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

• Dentin Dysplasia Type II (DD-2)
• Dentin Dysplasia, Shields Type 2

What is Coronal Dentin Dysplasia? (Definition/Background Information)

• Coronal Dentin Dysplasia is a rare mild form of dentin dysplasia, characterized by normal tooth roots but abnormal primary dentition
• Coronal Dentin Dysplasia is caused by mutations in the DSPPgene (4q21.3) coding for dentin sialophosphoprotein, a precursor for dentin sialoprotein and dentin phosphoprotein, which are involved in dentinogenesis. DD-2 follows an autosomal dominant pattern of inheritance
• In Coronal Dentin Dysplasia, features of primary dentition include amber translucent coloration, bulbous crowns, cervical constriction, tooth attrition, and short constricted roots. Pulp obliteration is also observed
• In the primary dentition, preformed stainless steel crowns on molars may be used to prevent tooth wear and maintain the occlusal vertical dimension. Appropriate care makes it possible to achieve good esthetic appearance and functional performance
• The prognosis of Coronal Dentin Dysplasia depends primarily on the age of diagnosis and the quality of management

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

Who gets Coronal Dentin Dysplasia? (Age and Sex Distribution)

• Coronal Dentin Dysplasia is a rare congenital disorder. The exact prevalence of this disorder is not known
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

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

• A positive family history may be an important risk factor, since Coronal Dentin Dysplasia may be inherited
• Currently, no other risk factors have been clearly identified for 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 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 Coronal Dentin Dysplasia? (Etiology)

• Coronal Dentin Dysplasia is caused by mutations in the DSPPgene (4q21.3) coding for dentin sialophosphoprotein, a precursor for dentin sialoprotein and dentin phosphoprotein, which are involved in dentinogenesis
• DD-2 follows an autosomal dominant pattern of inheritance

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

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 Coronal Dentin Dysplasia?

The signs and symptoms of Coronal Dentin Dysplasia in the first set of teeth (primary dentition), may include:

• Amber translucent coloration 
• Bulbous crowns 
• Cervical constriction
• Tooth attrition
• Short constricted roots 
• Pulp obliteration

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

How is Coronal Dentin Dysplasia Diagnosed?

Coronal Dentin Dysplasia is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Dental examination
• 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 Coronal Dentin Dysplasia?

The complications of Coronal Dentin Dysplasia may include:

• Tooth decay
• Low self-esteem due to deformed teeth

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

How is Coronal Dentin Dysplasia Treated?

There is no cure for Coronal Dentin Dysplasia, since it is a genetic condition. 

• Preformed stainless steel crowns on molars may be used to prevent tooth wear and maintain the occlusal vertical dimension 
• Appropriate care makes it possible to achieve good aesthetic appearance and functional performance

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

How can Coronal Dentin Dysplasia be Prevented?

Coronal Dentin Dysplasia may not be preventable, since it is a genetic disorder.

• 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

Regular medical screening at periodic intervals with tests and physical examinations are recommended.

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

• The prognosis of Coronal Dentin Dysplasia depends primarily on the age of diagnosis and the quality of management

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

Additional and Relevant Useful Information for Coronal Dentin Dysplasia:

Coronal Dentin Dysplasia is also known by the following names: 

• DTDP2
• DD-II
• Pulpal Dysplasia
• Pulp Stones

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

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