While rare, Amelogenesis Imperfecta still affects an estimated 23,000 people in the United States alone.* While unavoidable, there are dental and cosmetic treatments available to help patients overcome the aesthetics and practical challenges posed by this uncommon dental affliction.
What is Amelogenesis Imperfecta (AI)?
Amelogenesis is simply the formation of enamel. Amelogenesis imperfecta then is merely another way of saying the incorrect or inadequate formation or development of dental enamel.
Amelogenesis Imperfecta (AI) is a congenital dental disease that affects a patient’s dental enamel and the development of strong, healthy teeth. The teeth of patients with amelogenesis imperfecta are prone to rapid wear and breakage. As a result of compromised enamel, those with this rare disease often display teeth to be unusually small, discolored and pitted or grooved.
It is commonly referred to as teeth without enamel.
Amelogenesis Imperfecta Case Study - Drew
Teeth without enamel
Remember, enamel is the resilient outer layer of a tooth that protects the softer dentin and sensitive inner pulp from mechanical wear and tear and bacterial infection. When the enamel is weakened or compromised, the tooth is left exposed and vulnerable to infection, erosion, or physical damage.
There are 14 forms of Amelogenesis Imperfecta (AI) that can be broadly categorized into four primary types:
Type 1 Hypoplastic
Type 2 Hypomaturation
Type 3 Hypocalcified
Type 4 Hypomature Hypoplastic Enamel with Taurodontism
Many patients with one form or another of the disease may exhibit one, some, or all of the characteristics of the broad typologies listed above. Also, each broad typology can be further organized into various subtypes. Type 1E, for example, describes hypoplastic enamel that is of normal hardness but does not have the standard thickness (it is too thin).
Amelogenesis Imperfecta Types & Signs
Type 1: Hypoplastic
Due to abnormal enamel formation, the dental enamel is too thin.
Type 2: Hypomaturation
Due to abnormal enamel formation, the dental enamel is too soft.
Type 3: Hypocalcified
Due to abnormal enamel calcification, or the lack of adequate calcium in dental enamel, a patient’s dental enamel is vulnerable to demineralization.
Type 4: Hypomature Hypoplastic Enamel with Taurodontism
Due to abnormal enamel formation, the dental enamel display conditions associated with any of the previously described types (type 1-3) and also show signs of taurodontism.
All About the Genes
Amelogenesis Imperfecta is a congenital disease meaning that patients with the disease had to have received abnormal genes from both their parents to display symptoms. Those with only one set of abnormal genes from one parent are typically asymptomatic carriers who carry a risk of passing the mutated gene to their offspring.
The disease is typically diagnosed by the age of one-year-old with a simple hand instrument by a knowledgeable pediatric dentist. The final diagnosis is made with a combination of family history, visual confirmation, and x-ray examination.
Adults with amelogenesis imperfecta should seek genetic counseling and understand the risks of passing on their genetic mutations to their children.
Because Amelogenesis Imperfecta results from a genetic disorder, as opposed to an external pathogen in the case of dental caries, it is impossible to cure. Patients do, however, have a range of dental treatments available that can address the aesthetic (visual) and functional issues caused by the disorder. While the disorder is incurable, the symptoms can be addressed with cosmetic dentistry.
Don’t let amelogenesis imperfecta ruin your perfect smile!
Amelogenesis Imperfecta Case Study - Ninjah
Treating Amelogenesis Imperfecta (AI)
Amelogenesis Imperfecta presents a unique challenge to both patients and doctors. Without healthy enamel, teeth become extremely vulnerable to outside forces including trauma and infectious microbes. To combat this, there are a number of treatments available ranging from encapsulation to complete dental replacement therapy.
Treatments for Amelogenesis Imperfecta must not only address aesthetic concerns but functional considerations as well, such as eating, speaking, chewing, and biting.
The first step in treating Amelogenesis Imperfecta (AI) is to understand that enamel protection and retention is the goal.
Patients diagnosed with Amelogenesis Imperfecta should treat their enamel gently. Brush lightly with a soft brush and only as recommended by a dentist.
Avoid food and beverages that are harsh on teeth. This means avoiding highly acidic beverages, such as citrus juices and coffee, as well as eschewing hard or tough to chew foods, such as certain bread and hard candy.
Careful prevention is the best, easiest, and the most economical way to retain dental enamel.
"While the underlying genetic causes of amelogenesis imperfecta can’t be corrected, the symptoms, especially the visual imperfections, can be fixed!"
--- DR. REZA KHAZAIE, DDS. PROSTHODONTIST
Treatment Procedures for Amelogenesis Imperfecta
Next, patients should consider dental procedures that actively improve dental aesthetics and protect teeth. Common methods for addressing both form and function include dental veneers, full dental crowns, or when existing dentition is unsalvageable, complete dental replacement therapy.
Depending on the type and severity, any of the aforementioned treatment strategies could work alongside essential preventative care practices such as diet and routine dental hygiene.
Patients with mild amelogenesis imperfecta and largely healthy teeth can receive porcelain veneers to address aesthetic concerns.
Those with abraded or damaged teeth, including children, may require full crown restoration to help protect compromised teeth and prevent further degradation from biting and chewing.
Other important considerations include the use of desensitizing toothpaste to help manage dental sensitivity to heat and cold.
Remember, while the underlying genetic causes of amelogenesis imperfecta can’t be corrected, the symptoms, especially the visual imperfections, can be fixed!
Call Willow Pass Dental Care today and schedule a complimentary consultation with Dr. Reza Khazaie, DDS. Prosthodontist.