Dentinogenesis imperfecta

Dentinogenesis imperfecta is one of the many rare diseases associated with the mouth. Learn more about this condition, including its causes, symptoms and treatment.

Dentinogenesis imperfecta Definition

Generally speaking, this is a condition characterized by the abnormal formation of the dentin. It is usually inherited and is clinically heterogeneous. It may affect the teeth portion alone or can also be associated with Osteogenesis imperfecta.

The disorder was discovered by Barret as early as 1882. However, its name was coined only in 1939 by Robert and Schour.

Types of Dentinogenesis imperfecta

The condition is classified into various types, each of which has its own prominent characteristics:

Picture of Dentinogenesis imperfecta

Picture 1 – Dentinogenesis imperfecta

Dentinogenesis Imperfecta Type I

This is rare condition similar to Osteogenesis imperfecta, also known as Brittle Bone Disease.

Dentinogenesis Imperfecta Type II

This condition occurs in only 1 out of every 8000 births. The disorder may have a small incidence but has resulted in various problems and inconveniences.

Dentinogenesis Imperfecta Type III

This is a very rare condition that is typical to the triracial Brandywine population of Maryland. It is only not connected with other inherited diseases like osteogenesis imperfecta.

Dentinogenesis imperfect Symptoms

Know about the symptoms of each type of this condition:

Symptoms of Dentinogenesis imperfecta Type I

It is characterized by several tooth structure abnormalities, some of which include:

  • Bluish-gray teeth
  • Bulbous teeth crowns
  • Absent root canals
  • Absent root tooth
  • Amber colored teeth
  • Misaligned teeth
  • Recurring dental abscess
  • Blue sclera
  • Enamel separation from the ivory
  • Very small pulp chambers
  • Very small roots of the tooth
  • Very small canals of the tooth

Symptoms of Dentinogenesis Imperfecta Type II

It reveals very similar features present in Type I dentinogenesis imperfecta but pulp chamber obliteration which is prominent even prior to tooth eruption. It also reveals an abnormal crown and root morphology under radiographic evaluation. Moreover, it is not in association with osteogenesis imperfecta. Other specifics are as follows:

  • Thickened dentin
  • Resulted obliteration of the pulp chamber
  • Discoloration of the primary dentition producing yellow, brown, or opalescent gray color

Symptoms of Dentinogenesis Imperfecta Type III

Similarly though, this type of Dentinogenesis imperfecta exhibits pale-colored and lustrous teeth. It is awkwardly formed and is situated in the gums where it easily wears and breaks away easily. It is also called Brandywine symptom because it is interestingly distinct in southern Maryland.

Dentinogenesis Imperfecta Causes

Know about the causes of various forms of this condition.

Causes of Dentinogenesis Imperfecta Type I

The main cause of this condition is collagen gene mutation. It arises as a part of Osteogenesis imperfecta, resulting from mutations in any of various types of genes.

Causes of Dentinogenesis Imperfecta Type II

Research shows that the condition is a result of an autosomal dominant Mendelian trait that has a 100% penetration. The gene responsible for this condition has been revealed to be Dentin sialophophoprotein or DSPP. It has been located to chromosome 4121.3 and is a precursor protein cleaved into two dentin-specific proteins namely dentin phosphoroprotein (DPP) and dentin sialoprotein (DSP). However, the heterogenecity is still unknown.

As a clue, dentists often resolve this condition by looking at the pedigree of the family to see if any previous family member, suffered from the same condition. Since the gene has complete penetration, a recurrence within the family tree confirms the diagnosis of Dentinogenesis imperfecta.

Causes of Dentinogenesis Imperfecta Type III

It results from mutations occurring in the DSPP (Dentin sialophosphoprotein) gene that is hereditarily acquired in an autosomal dominant manner. The DSPP encodes two main proteins of the dentin extracellular matrix of the tooth.

Dentinogenesis Imperfecta Treatment

Dental treatment of this condition varies, depending on its severity. Patients have to be serious about their oral hygiene if they wish to preserve most part of their teeth. It is typical for dentists to conduct a thorough dental evaluation of the condition before conducting any treatment. The fragility of the jaw and the bone has to be analyzed to check if it is capable of hosting restorative dental treatment. Surgical correction is often necessary due to the autosomal recessive nature of this tooth condition. The maximum that dentists can do is to provide restorative treatment so that patients can still flash a normal smile and interact with others without inhibitions. Nevertheless, it may be more difficult to have all of the teeth preserved and protected from possible damage, especially if it is linked to Dentinogenesis Imperfecta Type I.

