• Tuesday July 7,2020

Dentinogenesis imperfecta

Under a Dentinogenesis imperfecta describes the physician an inheritable disease. Dentinogenesis imperfecta subsequently leads to a malformation of the dentin, which causes the enamel to dissolve and the dentin to be released. Due to the abrasion caused by chewing, the dentin breaks down to the gums.

What is a Dentinogenesis imperfecta?

As part of Dentinogenesis Imperfecta, the teeth have a mild bluish discoloration. The enamel is missing or cracked; sometimes there is also an underlying malformation because the dentin is malformed.
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Dentinogenesis imperfecta is an autosomal dominant inherited structural disorder or aberration of the dental ambitions; One in 8, 000 people is affected by Dentinogenesis Imperfecta. Dentinogenesis imperfecta is also referred to as Capdepont syndrome and is divided into three types:

  • Type I (also known as Shields DI Type I) manifests itself in osteogenesis imperfecta (OI - also known as vitreous bone disease) and sometimes results in progressive hearing loss occurring over time.
  • Type II (also known as Shields DI type II or hereditary opalescent type) is a genetic bias that can also occur as osteogenesis imperfecta.
  • Type III (shell teeth type or brandywine type) is comparable to type II and was documented for the first time in Brandywine (USA).


The cause of the development lies in the development phase of the teeth; a gene plays the essential role. This means that the predisposition of a Dentinogenesis imperfecta is also inheritable. Since the development of the hard tooth substance is an exchange of the cells, which has to do with dentin, there is a dentin deficiency, so that an increased water retention in the enamel can be observed.

Due to this condition, it subsequently comes to a tooth malformation. However, the malformations are visible only after breaking through the teeth. The malformations occur not only in the permanent teeth, but can already be observed in the deciduous dentition.

Symptoms, complaints & signs

As part of Dentinogenesis Imperfecta, the teeth have a mild bluish discoloration. The enamel is missing or cracked; sometimes there is also an underlying malformation because the dentin is malformed. The structure of the dentin is abnormal and also has an irregular arrangement. Due to the abrasion caused by the chewing, the enamel is subsequently destroyed.

The dentin is then exposed and "chewed" over time. Using radiographs, the physician can detect a significantly reduced contrast of the tooth tissue. Sometimes the physician also notes a shortening of the tooth roots; another indication that it is a dentinogenesis imperfecta. The pulp cavities and also the root canals are closed by dentin; However, if there is still a residual pulp, no so-called secondary dentin can be formed.

This is because no odontoblasts are available. As a rule, there is no pulp. The missing pulp also explains the absence of pain; even if the dentin was chewed to the gums and normally that condition would cause tremendous pain. If the Dentinogenesis imperfecta remains untreated, the tooth can actually be "chewed" to the gums.

Diagnosis & History

The physician makes the diagnosis on the basis of the disease history of the person concerned and the clinical examinations and radiological findings. However, it is - until not molecular genetic tests were carried out - for pure suspected diagnosis. Only after the genetic test can a backup diagnosis be made that there is actually a Dentinogenesis imperfecta.

If Dentinogenesis imperfecta is treated, the quality of life can certainly be increased; the prognoses vary depending on the type and extent of Dentinogenesis imperfecta, but are entirely positive. If the affected person leaves the Dentinogenesis imperfecta untreated, the condition of the teeth deteriorates. It is important that the teeth are filled with fillings, crowns and superstructures.

So it is possible that the teeth also get a "normal look". It is important that a doctor is contacted, who sometimes has experience in the treatment of Dentinogenesis imperfecta. There are certainly dentists who refuse the treatment of Dentinogenesis imperfecta patients.


Due to the dentinogenesis imperfecta it comes in most cases to complaints in the mouth. The patient suffers from severe malformations and a degradation of the enamel. The dentin can even break down to the gums and thus lead to complications in the mouth. The tooth roots are shortened in the patient and can even be closed.

If the dentin is actually chewed to the gums, it causes extreme pain. Due to the pain, the person concerned is no longer able to accept normal food intake and fluid intake, which leads to severe restrictions in everyday life. In many cases, patients also suffer from dehydration and weight loss. The pain can also lead to mental discomfort and depression. Often the patients are irritable and slightly aggressive.

The treatment of Dentinogenesis imperfecta is aimed primarily at minimizing abrasion. This avoids complete destruction. As a rule, cosmetic treatments are also necessary so that the affected person feels comfortable and does not suffer from inferiority complexes. The teeth are filled with a filling material. Furthermore, there are no further complications. In the worst case, the patient may suffer a tooth failure without treatment.

When should you go to the doctor?

