Horner's Syndrome

Horners
Syndrome
(Kneller, Lewis & Oliver 1972)
Group 1D
David Ho, Emma Josey, Steven Lin, Nhi Nguyen, Melitta Tanzer, Jessie Whiley
(George, Haydar & Adams 2008)
http://frontierdreams.blogspot.com.au/2011/04/hornerssyndrome.html
(Reede et al. 2008)
An Overview
Rare interruption of ocular sympathetic nerve innervation,

caused by preceding primary condition
First official descriptions date back to 1869
Group of symptoms including miosis, ptosis and facial

anihidrosis, refer to Figure 1
Figure 1
Preceding primary condition can be benign or life

threatening,
Variety of diagnoses used to find specific source of nerve

interruption which can then be treated accordingly
Trimester 2,
2015
Horners Syndrome
Group 1D
HMO102 Science of Vision
(Reede et al. 2008)
Horners Syndrome
The
History
(Reede et al. 2008)
Figure 2
http://fidoseofreality.com/wpcontent/uploads/2014/09/cocker_leo.jpg
4
Trimester 2,
2015
Horners Syndrome
Group 1D
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The History Significant Findings
The History Johann Friedrich Horner
An ophthalmologist, pictured in Figure 3, who studied

under Von Graefe, a former student of Claude
Bernard
What did Horner discover?

Examined a patient: Anna Brandli, 40yo healthy peasant
woman, who had the following symptoms:
Slight drooping of right upper eyelid
Upper lid sunken into orbit
Right pupil more constricted than left
Right side face never perspired
Different temperatures on right and left side of face
Diagnosis: ptosis due to paralysis of the musculus palpebrae
superioris as a result of damage of the sympathetic nerves
Trimester 2,
2015
Horners Syndrome
Group 1D
Figure 3
(Amonoo-Kuofi, HS 1999)
Horners Syndrome
The
Anatomy
(Reede et al. 2008)
The Anatomy Three-Order Neuron

Trail
Sympathetic innervation to the eye begins in the Central

Nervous System consisting of a pathway of a three-order
neuron trail, as detailed in Figure 4
First Order Neurons begin in posterolateral hypothalamus

with synapsing at the pons and the ciliospinal centre of the
Budge-Weller
Second-Order Neurons exit T1 spinal chord and ascend

sympathetic chain with synapsing between the internal
carotid artery and jugular vein
Third-Order Neurons exit the superior cervical ganglion, and

form plexus surrounding the internal carotid artery
Figure 4
Trimester 2,
2015
Horners Syndrome
Group 1D
(Reede et al. 2008)
The Anatomy Nerve Fibre

Organisation
Nerve fibres forming plexus around internal carotid

artery divide, as shown in Figure 5
Nerves associated with sweating follow external carotid artery

Remaining nerves travel through carotid canal to the
cavernous sinus
Nerve fibres at the cavernous sinus follow CNVI, join

with ophthalmic division of the trigeminal nerve, and
continue to enter the orbit through the ciliary
ganglion to separate and innervate different orbital
structures
Trimester 2,
2015
Horners Syndrome
Group 1D
Figure 5
(Reede et al. 2008)
The Anatomy Disruptions
Nerve disruption causing ptosis and inverse ptosis results from a

loss of Muller muscle and upper eyelid muscle function,
respectively. When combined, ptosis and inverse ptosis
effectively narrow the interpalpebral fissure
Nerve disruption causing pupil dilation results from a lack of force

against the iris constrictor muscle, this innervation is shown
again in Figure 5
Nerve disruption causing dilation lag results from passive

pupillary innervation of the iris sphincter muscle
Figure 5
Nerve disruption causing anihidrosis results from either First and

Second Order neurons or Third Order neurons depending on
position of affected area
Trimester 2,
2015
Horners Syndrome
Group 1D
(Reede et al. 2008)
Horners Syndrome
The
Etiology
(Reede et al. 2008)
The Etiology
Central
Preganglionic
Caused by variety of conditions being mild and benign to severe

and life threatening
Can also be caused by cluster headaches or migraines
Figure 6
Congenital
11
Most often caused by trauma during medical procedures or

Pancoast tumours (on apex of lung)
Postganglionic
Horners Syndrome originating from here is uncommon

