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Dentin Dysplasia Type I

Article
Dental Health
Diseases & Conditions
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Contributed byMaulik P. Purohit MD MPHNov 24, 2023

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

  • Opalescent Dentin Dysplasia Type I
  • Rootless Teeth (Dentin Dysplasia Type I)
  • Thistle Tube Teeth (Dentin Dysplasia Type I)

What is Dentin Dysplasia Type I? (Definition/Background Information)

  • Dentin Dysplasia is a rare, genetic disorder of the teeth that is characterized by:
    • Abnormal development of dentin (bone-like material situated under the enamel, which forms most part of the tooth and contains the pulp of the tooth)
    • Abnormal development of pulp tissues (The pulp is a soft tissue made up of blood vessels and nerves)
    • Enamel is normal: The layer over the teeth, called enamel, is usually not affected
    • Normal appearing crowns: Visually, the crowns of the teeth appear normal
    • Hypermobility of teeth
    • Dental abscesses and cysts
    • Underdevelopment of jaws
  • The various types of Dentin Dysplasia include:
    • Type I or radicular type Dentin Dysplasia
    • Type II or coronal type Dentin Dysplasia
  • Dentin Dysplasia Type I is also called as Radicular Dentin Dysplasia. In this condition:
    • The roots of the teeth are shorter than the normal length
    • Pulp chamber is absent or the pulp chamber is crescent-shaped in appearance
    • There is an early loss of teeth
    • However, the color of the teeth is normal
  • Dentin Dysplasia Type I is an inherited genetic disorder that is inherited in an autosomal dominant pattern. It is caused by the alteration or changes that occur in the DSPP gene, which is involved in dentinogenesis
  • The treatment of Dentin Dysplasia Type I includes dental implants and surgery in severe cases. The prognosis depends on early diagnosis and quality of the treatment and management methods. With proper treatment and care, aesthetic appearance and functional performance can be achieved

Who gets Dentin Dysplasia Type I? (Age and Sex Distribution)

  • Dentin Dysplasia Type I is an inherited disorder that is present at birth. It affects both the primary and permanent dentition (teeth development)
  • No racial, ethnic, or gender predominance is observed

What are the Risk Factors for Dentin Dysplasia Type I? (Predisposing Factors)

The only risk factor associated with Dentin Dysplasia Type I is a family history of the disorder.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones 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 Dentin Dysplasia Type I? (Etiology)

  • Dentin Dysplasia Type I is a rare, hereditary, and genetic disorder affecting the teeth
  • The disorder is inherited in an autosomal dominant pattern and is caused by alterations in the DSPP gene, which is involved in dentinogenesis (formation of dentin)

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 functioning 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 Dentin Dysplasia Type I?

The common signs and symptoms which distinguish Dentin Dysplasia Type 1 from other disorders include:

  • Lack of roots
  • Poor dental alignment
  • Risk of losing tooth very early
  • Crooked teeth
  • Underdeveloped jaw
  • Chipped teeth

How is Dentin Dysplasia Type I Diagnosed?

The diagnosis of Dentin Dysplasia Type I may involve:

  • Complete dental examination and evaluation of medical history
  • Checking for radiographic  features that include:
    • Abnormal roots
    • Pulp destruction    
    • Partially destroyed crescent-shaped pulp chamber
    • Pulp stones
  • Molecular genetic testing will help to confirm the diagnosis

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 Dentin Dysplasia Type I?

The possible complications of Dentin Dysplasia Type I may include:

  • Hypermobility of teeth
  • Dental abscesses and cysts
  • The roots of the teeth are shorter than normal length
  • Early loss of teeth

How is Dentin Dysplasia Type I Treated?

The treatment and management of Dentin Dysplasia Type I include:

  • Dental implants:
    • The early loss of teeth will necessitate dental implants
    • All the remaining teeth may be removed and a new set of teeth put in place
    • Some physicians may prefer replacing one tooth at a time as they are lost, instead of replacing the entire set
  • Rehabilitation:
    • Under this method, the dentist attempts to fix the lost tooth back in its place
    • Thus, the patient may be able to retain his/her original teeth and not rely on teeth made of artificial material
  • Surgical intervention: In severe cases, surgery is the only option to be recommended. Surgery may involve a combination of procedures that can include:
    • Sinus lifting
    • Bone augmentation
    • Bone grafting
    • Dental replacement

How can Dentin Dysplasia Type I be Prevented?

  • Currently, there are no specific methods or guidelines to prevent Dentin Dysplasia Type I, since it is a genetic condition
  • Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
  • 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
  • The best way to prevent the complications of Dentin Dysplasia Type I is to practice excellent oral hygiene and to take good care of the teeth

What is the Prognosis of Dentin Dysplasia Type I? (Outcomes/Resolutions)

  • The prognosis of Dentin Dysplasia Type I will depend on early diagnosis and quality of the treatment and management methods
  • With proper treatment and care, aesthetic appearance and functional performance can be improved

Additional and Relevant Useful Information for Dentin Dysplasia Type I:

There are still many unsolved issues with regards to the diagnosis and management of Dentin Dysplasia. However, early diagnosis and immediate treatment is still the essential prerequisite to prevent early loss of teeth.

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Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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