Horner syndrome
Kelly A. Walton and Lawrence M. Buono
Horner syndrome refers to the constellation of signs resulting
from the interruption of sympathetic innervation to the eye and
ocular adnexae. Classically, the clinical findings include a triad
of ipsilateral blepharoptosis, pupillary miosis, and facial
anhidrosis. The history, additional clinical examination features,
and pharmacologic testing may help localize the lesion and
suggest an etiology. An appropriate evaluation of Horner
syndrome and a timely elucidation of the etiology may allow for
a potentially life-saving intervention.
Keywords
Horner syndrome, oculosympathoparesis, pupillary
abnormalities, carotid artery dissection
Curr Opin Ophthalmol 14:357–363. ©2003 Lippincott Williams & Wilkins.
Neuro-Ophthalmology Service, Duke University Eye Center, Durham, North
Carolina, USA
Correspondence to Lawrence M. Buono, MD, Duke University Eye Center, Erwin
Road, Box 3802, Durham, NC 27710, USA
Tel: 919-684-3771; fax: 919-684-8509
Current Opinion in Ophthalmology 2003, 14:357–363
© 2003 Lippincott Williams & Wilkins
1040-8738
Introduction
Horner syndrome, or oculosympathoparesis, first de-
scribed by Johann Friedrich Horner in 1929, classically
presents with ipsilateral blepharoptosis, pupillary miosis,
and facial anhidrosis [1]. The syndrome results from in-
terruption of the sympathetic innervation to the eye and
ocular adnexae. The pathologic processes responsible for
the sympathetic denervation range from simple mi-
graines to life-threatening conditions such as carotid dis-
section and malignancy [2••]. Timely diagnosis and ap-
propriate evaluation may lead to life-saving intervention.
In this review we discuss the clinical features, pharma-
cologic diagnosis, and the various pathologic causes of
Horner syndrome with particular attention to the more
recent advances in and challenges of the disease.
Anatomy
The anatomic substrate for sympathetic innervation to
the eye and ocular adnexae follows a long and circuitous
route. The three-neuron pathway begins in the central
nervous system and concludes at the eye. The first-order
neurons originate from the posterolateral hypothalamus.
There are probably some synapses in the brainstem at
the level of the pons, but most of the fibers descend to
the synapse in the ciliospinal center of Budge–Waller,
located in the intermediolateral columns of the spinal
cord at the level of C8 to T2 [3].
Most of the second-order neuron fibers exit the spinal
cord at the level of T1 and ascend the sympathetic chain.
The second-order neurons synapse in the superior cer-
vical ganglion, located between the internal jugular vein
and the internal carotid artery. The third-order neurons
exit the superior cervical ganglion and form a plexus,
which surrounds the internal carotid artery. The fibers
responsible for sweat and piloerection of the face follow
the external carotid artery. The remainder of the sym-
pathetic plexus and the internal carotid artery enter the
skull base through the carotid canal and travel through
the middle cranial fossa into the cavernous sinus. The
sympathetic fibers briefly travel with the intracavernous
sixth cranial nerve before joining the ophthalmic division
of the trigeminal nerve on its course into the orbit. The
sympathetic fibers follow the course of the nasociliary
nerve through the superior orbital fissure. The fibers
pass through but do not synapse in the ciliary ganglion
[3]. Distal to the ciliary ganglion, the sympathetic fibers
divide and innervate various anatomic structures: the
middle ear, the orbital vasomotor fibers, the dilator
muscle of the pupil, the accessory levator muscle of the
357
358 Neuro-ophthalmology

upper eyelid (Müller’s muscle) and its analog in the Figure 2. Topical cocaine testing
lower eyelid, and the lacrimal gland [4].

