Ectodermal and Cleidocranial Dysplasia

If you’re reading this, then chances are you or someone you know has one of these developmental conditions. If it affects your child, then I urge you to read on because early intervention is very important from a dental persepective.

In this blog I will discuss two of many syndromes or conditions which may have implications in the mouth. Some examples of these include: ectodermal dysplasias, cleft lip and palate, Trisomy 21 (Down syndrome), Rieger syndrome, Williams’ syndrome and cleidocranial dysplasia.

Ectodermal dysplasias

Ectodermal dysplasias are a group of usually inherited conditions which someone is born with. ‘Ectodermal’ refers to the areas of the body which this disorder may affect. This includes: teeth, nails, skin, hair and sweat glands.

What to look for

Your male child may have:

  • Missing teeth
  • Small upper jaw
  • Lips that turn out (eversion)
  • Dry skin
  • Thin, brittle hair
  • The mouth and eyes may have pigmentation around them

In the dental clinic, I often see males with this condition have small and conically-shaped teeth. Sometimes, there may be a gap between their upper front teeth.

Females who have the gene for this condition, may have a missing tooth or a peg lateral incisor. A peg lateral incisor is when the front teeth on either side of your front centre teeth are smaller than usual.

What to do

Dental intervention usually starts when your son is 2-3 years old. At this age, his upper front teeth have come through and they may resemble fangs and have very pointy edges. We can re-shape these teeth using composite resin filling material. In cases where many teeth are missing or mis-shaped, dentures may need to be made. This will involve taking a mold of your child’s teeth and ongoing care and adjustment of their dentures. Later they will require implants, orthodontic treatment. The goal of dental treatment isn’t just to make a smile look good, but it also improves function when chewing and swallowing, speech and self-esteem.

Cleidocranial dysplasia

Cleidocranial dysplasia is a developmental disorder which affects the bones and teeth. Your child may have absent or poorly developed collarbones, are shorter than average, a prominent forehead, wide-set eyes and a flat nose.

What to look for

  • Delayed formation of the front of the skull
  • Small upper jaw
  • Lots of extra teeth
  • Delayed teeth erupting in the mouth
  • Cysts in their jaws

What to do

Early diagnosis is very important in kids with this condition. If your child’s teeth are delayed in coming through, you may want to see your dentist. If the baby teeth take too long to come out or there are extra teeth, then your dentist may need to help in removing them. Then, you will need to surgically expose the permanent teeth in order for them to come through. Orthodontic treatment will also be required when they have finished growing.

I hope you’ve found this blog helpful. I try and cover as many different topics related to oral health as possible. If you’d like me to discuss any specific issues, contact me on 8678 3538.

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