Ectodermal Dysplasia

Ectodermal dysplasia is a group of genetic disorders that primarily affect the development and/or homeostasis of two or more ectodermal derivatives tissues, which include the skin, hair, nails, sweat glands, and teeth. The specific effects of ectodermal dysplasia can vary widely depending on the type and severity of the condition. In terms of teeth, many individuals with ectodermal dysplasia experience dental abnormalities that can affect the restorability of their teeth. Here are some key points to consider:
One of the most common dental issues in individuals with ectodermal dysplasia is hypodontia, which refers to the underdevelopment or absence of some teeth. This can result in a reduced number of teeth, making restorability more challenging.
In some severe cases of ectodermal dysplasia, individuals may be completely missing some or all of their permanent teeth, a condition known as anodontia. In such cases, traditional dental restorations like fillings and crowns may not be applicable.
Some individuals with ectodermal dysplasia may have abnormally small teeth, which can make restorations more difficult due to the limited tooth structure available.
The teeth of people with ectodermal dysplasia may have unusual shapes and sizes, which can complicate the process of restoring them to normal appearance and function.
Enamel hypoplasia is a common feature in ectodermal dysplasia. Enamel is the hard outer layer of the teeth, and its reduced development can lead to increased susceptibility to tooth decay and sensitivity.
Ectodermal dysplasia can also affect the gums and oral mucosa, potentially causing gum problems and affecting the fit and durability of dental restorations.
Despite these challenges, dental professionals can still work to restore the appearance and function of teeth in individuals with ectodermal dysplasia. This may involve various treatments, including dental crowns, bridges, dentures, dental implants, and other prosthetic solutions, depending on the specific needs of the patient.
An “expert” in ectodermal dysplasia doesn’t really exist. Because the condition is rare, most doctors or dentists typically do not see enough cases to be considered an expert or have high level of experience treating these cases. The primary care doctor, such as a family medicine doctor, internist or pediatrician, who understands your needs, can be a valuable partner for your health care. They can help coordinate your care, especially if you need to see different specialists for multiple health problems. The key is to find a doctor willing to learn about this condition, be able to use all the knowledge combine with the latest technology to provide the best treatment possible for these patients. That’s why It’s important for individuals with ectodermal dysplasia to work closely with a multidisciplinary team that will refer to different specialist, including dentist specialized in prosthodontics who has more expertise restoring patients with this condition. The treatment plan will be tailored to the individual’s unique needs and may involve a combination of different dental procedures and technologies to achieve the best possible outcome in terms of teeth restorability and overall oral health.

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Dr. Karen Clemente has devoted years to assisting families with these chronic conditions. These children undergo extensive treatment primarily during the first and second decades of their lives. Given the significant role that function and aesthetics play in this condition, Dr. Clemente serves as a key coordinator throughout the treatment process. She has embraced this responsibility as her life’s passion. Patients with ectodermal dysplasia often require frequent adjustments to their prosthetics, and in many cases, replacements as they age. Dr. Clemente has established a network of professionals, including oral maxillofacial surgeons, pediatric dentists, endodontists, orthodontists, speech pathologists, etc., to support her patients and their families.
Dr. Karen Clemente

Dr. Karen Clemente, D.D.S., M.S.

Maxillofacial Prosthodontist

Advantages Of Proper Dental Treatment

Meeting the dental needs of people affected by Ectodermal Dysplasia is as important as meeting their medical needs. Early dental treatment for the child without teeth is especially necessary. We recommend that children with missing teeth get dentures by the time they start school, but children as young as 2 1/2 can successfully wear dentures.

Benefits of Dental Treatment for Children:

  • Allows you to eat a wide variety of foods, improving nutrition
  • Improves smiles
  • Creates an age-appropriate facial appearance, helping children to be accepted by their friends, families and others
  • Improves speech – sounds such as “ch, th, and sh” are easier following dental treatment, making words more easily understood
  • Stimulates tooth eruption when tooth buds are present
  • Positively affects psychological and emotional well-being

Different Dental Treatment Options:

  • Composite Bonding Restorations
  • Complete Dentures
  • Partial Dentures
  • Dental Implants At Proper Age

Benefits of Complete Dentures for Young Children:

  • Improved jaw development and tissue development around the mouth.
  • Improved esthetics: Dentures will create an age-appropriate facial appearance. The child does not have the “old man” appearance resulting from over closure of the jaws.
  • Improved social well-being: the child becomes happy and smiles instead of frowning, for the most part he looks like his peers. He is not afraid to interact with other children.
  • Improved psychological well-being: the child will feel better about himself and as a result will exhibit more self-confidence. Small children affected with an obvious physical deformity, such as the lack of teeth, run the risk of low self-esteem.
  • Improved mastication: The child will function better with teeth than without teeth and quite possibly will not be affected with some of the digestive disorders which may be associated with having no teeth.
  • Improved speech development: Speech will be much improved resulting in an increased vocabulary and communicative skills as well as interpersonal relations.
  • Psychological uplift to the entire family: By improving the child’s appearance we provide for a more normal physical development through better nutrition and psychological development through better social and emotional adjustment. The impact can be quite dramatic. The transition may not be without traumatic incidences. Problems do and will arise. But in most instances, these hurdles can be cleared through a cooperative effort between the child, parents and the dentist.
Source: Frank H. Farrington, D.D.S., M.S. Professor Emeritus Virginia Commonwealth University.
Nfed: National Foundation for Ectodermal Dysplasia.

Child Behavior

The management of child behavior in the dental office can be challenging for both the dental staff and parents. All children go through the same developmental stages, but the range of ages at which each occurs is different. Some young children may separate easily from their parents and adjust rapidly to new people, while another child, could be at 4 years-old and be more immature, which make hard the separation. Each of these children is behaving normally but at different end of the normal range of development.

Proper Age For Dental Treatment

When is the child ready for dental treatment? If dental care is needed and cannot be delayed the answer is NOW. The necessity of the needed treatment and the behavior of the child will determine how to best manage the problem. In most cases treatment for dentures is not an emergency or necessary for the child to live normally. Each child is different but as a general guideline a good time to consider denture treatment is once the child is out of diapers. This usually indicates a level of social and behavioral development at which the child is able to cooperate for denture treatment.
Source: Frank H. Farrington, D.D.S., M.S. Professor Emeritus Virginia Commonwealth University.
Nfed: National Foundation for Ectodermal Dysplasia.

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Dr. Karen Clemente Is Committed To Help Your Children And Family Walk The Journey

It Is A Life Treatment

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