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Sindrom Horner adalah gangguan dari jalur saraf okulosimpatetik yang bersumber di tempat
antara hipotalamus dan mata, biasanya menyebabkan ptosis, miosis pupil dan anhidrosis wajah,
serta enophthalmos, peningkatan amplitudo akomodasi, heterochromia, retraksi kelopak mata
kontralateral paradoks, penurunan transien tekanan intraokular, dan perubahan viskositas air
mata.
Horner Syndrome
Author: Christopher M Bardorf, MD, MS; Chief Editor: Hampton Roy, Sr, MD
Horner syndrome (Horners syndrome) results from an interruption of the sympathetic nerve
supply to the eye and is characterized by the classic triad of miosis (ie, constricted pupil), partial
ptosis, and loss of hemifacial sweating (ie, anhidrosis). The term Horner syndrome is commonly
used in English-speaking countries, whereas the term Bernard-Horner syndrome is common in
France. Von Passow syndrome is an association of Horner syndrome with iris heterochromia
(heterochromia iridis).[1]
Brainstem stroke or tumor or syrinx of the preganglionic neuron In one study, 33% of
patients with brainstem lesions demonstrated Horner syndrome [2]
Dissecting carotid aneurysm In one study, 44% (65/146) of patients with internal
extracranial carotid artery dissections had painful Horner syndrome, which remained
isolated in half the cases (32/65) [3]
Migraine
Horner syndrome is uncommon. No age, sexual, or racial predilections are known to exist. The
prognosis and the complications to be expected depend on the underlying cause of the syndrome,
as does treatment.
Pathophysiology
Horner syndrome may develop from lesions at any point along the sympathetic pathway.[4]
Abnormalities found in all patients, regardless of the level of interruption, include the following:
Slight elevation of the lower lid (upside-down ptosis), owing to denervation of the lower
lid muscle (analogous to the denervation of the Mller muscle in the upper lid)
Miosis and dilation lag, where pupillary dilation after psychosensory stimuli is slower in
the affected pupil than in the unaffected pupil
Depending on the level of the lesion, impaired flushing and sweating may be found ipsilaterally.
With central first-order neuron lesions, anhidrosis affects the ipsilateral side of the body. Lesions
affecting second-order neurons may cause anhidrosis of the ipsilateral face. With postganglionic
lesions occurring after vasomotor and sudomotor fibers have branched off the sympathetic chain,
anhidrosis is either absent or limited to an area above the ipsilateral brow. The pupils react
normally to light and accommodation.
Iris heterochromia (with the affected eye being hypopigmented) is seen in congenital Horner
syndrome or Horner syndrome that occurs in children younger than 2 years. Iris heterochromia
also may occur in long-standing Horner syndrome.
Etiology
Horner syndrome can be congenital, acquired, or purely hereditary (autosomal dominant). The
interruption of the sympathetic fibers may occur centrally (ie, between the hypothalamus and the
fibers point of exit from the spinal cord [C8 to T2]) or peripherally (ie, in cervical sympathetic
chain, at the superior cervical ganglion, or along the carotid artery).[5]
The common lesions that cause Horner syndrome interfere with preganglionic fibers as they
course through the upper thorax. Virtually all lesions producing postganglionic sympathetic
dysfunction are located intracranially or intraorbitally because the superior cervical ganglion is
near the skull. Preganglionic Horner syndrome indicates a serious underlying pathology and is
associated with a high incidence of malignancy. Postganglionic involvement has primarily
benign causes (ie, usually a vascular headache).