Clinical features
Interruption of the sympathetic innervation to the eye
and the ocular adnexae causes ipsilateral blepharoptosis,
pupillary miosis, and facial anhidrosis. Müller’s muscle
receives sympathetic innervation and acts as an accessory
elevator of the upper eyelid. Because Müller’s muscle is
responsible for only approximately 2 mm of upper eyelid
elevation, loss of sympathetic input results in subtle pto-
sis. Paresis of Müller’s muscle may also lead to the loss of
the upper lid crease [4]. The analogous, unnamed
smooth muscle of the inferior eyelid that is responsible
for lower eyelid retraction also receives sympathetic in-
nervation. In addition to an often subtle upper eyelid
ptosis with sympathetic denervation, lower eyelid retrac-
tion may occur, termed “inverse ptosis.” In combination,
this can result in narrowing of the interpalpebral fissure,
producing an enophthalmos that is more often apparent
than real [5].
(A) Horner syndrome (oculosympathoparesis) on the right side before instillation
The size of the pupil results from the balance of dilation of cocaine. Note the subtle anisocoria. (B) After instillation of two drops of
topical cocaine 10%. Note the increase in the amount of the anisocoria,
and constriction forces from the sympathetically inner- confirming the presence of oculosympathoparesis.
vated iris dilator muscle and the parasympathetically in-
nervated iris constrictor muscle. With sympathetic de-
nervation, the force of the iris constrictor muscle is
vated pupil will dilate more slowly compared with the
unopposed, resulting in pupillary miosis and subtle an-
normal contralateral pupil when the lights are turned off.
isocoria of approximately 1 to 1.5 mm. The amount of
This phenomenon is known as dilation lag. This occurs
anisocoria is greatest in dim illumination and is least in
because the process of pupillary dilation with sympa-
bright illumination (Fig. 1). The sympathetically dener-
thetic denervation is primarily passive, resulting from
relaxation of the iris sphincter alone. This feature can be
Figure 1. Horner syndrome (oculosympathoparesis) on the
right side
Figure 3. T1-weighted MR image with gadolinium
enhancement showing dissection of the right internal carotid
artery (arrow) approaching the skull base

(A) Note the subtle blepharoptosis and anisocoria in dim illumination.

Additionally, the right lower eyelid margin is higher compared with the left lower
eyelid margin, demonstrating “inverse ptosis.” (B) In bright illumination, the
amount of anisocoria decreases.

demonstrated clinically and is strongly indicative of ocu-
losympathoparesis as the cause of the anisocoria.
Facial anhidrosis results from disruption of the sympa-
thetic fibers traveling with the external carotid artery to
innervate the sweat glands of the face. The fibers trav-
eling to the medial forehead branch off with the third-
order sympathetic fibers, and the fibers supplying the
rest of the face branch off more proximally. For this
reason, central and preganglionic lesions should cause
anhidrosis of the entire side of the face whereas anhidro-
sis limited to the medial forehead suggests a third-order
etiology. Anhidrosis can be assessed clinically through a
variety of methods; however, testing is often awkward to
perform and is often inaccurate as a localizing sign [6].
Iris heterochromia occasionally accompanies Horner syn-
drome, particularly in congenital lesions. In brown-eyed
patients the lighter iris corresponds to the abnormal pu-
pil, and in blue-eyed patients the darker iris is usually
found on the affected side. Rarely, iris heterochromia can
be found in adults with acquired disease [7] and may be
delayed several years after injury to the sympathetic
chain in children [8]. The etiology of iris heterochromia
in Horner syndrome has not been firmly established;
however, some investigators have suggested that post-
ganglionic lesions in the sympathetic chain may disrupt
the neurotropic development of iris melanocytes [9].
The acute phase of Horner syndrome may present some-
what differently than more prolonged disease. Conjunc-
tival hyperemia may be observed initially as a result of
the loss of the vasoconstricting affects of the sympathetic
innervation. Corneal endothelial failure has been re-
ported in association with acute Horner syndrome [10].
Facial anhidrosis may be more apparent during the acute
phase before the sweat glands begin to respond more
vigorously to catecholamines in systemic circulation [4].
