Ectodermal Dysplasia

What is Ectodermal Dysplasia?

Ectodermal dysplasia is not a single disorder, but a band of closely related genetic disorders of which there are more than 175 different syndromes that have been identified to date. These conditions are known to affect the development of ectodermal organs such as teeth.

While the relative incidence of ectodermal dysplasia in the general population is relatively small, affecting 7 per 10,000 individuals, the condition is overwhelmingly prevalent in Caucasians, mainly Caucasians with very fair complexion.

Ectodermal Dysplasia Teeth

Many with ectodermal dysplasia are first diagnosed by their prosthodontist or dentist. The disorder affects many aspects of a person’s body and development, including hair, nails, sweat and salivary glands, facial features, as well as fingers and toes. One of the affliction's most noticeable traits is misshapen, cone or peg-shaped teeth that are abnormally spaced.

Ectodermal Dysplasia Causes

Ectodermal Dysplasias are directly caused by altered genes. These altered genes are often inherited or common genes that are defective (mutate) at the time of conception. The prospects of parents conceiving children with ectodermal dysplasia depend largely on the type of ectodermal dysplasia that exists in the family history.

It is important to remember that a person cannot choose or modify the genes that he or she has and the events surrounding a pregnancy do not change the genes one way or the other. Therefore, parents who have a child with ectodermal dysplasia should not think that they did anything to cause the defective gene and should not blame themselves for its existence. There is genetic counseling that is available for families or couples as well as new technology that can reveal unique family history with a simple DNA test.

One prevalent type of ectodermal dysplasia that affects males more than females is the X-linked form of hypohidrotic ectodermal dysplasia. There are other kinds which can affect males or females equally and can be inherited in various ways.

Prenatal diagnosis is available for some families with X-linked hypohidrotic ectodermal dysplasia, through the use of DNA probes. This is not possible for all families.

Other dental signs of ectodermal dysplasia include:

  1. Absence or malformation of one or more fingers or toes
  2. Frequent infections due to immune system deficiencies
  3. Impairment or loss of hearing
  4. Sensitivity to light
  5. Absence of hair growth
  6. Anodontia, or hypodontia, and associated underdevelopment of the alveolar ridges at the back of the mouth
  7.  Taurodontism of deciduous molars
  8. Sparse and limited permanent teeth formation
  9. Weak jaws
  10. Malformed and mal-occluded teeth
  11. cleft lip and/or palate
  12.  Irregular skin
  13. The absence of lingual frenulum or tongue web.

Ectodermal Dysplasia Treatment

Since many aspects of the teeth and mouth are related to ectodermal development, prosthodontists and dentists are often the first medical professionals to make an accurate diagnosis. They also are instrumental in the treatment and management of the effects of the condition including:

  1. Reshaping
  2. Realigning teeth
  3. Treatments for other Maxillofacial abnormalities

Due to a wide range of dental, orthodontic, and prosthodontic solutions available today that address both functional and aesthetic concerns, it is possible for a person suffering from ectodermal dysplasia to receive dental treatments that can dramatically improve and enhance their quality of life.

Treating the Dental Effects of Ectodermal Dysplasia

Due to the negative aesthetic and functional impact on a person's teeth and jaws, dental solutions play a critical role in ensuring this individual affected by ectodermal dysplasia can live a normal and happy life.

Dental management techniques may involve the employment of cosmetic surgery to correct certain deformities and the use of prosthetic dentures to address aesthetic and social issues.

An effective strategy for treating the dental and maxillofacial effects of ectodermal dysplasia should address four key areas of concern:

  1. Improving the ability to chew, eat and swallow.
  2. Managing the development of a young patient's mouth and facial structures.
  3. Improving speech functions
  4. Improving overall aesthetics for a beautiful smile.

4 Important Dental Challenges to Address for Children and Adults with Ectodermal Dysplasia

1. Eating, Chewing, Biting, and Swallowing

The ability to efficiently and comfortably eat, chew, bite and swallow is essential for basic survival, nutrition, and independent life.

Therefore, dental treatments and therapies should directly improve these practical concerns. In some cases of complete edentulism, drastic prosthodontic interventions may be necessary.

Treatment plans could include the fabrication of custom overdentures for both upper and lower jaws to simultaneously improve function and aesthetics. Remember treatment plans for treating ectodermal dysplasia should be developed in conjunction with a certified prosthodontist, particularly if young children are involved. It is critical that a child’s dental and facial development is guided, monitored, and if need be, gently corrected by a professional to ensure that he or she grows up healthy, strong, and beautiful.

2. Growth and Development Management

Many with ectodermal dysplasia are first diagnosed by their dentist or prosthodontist. Because the condition also significantly impacts the mouth and teeth, dentists and prosthodontists can and should play a critical role in the care and management of patients with ectodermal dysplasia.

As a person grows and develops from a child at the time of the initial diagnosis to an adult, their bodies will experience many physical changes. As a result, people with ectodermal dysplasia must often work very closely with a prosthodontist to ensure that static prosthesis and other dental implements are properly sized and fitted, functioning correctly, comfortable, and appealing aesthetically.

Age is also an important consideration, and a prosthodontist will know which age-appropriate solutions are best based on the patient’s particular needs.

A removable prosthesis, for example, is recommended for very young children who will likely outgrow their prosthetic teeth in 6 months or less. Meanwhile, for children older than 12, a more permanent implant-supported denture may be appropriate.

Remember, the key to effective management of the dental concerns caused by ectodermal dysplasia is to work closely with your prosthodontist.

3. Improving Speech Functions

Speech and the ability to enunciate and articulate words and sounds is another important aspect of life that is significantly impacted and diminished by ectodermal dysplasia.

Edentulism, mal-occluded and misaligned teeth, cleft palate, and other deformities of the mouth, jaws, gums, and teeth can significantly hinder a person’s capacity for communication. Correcting dental, face, and mouth issues aggressively and early on will give the patient the opportunity to speak normally, freely, and without fear of embarrassment or social anxiety.

4. Providing a Beautiful Smile

One of the biggest concerns for patients with ectodermal dysplasia and their families is how the disease will affect the way they look, and as a result the way others may view them. This can be a challenging situation for children as well as adults with ectodermal dysplasia who must learn to the navigate the social sphere of their lives with the condition that is not well known and may cause social anxiety, embarrassment, or worse.

That’s why treating the aesthetic concerns and providing a beautiful, natural-looking smile is so important. Patients and their families want the opportunity to live as healthy and normal a life as possible. Modern day dentistry techniques, treatments, and procedures can help make that possible.

Do you have questions or concerns about Ectodermal Dysplasia? Are you looking for a prosthodontist to work closely with to provide you or a loved one with the care that they deserve? Look no further than Dr. Reza Khazaie of Willow Pass Dental Care.

Willow Pass Dental Care is regarded as one of the leading dentists in Concord, CA. We offer a broad range of dental services in a friendly and professional environment you can call home. Contact us at 925-326-6114 or schedule an appointment below.