What are the other Names for this Condition? (Also known as/Synonyms)

• Bader Syndrome
• Multiple Odontomas with Dysphagia
• Odontomatosis with Dysphagia

What is Odontoma-Dysphagia Syndrome? (Definition/Background Information)

• Odontoma-Dysphagia Syndrome (or Odontomatosis-aortae esophagus stenosis syndrome) is a malformation syndrome, characterized by odontomas (undifferentiated mass of the esophagus) and severe dysphagia. Three of the reported patients manifested multiple odontomas
• Occasionally, cardiac (stenosis of the intrathoracic descendent aorta, interstitial myocarditis), renal (pyelonephritis) and hepatic (hepatic sclerosis) involvement has been described
• Hypertrophy and dysmotility of the esophageal smooth muscles is suggested to have causative role for dysphagia

(Source: Odontomatosis-aortae esophagus stenosis syndrome; Orphanet, National Institute of Health and Medical Research (INSERM), Paris.)

Who gets Odontoma-Dysphagia Syndrome? (Age and Sex Distribution)

• Odontoma-Dysphagia Syndrome is an extremely rare disorder and only about 10 cases have been so far described in the medical literature
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Odontoma-Dysphagia Syndrome? (Predisposing Factors)

• Currently, no risk factors have been clearly identified for Odontoma-Dysphagia Syndrome

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Odontoma-Dysphagia Syndrome? (Etiology)

• Hypertrophy and dysmotility of the esophageal smooth muscles is suggested to have causative role for dysphagia

(Source: Odontoma-Dysphagia Syndrome; Orphanet, National Institute of Health and Medical Research (INSERM), Paris.)

What are the Signs and Symptoms of Odontoma-Dysphagia Syndrome?

The signs and symptoms of Odontoma-Dysphagia Syndrome may include:

• Abnormality of esophagus morphology

Very frequently present symptoms in 80-99% of the cases include:

Occasionally present symptoms in 5-29% of the cases: Myocarditis

(Source: Odontoma-Dysphagia Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How is Odontoma-Dysphagia Syndrome Diagnosed?

Odontoma-Dysphagia Syndrome is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests
• Imaging studies
• Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Odontoma-Dysphagia Syndrome?

The complications of Odontoma-Dysphagia Syndrome may include:

• Liver dysfunction and liver failure
• Cardiac abnormalities
• Gastrointestinal tract abnormalities

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Odontoma-Dysphagia Syndrome Treated?

There is no cure for Odontoma-Dysphagia Syndrome. The treatment is usually given to manage the signs and symptoms and any complications that develops.

How can Odontoma-Dysphagia Syndrome be Prevented?

• Currently, Odontoma-Dysphagia Syndrome is not preventable
• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Odontoma-Dysphagia Syndrome? (Outcomes/Resolutions)

• The prognosis of Odontoma-Dysphagia Syndrome is dependent upon the severity of the signs and symptoms and associated complications, if any
• Individuals with mild conditions have better prognosis than those with severe symptoms and complications
• Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Odontoma-Dysphagia Syndrome:

Odontoma Dysphagia Syndrome may also be known by the following synonym:

• Odontomatosis-aortae esophagus stenosis syndrome

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/