Under a dentinogenesis imperfecta, the medical profession describes a hereditary disease. Dentinogenesis imperfecta subsequently leads to a malformation of the dentin, which results in the tooth enamel becoming detached and the dentin being released. Due to the abrasion caused by chewing, the dentin breaks down to the gums.
What is dentinogenesis imperfecta?
Dentinogenesis imperfecta is an autosomal-dominant inherited structural disorder or maldevelopment of tooth dentition; one in 8,000 people is affected by dentinogenesis imperfecta. Dentinogenesis imperfecta is also known as capdepont syndrome and is divided into three types:
- Type I (also known as Shields DI Type I) manifests as osteogenesis imperfecta (OI – also known as brittle bone disease) and sometimes results in progressive hearing loss over time.
- Type II (also known as Shields DI type II or hereditary opalescent type) is a genetic predisposition that can also occur like osteogenesis imperfecta.
- Type III (shell teeth type or Brandywine type) is comparable to type II and was first documented in Brandywine (USA).
Causes
The cause of the development lies in the developmental phase of the teeth; a gene plays the essential role. This means that the predisposition of dentinogenesis imperfecta is also hereditary. Since in the course of the development of the tooth hard substance an exchange of the cells is formed, which has to do with dentin, a dentin undersupply takes place, so that an increased water storage in the tooth enamel can be observed. Due to this condition, tooth malformation occurs subsequently. However, the malformations only become apparent after the teeth have erupted. The malformations occur not only in the permanent teeth, but can be observed even in the primary dentition.
Symptoms, complaints and signs
In the context of dentinogenesis imperfecta, the teeth show a soft bluish discoloration. The enamel is missing or becomes cracked; sometimes there is also a substructure 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 chewing, the enamel is subsequently destroyed. The dentin is then exposed and “chewed off” over time. By means of X-rays, the physician can see a clearly reduced contrast representation of the tooth tissue. Sometimes the physician also notices a shortening of the tooth roots; another sign that dentinogenesis imperfecta is involved. 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, however, no pulp is present. The lack of pulp also explains the absence of pain, even if the dentin has been chewed down to the gum, a condition that would normally cause enormous pain. If dentinogenesis imperfecta is left untreated, the tooth can actually be “chewed” down to the gums.
Diagnosis and progression
The medical professional makes the diagnosis based on the affected person’s medical history and clinical examinations and radiological findings. However, until molecular genetic testing has been performed, the diagnosis is purely tentative. Only after genetic testing can a confirmatory diagnosis be made that dentinogenesis imperfecta is actually present. If dentinogenesis imperfecta is treated, the quality of life can very well be improved; the prognosis varies – depending on the type and extent of dentinogenesis imperfecta – but is consistently positive. If the affected person leaves dentinogenesis imperfecta untreated, the condition of the teeth will deteriorate. Therefore, it is important to treat the teeth with fillings, sub-crownings as well as abutments. In this way it is possible that the teeth also get a “normal appearance”. It is important to contact a dentist who has experience in the treatment of dentinogenesis imperfecta. There are very well dentists who refuse the treatment of dentinogenesis imperfecta patients.
Complications
Due to dentinogenesis imperfecta, in most cases there is discomfort in the oral cavity. In this case, the patient suffers from severe deformities and degradation of the tooth enamel. The dentin can even degrade towards the gums, leading to complications in the oral cavity. The roots of the teeth are also shortened in the patient and may even be occluded in the process. If the dentin is actually chewed down to the gums, there is extremely severe pain. Due to the pain, it is no longer possible for the affected person to take in normal food and liquids, so that there are severe restrictions in everyday life. In many cases, patients also suffer from dehydration and weight loss. The pain can also lead to psychological discomfort and depression. Patients often appear irritable and slightly aggressive. Treatment of dentinogenesis imperfecta is mainly aimed 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 other complications. In the worst case, the patient may suffer tooth loss without treatment.
When to go to the doctor?
If a slight bluish discoloration of the teeth is noticed, this indicates dentinogenesis imperfecta. A visit to the dentist is recommended if this blue discoloration does not recede on its own after a few days or if other complaints are added. In the further course, the hereditary disease manifests itself, for example, through visible redness and inflammation. In the case of these symptoms, a doctor must be consulted immediately, who will clarify the cause and, if necessary, treat it directly. At the latest, if pain develops, an immediate visit to the doctor is indicated. Patients who already suffer from other dental complaints are particularly susceptible to dentinogenesis imperfecta. They should visit the dentist regularly and have any signs of the disease clarified immediately. People who have already been diagnosed with the disease but have not yet developed it are also best to arrange regular check-ups at the dentist’s office. Occasionally, dentinogenesis imperfecta can be treated before its onset. Affected individuals should talk to their dentist about the possibilities of such early treatment.
