Horner's Syndrome

Horner’s SyndromeGroup 1D

David Ho, Emma Josey, Steven Lin, Nhi Nguyen, Melitta Tanzer, Jessie Whiley

(Reede et al. 2008) (George, Haydar & Adams 2008)

(Kneller, Lewis & Oliver 1972)

http://frontierdreams.blogspot.com.au/2011/04/horners-syndrome.html

2

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

◊ 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

Horner’s Syndrome Group 1D HMO102 Science of Vision

Figure 1 (Reede et al. 2008)

Horner’s Syndrome

The History




4

The History – Significant Findings

Trimester 2, 2015


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5

The History – Johann Friedrich Horner

Trimester 2, 2015


◊ 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

Figure 3 (Amonoo-Kuofi, HS 1999)

Horner’s Syndrome

The Anatomy




7

The Anatomy – Three-Order Neuron Trail

Trimester 2, 2015


◊ 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


8

The Anatomy – Nerve Fibre Organisation

Trimester 2, 2015


◊ 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 Figure 5 (Reede et al. 2008)

9

The Anatomy – Disruptions

Trimester 2, 2015


◊ 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

◊ Nerve disruption causing anihidrosis results from either First and Second Order neurons or Third Order neurons – depending on position of affected area


Horner’s Syndrome

The Etiology




11

The Etiology

Trimester 2, 2015


◊ Central◊ Horner’s Syndrome originating from here is uncommon◊ Usually due to cervical cord lesions from trauma or

syringomyelia (cyst causing disease) shown in Figure 6

◊ Preganglionic◊ Most often caused by trauma during medical procedures or

Pancoast tumours (on apex of lung)

◊ Postganglionic◊ Caused by variety of conditions being mild and benign to

severe and life threatening ◊ Can also be caused by cluster headaches or migraines

◊ Congenital◊ Rare but can be caused from multitude of medical conditions

or birth-related trauma

Figure 6 http://www.adlergiersch.com/post-traumatic-syringomyelia/

Horner’s Syndrome

The Pathophysiology




13

The Pathophysiology

Trimester 2, 2015


◊ 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 Horner’s Syndrome with pupil light reactions and accommodation remaining unaffected)

Figure 7 (Gralapp, C 2015)

14

The Pathophysiology – Common Symptoms

Trimester 2, 2015


◊ 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)

Figure 8 http://patient.info/doctor/horners-syndrome

15

The Pathophysiology – Individual Neuron Damage

Trimester 2, 2015


First

Order

Neurons

• Pathway: Originating from hypothalamus, through midbrain and extending into upper spinal chord

• Affected Area: Ipsilateral side of body• Symptoms: Anhidrosis, abnormal eye movements, ipsilateral limb ataxia and

dissociated sensory loss

Second Order

Neurons

• Pathway: Originating from upper spinal chord, through upper chest and extending into side of neck

• Affected Area: Ipsilateral face• Symptoms: Anhidrosis (shown in Figure 9), facial flushing

Third

Order

Neurons

• Pathway: Originates from side of neck and into face, innervating facial skin and muscles of iris and eyelids

• Affected Area: Area above ipsilateral brow or forehead• Symptoms: Minimal anihidrosis, orbital pain/headaches

16

The Pathophysiology - Anhidrosis

Trimester 2, 2015


Figure 9 https://classconnection.s3.amazonaws.com/448/flashcards/56448/png/capture1316217505465.png

17

The Pathophysiology - Congenital Horner’s Syndrome

Trimester 2, 2015


◊ 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 Horner’s Syndrome)

Figure 10 (Mirzai, H & Baser, EF 2006)

Horner’s Syndrome

The Epidemiologies




19

The Epidemiologies

Trimester 2, 2015


◊ 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 Figure 11 http://www.umweltbundesamt.de/en/topics/health/assessing-environmentally-

related-health-risks/epidemiology

Horner’s Syndrome

Diagnoses & Treatment




21

The Treatments – Pharmacological Tests

Trimester 2, 2015


◊ Apraclonidine Eye Drop Test

Effective at 0.5% concentration with good ocular penetration

Direct Alpha adrenergic receptor agonist (reacts strongly to alpha 2 and weaker to alpha 1 receptors)

