Imperfect amelogenesis: what is it and how is it treated?

Dental enamel is the outermost hard tissue that covers and protects teeth. There are some rare conditions that can affect this property. Imperfect amelogenesis is one and we will talk about it in this article.

We know by the name of “enamel” the hardest tissue of the body, which covers and protects the visible part of the teeth. It is formed from calcium phosphate, in the form of hydroxyapatite crystals. Also, it is what gives the smile its characteristic whitish appearance.

However, during the formation of teeth, anomalies can occur that affect the correct development of the tissues. In amelogenesis imperfecta, enamel does not form normally. Why does this happen? Discover!

What is amelogenesis imperfecta?

Amelogenesis imperfecta is a genetic condition that interferes with the normal development of tooth enamel. This pathology can occur both in milk and in permanent teeth.

The origin of this dental disease is in mutations genetics that are passed on from parent to child. In any case there may also be alterations of the genetic material without any history in the family group.

Several genes are involved during enamel formation, a process known as amelogenesis. The origin of the disorder it could be due to mutations in any of these pieces of genetic material.

As the disease develops, the teeth undergo changes in their appearance and structure. In particular, they are smaller, more fragile and often have spots. They are also more likely to suffer from sensitivity, wear and tear, tooth decay or falls.

What is amelogenesis imperfecta?
Amelogenesis imperfecta is often detected in infancy with the eruption of milk or permanent teeth.

Types of amelogenesis imperfecta

Tooth enamel alteration is the main feature of amelogenesis imperfecta. Even so, there are several clinical forms of the disease that differ in their manifestations. This largely depends on the gene involved and the enamel formation process in which it is involved.

That means, the symptoms of the disease vary depending on when the alteration occurs. In some cases, different types can coexist in the same patient.

In his opinion Center for Genetic Information and Rare Diseases, there are 4 main variants. These, in turn, are divided into 17 or 18 subtypes, depending on the genetic mutations that give rise to them.

Type I or hypoplastic

Change happens at the beginning. Cells that should be specialized to take care of enamel formation fail to differentiate into ameloblasts. The result is the presence of a very thin outer tooth layer.

The teeth have a rough surface with linear depressions and are often brown in color. Also, it is normal for teeth to be smaller and poorly set.

Type II or hypomature

The defect occurs at the end of the enamel formation process. Tissue calcification is incomplete and defective. The enamel of these teeth usually has an altered texture and is less hard than usual.

In this way, it fails to fulfill its protective function and it is common for teeth to break and get damaged easily. Meanwhile, the coloring is usually yellow-brown and its surface is smooth. Some cases have a mottled appearance or vertical streaks.

When acquiring radiographic images, there is not such a clear contrast between enamel and dentin, as occurs in healthy pieces. It is common for patients with this type of amelogenesis imperfecta to have tooth sensitivity and have problems with occlusion associated, such as the open bite.

Type III or hypocalcified

In this type of amelogenesis imperfecta, the problem occurs during the calcification phase of the organic matrix. In this case the amount and thickness of the enamel is normal, but the mineralization is less.

The teeth are yellow-brown in color and their texture is soft. As in type II, They are very brittle, often chipped and break easily. On radiographic images, the enamel appears less radiopaque than usual.

Due to the poor quality of the glaze, these pieces are easily affected by the loss or wear of the fabric due to external factors. This happens early, exposes the underlying dentin and causes sensitivity problems.

Type IV or hypomature-hypoplastic

In this case, the characteristics of amelogenesis imperfecta of types I and II are combined. Often occurs with taurodontism an abnormality in the shape and size of the pulp chamber of the teeth.

The enamel layer is thin and smooth, white, brown or yellowish in color with spots. A differential diagnosis should be made with dental fluorosis, as the appearance is similar.

Complications of amelogenesis imperfecta

Insufficient or poor quality enamel means that teeth with amelogenesis imperfecta do not have sufficient protection. Therefore, habitual practices, such as eating, chewing or brushing your teeth They can cause wear and tear of tooth tissue.

The disorder leads to the exposure of the underlying dentin. With this, the person suffers from dental sensitivity to contact with cold, hot or sweet substances; even when it bites. In some cases, the pain can become quite intense and chronic.

Likewise, the susceptibility to the action of bacteria and their acids increases. Therefore, the appearance of cavities is quite common. It’s also common for teeth to break or fracture easily, even during common activities like chewing.

Among other things, this pathology can affect the tissues surrounding the teeth. The gums, root cementum, periodontal ligament and alveolar bone may be affected.

And since defective enamel affects dental aesthetics, self-esteem and security issues are common. Even for some it is grounds for rejection or ridicule.

Diagnosis and treatment

The changes in the appearance of the teeth cause the diagnosis of amelogenesis imperfecta to be made as soon as the teeth appear in the mouth. This will be in infants or older children, depending on whether the affected items are milk or definitive.

The oral examination and radiographic images are usually sufficient to arrive at a diagnosis. Even so, family history and complementary and genetic studies help confirm the disorder.


Treatment will depend on the type of amelogenesis imperfecta diagnosed and the age of the patient. Therapies will change and renew themselves as the child grows to accompany their development and their new needs.

In some cases, direct resin fillings and sealants may help. However, restorations with crowns that cover and protect the entire tooth are the most commonly used measure.

With these procedures, not only are the associated complications avoided, but they as well it is obtained that the elements acquire a colour, shape and size similar to the normal ones. That is, it provides aesthetics and functionality to the mouth.

When the children are older, it is important to evaluate the need for orthodontic or surgical treatment, because as we have said, malocclusions are frequent in these patients and their correction over time will prevent future problems.

Preventive procedures are also part of the care of patients with amelogenesis imperfecta. These are some of the measures that help take care of this type of prosthesis:

  • Adequate oral hygiene.
  • Healthy diet, low in sugars and acids.
  • Topical application of fluoride.
  • Placement of the sealant.
  • Rinses with chlorhexidine.
  • Use of special pastes for tooth sensitivity.

In the most serious cases, the extraction of teeth with a poor prognosis and their subsequent prosthetic rehabilitation will be indicated.

Little girl brushing her teeth
Proper dental hygiene is essential to reduce the risk of complications from amelogenesis imperfecta.

Caring for children with amelogenesis imperfecta

Amelogenesis imperfecta is a rare oral disease. However, for those who suffer from it, This condition alters the aesthetics and functionality of your teeth. Furthermore, it predisposes to suffer from annoying complications such as sensitivity, fractures and cavities.

Taking children to the pediatric dentist early is a key action to detect and address this problem in time. The professional accompanies the child with the therapeutic measures appropriate to his age, which improve his quality of life.

Home care is also very important. Take care to maintain proper oral hygiene and a healthy diet low in sugar and acidic foods helps prevent complications associated with these unprotected teeth.

The post Amelogenesis imperfecta: what is it and how is it treated? first appeared on research-school



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