Treatment and therapy
In the context of dentinogenesis imperfecta, treatment is predominantly aimed at minimizing abrasion – as much as possible. Only in this way can the teeth be rehabilitated when a tremendous amount of wear is already visible. Likewise, the esthetics can be optimized; possible further possibilities are preventions, so that no consequential damages occur. The molars are usually provided with dental crowns. This reduces wear of the teeth and both the lower and upper jaws can be stabilized. The dentist places stainless steel crowns on the back teeth; the front teeth are subsequently treated with a filling material. Above all, treatment with filling material is essential for permanent teeth. However, at the beginning of the treatments, the dentist also takes care to treat any carious defects. Caries is eliminated by means of dental fillings. Furthermore, regular control visits must be perceived, so that in the event of any changes or deterioration, further measures can be set that the medical professional can prevent consequential damage or the failure of the teeth.
Outlook and prognosis
Dentinogenesis imperfecta is not curable. Despite intensive dental treatment, tooth wear progresses. In addition, tooth mobility is increased due to rudimentarily formed tooth roots. Finally, the structural abnormalities of the teeth often lead to edentulism at an early age. The classical treatment measures can only delay the destruction of the teeth somewhat. To minimize the extreme signs of wear, the teeth must be protected by crowns. Since there is no connection between the enamel and the dentin, the slightest stress on unprotected teeth causes the enamel to flake off. Another problem results from the yellow-brown to blue-gray discoloration of the teeth. In addition to the functional disorders of the teeth, the affected patients also suffer from considerable psychological pressure. In today’s society, aesthetics has a high priority.Patients suffering from dentinogenesis imperfecta do not fit into the generally accepted well-groomed appearance. This often leads to social exclusion. Thus, those affected often withdraw. They suffer from the general expectations of society, which only accepts an aesthetic overall appearance. As a result, psychological abnormalities can develop, often leading to depression and suicidal thoughts. However, the signs of wear, discoloration and loss of teeth cannot be stopped in the long run with the classical methods of treatment. In perspective, only a complete tooth replacement can stabilize the patient’s self-esteem again.
Prevention
Dentinogenesis imperfecta cannot be prevented. This is because it is a genetic disease. There are neither favoring nor preventive measures so that dentinogenesis imperfecta can be prevented. It is important that patients with dentinogenesis imperfecta receive early treatment or regular check-ups.
Follow-up
Direct follow-up is not possible for dentinogenesis imperfecta, as it is a hereditary condition. It must therefore be treated symptomatically, as causative treatment is not possible in this case. However, those affected by dentinogenesis imperfecta must always take care of their teeth and avoid sweet foods as much as possible so as not to promote the development of caries. Life expectancy is usually not negatively affected by dentinogenesis imperfecta. If pain or other unpleasant oral discomfort occurs, the affected person should always consult a doctor. Likewise, very warm and very cold foods and drinks should be avoided in order not to further irritate the teeth. A healthy diet generally has a very positive effect on the course of dentinogenesis imperfecta. In most cases, the disease is treated by surgical intervention in the oral cavity. Furthermore, the teeth must be cared for regularly. In case of psychological discomfort due to the esthetic limitations, a visit to a psychologist is also recommended in order to avoid depression and other psychological upsets. Especially in children, early diagnosis and treatment have a positive effect on the further course of the disease.
What you can do yourself
Dentinogenesis imperfecta must be treated by a doctor in any case. Accompanying dental therapy, some self-help measures as well as various household and natural remedies can promote dental health. First of all, care must be taken to maintain good dental hygiene. Teeth should be brushed with a medicated toothpaste at least three times a day and flossed daily. Furthermore, a dentist should be visited regularly, who can check the progression of the disease and react quickly in case of complications. If secondary damage has already developed or the teeth have even fallen out, there is not much that can be done by the patient. Specialist treatment is then absolutely necessary. Until this takes place, irritating substances should be avoided in order to prevent inflammation of the tooth necks and the oral mucosa. With dentigonesis imperfecta, attention should generally be paid to a healthy diet that is as sugar-free as possible. All substances that could further irritate the gums and teeth should be avoided. Teeth grinding and similar compulsive actions should be restricted if possible, as this will aggravate the symptoms in the long term. In case of doubt, therapeutic counseling is also advisable.