Pharmacologic diagnosis and localization
Pharmacologic pupillary testing can be used to confirm a
diagnosis of Horner syndrome and to localize anatomi-
cally the lesion causing sympathetic denervation. Co-
caine testing can be used to confirm the presence of
Horner syndrome. Phenylephrine has also been used for
confirmation, although with limited reliability [11]. Once
the diagnosis is established, hydroxyamphetamine
(Paredrine) can be used to distinguish between central
and preganglionic lesions from postganglionic lesions.
The cocaine test
The normal pupillary response to topical cocaine is dila-
tion. Cocaine inhibits the reuptake of norepinephrine at
the synaptic junction of the postganglionic fibers and the
iris dilator muscle. If the sympathetic innervation has
been disrupted, norepinephrine is not released from the
Horner syndrome Walton and Buono 359
nerve terminal and topical cocaine does not produce pu-
pillary dilation. Failure of pupillary dilation after instil-
lation of topical cocaine confirms the presence of sym-
pathetic denervation; however, it does not determine the
location of the lesion.
The topical cocaine test is performed by measuring both
pupils in darkness before instillation of two drops of co-
caine 10% into both eyes. One hour later, the pupils are
again measured [Fig. 2]. Kardon et al. [12] demonstrated
that pupillary inequality of at least 0.8 mm after admin-
istration of cocaine 10% correlated with a mean odds
ratio of 1050:1 for having Horner syndrome. Because ac-
curate pupillary measurements of less than 1 mm are
difficult to achieve, some have adopted a 1 mm or more
difference in anisocoria as being positive [2••]. Beware
that cocaine metabolites may be present in the urine for
as long as to 2 days and patients should be warned that a
urine drug-screening test might be positive for cocaine
[13].
An interesting alternative to cocaine testing is the use of
apraclonidine, an ␣-adrenergic agonist. In a series of six
patients with Horner syndrome including both pregan-
glionic and postganglionic lesions, one drop of apracloni-
dine 1% resulted in mydriasis of 1.0 to 4.5 mm in the
affected pupil in all patients [14]. Unaffected eyes ex-
perienced 0 to 0.5 mm of mydriasis, effectively reversing
the anisocoria. The authors hypothesized that the my-
driasis was the result of denervation supersensitivity of
the pupillary dilator muscle ␣-1 receptors.
The hydroxyamphetamine test
After confirmation with the topical cocaine test, topical
hydroxyamphetamine (Paredrine) can be used to distin-
guish central and preganglionic sympathetic lesions from
postganglionic sympathetic lesions. Topical hydroxyam-
phetamine produces pupillary mydriasis by releasing
norepinephrine from the sympathetic synaptic terminal.
Pupillary mydriasis occurs only if the third-order neuron
is intact. Pupillary inequality of 1 mm or more after in-
stillation of hydroxyamphetamine suggests a third-order
lesion.
There are, however, significant drawbacks to the use of
hydroxyamphetamine. Because cocaine inhibits the up-
take of hydroxyamphetamine from the nerve terminal,
the two tests cannot be performed on the same day
[4,15]. The hydroxyamphetamine test may yield false-
negative results in acute Horner syndrome because nor-
epinephrine may not be completely depleted from the
terminal of the damaged postganglionic neuron
[16,17,18•]. A more practical drawback to the test is that
hydroxyamphetamine is no longer available commer-
cially [19]. Pholedrine, the n-methyl derivative of hy-
droxyamphetamine, is available commercially and has
been suggested as an alternative [20]. In 13 patients with
Horner syndrome, pholedrine correctly distinguished all
360 Neuro-ophthalmology

the patients with preganglionic lesions from those with
postganglionic lesions in the same manner as hydroxy-
amphetamine [20].
