Dental Problems in Angelman Syndrome
People with Angelman syndrome can have dental problems in addition to the mental and physical disabilities that mark this disease. Many of these problems are cosmetic. However, some can lead to an increased risk of cavities, difficulty eating or drinking, and drooling.
Here is more information about how a child’s mouth and teeth may be affected by Angelman syndrome, and possible treatment options.
What dental problems are common in Angelman syndrome?
Angelman syndrome patients have several characteristic abnormalities related to their mouth and teeth. Many have diastemas (gaps between their teeth), prognathism (an “underbite” or “overbite,” where the upper and lower jaw are misaligned), forward thrusting of the tongue, which can lead to an opening between the top and bottom teeth, and drooling.
Patients may also frequently like to suck or chew on objects or their hands. They are also known to grind their teeth during the day.
How might Angelman syndrome affect the teeth?
It is not clear how Angelman syndrome causes such oral characteristics as spaces between teeth and prognathism.
A recent study found that the enamel (the hard outside covering of teeth) of children with Angelman syndrome was unusually thin and contained nitrogen, while that of unaffected children did not. Both sets of children’s dentin (the layer just below the enamel) contained nitrogen, but the enamel of patients’ teeth alone had nitrogen in quantities similar to the dentin. This led the investigators to propose that patients’ tooth enamel may have some of the same proteins as the dentin. They also found that several of the patients’ teeth had variations in different minerals, which may lead to an increased risk of cavities.
Dental treatments of Angelman syndrome patients
The primary form of dental care for Angelman patients is good oral hygiene, and limits on consumption of sweets and sugary beverages. Since most patients have developmental delays and motor challenges, parents will need to ensure proper brushing. Regular dental checkups can also help to prevent cavities. Since children with Angelman syndrome may not be cooperative during dental procedures, restraint or general anesthesia may be necessary.
Treatment is usually not necessary for spaces between teeth and prognathism unless these impair the patient’s ability to eat and acquire sufficient nutrition. In such cases, jaw surgery or orthodontic devices like a chincup or face mask — or a combination of the two — may help.
Last updated: Jan. 4, 2020
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