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A Dental Guide to the

Ectodermal Dysplasias


DEDICATION This booklet is dedicated to the memory of Robert Michael Eisenberg, D.D.S., and all dentists like him, who care deeply, who share ceaselessly, and who love endlessly. L. Stefan Levin, DDS, MSD, whose wisdom and humor enriched our lives. Nancy Esterly, MD, who continues to set an unparalleled example of excellence. Virginia P. Sybert, MD, who is the quintessential pediatric dermatologist.

Copyright by NFED 1986 1st Revision 1990, 2nd Revision 1999, 3rd Revision 2006, 4th Revision 2015 This book was authored by the National Foundation for Ectodermal Dysplasias Scientific Advisory Council.


INTRODUCTION The ectodermal dysplasias are a group of inherited disorders that involve defects of the hair, nails, teeth, skin and sweat glands. However, the ectodermal dysplasias are a remarkably diverse group of human disorders, so that other parts of the body may be affected also. The many types of ectodermal dysplasias are recognized by the combination of physical features that an affected person has and the way in which they are inherited. There are more than 180 different syndromes.

SPECIAL NOTE Information on treatment is suggested in this booklet as a guide to what might be expected or recommended by medical professionals who are consulted by our families. It is important for you to understand that the statements are generalizations about a large number of the ectodermal dysplasias and no absolute recommendations are made or implied. Trust the clinicians whom you consult to do the right things, but also be an advocate for yourself or your child. Your medical professional may appreciate a copy of this booklet in order to increase his or her awareness of the ectodermal dysplasias.

The mention of any “brand name� drug in this booklet is presented as an example only and must not be interpreted as endorsement of a specific product or brand by the National Foundation for Ectodermal Dysplasias (NFED) or its Scientific Advisory Council.


DENTAL PROBLEMS Since each ectodermal dysplasias has a specific pattern of dental problems, it is helpful to know what syndrome you or your child has to understand the dental needs. The problems that may exist include unpredictable patterns of tooth eruption and loss, widely spaced teeth, poorly shaped teeth, cavity prone teeth, and missing teeth.

IMPORTANCE OF DENTAL TREATMENT Meeting the dental needs of people affected by ectodermal dysplasias is as important as meeting their medical needs. Proper dental treatment has several advantages. It allows you to eat a wide variety of foods, improves

a smile, and creates age appropriate facial appearances. Tooth replacement also improves speech. Sounds such as “ch, th, and sh� are easier following dental treatment, making words more easily understood. Dentures, for those who need them, may stimulate tooth eruption when tooth buds are present. Most importantly, dental treatment has a positive effect on the psychological and emotional well-being of children affected by ectodermal dysplasias. The improved appearance resulting from the treatment helps children to be accepted by their friends, families, and others.


BEGINNING DENTAL TREATMENT Consult a dentist as soon as a diagnosis of an ectodermal dysplasia is suspected or confirmed. The early consultation provides an overview of treatment options and introduces children to the dental office in a non-threatening situation. Although treatment may not be necessary in infancy, it is important to develop the habit of dental care and visits to the dentist. Most children can begin treatment for tooth replacement at three years of age. However, just as with many other aspects of child development, there are children who are ready earlier and some who are not ready until a year or two later. A child, who is successfully out of diapers, at least during the day, usually has the emotional and behavioral maturity to begin treatment.


Parents must understand that although the making of dentures takes place in the dental office, learning to wear them, learning to eat, and learning to be comfortable with them occurs at home. You must be as ready as the child to undertake dental care. In some instances, certain children will not be emotionally or developmentally ready to accept some types of treatment. This can be especially true when treating the very young

child. In such cases, it might be advisable to delay some elective aspects of care until the child is older.

CHOOSING THE RIGHT KIND OF DENTIST Any dentist with appropriate training, interest, or experience can provide treatment for people affected by ectodermal dysplasias. A general practitioner


can treat most dental problems of adults affected by ectodermal dysplasias and may choose to treat children as well. A pediatric dentist, however, has been uniquely trained to deal with children’s behavior and their special dental needs. In instances where the restorative dental needs are complex, a dentist trained in the specialty of prosthetic dentistry, may be consulted. If your family dentist feels this would be helpful, he or she will refer you to a prosthodontist. When contacting a dentist for the first time, ask whether he or she has had experience in treating patients affected by ectodermal dysplasias or is interested in treating people with special needs. One of the best ways to locate a dentist is to talk to families who have had good experiences and successful treatment.


the right clinician for your needs.