Differential diagnosis
The sympathetic pathway can be interrupted anywhere
along its course and, depending on the location of the
lesion, Horner syndrome can occur in isolation or in as-
sociation with other neurologic abnormalities. Historical
and clinical examination findings, along with pharmaco-
logic testing, may suggest the locus of the oculosympa-
thoparesis as central, preganglionic, or postganglionic.
Central Horner syndrome
Central Horner syndrome is relatively uncommon. In a
series of 450 patients with Horner syndrome that in-
cluded both inpatients and outpatients, 270 patients
(60%) had an identifiable cause [21]. A central lesion was
found in 34 patients (13%), preganglionic lesion in 120
patients (44%), and postganglionic lesion in 116 patients
(43%). Because of the proximity of the corticospinal
tracts and cranial nerves to the sympathetic chain in the
brainstem, central Horner syndrome (first-order neuron)
occurs uncommonly in isolation and is usually one of a
constellation of neurologic findings. The most common
presentation of a first-order neuron lesion is part of the
Wallenberg lateral medullary syndrome [22], resulting
from infarction of the territory supplied by the posterior
inferior cerebellar artery. Patients with Wallenberg syn-
drome exhibit dysphagia, ipsilateral facial analgesia, con-
tralateral analgesia of the trunk and extremities, cerebel-
lar ataxia, rotary nystagmus, and skew deviation, and as
many as three quarters of these patients also demonstrate
ipsilateral oculosympathoparesis [22,23].
Because the first-order neuron courses through the cer-
vical spinal cord, central Horner syndrome can result
from a cervical cord lesion. Such lesions include syringo-
myelia [24] and trauma [15]. Patients with Brown–
Sequard syndrome from any cause may have Horner syn-
drome on the same side as the loss of light touch and
motor function, and on the opposite side as the loss of
temperature and pain sensation [25].
Preganglionic Horner syndrome
Preganglionic Horner syndrome is most often caused by
trauma or tumor [21]. Direct spinal cord trauma or trac-
tion on the brachial plexus may distort the ventral roots
and cause interruption of the sympathetic innervation.
The trauma responsible for the injury is often iatrogenic.
Particularly in forceps delivery, brachial plexus injury
(Klumpke palsy) may be associated with dislocation of
the C8 and T1 dorsal and ventral nerve roots, and may
cause oculosympathoparesis in the newborn [9]. Other
iatrogenic causes of preganglionic Horner syndrome in-
clude lumbar epidural anesthesia [26], chest tube place-
ment [27], and coronary artery bypass surgery [28] among
others (Table 1).
Lung and mediastinal tumors can impinge on the sec-
ond-order sympathetic neurons. A tumor from the apex
of the lung may cause Horner syndrome that is accom-
panied by shoulder and arm pain, also known as Pancoast
syndrome [29]. Therefore, occult malignancy should be
considered in any patient with shoulder or arm pain and
a new Horner syndrome without a history of trauma.
Postganglionic Horner syndrome
Postganglionic Horner syndrome may result from a vari-
ety of causes ranging from benign to life-threatening

Table 1. Topographic diagnosis of Horner syndrome

Central Preganglionic Postganglionic
Hypothalamus/thalamus/brainstem Cervicothoracic spinal cord Superior cervical ganglion
Ischemia (infarction) Trauma Forceps trauma
Wallenberg syndrome Tumor Ganglionectomy
Tumor Syrinx Cervical adenopathy
Hemorrhage AVM Jugular venous ectasia
Demyelination Cervical disc herniation Internal carotid artery
Cervical spinal cord Epidural spinal anesthesia Dissection
Tumor Brachial plexus Direct trauma
Trauma Forceps trauma Surgical trauma (carotid endartectomy)
Syrinx Pleural apex Thrombosis
AVM Apical lung tumor (Pancoast) Tumor (nasopharyngeal carcinoma at
Surgical trauma (chest tube placement, skull base)
cardiothoracic surgery) Cavernous sinus
Anterior neck Tumor (pituitary lesion, meningioma)
Trauma Carotid cavernous fistula
Surgical trauma (radical neck dissection, internal Carotid aneurysm
jugular catheterization) Inflammation
Tumor (thyroid) Infection
Thrombosis
Vascular headaches
Migraine headache
Cluster headache
AVM, arterovenous malformation.

