Rare Gastroenterology News

Disease Profile

Eagle syndrome

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

#N/A

ICD-10

#N/A

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

no.svg

Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

no.svg

X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

no.svg

X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

no.svg

Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

no.svg

Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

no.svg

Not applicable

no.svg

Other names (AKA)

Elongated styloid process which causes cervico facial pain tinnitus and otalgia; Elongated styloid process syndrome; Styloid-stylohyoid syndrome;

Summary

Eagle syndrome is characterized by recurrent pain in the middle part of the throat (oropharynx) and face. "Classic Eagle syndrome" is typically seen in patients after throat trauma or tonsillectomy. Symptoms include dull and persistent throat pain that may radiate to the ear and worsen with rotation of the head. Other symptoms may include difficulty swallowing, feeling of something stuck in the throat, tinnitus, and neck or facial pain. A second form of Eagle syndrome unrelated to tonsillectomy causes compression of the vessel that carries blood to the brain, neck, and face (carotid artery). This form can cause headache and dizziness.[1]

Eagle syndrome is due to a calcified stylohyoid ligament or an elongated styloid process.[1] The styloid process is a pointed part of the temporal bone that serves as an anchor point for several muscles associated with the tongue and larynx.[1][2]

The mainstay treatment for Eagle syndrome is surgery to shorten the styloid process (styloidectomy).[3] Medical management may include the use of pain and anti-inflammatory medications, antidepressants, and/or steroids.[1][3] The overall success rate for treatment (medical or surgical) is about 80%.[1]

Treatment

The mainstay treatment for Eagle syndrome is surgery to shorten the styloid process (styloidectomy).[3] Traditionally, this surgery has been done using either an intraoral (through the mouth) or extraoral (through the neck) approach.[1][3]

The intraoral approach usually requires tonsillectomy, and access to the styloid process is limited.[3] There is also risk of injury to major vessels.[1] However, this method reportedly is safe, more simple, and an external scar is avoided.[1]

The extraoral approach may provide better exposure of the process and its surrounding structures. However, this approach results in a scar, requires going through connective tissue and may carry an increased risk of trauma to surrounding structures.[3]

In recent years, more minimally-invasive techniques have been used for head and neck surgery. Some patients with Eagle syndrome have undergone an endoscope-assisted approach.[3] An endoscope is a long, thin tube with a camera attached at the end. According to the authors of a study published in 2017, this approach reportedly has the benefits of providing direct surgical access, satisfactory exposure, and minimal invasion.[3]

Medical management of Eagle syndrome may include the use of pain and anti-inflammatory medications, antidepressants, and/or steroids for pain.[1][3]

The overall success rate for treatment (medical or surgical) is about 80%.[1]

People with questions about personal treatment options and recommendations for Eagle syndrome should speak with their doctor.

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    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.

    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.
    • PubMed is a searchable database of medical literature and lists journal articles that discuss Eagle syndrome. Click on the link to view a sample search on this topic.

      References

      1. Rinaldi V. Eagle Syndrome. Medscape. May 6, 2016; https://emedicine.medscape.com/article/1447247-overview#a6.
      2. Baghat, M. et al.. Eagle's syndrome, a rare cause of neck pain.. BMJ Case report. July 25, 2012; https://www.ncbi.nlm.nih.gov/pubmed/?term=22843755. Accessed 8/3/2016.
      3. Chen R, Liang F, Han P, Cai Q, Yu S, Huang X. Endoscope-Assisted Resection of Elongated Styloid Process Through a Retroauricular Incision: A Novel Surgical Approach to Eagle Syndrome. J Oral Maxillofac Surg. January 26, 2017; https://www.ncbi.nlm.nih.gov/pubmed/28215854.
      4. Bokhari MR, Graham C, Mohseni M. Eagle Syndrome.. StatPearls. Updated Jul 20, 2020; https://pubmed.ncbi.nlm.nih.gov/28613540/.
      5. Westbrook M, Kabbaz VJ, Showalter CR. Eagle's syndrome, elongated styloid process and new evidence for pre-manipulative precautions for potential cervical arterial dysfunction. Musculoskelet Sci Pract. Dec 2020; 50:102219:https://pubmed.ncbi.nlm.nih.gov/32891576/.
      6. Badhey A, Jategaonkar A, Anglin Kovacs AJ, Kadakia S, De Deyn PP, Ducic Y, Schantz S, Shin E. Eagle syndrome: A comprehensive review. Clin Neurol Neurosurg. Aug 2017; 159:34-38. https://pubmed.ncbi.nlm.nih.gov/28527976/.

      Rare Gastroenterology News