Usually due to cervical cord lesions from trauma or syringomyelia
(cyst causing disease) shown in Figure 6
Rare but can be caused from multitude of medical conditions or

birth-related trauma
Trimester 2,
2015
Horners Syndrome
Group 1D
http://www.adlergiersch.com/post-traumaticsyringomyelia/
Horners Syndrome
The
Pathophysi
ology
(Reede et al. 2008)
The Pathophysiology
Lesions along any point of sympathetic pathway

to eye
Effects vary depending on location of nerve

disruption, as pictured in Figure 7:
First Order/Central Neuron Disorder
Second Order/Pre-Ganglionic Neuron Disorder
Third Order/Post-Ganglionic Neuron Disorder

(known as Partial Horners Syndrome with pupil light reactions and
accommodation remaining unaffected)
Figure 7
13
Trimester 2,
2015
Horners Syndrome
Group 1D
(Gralapp, C 2015)
The Pathophysiology Common

Symptoms
14
Mild/moderate Ptosis, as pictured in

Figure 8
(drooping of upper eyelid)
Upside down Ptosis

(slight elevation of lower eyelid)
Miosis, as pictured in Figure 8

(constant pupil constriction)
Dilation Lag
Anhidrosis
(lack of sweating, leading to dry skin)
Trimester 2,
2015
Horners Syndrome
Figure 8
http://patient.info/doctor/horners-syndrome
Group 1D
The Pathophysiology Individual

Neuron Damage
Pathway: Originating from hypothalamus, through midbrain and extending into upper
spinal chord
Affected Area: Ipsilateral side of body

First
Order
Symptoms: Anhidrosis, abnormal eye movements, ipsilateral limb ataxia and
Neurons
dissociated sensory loss
Pathway: Originating from upper spinal chord, through upper chest and extending into
side of neck
Second Affected Area: Ipsilateral face

Order
Symptoms: Anhidrosis (shown in Figure 9), facial flushing
Neurons
Pathway: Originates from side of neck and into face, innervating facial skin and muscles
of iris and eyelids
Third
Affected Area: Area above ipsilateral brow or forehead
Order
Symptoms: Minimal anihidrosis, orbital pain/headaches
Neurons
15
Trimester 2,
2015
Horners Syndrome
Group 1D
The Pathophysiology - Anhidrosis
Figure 9
16
Trimester 2,
2015
Horners Syndrome
https://classconnection.s3.amazonaws.com/448/flashcards/56448/png/capture1316217505465.png
Group 1D
The Pathophysiology - Congenital

Horners Syndrome
Occurring in children under 2 years of age
Symptoms:
Iris Heterochromia, as pictured in Figure 10

(hypopigmentation of the eye, also common in long-term
cases of Horners Syndrome)
Figure 10
17
Trimester 2,
2015
Horners Syndrome
Group 1D
(Mirzai, H & Baser, EF 2006)
Horners Syndrome
The
Epidemiolo
gies
(Reede et al. 2008)
The Epidemiologies
19
Male to female ratio is 5:1
Equally distributed amongst all age groups
Congenital cases occur 1 in every 6,250

births
1.42 cases per 100,000 patients under

19yo
Trimester 2,
2015
Horners Syndrome
Figure 11
Group 1D
http://www.umweltbundesamt.de/en/topics/health/assessing-environmentallyrelated-health-risks/epidemiology
Horners Syndrome
Diagnose
s&
Treatmen
t
(Reede et al. 2008)
The Treatments Pharmacological

Tests
Apraclonidine Eye Drop Test
Effective at 0.5% concentration with good ocular penetration
Direct Alpha adrenergic receptor agonist
87% accuracy
Currently accepted alternative to cocaine eye drop test
(reacts strongly to alpha 2 and weaker to alpha 1 receptors)
Normal Pupil
Horners
Pupil
21
Trimester 2,
2015
Horners Syndrome
Denervated
Dominant
alpha 2
receptor
activity
Miosis or no
effect on
size
Increased
alpha 1
receptor
numbers
Group 1D
Mydriasis
The Diagnosis Pharmacological

Tests
Cocaine Eye Drop Test
Contains 4 10% of Topical Cocaine
Blocks noradrenalin uptake at the synaptic cleft

between postganglionic ciliary nerve and iris dilator
muscle
Normal pupil dilates or Mydriasis, as shown in

