Turner Syndrome & Dental Health

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Smile; October is National Dental Hygiene Month! Individuals with Turner syndrome (TS) are more susceptible to dental health problems than others. Dental problems can also slowly impact a person’s overall health. The Turner Syndrome Foundation (TSF) believes that all patients with TS deserve comprehensive care that encompasses the whole person throughout their lifetime. Let’s first look at some common dental issues affecting the general population and then learn about oral health and TS.

Healthy Teeth Lead to Happy Smiles

October is National Dental Hygiene Month, and it is time to shine a spotlight on the importance of a healthy smile. The basics of oral health are universally taught as brushing twice a day and flossing every day. These recommendations are easy to follow, and unwanted complications can arise when they are ignored. 

Proper care is especially important when it comes to pediatric dental hygiene. Baby teeth first begin to emerge by six months of age. By six to seven years of age, the first set of teeth are slowly replaced by the secondary, permanent set of teeth. With periodic dental care and education, common oral health problems can be avoided. Poor dental hygiene can slowly start to affect the overall quality of life.

The issues that cause the most concern are cavities, severe gum disease, and severe tooth loss. About one out of  five children aged 5 to 11 years (20%) have at least one untreated, decayed tooth. It is 

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estimated that oral diseases affect nearly 3.5 billion people. For an individual with TS, dental health may present a different set of concerns that require careful attention.

Dental Health & TS

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TS is one of the most common chromosomal conditions, and is estimated to affect one in every 2,000 live female births. It is a condition in which one of the X chromosomes is  completely or partially missing. 

TS can cause a variety of medical and developmental issues, and dental health can be a frequent but often overlooked issue. Professionals recommend that a dental evaluation be conducted at the time of a TS diagnosis if ongoing care has not already been established.

"Females with Turner Syndrome (TS) encounter unique challenges in creating and maintaining oral and dental health ... Because of these challenges, serving TS patients successfully requires diverse and creative solutions."

Dental Health Issues To Be Aware of

When it comes to dental health issues, people with TS can have problems with either oral development (facial bones/palate, or roof of the mouth) or dental development (teeth). Some of the more common problems are described below:

Oral Development Issues

  • Asymmetry of facial bones: Individuals with TS may experience craniofacial abnormalities, which can lead to problems with improper development of the mandible (jaw) bone, such as:
    •  Mandibular hypoplasia or micrognathia: underdeveloped or unusually smaller lower jaw;
    • Retrognathia: posterior shifting of the jaw due to either underdevelopment of the maxilla (upper jaw) or mandible (lower jaw); and
    •  Narrow maxillary alveolar arch: top arch of the teeth is narrower than usual.
  • Malocclusion and bite abnormalities include the following:
    • Malocclusion: misalignment of the upper and lower teeth;
    • Anterior and lateral open bite: tongue can stick out between the teeth when biting down; and
    •  Lateral crossbite: upper teeth fit inside the lower teeth, rather than outside.
American Journal of Orthodontics & Dexofacial Orthopedics
  • High-arched and narrow palate: “Historically, it was believed that females with TS have a ‘high-arched palate. Recent research indicates that palatal height is not affected, as a narrower dental maxillary arch with the presence of lateral palatal ridges gives the false illusion of increased palatal height” (2007 Clinical Care Guidelines).
  • Low tongue position: A greater distance between the palate and tongue has been observed in individuals with TS. This may be due to primary palatal malformation.

Dental Development Issues

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  • Hypoplastic tooth enamel/dentin decrease: Enamel is the hard, shiny, white outer layer of teeth. Since the X chromosome plays a part in enamel creation, any abnormalities can lead to thin enamel. As a result, there is a higher risk for cavities and tooth sensitivity.
  • Decreased dentin/reduced tooth size: There may be a decreased amount of dentin (the layer that lies immediately beneath the enamel and protects the teeth’s pulp). This leads to a reduced permanent tooth size in individuals with TS, particularly in the first molars, when compared to people without TS.
  • Premature and crowded teeth: According to Robert Korwin, DMD, MICOI, MAGD, and TSF Medical Advisory Board Member, “Unlike skeletal development, which tends to be delayed by 2+ years in TS females, dental development tends to be early. Therefore, secondary teeth may erupt prematurely. The first permanent molars appear between 1-1/2 and 4 years of age, rather than the typical 5-6 years of age” (Dental Health Considerations & Solutions in Patients with Turner Syndrome). This can lead to crowding in the presence of already underdeveloped jaws.
  • Root resorption/ decayed or missing teeth: Root resorption is the progressive loss of part of a tooth, which can result in complete tooth loss. One study showed a higher incidence of decayed or missing teeth in TS patients, compared to control subjects

Dental Care & Treatment

Dental care should always start early in a child’s life, especially if they have TS. The American Academy of Pediatrics recommends that children with TS be seen by a dentist within six months of the eruption of the first tooth or 12 months of age, whichever comes first. 

Along with a dental evaluation, a full assessment should involve a pediatrician, endocrinologist, and/or a cardiologist. A cardiologist may be needed if the child has a congenital heart defect (which can occur in up to 50% of individuals with TS). They can recommend any necessary antibiotics for subacute bacterial endocarditis (an inflammation of the heart valves) before any dental procedures.

An orthodontist and/or oral surgeon may be needed for certain surgeries and treatment for problems, such as severe bite abnormalities, mandibular issues, teeth crowding, etc.

Another consideration for a child with TS is whether they are undergoing growth hormone treatment, as “this can alter craniofacial proportions and lead to further orthodontic concerns” (Dental Health Considerations & Solutions in Patients with Turner Syndrome, Korwin).

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Because of the factors mentioned above, it is very important that children have regular dental check-ups as your they grow older. The same is true for adults!

Steps You Can Take for a Healthy Smile

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  • Toothbrush: A soft-bristled toothbrush should be used after the appearance of the first tooth. The motions of toothbrushing can be up and down, in circles, or side-to-side, but extra attention should be paid to thoroughly cleaning teeth from all sides.
  • Toothpaste: At age three, children can start using a pea-sized amount of fluoride toothpaste, which helps prevent cavities. Make sure to teach your child not to swallow the toothpaste, as “swallowing too much fluoride toothpaste can make white or brown spots on your child’s adult teeth.” (Dental Health & Hygiene for Young Children, healthychildren.org
  • Diet: Watching your child’s diet plays an important role in preventing cavities. Avoid giving them large amounts of sugary drinks and snacks, and always make sure they brush after eating or drinking sweets. This will prevent sugar from staying on teeth for long periods of time and causing damage.
  • Dental check-ups: Routine exams should occur every six months, or as determined by your child’s dentist. During these visits, the dentist will check your child’s teeth and gums, and a referral to a pediatric dentist or orthodontist can be made for further evaluation/treatment if necessary.

For further information regarding proper dental hygiene and dental health in individuals with TS, browse through the links below. And remember to smile; it’s National Dental Hygiene Month!

Written by Ritu Bhavani Kasarapu, TSF volunteer blog writer. Edited by Susan Herman, TSF Blog Coordinator, and Kayla Ganger, PA-C, TSF Professional Membership Liaison. 

© Turner Syndrome Foundation, 2022

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