Early treatment is the best solution to help resolve this condition. This is the only solution that can possibly save your teeth from deterioration. Other benefits of early detection include:

  • Preservation of dental health
  • Preservation of form, vitality, and size of your teeth
  • Prevention of vertical dimension loss
  • Maintenance of arch length
  • Normal growth of facial bones and the tempero mandibular joint
  • Provision of functional dentition
  • Aesthetic appearance at an early age
  • Freedom from humiliation
  • Regain self confidence at an earlier stage

Treatment of this condition is very challenging. It requires a multi-disciplinary approach and is limited to highly skilled dental surgeon and dental practitioners. It often requires a collaborative approach, with an orthodontist and a prosthodontist, for the completion of the entire dental restoration and prevention procedure.

Dentinogenesis imperfecta Treatment Options

The following forms of dentinogenesis imperfecta treatment have been recommended by many experts:

  • Stainless steel crowns
  • Overdentures
  • Jacket crowns
  • Pin-retained cast gold thimbles under the acrylic resin crown

One of these or a combination of these methods can be used for the aesthetic restoration of the tooth. It is a long procedure and its success involves a lengthy healing process combined with strict adherence to oral hygiene. The patient has to be oriented with all the procedure that will be done and his consent has to be taken before any of these methods can be performed. Moreover, the financial capacity of the patient should also be checked to guarantee that all of the expenses can be truly met.

Indirect Resin Crown (IRC)

This technique helps restore the maxillary incisors and is often used in the treatment of severe enamel hypoplasia and attrition. It is preferred due to its long lasting intermediate restoration and affordability in comparison with other advanced, yet expensive, forms of treatment for this disease. This method can be used to cure all types of the disorder without posing any threat to the patient.

Treatment of Dentinogenesis Imperfecta in Children

Image of Dentinogenesis imperfecta

Picture 2 – Dentinogenesis imperfecta Image

There are published cases of treatment of this disorder in children. However, treatment involves certain problems like:

  • High cost of operation, since it involves minors
  • Possible risk, due to the application of general anesthesia
  • Technical problems in the restoration of the teeth
  • Postponement of treatment due to the need for general anesthesia application

However, early treatment of this disorder yield various advantages like:

  • Preventing possible TMJ problems and arch length complication.
  • Boosting self-esteem and self-confidence. Children affected with this disorder require socialization and deprivation of this would lead to loss of valuable time needed for them to have psychological development. Psychological problem is often the hardest thing to resolve in patients.

Early treatment of this condition among children is still the subject of an on-going debate. Nevertheless, the wishes of the parents are taken into high consideration. Their consent is enough for dentists and medical professionals to proceed with the option that they deem necessary to resolve the inconvenience felt by affected children.

Dentists require an evaluation of possible Osteogenesis imperfecta associated with the child’s condition as it means pursuing a different treatment approach.

Dentinogenesis Imperfecta Research Organizations

There are many research organizations dedicated to the understanding of this rare condition, which include:

Osteogenesis Imperfecta Foundation

This organization is concentrated in providing information about Osteogenesis imperfecta. It is a rare disease that is not known to many, but is often associated with Dentinogenesis imperfecta type I. It supports researches and individuals suffering from this condition cope up with their daily struggles.

National Institute of Dental and Craniofacial Research

This research group aims to help improve the dental health of the American population and the world through the efforts of their research team. It is not entirely involved with the disorder but all other diseases associated with the mouth and the craniofacial features of the body.

MUM National Parent-to-Parent Network

It provides the much-needed support to parents of affected children. The organization also offers alternative health solutions that can be used for the assistance of your child.

 

Although Dentinogenesis imperfecta has not been fully understood yet, researchers and medical communities are striving constantly to make lives better for patients of this disease and their family members.

References:

http://ghr.nlm.nih.gov/condition/dentinogenesis-imperfecta

http://rarediseases.info.nih.gov/GARD/Condition/6258/Dentinogenesis_imperfecta_1.aspx

http://www.nacd.in/ijda/volume-02-issue-02/52-dentinogenesis-imperfecta-hereditary-opalescent-dentin

http://www.rightdiagnosis.com/d/dentinogenesis_imperfecta_type_i/intro.htm

 

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