Figure 12
Only available in Australia in public hospital settingsFigure 12

Horners
Pupil
22
Trimester 2,
2015
Horners Syndrome
Denervate
d
https://lh4.googleusercontent.com/489j2ewJXWc_A2got6CJ0ix8o4G_T8tyjWm0PPr_NZhZok8miOURWZrt1pMRz1smEDMikdBi_rCiM3aSzZL2W61796Bw_5Ig2gWhOS5uwRMn0ypGeEj1BIl
No effect
on size
Group 1D
The Diagnosis Localisation
Hydroxyamphetamine Eye Drop
Helps to distinguish between first or second order neuron lesion from Third order
(postganglionic) neuron lesion and is pictured in Figure 13
Triggers release of stored noradrenaline from postganglionic neuron into the

neuromuscular junction at the iris dilator muscle
Applied 24-48 hours after the cocaine eye drop test to prevent
Interference with drug uptake
23
Trimester 2,
2015
Horners
Pupil with
intact
postgangli
onic
neuron
Noradrenal
ine present
Mydriasis
Horners
Pupil with
damaged
postgangli
onic
neuron
Noradrenal
ine absent
No effect
on size
Horners Syndrome
Figure 13
http://images.medscape.com/pi/features/drugdirectory/octupdate/AKN07040.jpg
Group 1D
The Diagnosis Neuroimaging
Assists in differential diagnosis and localization of lesion
MRI or CT scans
MRI, as pictured in Figure 14, is mainly used for soft tissue examination, takes
approximately 30 minutes to conduct and can be relatively expensive
CT is mainly used on bones, lung and chest injuries or cancers, takes
approximately 5 minutes to conduct, uses radiation but can be cheaper
Neuroimaging results are often combined with pharmacological

testing
Region of body undergoing medical imaging depends upon

suspected cause and location
Painful Horner syndrome may indicate carotid artery dissection MRI of brain and
neck
Stroke CT of head
Figure 14
(George, Haydar
& Adams 2008)
24
Trimester 2,
2015
Horners Syndrome
Group 1D
The Treatment Options
25
There is no effective treatment, as the underlying cause varies between

cases and each cause required appropriate treatment
Knowing cause of Horners Syndrome and eradicating underlying disease

can improve the symptoms
Horners Syndrome is diagnosed by an optometrist or doctor and patients

are referred to appropriate professionals for further investigation
Trimester 2,
2015
Horners Syndrome
Group 1D
Horners Syndrome
Case
Studies
(Reede et al. 2008)
Summary of Etiology
Figure 15
27
Trimester 2,
2015
Horners Syndrome
Group 1D
Case Study #1
52 year old man who developed Horners Syndrome after sneezing

(Bazari, F, Hind, M & Ong, Y 2010)
The sneeze resulted in a spontaneous dissection of his carotid artery,

which damaged the surrounding nerve fibres
Figure 16 shows the mans right eye with miosis and partial ptosis (A) and
haematoma at the site of the carotid artery dissection
Figure 16
28
Trimester 2,
2015
Horners Syndrome
(Bazari, F, Hind, M & Ong, Y 2010)
Group 1D
Case Study #2
25 year old man was attacked and stabbed (Dubois-Marshall, S & De Kock, S
2011)
Doctors treated and stitched, however a few days later the patient noticed
signs of Horners Syndrome, as shown in Figure 17
An X-ray was taken at the location of the original stab wound, shown in Figure
18, and it showed that the blade of the knife was still inside his neck, lodged in
a position that was disrupting nerve innervation
Figure 17 (Dubois-Marshall, S & De Kock, S

2011)
29
Trimester 2,
2015
Horners Syndrome
Figure 18 (Dubois-Marshall, S & De Kock, S

2011)
Group 1D
Case Study #3
30
5 year old boy with Attention Deficit Disorder (ADD) presented to

emergency department with complaints of unequal pupils after falling from
his bike and hitting his neck on the bike handlebars (Starr, BE, Shubert, RA
& Baumann, B 2004)
Showed symptoms of Horners Syndrome with a larger right pupil (R =

