent elasticity---
  • 23.
    Ciliospinal reflex  consistsof dilation of the pupil on painful stimulation of the skin of I/L neck  Local cutaneous stimulation (e.g., scratching the neck)--activates sympathetics through connections with the ciliospinal center at C8- T2 --sympathetic nervous system—dilator pupillae--I/L pupil dilate  intact ciliospinal reflex is evidence of brainstem integrity when evaluating a comatose patient
  • 24.
     Oculosensory /oculopupillaryreflex consists of either constriction of the pupil or dilation followed by constriction in response to painful stimulation of the eye or its adnexa  Piltz-Westphal reaction pupils normally constrict on attempted lid closure  Cochleopupillary reflex/Vestibulopupillary reflex constriction followed by dilation occurs in response to a loud noise/stimulation of the labyrinthine system  Psychic reflex dilate in response to fear, anxiety, mental concentration because of sympathetic nervous system activity
  • 25.
    Large Pupils  pupillaryparasympathetics occupy a position on the dorsomedial periphery of the nerve as it exits the brainstem  compressive lesions -- affect the pupil (aneurysm)  Ischemic lesions-- spare pupil (diabetic third nerve palsies) because the periphery of the nerve has a better vascular supply  This rule is not absolute: pupil-sparing third nerve palsies have been reported with aneurysms (in up to 10% of cases), as have diabetic palsies involving the III rd CN palsy Adie's tonic pupil
  • 27.
    PUPIL RULE --- BARTON Completepupil sparing with otherwise complete and isolated palsy of CN III is never due to an aneurysm
  • 28.
    HUTCHINSON’S PUPIL  Pupilis involved early and prominently with third nerve compression due to uncal herniation
  • 29.
    Cavernous sinus lesions  ocularsympathetics are involved along with CN III  pupil may be midposition  compression of both CN III and the pericarotid sympathetics, leaving the pupil mid-size but unreactive  should not be mistaken for pupil sparing
  • 30.
    Adie's (Holmes-Adie) tonicpupil  Asymptomatic Young woman suddenly noticing Unilaterally enlarged pupil  Pupillary reaction to light absent  Reaction to near slow but preserved  Ciliary ganglion or short ciliary nerves or both  Depressed or absent deep tendon reflexes, particularly in the lower extremities  Old adie's pupil --unilateral miosis
  • 31.
  • 32.
    HORNER’S SYNDROME [J.F. Horner]  Sympathetic dysfunction Ptosi s Miosis Anhidrosis Apparent enophthalmo s Loss of the ciliospinal reflex Ocular hypotony Increased amplitude of accommoda tion vasodilatio n in affected distributio n
  • 33.
     Small pupildilates poorly in the dark  Pupillary asymmetry greater in the dark than in the light generally means Horner's syndrome  The pupil in Horner's syndrome not only dilates less fully, it dilates less
  • 34.
     Ptosis ofthe upper lid due to denervation of Müller's muscle is only 1 mm to 3 mm  The lower lid is frequently elevated 1 mm to 2 mm because of loss of the action of the lower lid accessory retractor that holds the lid down (inverse ptosis)  Resulting narrowing of the palpebral fissure causes apparent enophthalmos  Fibers mediating facial sweating travel up the external carotid--lesions distal to the carotid bifurcation produce no facial anhidrosis except for perhaps a small area of medial forehead that is innervated by sympathetic fibers traveling with the internal carotid
  • 36.
    FIRST ORDER HORNER'SSYNDROME  Interruption of the sympathetic pathways between the hypothalamus and the spinal cord (Wallenberg's syndrome) SECOND ORDER HORNER'S SYNDROME  2nd order neuron lies in the ciliospinal center at C8- T2  Lesion involving this portion of the pathway (e.g., syringomyelia, C8 root lesion) THIRD ORDER HORNER'S SYNDROME  third order neuron lies in the superior sympathetic
  • 38.
    CAUSES OF HORNERS SYNDROME Brainstem lesions (especially of the lateral medulla)  Internal carotid artery thrombosis or dissection  Cavernous sinus disease  Apical lung tumors  Neck trauma  Cluster headache  Isolated manifestation of syringomyelia
  • 39.
    Behaviour of unequalpupils in light & dark
  • 40.