87% accuracy

Currently accepted alternative to cocaine eye drop test

Normal Pupil

Dominant alpha 2 receptor activity

Miosis or no effect on

size

Horner’s Pupil Denervated

Increased alpha 1 receptor numbers

Mydriasis

22

The Diagnosis – Pharmacological Tests

Trimester 2, 2015


◊ 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 settingsHorner’s

PupilDenervate

d No effect on size

Figure 12https://lh4.googleusercontent.com/489j2ewJXWc_A2got6CJ0ix8o4G_T8tyjWm0PP-

r_NZhZok8miOURWZrt1pMRz1smEDMikdBi_rCiM3aSzZL2W61796Bw_5Ig2gWhOS5uwRMn0ypGeEj1BIl

23Trimester 2,

2015Horner’s Syndrome Group 1D HMO102 Science of Vision

Horner’s Pupil with damaged

postganglionic

neuron

Noradrenaline absent

No effect on size

◊ 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

Horner’s Pupil with

intact postgangli

onic neuron

Noradrenaline present Mydriasis

Figure 13 http://images.medscape.com/pi/features/drugdirectory/octupdate/AKN07040.jpg

The Diagnosis – Localisation

24

The Diagnosis – Neuroimaging

Trimester 2, 2015


◊ 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)

25

The Treatment Options

Trimester 2, 2015


◊ There is no effective treatment, as the underlying cause varies between cases and each cause required appropriate treatment

◊ Knowing cause of Horner’s Syndrome and eradicating underlying disease can improve the symptoms

◊ Horner’s Syndrome is diagnosed by an optometrist or doctor and patients are referred to appropriate professionals for further investigation

Horner’s Syndrome

Case Studies




27

Summary of Etiology

Trimester 2, 2015


Figure 15 (George, Haydar & Adams 2008)

28

Case Study #1

Trimester 2, 2015


◊ 52 year old man who developed Horner’s 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 man’s right eye with miosis and partial ptosis (A) and haematoma at the site of the carotid artery dissection

Figure 16 (Bazari, F, Hind, M & Ong, Y 2010)

29

Case Study #2

Trimester 2, 2015


◊ 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 Horner’s 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) Figure 18 (Dubois-Marshall, S & De Kock, S

2011)

30

Case Study #3

Trimester 2, 2015


◊ 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 Horner’s 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)

31

Case Study #4

Trimester 2, 2015


◊ 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 Horner’s Syndrome caused by blunt force trauma to sympathetic innervation of left eye

Figure 19 (Ganca, A et al. 2005)

32

Horner’s Syndrome – A summary

Trimester 2, 2015


◊ 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 Horner’s Syndrome

Horner’s SyndromeGroup 1D

David Ho, Emma Josey, Steven Lin, Nhi Nguyen, Melitta Tanzer, Jessie Whiley

Thank you




34

References

Trimester 2, 2015


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, ‘Horner’s 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, O’Day, 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, Horner’s Syndrome, infant diagnosed with Horner’s 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>.

http://www.umweltbundesamt.de/en/topics/health/assessing-environmentally-related-health-risks/epidemiology

http://fidoseofreality.com/health-condition-threatens-dog-vision/

http://www.bioone.org/doi/full/10.1647/2004-017

https://www.clinicalkey.com.au/

http://www.aao.org/image/sympathetic-pathway-3

https://www.studyblue.com/notes/note/n/exam-2/deck/1122222

http://dx.doi.org/10.1111/j.1748-5827.1972.tb06803.x%3E

http://www.uptodate.com/contents/horner-syndrome

http://patient.info/doctor/horners-syndrome

https://www.clinicalkey.com.au/

http://www.adlergiersch.com/post-traumatic-syringomyelia/




http://ophthalmology.stanford.edu/blog/archives/2013/09/neuro-ophthalmology-question-of-the-week-anisocoria-6-horner-syndrome-cocaine-test-continued.html

http://www.webmd.com/drugs/2/drug-64194/paremyd-ophthalmic/details#images

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Horner's Syndrome