Do not be discouraged if none of the dentists you contact has had experience treating a patient affected by ectodermal dysplasias. A clinician’s desire to provide care can offset a lack of treatment experience.

The key to establishing a good professional relationship is to find a dentist who will listen to your concerns and requests, answer your questions, explain in a way you can understand what the plan of treatment is, and has a good relationship with the child.

Even though you may have ideas about the treatment you feel is necessary, there may be very good reasons for delaying treatment or changing the treatment to During the initial call be provided. If the dentist or visit, you must tell cannot explain in an the dentist about the understandable manner diagnosis and describe why a certain course of as best you can what the treatment is necessary, special needs are. Do or why changes in plans not be discouraged by need to be made, do not a dentist who says that hesitate to get a second treatment cannot be done. opinion about treatment Ultimately, you will find options.


FINDING A DENTIST If a dentist in your hometown cannot provide dental treatment, you may need to travel to a major metropolitan area to see a specialist or to a dental school or medical center. Some community hospitals have dental departments. Dentists in local areas may, in fact, make referrals to one of these centers for advice on how to proceed with treatment. Also, the NFED maintains a database of dentists that affected individuals or their families have referred to us.

PREPARING YOUR CHILD FOR DENTAL TREATMENT Most public libraries have children’s books on “the first visit to the dentist”. Reading such books to the child can be very helpful. Ask the dentist who will be providing treatment what his or her preference is in preparation for the first visit. Meanwhile, talk to the child about the need for healthy teeth, the effect of treatment on appearance, and the variety of foods that can be eaten after treatment. If anyone in the family wears dentures, ask if the child can watch them put the dentures in or take them out and clean them. We have published a wonderful children’s photo book,


Carver’s New Smile, about a boy affected by ectodermal dysplasias and the process he goes through to get new dentures.

FIRST VISIT The activities of the first visit depend on the patient and dentist. For very young children, the first visit or two may be “get acquainted� sessions. The dentist will talk to the child about special needs. The child has the opportunity to become comfortable

with the dentist and the office. For older children, the first visits may be more comprehensive and include a thorough examination of the mouth, x-rays, photographs and impressions for molds of the jaws. Because the oral structures and teeth of the child are growing and constantly changing, some of the early treatment may be temporary and have to be changed as the child grows.


Follow-up observation of treatment is essential since the jaws change with growth, new teeth may erupt and temporary treatment may need to be made permanent. Most permanent treatment cannot be completed until growth is complete. The length of the follow-up and intervals between appointments can be judged best by the dentist providing treatment.

AN UNCOOPERATIVE CHILD Children may fear initial visits to the dentist just as they may fear any new experience. The dentist and staff will do their best to relieve this fear. Do not be alarmed if your child cries before, during or after a visit. Crying or fussing is often used to gain sympathy from parents rather than to express discomfort. Dentists know this and may even ask parents to remain outside the room during treatment. Behavior problems should disappear as rapport is established. If problems persist, you may have to do treatment in small stages, delay it for a short period or have to get a referral to another dentist.

TREATMENT OPTIONS Complete dentures can be made for people of all ages. For individuals with some teeth, partial dentures, using existing teeth for support, can be made. For some people, over-dentures can be made to fit over existing teeth. Whatever type of denture is chosen, it is important to maintain natural teeth to preserve the bones of the jaws which serve as foundations for dentures.


For people with cleft palates, dentists can make special dentures, which cover any holes in the mouth. It is essential that dentures fit properly. Consider changes in the size and shape of the denture as children grow. If upper and lower dentures are needed, make both at the same time and wear them together. You can also wear a fixed bridge to replace missing teeth. Fixed bridges are cemented to natural teeth on either side of the spaces caused by missing teeth and are not removable. Fixed bridges made of plastic may be considered for temporary

treatment. Metal bridges should be selected for permanent treatment. Other techniques, including bonding, can be used to replace missing teeth or alter the shape of natural teeth. Crowns (caps) can be used to restore teeth whose enamel is defective. They can also be used to change the color, shape and contour of natural teeth. Orthodontic treatment (braces) may be necessary to straighten or realign teeth. Oral surgery may be necessary to remove teeth that fail to erupt, are severely malformed, cannot be used to support dentures


or bridges, or when dental implants are needed to support dentures or bridges. The use of dental implants has gained acceptance in the treatment of ectodermal dysplasias. Almost all implants placed today are termed endosseous implants as they are placed into the bone. Once the implants are placed, they are used to support a fixed denture or bridge, or a removable complete or partial denture. In some situations a bone graft may be necessary to provide sufficient bone for implant placement.