    Reverse Horner's syndrome (Porfourdu Petit syndrome)  Unilateral mydriasis, facial flushing and hyperhidrosis  Due to transient sympathetic overactivity in the early stages of a lesion involving the sympathetic pathways to one eye
  • 41.
    Pharmacologic testing toassess level of lesion  Cocaine drops instilled into the eye can confirm the presence of Horner's syndrome  Cocaine blocks the reuptake of norepinephrine from the nerve terminals, increasing its effect  With Horner's syndrome of any type, there is less norepinephrine being released, less accumulates at the pupillodilator, and cocaine will fail to dilate the affected pupil
  • 42.
    Hydroxyamphetamine  can distinguisha third order from other types of Horner's syndrome  release of norepinephrine, but only from intact nerve endings  If the third order neuron is intact, as with first or second order Horner's syndrome, the pupil will dilate in response to hydroxyamphetamine  In a third order Horner's syndrome, there are no surviving nerve endings in the eye to release norepinephrine and the pupil will fail to dilate
  • 44.
    Congenital Horner's syndrome may cause sympathetic heterochromia iridis and other trophic changes of the head and face Pseudo-Horner's syndrome  Thompson et al. described a group of patient's with unilateral ptosis and miosis of unrelated origin simulating oculosympathetic paresis,  majority of patients had simple, physiologic anisocoria accompanied by incidental ptosis due to blepharochalasis
  • 45.
    Argyll Robertson Pupil small (1 mm to 2 mm)  irregular in outline  light near dissociation --react poorly or not at all to light, but very well to near  Anterior visual pathway function must be normal  generally bilateral and symmetric  lesion lies in the periaqueductal region, pretectal area and rostral midbrain dorsal to the
  • 46.
    Mechanism:  Pupillary lightreflex fibers enter the dorsal brainstem  Near response fibers ascend to the EW nucleus from the ventral aspect  Disorders that affect the dorsal rostral brainstem may affect the light reaction but leave the near reaction intact
  • 47.
    CAUSES OF LIGHTNEAR DISSOCIATION  Neurosyphilis (mandate serological testing if present)  Diabetic autonomic neuropathy (tabes diabetica)  Myotonic muscular dystrophy  Amyloidosis  Adie's pupil  Lyme disease  Chronic alcoholism  Chiasmal lesions  Sarcoidosis  Multiple sclerosis (MS)  Aberrant regeneration of CN III
  • 48.
    Afferent Pupillary Defect Key clinical technique in the evaluation of suspected optic neuropathy  detect a side-to-side difference even when the lesion is mild and there is no detectable difference in the direct light reflex when testing each eye individually
  • 49.
  • 50.
     OPTIC NERVELESION--brain detects a relative diminution in light intensity and the pupil may dilate a bit in response  The pupil in the other eye dilates as well because the consensual reflex constricting the pupil in the good eye is less active than its direct reflex  On moving the light back to the good eye, the more active direct response causes the pupil to constrict  On moving back to the bad eye, the pupil dilates because the direct light reflex is weaker than the consensual reflex that had been holding it down  As the light passes back and forth, the pupil of the good eye constricts to direct light stimulation and the pupil of the bad eye dilates to direct light stimulation  DYNAMIC ANISOCORIA  weaker direct response or the paradoxical dilation of the light-stimulated pupil is termed an afferent pupillary defect (APD), or Marcus Gunn pupil
  • 51.
    Hippus / pupillaryplay/ athetosis  Normal pupils may display constant, small amplitude fluctuations in size under constant illumination  no clinical significance, even when pronounced  but can cause confusion in the evaluation of an afferent pupillary defect  Hippus is random; a true APD will be consistent over multiple trials  Pay attention to the first movement of the pupil-- consistently a dilation movement the patient has an APD and not hippus
  • 53.
    UNSUAL PUPILLARY ABNORMALITIES  Paradoxicalpupil  Constrict in darkness  Congenital retinal and optic nerve disorders  Mechanism is unknown  Tadpole pupil  Pupil intermittently and briefly becomes comma- shaped
  • 54.
     Springing pupil (benign,episodic pupillary dilation; mydriasis á bascule) Intermittent, sometimes alternating, dilation of one pupil lasting minutes to hours Young, healthy women Often followed by headache  Periodic unilateral mydriasis migraine and as an ictal phenomenon  Scalloped pupils  familial amyloidosis  Oval pupilsmajor intracranial pathology /may be a transient phase in evolving injury to the third nerve nuclear complex