5mm, L = 2mm) and a lower left eyelid (1-2mm lower than right eyelid)
Further investigations and neurological scans were taken due to the blunt
trauma to the boys neck suspected to have caused a carotid artery
dissection (much like Case Study #1)
Trimester 2,
2015
Horners Syndrome
Group 1D
Case Study #4
3 year old red bellied parrot who was pinned to the ground by a white
cockatoo who proceeded to apply pressure to the parrots upper back and
lower neck
(Ganca, A et al. 2005)
Following the incident, the parrot showed ptosis of the left eye, whilst the
right eye was normal, this change in symptoms is respectively shown in
Figure 19
Parrot was diagnosed with Horners Syndrome caused by blunt force

trauma to sympathetic innervation of left eye
Figure 19
31
Trimester 2,
2015
Horners Syndrome
(Ganca, A et al. 2005)
Group 1D
32
Horners Syndrome A
summary
Distinguished as an official condition in 1869 after previous nerve experimentation and research of nerves in dogs
Is a rare condition caused by disruption of oculosympathetic nerve pathway (at either central, ganglionic or
postganglionic locations) with main symptoms of miosis, ptosis and anhidrosis
Location of nerve interruption determines effect upon ocular region
Equal prevalence amongst age groups, with males more likely to contract and congenital cases being more common
Diagnosed mainly by Apraclonidine eye drop testing, MRI or CT scans, with localisation determined by
Hydroxyamphetamine eye drops
No effective treatment for condition, but there is usually a treatment for the underlying cause and once treated
symptoms can be reduced
Despite small prevalence of disease, large number of case studies are reported to effectively inform health
professionals and general public of underlying health complications that can be contributing to Horners Syndrome
Trimester 2,
2015
Horners Syndrome
Group 1D
Thank
you
Horners
Syndrome
Group 1D
David Ho, Emma Josey, Steven Lin, Nhi Nguyen, Melitta Tanzer, Jessie Whiley
(Reede et al. 2008)
References
4designersart 2013, Epidemiology, Umwelt Bundesamt, 21/09/2015, <http://www.umweltbundesamt.de/en/topics/health/assessing-environmentally-related-health-risks/epidemiology>.
Amonoo-Kuofi, HS 1999, 'Horner's syndrome revisited: with an update of the central pathway', Clinical Anatomy (New York, N.Y.), vol. 12, no. 5, pp. 345-61George, A, Haydar, A & Adams, W 2008a, 'Imaging
of Horner's Syndrome', Clinical Radiology, vol. Volume 63, no. Issue 5, pp. pp. 499-505.
Bazari, F, Hind, M & Ong, Y 2010, 'Horner's Syndrome - not to be sneezed at ', The Lancet, vol. 375.
Bryant, C 2014, Health Condition Threatens Dog Vision, 21/09/2015, <http://fidoseofreality.com/health-condition-threatens-dog-vision/>.
Dubois-Marshall, S & De Kock, S 2011, 'Two days with a broken knife blade in the neck - an interesting case of Horner's Syndrome', Emergency Medical Journal, vol. 28, pp. 629-31.
Ganca, A, Malka, S, Sandmeyer, L, Cannon, M, Smith, D, Taylor, M 2005, Horners syndrome in a red-bellied parrot (Poicephalus rufiventris), Journal of Avian Medicine and Surgery, vol. 19, no. 1, pp. 30-34,
retrieved 21 September 2015, <http://www.bioone.org/doi/full/10.1647/2004-017>
George, A, Haydar, A & Adams, W 2008, Imaging of Horner's Syndrome, Clinical Radiology, <https://www.clinicalkey.com.au/#!/content/journal/1-s2.0-S0009926008000123>.
Gralapp, C 2015, Sympathetic Pathway, ONE Network, 21/09/2015, <http://www.aao.org/image/sympathetic-pathway-3>.
H., D 2011, Exam 2, Studyblue, 18/09/2015, <https://www.studyblue.com/notes/note/n/exam-2/deck/1122222>.
Kelly A. Walton, LMB 2003, 'Horner syndrome', Current Opinion in Ophthalmology, vol. 14, no. 1, pp. 357-63.
Kexin, M, Wei, T, Zhenye, LV & Xiangyang, S 2015, 'Horner's syndrome subsequent to minimally invasive video-assisted thyroidectomy in two patients', Oncology Letters, vol. 10, no. 1, pp. 459-62.
Kneller, SK, Lewis, RE & Oliver, JE 1972, Horner's syndrome following common carotid artery catheterization in cats, 10, Blackwell Publishing Ltd, 1748-5827, <
http://dx.doi.org/10.1111/j.1748-5827.1972.tb06803.x>.
Kong, YX, Wright, G, Pesudovs, K, ODay, J, Wainer, Z & Weisinger, HS 2007, 'Horner syndrome', Clinical and Experimental Optometry, vol. 90, no. 5, pp. 336-44.
Mirzai, H & Baser, EF 2006, 'Congenital Horner's syndrome and the usefulness of the apraclonidine test in its diagnosis', Indian J Ophthalmol, vol. 54, no. 3, pp. 197-9.
Newman JN, Kedar S & Biousse V 2015, Horner Syndrome, UpToDate, retrieved 20 September 2015, http://www.uptodate.com/contents/horner-syndrome
Payne, J 2015, Horner's Syndrome, Patient, 20/09/2015, <http://patient.info/doctor/horners-syndrome>.
Reede, D, Garcon, E, Smoker, W & Kardon, R 2008, Horner's Syndrome: Clinical and Radiographic Evaluation, Neuroimaging Clinic of North America <
https://www.clinicalkey.com.au/#!/content/journal/1-s2.0-S1052514907001219?scrollTo=%231-s2.0-S1052514907001219-gr1>.
The Reed Group 2014, Post Traumatic Syringomyelia, diagram, retrieved 21 September 2015, http://www.adlergiersch.com/post-traumatic-syringomyelia/
Spring, N 2011, Horners Syndrome, infant diagnosed with Horners Syndrome, Frontier Dreams, retrieved 23/09/2015, http://frontierdreams.blogspot.com.au/2011/04/horners-syndrome.html
Starr, BE, Shubert, RA & Baumann, B 2004, 'A child with isolated Horner's syndrome after blunt neck trauma', The Journal of Emergency Medicine, vol. 26, no. 4, pp. 425-7.
tcooper 2013, Neuro-ophthalmology, Stanford Medicine Ophthalmology, 20/09/2015, <
http://ophthalmology.stanford.edu/blog/archives/2013/09/neuro-ophthalmology-question-of-the-week-anisocoria-6-horner-syndrome-cocaine-test-continued.html>.
WebMD 2015, Paremyd Ophthalmic, 21/09/2015, <http://www.webmd.com/drugs/2/drug-64194/paremyd-ophthalmic/details#images>.
34
Trimester 2,
2015
Horners Syndrome
Group 1D

