Horner’s syndrome (also called oculosympathetic paresis, or Horner syndrome) comprises of the classic triad of ptosis, miosis and anhidrosis.
It results from a lesion to the sympathetic pathway that supply the head and neck region.
The causes of Horner’s syndrome varies with the age of the patient and site of the lesion.
Prompt evaluation is necessary to detect and treat life-threatening conditions.
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Horner's syndrome
1. H O R N E R ’ S
S Y N D R O M E
D R R A J A M A R H A M A H ,
O P H T H A L M O L O G Y
D E P A R T R M E N T O F H R P 2 I I K O T A
B H A R U K E L A N T A N M A L A Y S I A
2. • Horner’s syndrome (also called oculosympathetic paresis, or Horner syndrome)
comprises of the classic triad of ptosis, miosis and anhidrosis.
• It results from a lesion to the sympathetic pathway that supply the head and
neck region.
• The causes of Horner’s syndrome varies with the age of the patient and site of
the lesion.
• Prompt evaluation is necessary to detect and treat life-threatening conditions.
3. P A T H O P H Y S I O L O G Y
• Horner's syndrome results from a lesion to the
sympathetic pathways that supply the head and neck,
including the oculosympathetic fibers.
• Disruption of the sympathetic pathway that supplies the
head, eye, and neck
4. R E L E V A N T A N A T O M Y
• The pupil is innervated by sympathetic and
parasympathetic fibers.
• Pupillary dilation is mediated by a three-
neuron sympathetic pathway that originates in
the hypothalamus.
• FIRST ORDER NEURON (FON)- central sympathetic
fibers
• SECOND ORDER NEURON (SON)- preganglionic
pupillomotor fibers
• THIRD ORDER NEURON (TON)- post ganglionic
pupillomotor fibbers
sympathetic Pathway for pupillary Innervation
5. F I R S T O R D E R ( C E N T R A L ) N E U R O N
• The first order (central) neuron descends
caudally from the hypothalamus to the first
synapse in the cervical spinal cord (C8-T2
level-also called the ciliospinal center of
Budge).
• The descending sympathetic tract is in close
proximity to other tracts and nuclei in the
brainstem.
6. F I R S T O R D E R
N E U R O N
Sympathetic Pathway for pupillary
Innervation
ciliospinal centre of budge
7. S E C O N D O R D E R
( P R E G A N G L I O N I C )
N E U R O N
• The second order (preganglionic) neuron exits
the spinal cord and travels in the cervical
sympathetic chain through the brachial plexus,
over the pulmonary apex and synapses in the
superior cervical ganglion.
• The superior cervical ganglion is located near the
angle of the mandible and bifurcation of the
common carotid artery.
9. T H I R D O R D E R ( P O S T G A N G L I O N I C )
N E U R O N
• The third order
(postganglionic) neuron for the
orbit enters the cranium within
the adventitia of the internal
carotid artery into the
cavernous sinus.
• It exit the internal carotid
artery in close proximity to the
trigeminal ganglion and the
6th CN and
• join the 1st division of the
trigeminal nerve (5th CN) to
enter the orbit.
optic chiasmia Internal carotid artery
sphenoidal air sinus
Temporal
lobe of
cerebrum
10. • The vasomotor and sudomotor
fibers to the face exit the superior
cervical ganglion and ascend in
the external carotid artery.
11. • The fibers (long ciliary nerve) innervate
the dilator muscles of the iris and the
smooth muscle (Müller’s muscle) in the
upper and lower eyelid (inferior
retractors).
• Pupillary constriction is produced by
parasympathetic (cholinergic) fibers that
travel with the 3rd CN (oculomotor) .
Contd.....THIRD ORDER NEURON
12. E T I O L O G Y
• Varies with the patient age and site of lesion.
• Remains unknown in 35-40% of cases.
13. E P I D E M I O L O G Y I N A D U L T S
First-order lesions
1)Brainstem disease.
-commonly stroke: eg. Lateral medullary
infarction (Wallenburg syndrome).
