×

Please Remove Adblock
Adverts are the main source of Revenue for DoveMed. Please remove adblock to help us create the best medical content found on the Internet.

Tricho-Dental Syndrome

Last updated April 24, 2018

Approved by: Maulik P. Purohit MD, MPH

Tricho-Dental Syndrome is characterized by the association of fine, dry and short hair with dental anomalies.


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

  • Trichodental dysplasia
  • Trichodental syndrome
  • Tricho-dental dysplasia

What is Tricho-Dental Syndrome? (Definition/Background Information)

  • Tricho-Dental Syndrome is characterized by the association of fine, dry and short hair with dental anomalies
  • The disorder has been described in less than 10 families worldwide
  • Tricho-Dental Syndrome is inherited in an autosomal dominant manner

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

Who gets Tricho-Dental Syndrome? (Age and Sex Distribution)

  • Tricho-Dental Syndrome is a rare congenital disorder, with fewer than 10 affected families reported in the scientific literature
  • The presentation of symptoms may occur in childhood
  • Both males and females may be affected
  • Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Tricho-Dental Syndrome? (Predisposing Factors)

  • A positive family history may be an important risk factor, since Tricho-Dental Syndrome can be inherited
  • Currently, no other risk factors have been clearly identified for Tricho-Dental 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 Tricho-Dental Syndrome? (Etiology)

  • The genetic cause of Tricho-Dental Syndrome is not known at the present time
  • Tricho-Dental Syndrome is inherited in an autosomal dominant manner

Autosomal dominant: Autosomal dominant conditions are traits or disorders that are present when only one copy of the mutation is inherited on a non-sex chromosome. In these types of conditions, the individual has one normal copy and one mutant copy of the gene. The abnormal gene dominates, masking the effects of the correctly function gene. If an individual has an autosomal dominant condition, the chance of passing the abnormal gene on to their offspring is 50%. Children, who do not inherit the abnormal gene, will not develop the condition or pass it on to their offspring

What are the Signs and Symptoms of Tricho-Dental Syndrome?

The signs and symptoms of Tricho-Dental Syndrome may include:

  • Small mouth
  • Short, incompletely developed tongue (hypoglossia)
  • Absent, partially missing, or shortened fingers and/or toes
  • Jaw abnormalities such as micrognathia, retrognathia (receding jaw), or partially missing mandible (lower jaw)
  • High-arched, narrow, or cleft palate
  • Absent or unusually formed arms and/or legs
  • Missing teeth
  • Absence of major salivary glands
  • Abnormality of oral frenula
  • Aglossia
  • Epicanthus
  • Microglossia

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

How is Tricho-Dental Syndrome Diagnosed?

Tricho-Dental 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 Tricho-Dental Syndrome?

The complications of Tricho-Dental Syndrome may include:

  • Inability to chew food due to abnormal development of teeth
  • Swallowing difficulties due to tongue hypoplasia
  • Loss of teeth
  • Problems with development of speech, due to abnormal tongue development
  • Low self esteem

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

How is Tricho-Dental Syndrome Treated?

There is no cure for Tricho-Dental Syndrome, since it is a genetic condition. The treatment is usually given to manage the signs and symptoms and any complication that develops

How can Tricho-Dental Syndrome be Prevented?

  • Tricho-Dental Syndrome may not be preventable, since it is a genetic disorder
  • If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
  • Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders
  • Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Tricho-Dental Syndrome? (Outcomes/Resolutions)

  • The prognosis of Tricho-Dental 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 Tricho-Dental Syndrome:

Tricho-Dental Syndrome is also known by the following additional synonym:

  • Kersey syndrome

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

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

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: April 24, 2018
Last updated: April 24, 2018