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Horner’s syndrome (also known as Bernard-Horner Syndrome or Oculosympathetic Palsy) is a rare medical condition that affects the eyes and parts of the face.
Horner syndrome is typically found in children and adolescents, occurring in about 1.42 out of 100,000 people below 19 years old.1
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Horner’s syndrome is associated with three classic symptoms:
Signs and symptoms may appear at birth, or they may show up at a later age.
They usually affect one side of the face. But they can also affect both sides and cause bilateral symptoms like complete ptosis.2
Rare diseases such as Horner syndrome may produce uncommon symptoms such as:
Other signs include conjunctival redness, decreased eye pressure, and changes in tear viscosity on the affected side of the body.
Sympathetic nerves connect the brain to different parts of the body. The sympathetic nervous system (SNS) triggers involuntary actions in response to dangerous or stressful situations.
Some of these nerves — called the cervical ganglia — are connected to the eyes and face. They are responsible for facial sweating and the dilation (enlargement) of eye pupils.
When the impulses along these nerves are disrupted in some way, Horner syndrome develops. Common causes include injury or impaired blood flow to the cervical ganglia or the presence of tumors and inflammation in surrounding areas.
More than half of cases (55%) are congenital or present at birth. Less than half (45%) are acquired at a later age.1 Whether it is congenital or acquired, each is associated with different causes.
The cervical ganglia has three nerve pathways. Depending on which pathway is affected, it can lead to one of three types of Bernard-Horner’s syndrome:6
Also known as Central Horner Syndrome, it affects the first order neurons (FON) found along the hypothalamus in the brain and the spinal cord.
Possible causes include spinal cord lesions, impaired blood flow to the brain stem, and tumors in the hypothalamus.
Also known as Preganglionic Horner Syndrome, it affects the preganglionic nerves along the chest, the top of the lungs, and the carotid artery in the neck. It may be caused by tumors or trauma in the upper lungs or chest cavity which can result from surgery or an accident.
Also known as Postganglionic Horner Syndrome, it affects the postganglionic nerves which run across the neck, the middle ear, and the eye.
Migraines, cluster headaches, middle ear infections, carotid artery lesions, and skull injuries may lead to this condition.
Horner syndrome does not cause any significant effects on a person’s vision and overall health. However, it may be a sign of oculosympathetic nerve damage.
Damage to this nerve may result from serious and sometimes life-threatening medical conditions.
Serious health problems that are associated with Horner’s syndrome include:
Malignant (cancerous) and benign tumors (noncancerous) around the cervical ganglia have also been linked to the development of Horner’s syndrome.
You should see a doctor as soon as you notice signs. Horner’s syndrome may occur due to serious conditions, so you must get a prompt and accurate diagnosis.
Emergency care is needed if symptoms appear suddenly after a traumatic injury on the head or neck, or if the following signs accompany them:
A doctor may initially assess for signs of Horner’s syndrome and check for possible causes.
In addition, they may perform a series of tests to confirm a positive diagnosis and identify the site of nerve damage.
Diagnosis begins with the doctor conducting a physical examination. They will check for visible signs and symptoms of the condition.
A physical eye exam usually includes checking for the following:
Once the doctor confirms the presence of symptoms, they will review your medical history to see if they can trace its underlying causes.
Obtaining a patient’s history will also help them identify where the lesions are located.
An ophthalmologist may use different eye drops to see how your pupils react. Pharmacologic tests can determine whether or not you have Horner’s syndrome and which type you have.
Tests may include:
A doctor may request pharmacologic and imaging tests to identify which type of Horner’s syndrome a person has and whether it is caused by a tumor, lesion, or structural abnormality.
Tests may include:
According to the Genetic and Rare Diseases (GARD) Information Center, the treatment for Horner syndrome depends on the underlying cause.
When appropriate, a doctor may recommend surgical interventions to alleviate conditions such as aneurysm, carotid artery dissection, and tumor growths.
The prognosis of Horner’s syndrome depends on the causative factors. While the condition itself won’t affect your eyesight or quality of life, there may be a severe and life-threatening condition causing it that requires immediate treatment.
(2) “ Bilateral Horner's syndrome: detection and occurrence.” National Center for Biotechnology Information.
(3) “ Horner's syndrome.” National Center for Advancing Translational Sciences.
(4) “ Congenital Horner Syndrome with Heterochromia Iridis Associated with Ipsilateral Internal Carotid Artery Hypoplasia.” National Center for Biotechnology Information.
(5) “ Enophthalmos Is Not Present in Horner Syndrome.” National Center for Biotechnology Information.
(6) “ Horner Syndrome.” National Center for Biotechnology Information.