SYMPTOMS:
• Cerebellar ataxia
• Vertigo
• Dysphagia
• Dysarthria
• Loss of pain and temperature over one side of
face
-Tumor or demyelinating
15. S E C O N D - O R D E R L E S I O N S
• Trauma/surgery of spinal cord or brachial
plexus
• Apical lung tumors (Pancoast tumor)
• Lumbar epidural anesthesia
• Chiropractic manipulation
16. T H I R D O R D E R N E U R O N
• 1) Internal Carotid Artery Dissection
• 2) Nasopharyngeal tumor
• 3) Cavernous sinus mass-Tumor
extension, including nasopharyngeal
carcinomas
• 4) Otitis media
17. I N T E R N A L C A R O T I D A R T E R Y
D I S S E C T I O N ( I C A D )
• Average age a/w Horners Syndrome 40–50 years
old
• 65% males
• Traumatic or spontaneous
• Due to a disruption of layers of the artery
causing blood to collect between the layers
• HS is seen in nearly 40% of (ICAD)
18. I C A D : O T H E R O P H T H A L M O L O G I C
M A N I F E S T A T I O N S
• 52% of patients with ICAD
presented with ophthalmologic
symptoms or signs.
• Monocular visual loss 28%
• 76% of those were painful.
• Scintillations
• Ischemic optic neuropathy
• Diplopia
Scintillations
19. 5)Pituitary lesions
6) Herpes zoster - infections at the level of
cervical and thoracic
7)Tolosa-Hunt syndrome
8)Cluster headaches (HS lasts less than 1–2
hours)
contd... CAUSE OF THIRD ORDER NEURON
20. 9) Giant cell arteritis- inflammatory lesions of the
internal carotid artery can damage the
sympathetic nerve fibres
10) Tonscillectomy or radiofrequency tonsil
ablation -as the ganglion lies about 1.5 cm
behind the palatine tonsil
11)Eagle syndrome (pressure from an elongated
styloid process on the carotid artery)
12)Idiopathic
contd... CAUSE OF THIRD ORDER NEURON
21. E P I D E M I O L O G Y I N C H I L D R E N
ACQUIRED CONGENITAL
Neuroblastoma
-In children presenting with HS, 33% had a
tumor and 22% had a neuroblastoma
- 2% of patients with neuroblastoma
Birth trauma-brachial plexus injury
Head, neck, and chest surgery Vascular anomaly
Infection Neoplasm
- neuroblastoma and neurofibroma
Idiopathic: 67% OF CASES
22. • Presence of pain (neck pain, facial pain, headache)-suggestive for a
carotid artery dissection (75% of the cases)
• History of trauma- to exclude concurrent traumatic mydriasis and
traumatic ptosis
• Review of old patient photographs- new anisochoria can be seen when
compared with childhood photo
• Brainstem signs- suggestive of first order lesion (Diplopia, vertigo, ataxia,
lateralized weakness or sensory loss)
• Bilateral muscle or sensory weakness, bowel/bladder impairment-
Suggestive cervicothoracic cord injury (first-order lesion)
23. • Arm pain/weakness
Brachial plexus injury (second-order lesion)
Particularly important in the assessment of
pediatric patients- Discuss birth history,
complications, forceps use
• Chiropractic manipulation
Several reports of subsequent carotid dissection
leading to HS
An especially important consideration for
patients less than 45 years old (given the
increased risk of ICAD)
24. • Diplopia- if worse in lateral
gaze, suggestive of CN VI palsy
6th CN palsy due to cavernous
sinus lesion (third-order lesion)
6th CN is most commonly
affected
Involvement of other CN (in
cavernous sinus):
• 3rd CN
• 4th CN
• opthalmic V1 and maxillary
division V2 of trigeminal nerve
25. C L I N I C A L
F E A T U R E S
• Classic triad of ptosis, miosis and facial anhidrosis
• Mild upper-lid ptosis (1–3 mm)- Ptosis can be subtle and reportedly
absent in 12% of HS patients
• Lower-eyelid elevation ("reverse ptosis")- can be subtle or absent
• Pupillary miosis
- anisochoria is greater in the dark
- dilatation lag: slow to dilate when removal of light
26. • Ipsilateral facial anhidrosis
Only present in first- and second-order lesions, as facial sweat-gland
fibers branch off at the superior cervical ganglion
Variable presentation: Involvement ranges from entire half of face to
small patch on the forehead, depending on lesion location.
This can present as impaired facial flushing (Harlequin sign) in children.