If a slight bluish discoloration of the teeth is noticed, this indicates a dentinogenesis imperfecta. A visit to the dentist is recommended, if this blue color does not return by itself after a few days or if further complaints are added. In the further course, the hereditary disease manifests itself, for example, as visible redness and inflammation. For these symptoms, a doctor must be consulted immediately, clarifying the cause and if necessary treated directly.

At the latest, when pain sets in, an immediate visit to the doctor is indicated. Patients already suffering from other dental problems are particularly prone to dentinogenesis imperfecta. These should visit the dentist regularly and have signs of a disease clarified immediately.

People who have already been diagnosed with the disease but have not yet started have regular check-ups in the dental office. Occasionally, Dentinogenesis imperfecta may be treated before it breaks out. Patients should talk with the dentist about the possibilities of such early treatment.

Treatment & Therapy

In Dentinogenesis imperfecta, the main aim of the treatment is to minimize abrasion as much as possible. Only then can the teeth be rehabilitated, if already an enormous wear phenomenon is visible. Also, the aesthetics can be optimized; Any other options are preventive, so that no consequential damage occurs. The molars are usually provided with dental crowns.

This reduces wear on the teeth and the lower and upper jaws can be stabilized. The physician places stainless steel crowns on the back teeth; the anterior teeth are subsequently treated with a filling material. Above all, treatment with permanent teeth filler is essential.

At the beginning of the treatment, however, the physician also makes sure that he treats any carious defects. Tooth decay is eliminated by means of dental fillings. Furthermore, regular inspection visits must be carried out so that further measures can be taken in the event of any changes or deterioration, so that the physician can prevent consequential damage or tooth failure.

Outlook & Forecast

Dentinogenesis imperfecta is not curable. Despite intensive dental treatment, the wear of the teeth progresses. By rudimentary tooth roots also the tooth mobility is increased. Finally, the structural anomalies of teeth often lead to edentulousness at an early age.

The classical treatments can only delay the destruction of the teeth. In order to minimize the extreme signs of wear, the teeth must be protected by crowns. Since there is no connection of the enamel to the dentin, the lowest stresses on unprotected teeth cause the enamel to flake off.

Another problem arises from the yellow-brown to blue-gray discoloration of the teeth. The affected patients also suffer from considerable mental pressure in addition to the functional disorders of their teeth. In today's society, the aesthetics have a high priority. Patients suffering from dentinogenesis imperfecta do not fit into the generally accepted well-groomed appearance.

This often leads to social exclusion. So sufferers pull back often. They suffer from the general expectation of society, which only accepts an aesthetic overall picture. As a result, mental disorders may develop, often leading to depression and suicidal thoughts.

With the classic treatment methods, however, the signs of wear, discoloration and the loss of teeth can not be stopped permanently. From a perspective perspective, only full dentures can stabilize patients' self-esteem.


Dentinogenesis imperfecta can not be prevented. This is because it is a genetic disease. There are no favorable or preventive measures to prevent Dentinogenesis Imperfecta. It is important that patients with a Dentinogenesis imperfecta be treated early or regular medical check-ups take place.


Dentinogenesis imperfecta does not provide direct follow-up because it is a hereditary disease. It must therefore be treated symptomatically, as a causal treatment is not possible. The person affected by Dentinogenesis imperfecta, however, must always protect his teeth and avoid sweet foods as much as possible, so as not to promote the development of tooth decay.

Life expectancy is usually not adversely affected by dentinogenesis imperfecta. In case of pain and other unpleasant oral complaints, the person should always see a doctor. Similarly, very warm and very cold food and drinks should be avoided so as not to irritate the teeth in addition.

A healthy diet generally has a very positive effect on the course of dentinogenesis imperfecta. In most cases, the disease is treated by oral surgery. Furthermore, the teeth must be maintained regularly.

In the case of mental health problems due to aesthetic limitations, visiting a psychologist is also recommended to avoid depression and other mental disorders. Especially in children, an early diagnosis and treatment have a positive effect on the further course of the disease.

You can do that yourself

Dentinogenesis imperfecta must always be treated by a doctor. Accompanying dental therapy, some self-help measures as well as various household and natural remedies can promote dental health.

First, care must be taken to ensure good dental hygiene. The teeth should be brushed at least three times a day with a medical toothpaste and flossed daily. Furthermore, a dentist should be consulted regularly, who can control the progression of the disease and respond quickly to complications.

If consequential damage has already developed or the teeth have even failed, not much can be done. Specialist treatment is essential. Until this occurs, irritants should be avoided to prevent inflammation of the necks and oral mucosa.

With a Dentigonesis imperfecta should be paid in principle to a healthy, sugar free as possible. All substances that could irritate the gums and teeth should be avoided. Teeth grinding and similar acts of coercion should be restricted as far as possible, because this will increase the symptoms in the long run. In case of doubt, a therapeutic consultation is useful.

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