Horner's syndrome

Title

Other Names:

Bernard-Horner Syndrome; Oculosympathetic Palsy

Summary Summary

Horner's syndrome is a rare condition characterized by miosis (constriction of the pupil), ptosis (drooping of the upper eyelid), and anhidrosis (absence of sweating of the face).^[1]^[2] It is caused by damage to the sympathetic nerves of the face. The underlying causes of Horner's syndrome vary greatly and may include a tumor, stroke, or other damage to a part of the brain called the brain stem; injury to the carotid artery; and trauma to the brachial plexus. In rare cases, Horner's syndrome is congenital (present from birth) and associated with a lack of pigmentation of the iris (colored part of the eye).^[1]^[2]^[3] Treatment of Horner's syndrome depends on the underlying cause.^[3]

Last updated: 1/3/2017

Symptoms Symptoms

Symptoms of Horner's syndrome typically include drooping of the upper eyelid (ptosis), constriction of the pupil (miosis), sinking of the eyeball into the face, and decreased sweating on the affected side of the face (anhidrosis). These symptoms may vary and other symptoms may occur depending on the underlying cause of the condition.^[1]^[2] Other symptoms which may be seen include the inability to completely close or open the eyelid, facial flushing, headaches, and pain.^[2]^[3]

Heterochromia iridium (i.e., a relative deficiency of pigment in the iris of affected side of the face) is usually present when the syndrome is congenital or caused by a lesion that has occurred before the age of 1-2 years of age.^[3]

Last updated: 1/3/2017

The Human Phenotype Ontology (HPO) provides the following list of features that have been reported in people with this condition. Much of the information in the HPO comes from Orphanet, a European rare disease database. If available, the list includes a rough estimate of how common a feature is (its frequency). Frequencies are based on a specific study and may not be representative of all studies. You can use the MedlinePlus Medical Dictionary for definitions of the terms below.

Signs and Symptoms	Approximate number of patients (when available)
Autosomal dominant inheritance	-
Congenital Horner syndrome	-
Deeply set eye	-
Heterochromia iridis	-
Ipsilateral lack of facial sweating	-

Last updated: 5/8/2017

Cause Cause

There are many potential causes of Horner's syndrome. It can be caused by any interruption of the sympathetic nerve fibers, which start in the hypothalamus and run via the upper spinal cord near the carotid artery to the face. Sympathetic nerve fiber injuries can result from a stroke in the brainstem, injury to the carotid artery, a tumor in the upper lobe of the lung, migraines, and cluster headaches.^[1]

Horner's syndrome can also be caused by the following:^[2]^[3]

Neck trauma and neck surgery
A tumor in the brainstem or in the hypothalamus
Diseases that cause damage to the protective covering that surrounds nerve fibers (demyelinating disease)
Development of a fluid-filled cavity or cyst within the spinal cord (syringomyelia)
Arnold-Chiari malformation

Last updated: 1/3/2017

Diagnosis Diagnosis

An ophthalmologist may confirm the diagnosis by special eye tests.^[4] In addition, a careful neurological exam may be necessary to find the cause by determining which, if any, other parts of the nervous system are affected. Tests may include^[1]:

MRI of the head
Carotid ultrasound
Chest x-ray
CT scan of the chest
Blood tests
Angiogram
Eye drop tests

Last updated: 1/3/2017

Treatment Treatment

Treatment depends on the underlying cause. There is no specific treatment for Horner's syndrome itself.^[1] In many cases, no effective treatment is known.^[3]

Last updated: 1/3/2017

Do you have updated information on this condition? Let us know.

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.
ClinicalTrials.gov lists trials that are studying or have studied Horner's syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations Organizations

Nonprofit support and advocacy groups bring together patients, families, medical professionals, and researchers. These groups often raise awareness, provide support, and develop patient-centered information. Many are the driving force behind research for better treatments and possible cures. They can direct people to research, resources, and services. Many groups also have experts who serve as medical advisors. Visit their website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Social Networking Websites

Visit the Ross, Harlequin, Holmes-Adie and Horner's Syndrome group on Facebook.

Do you know of an organization? Send us your suggestions.

Living With Living With

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Genetics Resources

To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by GeneTests, the American College of Medical Genetics, and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from Genetics Home Reference.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

Genetics Home Reference contains information on Horner's syndrome. This website is maintained by the National Library of Medicine.
MayoClinic.com offers additional information about Horner syndrome. Click on the above link to access this information.
MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
PubMed is a searchable database of medical literature and lists journal articles that discuss Horner's syndrome. Click on the link to view a sample search on this topic.

GARD Answers GARD Answers

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I have Horner's Syndrome which is believed to have been caused by a thoracic spinal tumor. I have all of the symptoms, except that of my eye retracting back into the socket. Will the syndrome keep progressing now that the tumor has been removed? Is that something I should be watching for? See answer
My son was recently diagnosed with Horner syndrome. Is it possible that he got this after having his neck and back aligned by a chiropractor? Also as a parent is there anything I should be concerned about? Do you recommend any restrictions to his playing high school sports? See answer
My friend was recently diagnosed with Horner syndrome. I am trying to find information and resources regarding this topic. See answer
My sister was recently diagnosed with Horner syndrome. Last year I was diagnosed with cluster headaches. Are these two condition related? See answer
My daughter sustained an injury which required her to be placed on an ECMO machine for several days. The machine damaged a nerve which caused Horner syndrome. Can you provide me with more information about this condition? See answer