• How to test:
• 1. Sweat Friction test
• 2. Starch -iodine test
C L I N I C A L F E A T U R E S
28. A N H I D R O S I S T E S T
Sweat friction test
• A standard prism bar and the forehead are cleaned with an alcohol
pad and allowed to dry.
• The bar is then placed flat against 1 side of the forehead and drawn
down with mild pressure.
• The same is done on the other side.
• The side with anhidrosis will provide significantly less resistance
compared to the normal side.
Starch-iodine test
• Iodine is applied to an area and allowed to dry.
• A thin layer of starch is then applied.
• Areas of anhidrosis will appear a dark bluish color.
29. • Iris heterochromia
Secondary to a congenital Horner syndrome, where ipsilateral iris is lighter
Occurs in approximately 12% of pediatric HS (Mahoney, Arch Ophthalmol 2011)
Other signs: ipsilateral conjunctival injection, nasal stuffiness, increased near point of
accommodation
C L I N I C A L
F E A T U R E S
30. T E S T S T O E S T A B L I S H D I A G N O S I S
• 1. COCAINE TEST
• Methods: 2 drops of cocaine 4% (paeds) or 10%
(adults) should be placed and evaluate for anisocoria
in 45 minutes.
• The pupils are tested in dim lighting
• Effects: dilates unaffected eye, anisocoria
• Explaination: Cocaine blocks reuptake of
catecholamines (norepinephrine) secreted at
POSTGANGLION nerve endings. Accumulation of NE
cause dilatation of pupil
• Norepinephrine is not released in impaired
sympathetic innervation, therefore cocaine has no
effect on HS eyes.
32. T E S T S T O E S T A B L I S H D I A G N O S I S
2. Apraclonidine Test
Method: 1 drop of Apraclonidine (0.5% or 1%) instilled
into BE, pupil in checked in 30 minutes and 45 minutes
Result: Horner pupil will dilate and improves ptosis, but
normal pupil will not affected.- reverses anisocoria.
-90% sensitive and 100% Specific
Explanation: Apraclonidine is alpha-2 agonist.
Due to denervation hypersensitivity in HS, the
upregulated alpha-1 receptors result in pupil dilation,
while weakly dilate normal pupil.
May takes 36 hours to 14 days to become positive.
34. T E S T T O L O C A T E P R E O R P O S T
G A N G L I O N L E S I O N
1. Phenylephrine 1%
• Effects:
• Causes dilation of TON,
• No dilation of a FON and only minimal
dilation of a SON or normal pupil
• Explanation: based on supersensitivity of
alpha receptors from denervation
• Postganglionic (TON)- dysfunction of
local motor nerve causing denervation.
• Hypersensitivity to adrenergic
neurotransmitter causing pupil
dilatation
35. 2. Hydroxyamphetamine 1%
• Methods: 2 drops instilled into BE
• more sensitive than phenylepinephrine
• Results: POSTGANGLIONIC Horner will NOT dilate.
• Preganglion horner or normal will dilate and
improves ptosis
• Explanation: hydroxyamphetamine potentiates the
release of NE from functioning postganglionic
nerve endings.
• Lesion in post ganglion (TON) will cause
dysfunctioning nerve, no release of NE.
T E S T T O L O C AT E P R E O R P O S T
G A N G L I O N L E S I O N
36. I N V E S T I G A T I O N S
• Seek second opinion from neuroophthal or
neuromedical for assessment.
• An acute presentation is an Emergency
• CT or MRA examining the region from aortic
arch to the circle of Willis- (including carotid,
apical of lungs, thyroid and skull base lesions.
• MRI may be used to exclude brainstem stroke.
• Plain xray and Carotid ultrasound -limited utility
• If symptoms more than 1 year and no localising
sign- not for urgent radiology investigation.
37. D I F F E R E N T I A L D I A G N O S I S
• Other causes of ptosis
• Pharmacologic pupil
• CN III palsy
• Contralateral iris sphincter tear (Traumatic mydriasis)
• Physiologic anisocoria
38. T R E AT M E N T
• Management based on underlying cause
39. R E F E R E N C E S
• https://www.aao.org/oculoplastics-center/horner-syndrome-8
• kanski’s clinical ophthalmology A systemic Approach 8th edition