Horner&#39;s Syndrome

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Horners

(Kneller, Lewis & Oliver 1972)

(Reede et al. 2008)

Rare interruption of ocular sympathetic nerve innervation,

First official descriptions date back to 1869

Group of symptoms including miosis, ptosis and facial

Preceding primary condition can be benign or life

Variety of diagnoses used to find specific source of nerve

HMO102 Science of Vision

(Reede et al. 2008)

(Kneller, Lewis & Oliver 1972)

(Reede et al. 2008)

The History Significant Findings

HMO102 Science of Vision

The History Johann Friedrich Horner

An ophthalmologist, pictured in Figure 3, who studied

What did Horner discover?

HMO102 Science of Vision

(Kneller, Lewis & Oliver 1972)

(Reede et al. 2008)

The Anatomy Three-Order Neuron

Sympathetic innervation to the eye begins in the Central

First Order Neurons begin in posterolateral hypothalamus

Second-Order Neurons exit T1 spinal chord and ascend

Third-Order Neurons exit the superior cervical ganglion, and

HMO102 Science of Vision

(Reede et al. 2008)

The Anatomy Nerve Fibre

Nerve fibres forming plexus around internal carotid

Nerves associated with sweating follow external carotid artery

Nerve fibres at the cavernous sinus follow CNVI, join

HMO102 Science of Vision

(Reede et al. 2008)

The Anatomy Disruptions

Nerve disruption causing ptosis and inverse ptosis results from a

Nerve disruption causing pupil dilation results from a lack of force

Nerve disruption causing dilation lag results from passive

Nerve disruption causing anihidrosis results from either First and

HMO102 Science of Vision

(Reede et al. 2008)

(Kneller, Lewis & Oliver 1972)

(Reede et al. 2008)

Caused by variety of conditions being mild and benign to severe

Most often caused by trauma during medical procedures or