(Table 1). Many processes that cause postganglionic
Horner syndrome are associated with ipsilateral facial
pain or headache. Cluster headaches are defined as at
least five episodes of one-sided headaches with ipsilat-
eral tearing, conjunctival injection, rhinorrhea, nasal con-
gestion, and postganglionic Horner syndrome [30]. The
etiology of cluster headache is not well understood, but
up to two thirds of patients with cluster headaches ex-
perience Horner syndrome at some point in the disease
[31]. Raeder paratrigeminal neuralgia, which may be a
variant of cluster headache, refers to the combination of
unilateral headache, facial pain in the ophthalmic divi-
sion of the trigeminal nerve distribution, and ipsilateral
oculosympathoparesis [32].
Carotid artery dissection may be the most important eti-
ology of postganglionic Horner syndrome to consider.
This occurs when blood from the lumen of the artery
dissects into the wall of the vessel and creates a false
passage, potentially compromising the true arterial lu-
men [33]. Carotid artery dissection may be the result of
minor trauma or may occur spontaneously in patients
with fibromuscular dysplasia, syphilis, Marfan’s, or
Ehlers Danlos [34,35]. In addition to oculosympathopa-
resis, manifestations of carotid artery dissection include
amaurosis fugax, ischemic optic neuropathy, ophthalmic
or retinal arterial occlusion, and cranial nerve palsies
[36,37]. However, oculosympathoparesis is the most
common neurologic finding associated with dissection
and it occurs in approximately half the cases [34,38].
In addition to dissection, carotid artery thrombosis can
also cause postganglionic Horner syndrome. Branches of
the external carotid artery provide the blood supply for
the superior cervical ganglion, and branches of the inter-
nal carotid artery supply the carotid plexus. If the throm-
bosis compromises cerebral blood flow, the oculosympa-
thoparesis may be accompanied by contralateral
hemiplegia [39].
Horner syndrome accompanied by oculomotor, trigemi-
nal, or abducens palsy suggests a lesion in the cavernous
sinus, superior orbital fissure, or orbital apex. Lesions of
the superior orbital fissure are more likely to spare V2
than cavernous sinus lesions. Orbital apex lesions may be
distinguished by visual loss secondary to compromise of
the optic nerve [15].
Congenital Horner syndrome
Congenital Horner syndrome is an unusual but impor-
tant presentation. It has been estimated that less than 5%
of cases of oculosympathoparesis can be classified as con-
genital [9]. As discussed previously, iris heterochromia
accompanies most, but not all, cases of congenital Horner
syndrome [40]. Asymmetric facial flushing, also known as
the “harlequin” sign, is the clinical equivalent of anhi-
Horner syndrome Walton and Buono 361
drosis seen in adults, and may be easier to quantitate
than anhidrosis in a child [6].
Although delivery-related brachial plexus injury is one of
the most common causes of congenital Horner syn-
drome, a lesion anywhere along the sympathetic chain
may result in oculosympathoparesis [15]. Agenesis of the
internal carotid artery [41], vascular occlusion [42], basal
skull fracture through the carotid canal [9], and pneumo-
thorax [43] have all been reported to cause congenital
Horner syndrome. An important cause to consider is pri-
mary thoracic neuroblastoma. In a series of 405 patients
with neuroblastoma, 14 patients (3.5%) demonstrated
Horner syndrome [44]. Timely diagnosis is particularly
important because the cure rate for primary mediastinal
neuroblastoma approaches 100% if treated during the
first year of life [45].
