Horner's syndrome

Title

Other Names:

Bernard-Horner Syndrome; Oculosympathetic Palsy

Summary Summary

Horner's syndrome consists of miosis (constriction of the pupil), ptosis (drooping of the upper eyelid), and anhidrosis (absence of sweating of the face).^[1]^[2]^[3] It is caused by injury to the sympathetic nerves of the face. 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]

Last updated: 10/1/2014

Symptoms Symptoms

Symptoms typically include drooping of the eyelid, constriction of the pupil, sinking of the eyeball into the face, and decreased sweating on the affected side of the face. These symptoms may vary.^[1]^[2]^[3] Other symptoms which may be seen include the inability to close the eyelid, a transient decrease in intraocular pressure, changes in tear viscosity (thickness), absence of horizontal eyelid fold or crease in the drooping eyelid, and red conjunctiva.^[3]^[4] Other symptoms associated with the underlying cause may also occur.

The most reliable clinical sign of Horner's syndrome is the anisocoria (unequal size of the pupils).^[4] Heterochromia iridium (i.e., a relative deficiency of pigment in the iris (colored part of the eye) of the Horner's side, is usually present when the syndrome is congenital or caused by a lesion that has occurred before the age of 1-2 years.^[3] Also, cluster headaches (tearing, congestion, and pain around one eye) is associated with intermittent Horner's syndrome.^[4]

Last updated: 3/6/2012

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: 9/1/2016

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, and cluster headaches.^[1]

Horner's syndrome can also be caused by the following ^[3]^[4]:

Certain eye drops and medications
Neck trauma and neck surgery
Infarction in the brainstem
Infarction in the hypothalamus
A tumor in the brainstem or in the hypothalamus
A multiple sclerosis plaque
Syringomyelia
Infection of the middle ear
A parasellar tumor located in the middle cranial fossa

Last updated: 3/6/2012

Diagnosis Diagnosis

The physical signs of Horner's syndrome may be so subtle that they often go undetected. An ophthalmologist may confirm the diagnosis by special eye tests.^[5] 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: 3/6/2012

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.^[4]

Last updated: 3/6/2012

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.

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

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

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

Have a question? Contact a GARD Information Specialist.

References References

Horner syndrome. MedlinePlus. May 20, 2014; http://www.nlm.nih.gov/medlineplus/ency/article/000708.htm. Accessed 10/1/2014.
Horner's syndrome. National Organization for Rare Disorders (NORD). 2003; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/864/viewAbstract. Accessed 10/1/2014.
Rolf Salvesen. Horner Syndrome. NORD Guide to Rare Disorders. Philadelphia, PA: Lippincott Williams & Wilkins; 2003;
Parmar MS. Horner Syndrome. eMedicine. June 5, 2008; http://emedicine.medscape.com/article/279394-overview. Accessed 3/27/2009.
Dennis Robertson. Horner syndrome: What causes it?. MayoClinic.com. March 23, 2007; http://www.mayoclinic.com/health/horner-syndrome/HQ00858. Accessed 3/27/2009.