Arnold-Chiari malformation
Intrapontine hemorrhage
Neck trauma (eg, traumatic dislocation of cervical vertebrae or traumatic dissection of the
vertebral artery) - Horner syndrome occurring in association with spinal cord trauma
suggests a high cervical cord lesion because it does not occur with lesions below T2 or
T3
Pituitary tumor
Syringomyelia
Second-order neuron lesions that may give rise to Horner syndrome include the following:
Pancoast tumor (tumor in the apex of the lung, most commonly squamous cell
carcinoma)
Cervical rib
Trauma or surgical injury (eg, due to radical neck dissection, thyroidectomy, [7] carotid
angiography, radiofrequency tonsil ablation, [8] chiropractic manipulation, [9] or coronary
artery bypass grafting)
Chest tubes
Neuroblastoma [10]
Third-order neuron lesions that may give rise to Horner syndrome include the following:
Internal carotid artery dissection (associated with sudden ipsilateral face or neck pain) [11]
Herpes zoster
Drugs that may cause symptoms similar to Horner syndrome include the following:
Acetophenazine
Alseroxylon
Bupivacaine
Butaperazine
Carphenazine
Chloroprocaine
Chlorpromazine
Deserpidine
Diacetylmorphine
Diethazine
Ethopropazine
Etidocaine
Fluphenazine
Guanethidine
Levodopa
Lidocaine
Mepivacaine
Mesoridazine
Methdilazine
Methotrimeprazine
Oral contraceptives
Perazine
Prilocaine
Procaine
Prochlorperazine
Promazine
Promethazine
Propoxycaine
Reserpine
Thioproperazine
Thioridazine
Trifluoperazine
Clinical Presentation
Patient history
Obtaining a careful history is very helpful in the localization of lesions causing Horner
syndrome. The symptoms reported by the patient will depend on the underlying cause, as
follows:
Third-order neuron lesions Diplopia from sixth nerve palsy, numbness in the
distribution of the first or second division of the trigeminal nerve (cranial
nerve [CN] V), and pain
Although Horner syndrome is commonly an incidental finding related to a benign cause, it
occasionally may be a manifestation of a serious and life-threatening disorder. Careful direction
of the history to rule out such life-threatening disorders is vital.
Patients may not be able to open the affected eye completely and may not sweat on that side of
the face. The presence, absence, or location of anhidrosis is an important localizing sign.
Patients with preganglionic lesions may have facial flushing. This symptom (ie, harlequin effect)
occurs with physical exercise in some patients. Patients with postganglionic lesions may have
ipsilateral orbital pain or a migrainelike headache.
Raeder described a combination of orbital pain, miosis, and ptosis and termed it paratrigeminal
syndrome.[18] If this set of symptoms is associated with lesions of CNs III-VI on the ipsilateral
side, a mass lesion in the middle cranial fossa (ie, type I Raeder paratrigeminal syndrome) should
be suspected. A benign form characterized by episodic retrobulbar or orbital pain, with miosis
and ptosis but without associated cranial nerve findings, is considered a migraine variant (ie, type
II Raeder paratrigeminal syndrome).
Patients with carotid artery dissection may present with ipsilateral head, neck, or facial pain.
Physical examination
Important aspects of the physical examination include the following:
Measurement of pupillary diameter in dim and bright light and the reactivity
of the pupils to light and accommodation
Examination of the lower lids for upside-down ptosis (eg, the position of the
lower lid with respect to the inferior limbus)
Observation of extraocular movements
The pupil on the affected side may be round and constricted (ie, miosis). Patients may have a loss
of the ciliospinal reflex (ie, afferent C2, C3). The pupil fails to dilate when the skin on back of
the neck is pinched. (Most authors, however, consider this finding unreliable.) Patients
anisocoria is greater in darkness. The affected pupil dilates more slowly than the normal pupil
does because the affected pupil lacks the pull of the dilator muscle (ie, dilation lag).
Patients have dry skin (ie, anhidrosis) on the same side of their face as the affected pupil. The
pattern of a patients inability to sweat may be helpful in localizing the lesion. If a patient has a
lesion in the area of the common carotid artery, loss of sweating involves the entire side of the
face. With lesions distal to the carotid bifurcation, the lack of sweating is confined to the medial
aspect of the forehead and the side of the nose.
Partial ptosis
Iris heterochromia (heterochromia iridis) The affected iris may remain blue
when the other iris changes to brown; this may be present if the lesion is in a
child younger than 2 years but is uncommon in older patients; iris
pigmentation is under sympathetic control during development, which is
completed by the age of 2 years
Differential Diagnosis
Horner syndrome in the presence of pain merits special consideration.
Horner syndrome in the presence of axial, shoulder, scapula, arm, or hand pain may be indicative
of compression by an apical lung tumor (Pancoast tumor).
Horner syndrome in the presence of acute-onset, ipsilateral facial or neck pain may indicate
carotid artery dissection, which may be caused by cardiovascular disease, arteriopathy (eg,
fibromuscular dysplasia or collagen disorders), or trauma (even minor trauma, such as results
from quick head turns). If carotid artery dissection is suspected, especially if there are signs or
symptoms of retinal ischemia, urgent neuroimaging studies (magnetic resonance imaging or
magnetic resonance angiography) should be obtained along with neurologic consultation.
Postganglionic Horner syndrome associated with ipsilateral headache has several causes. Patients
with spontaneous carotid artery dissection may present with Horner syndrome and ipsilateral
headache. Patients with cluster headaches may develop ipsilateral Horner syndrome during an
acute attack.