Bilateral Horner syndrome
Patients with diffuse autonomic neuropathy or poten-
tially bilateral defects in the sympathetic chain provide a
diagnostic dilemma. Anisocoria may not be present to
help guide the evaluation. The presence of “redilation
lag” can be used to help diagnose bilateral Horner syn-
drome. In a study of 65 healthy subjects, 47 with unilat-
eral and 20 with bilateral Horner syndrome, infrared TV
pupillometry was used to measure baseline pupillary di-
ameters in the dark, then the eyes were exposed to
bright light. The time to reach three quarters of the
original pupillary diameter after removal of the light was
abnormally prolonged (redilation lag) in the eyes of pa-
tients with bilateral Horner syndrome [46].
Neuroimaging
History, clinical examination, and pharmacologic testing
can help direct appropriate imaging. However, because
localization through pharmacologic testing can be incor-
rect and misleading, imaging of the entire sympathetic
pathway is warranted if the accuracy of localization can-
not be ensured.
MRI of the brain should reveal stroke, tumor, or demy-
elination causing central Horner syndrome. CT of the
chest will reveal most tumors of the lungs causing a pre-
ganglionic Horner syndrome. For many years the gold
standard for evaluation of the carotid arteries has been
carotid angiography. Angiography, however, is not with-
out risk. The risk of minor stroke as a result of diagnostic
angiography ranges from 1.3 to 4.5%, and the risk of
major stroke ranges from 0.6 to 1.3% [47–49]. Newer
modalities including ultrasonography, MR angiography
[Fig. 3] and CT angiography provide for a safer alterna-
tive for carotid dissection evaluation.
Ultrasonography has the advantages of speed and non-
invasiveness, but is limited by technician reliability and
variability of the arterial anatomy. Often only the com-
362 Neuro-ophthalmology
mon and proximal carotid artery segments are detectable
by the acoustic window with ultrasound. However, in
one series of 43 patients who underwent carotid artery
dissection, a combination of extracranial, transcranial,
and duplex sonography correctly diagnosed the dissec-
tion in more than 95% of cases [50].
MR angiography, however, provides the capability of im-
aging the more distal internal carotid artery segment and
has largely supplanted other techniques in the diagnosis
of carotid artery dissection. In one series of 18 patients,
the sensitivity and specificity for diagnosis of carotid ar-
tery dissection were 95% and 99% respectively [51].
More recently, CT angiography has been used to diag-
nose carotid artery dissection. In a series of 16 patients
with angiographically confirmed carotid artery dissec-
tion, CT angiography provided the correct diagnosis in
all cases [52]. CT angiography has two major advantages
over MR angiography: general availability of the hard-
ware and decreased scan acquisition time. The disadvan-
tage of CT angiography is radiation exposure.
Treatment
In those cases in which an etiology of the oculosympa-
thoparesis can be found, the management of Horner syn-
drome is directed at treating the underlying disease. An-
isocoria is unlikely to be bothersome to patients, but
some may troubled by the blepharoptosis. Surgical cor-
rection with blepharoplasty is an option for these pa-
tients. Topical phenylephrine drops are an alternative to
surgery. Phenylephrine, an adrenergic agonist will act on
the sympathetically innervated Müller’s muscle of the
affected eye and will elevate the upper lid as much as 2
mm [8]. Iris heterochromia may be addressed with col-
ored contact lenses [8].
Conclusion
The signs of Horner syndrome may be subtle. A thor-
ough evaluation requires a focused history and physical
examination as well as a selection of pharmacologic test-
ing and suitable imaging studies. Important physical
signs include subtle ptosis, miosis, and anhidrosis. Pupil-
lary response to topical cocaine testing provides diagnos-
tic confirmation, and hydroxyamphetamine may help lo-
calize the lesion. In most situations, imaging of the entire
three-neuron sympathetic pathway is warranted. The ap-
propriate assessment of the disease provides the eye care
provider with the opportunity to make a potentially life-
saving diagnosis.
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