DENTAL TREATMENT COSTS The cost of dental treatment for ectodermal dysplasias can be considerable. Always ask for alternative treatment plans to consider. Treatment plans may vary in cost and that is because the more complicated a plan becomes, the more expensive it will be. Remember that there are care options for everyone. The improvements in appearance, speech and function outweigh the cost. Many NFED families have utilized medical insurance benefits to pay for their dental treatment. This can be a lengthy process that takes determination but can be successful and worth the effort. We can assist you with the steps in this process.



worn by adults should be relined or remade every four to seven years. Information regarding school age children tends to indicate that removable appliances need to be remade every two to three years.

PROBLEMS TO EXPECT The need for replacement of dentures is determined for children largely by changes in oral structures caused by growth and tooth eruption. Several sets of dentures may be necessary from infancy to adulthood. For adults, tissue response to the dentures and changes in health determine the replacement schedule. The schedule for replacement differs from person to person and is best determined by the dentist providing treatment. Some studies have concluded that removable dentures

There are many problems associated with dentures. If dentures are lost or broken, they must be replaced or repaired by a dentist. If dentures cause sore spots, a dentist must adjust the fit. If dentures affect speech, special efforts in making sounds must be made to accommodate the appliance or a dentist may have to adjust the dentures. Some people with dentures experience increased saliva flow or gagging while inserting or wearing the dentures. Discuss these problems and others with the dentist.


MY CHILD WON’T WEAR DENTURES The first step in dealing with a child who will not wear dentures is to determine the reason. With your dentist, determine that the dentures fit, are comfortable and are functional. Determine whether the child refuses because of fears such as swallowing the dentures, breaking them while eating or looking different with dentures. It may be useful to have the child wear the dentures for short periods of time, praising the appearance and clearer speech produced by the dentures and discussing the new types of food that can be eaten once he or she learns how to chew properly. You can do any number of things, including positive reinforcement, to help the child want to wear the dentures. Keep a record book of the number of hours per day that the child wears the dentures. Provide special treats when set goals of wear are reached. Compare before and after pictures. Whatever you do, be positive about the advantages of the dentures and be firm in demanding that the child wear them. If the child complains of a sore spot, contact the dentist immediately to minimize the child’s discomfort and annoyance with the dentures. Help the child realize that the advantages of wearing dentures far outweigh the disadvantages. Once the child learns that the dentures help with eating, speaking, and appearance, it is hard to get the child to give them up even for short periods that might be needed for adjustments and repairs.


EATING WITH DENTURES Chewing with dentures is a learned skill which takes practice. People with dentures, especially children, should modify their diets until they are accustomed to eating. Chewing small bites of soft food on both sides of the mouth at the same time helps people adjust to their new teeth. Gradually, introduction of more and harder foods will build confidence

in chewing and eating. Remember, dentures are artificial appliances and will never function as natural teeth. Accommodation is necessary, but with time and effort a normal diet may be possible.


RECOMMENDED HOME CARE While adults must be responsible for their own care, if you are parent of an affected child, you must assume responsibility for their dental care in the home. You must keep the natural teeth, dentures, bridges and crowns immaculately clean. Use a good quality toothbrush to clean natural teeth, their supporting tissues and appliances that are not removable. Use a soft toothbrush to clean outside and inside of the dentures or other appliances that are removable. Denture toothbrushes and pastes are suitable for such cleaning. Use denture soaking solutions, such as Polident or Efferdent or a dilute solution of white vinegar. Never use abrasive compounds, such as baking soda, home cleaners or bleach, on dentures. These products may wear down the plastic and affect the denture’s fit and appearance. Remove dentures at night to give the gums a rest. This is also a good time to soak the dentures. Use fluoride toothpaste to clean natural teeth. Parents need to