The term Raeder paratrigeminal syndrome is applied to patients, usually middle-aged males, who
have Horner syndrome and daily unilateral head pain. In the original Raeder syndrome, the pain
is trigeminal pain associated with hypoesthesia or anesthesia in the distribution of the trigeminal
nerve (cranial nerve [CN] V). Pain related to Raeder syndrome can be distinguished from that
related to cluster headaches or carotid disease in that the latter conditions occur without
impairment of trigeminal nerve function.
Adie pupil
Anisocoria
Senile miosis
Laboratory Studies
In general, laboratory studies do not play a significant role in the diagnosis and management of
Horner syndrome. However, depending on the localization and suspected etiology, certain
laboratory tests may be considered, in conjunction with appropriate medical consultation. These
include the following:
Urine test (ie, vanillylmandelic acid [VMA] and homovanillic acid [HVA]) to rule out
neuroblastoma
Imaging Studies
Imaging studies may be ordered in conjunction with appropriate medical or surgical consultation,
depending on the localization and suspected etiology.[19]
A chest radiograph should be obtained; apical bronchogenic carcinoma is the most common
cause of Horner syndrome. If stroke is suspected, computed tomography (CT) of the head should
be performed.
Painful Horner syndrome should alert the physician to the possibility of carotid artery dissection,
and the patient should undergo further testing (ie, magnetic resonance imaging [MRI]/magnetic
resonance angiography [MRA] of the brain and neck) to exclude this possibility. Internal carotid
artery dissection is life-threatening and carries a risk that the patient will experience a disabling
stroke.
Ultrasonography may be considered but has not been found to be reliable for diagnosing carotid
artery dissection in patients with isolated Horner syndrome.
Pharmacologic Testing
Pharmacologic testing is very helpful in the diagnosis of Horner syndrome.[21] The following
pharmacologic tests document the presence or absence of an ocular sympathetic lesion and
identify the level of involvement (ie, preganglionic or postganglionic). Localizing the lesion is
important because preganglionic lesions are associated with a higher incidence of malignancy
that necessitates extensive investigations.
The test is performed by instilling cocaine solution (2-4% or, according to some, 4-10%) into
each eye. Cocaine instilled in an eye with intact sympathetic innervation causes the pupil to
dilate. A sympathetically denervated pupil (such as is present in Horner syndrome) dilates poorly
to cocaine, regardless of the level of the sympathetic interruption, because of the absence of
endogenous norepinephrine in the synapse.
For optimal accuracy, test results should be evaluated 30 minutes or longer after cocaine is
administered. The maximal response is seen 40-60 minutes after instillation of the drops.
Postcocaine anisocoria greater than 0.8 mm is sufficient to diagnose Horner syndrome.
Apraclonidine is an ocular hypotensive agent that acts as a weak alpha1-agonist and a strong
alpha2-agonist.[25, 22, 21, 26] Typically given in a 0.5% or 1% solution, it has little to no effect on a
normal pupil but has a mydriatic effect on an abnormal pupil.
In acute cases, false-negative test results may occur because the alpha1-receptor upregulation on
which the effect of apraclonidine depends may take 5-8 days.[27, 28] Accordingly, a negative
apraclonidine test result, especially in acute settings, does not exclude Horner syndrome. In such
cases, a cocaine test should be performed to exclude Horner syndrome.
Apraclonidine 0.5% or 1% may cause lethargy, bradycardia, and respiratory depression in infants
younger than 6 months as a consequence of the immaturity of the blood-brain barrier.[29]
To perform the test, 2 drops of 1% hydroxyamphetamine solution are instilled into each eye. A
period of 24-48 hours must be allowed to elapse between the cocaine test and the
hydroxyamphetamine test because cocaine has the ability to inhibit the uptake of
hydroxyamphetamine into the presynaptic vesicles, thereby reducing the accuracy of the latter
test.
Hydroxyamphetamine drops instilled into an eye with Horner syndrome with intact
postganglionic fibers (ie, first- or second-order neuron lesions) dilate the affected pupil to an
equal or greater extent than they do the normal pupil. However, hydroxyamphetamine drops
instilled into an eye with Horner syndrome with damaged postganglionic fibers (ie, third-order
neuron lesions) do not dilate the affected pupil as well as they do the normal pupil.
Whether surgical care is indicated and what type is appropriate depend on the particular cause of
Horner syndrome. Potential surgical interventions include neurosurgical care for aneurysm-
related Horner syndrome and vascular surgical care for causative conditions such as carotid
artery dissection or aneurysm.
For optimal management of the underlying cause, the following specialist consultations may be
required:
Pulmonology
Internal medicine
Neurology or neuro-ophthalmology
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