be aware of a child’s ability to brush the teeth properly. Give children the opportunity to brush their own teeth but you must be ready to check to see that brushing was done properly and brush the teeth for the child if necessary. Children under the age of six will usually need an adult to brush the teeth properly. Home fluoride treatment of natural teeth is recommended for those who wear partial dentures or over-dentures. For children wearing overdentures covering natural teeth, consult your dentist about the use of fluoride gels. You can place the gel in the denture to carry the fluoride to the teeth. Parents, be aware of the dangers of the use of a baby bottle at night and as a pacifier. By the time children are about one year old, they need to start developing fine motor skills. One way is by learning to use a cup.

Children using a baby bottle tend to take longer to drink than children using a cup. The longer it takes a child to drink, the longer the liquid in the bottle is in contact with the teeth. The common juices given to children have a high level of sugar. It is well known that the more frequently and longer teeth are exposed to these high sugar liquids, the higher the numbers of cavities children develop. Because of the dental problems children affected by ectodermal dysplasias have, there may be a tendency for parents to let the child use a baby bottle well past the first birthday. The same can be said for frequent snacking. The more frequent the use of highly sugared snacks, the greater the risk of tooth decay. Snacking between meals should be limited to a few occasions each day, such as after school. The


use of fresh fruits and vegetables as snacks should be encouraged as these are better for the child’s overall diet as well as the teeth.

DENTURE ADHESIVES Denture adhesives, such as Oroflex and Super Wernets, may help retention of dentures. If you use, completely remove the adhesive material from the dentures daily. Do not use homemade adhesives.

DENTAL IMPLANTS Dental implants are metal or ceramic devices in the shape of a screw, cylinder or blade which are placed on or into the jaw bone to anchor dentures or other replacement teeth. At least one surgical procedure is required to place implants on or into the bone. There are many commercial implant systems which incorporate different designs and materials. (You should use a system approved by the American Dental Association.)


Dentists must carefully evaluate candidates for implants to determine which system best meets their needs. For most people affected by ectodermal dysplasias, this means the installation of several screw-like fixtures into the jaw bone. This process usually requires three steps: 1. Installation of the fixtures into the bone, 2. Additions of the abutments which ultimately hold the replacement teeth, and 3. Construction and placement of a fixed bridge or denture. Because implants are a complicated procedure and require a number of visits, it is best to locate a team of doctors who have specialized training and

experience. Discuss all aspects of the treatment so that you are clear before the procedure starts.

BENEFITS OF DENTAL IMPLANTS Dental implants provide a very stable dentition, improving chewing ability, speech, appearance and selfconfidence. In some cases, dental implants also prevent shrinkage of bone supporting the implants and dentures. Most adults who have selected this type of treatment are pleased with the results.

DRAWBACKS OF DENTAL IMPLANTS Dental implants have been used for more


than 30 years and have become an important and accepted treatment for the replacement of missing teeth in adults. The long term effects of implants on children in whom facial growth and development is not complete are not known. At the present time, it is recommended that, whenever possible, the use of implants should be delayed until the major thrust of puberty (age 14 for girls and 16 for boys). The placement of implants is a surgical procedure and careful planning is required by all those involved in the patient’s care before treatment is started. While many surgical procedures on adults can be done in the office using local anesthesia, these same procedures on a child may require a general anesthetic in the hospital. The discomfort and negative impression that this surgery may have on the child needs to be considered when considering the use of implants in children. As the number of children having implants grows, many of the questions about their use will be answered.

LEARN MORE ABOUT DENTAL IMPLANTS Most dentists are able to provide information on implants or suggest other sources of information. The American Dental Association, dental schools and the National Institute for Dental and Craniofacial Research also have information.



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Profile for National Foundation for Ectodermal Dysplasias

A Dental Guide to the Ectodermal Dysplasias  

Our guide addresses the dental treatment process for the ectodermal dysplasias. We’ll go through when and where to begin, how to prepare you...

A Dental Guide to the Ectodermal Dysplasias  

Our guide addresses the dental treatment process for the ectodermal dysplasias. We’ll go through when and where to begin, how to prepare you...

Profile for nfed