Oral and Dental

Dental

Oral and Dental anomalies are commonly seen in children with Emanuel Syndrome. Baby teeth are typically late to appear. Teeth can come in crooked and crowded and may appear abnormally shaped. Sometimes this is because of a smaller than usual jaw. 

Some of our children are missing a few adult teeth – with no teeth to come in behind the baby teeth that fell out. 

It is not uncommon for our children to have enamel defects, which may make them prone to getting cavities, or for our children to require dental surgery to have extra teeth removed, or baby teeth removed that did not become loose and fall out on their own. Most articles published on Emanuel Syndrome, as well as members of our parent group, report these common findings.

“Maia had teeth that came in with enamel defects. She also was missing some adult teeth as shown on an x-ray. Some of her baby teeth didn’t come out and had to be removed. Her teeth are not lined up properly.” 

Dental Care

Within our group, our shared experience is that our children can be challenging to examine for routine checkups or cleanings or to obtain radiographs. Often, it can be a risk to sedate our children for a thorough dental examination. Some parents have been able to arrange a dental assessment or radiographs when a child has to be sedated for a different procedure, for example when ear tubes are being placed.

As our children are unlikely to express pain in the mouth due to an infection or cavity, regular dental care, including assessment, cleaning and obtaining radiographs, is recommended.

Joaquin was schedule for oral surgery along with a sedated hearing exam. Here he is pre-op, post-op, and showing off his new smile a few days later!

Articles specifically detailing dental or oral findings are scarce in the literature, with one recent exception:

Puranik, C. P., & Katechia, B. (2019). Oral and dental findings in Emanuel syndrome. International Journal of Paediatric Dentistry, 29(5), 677–682. https://doi.org/10.1111/ipd.12502

Carter, M. T., St Pierre, S. A., Zackai, E. H., Emanuel, B. S., & Boycott, K. M. (2009). Phenotypic delineation of Emanuel syndrome (supernumerary derivative 22 syndrome): Clinical features of 63 individuals. American Journal of Medical Genetics. Part A, 149A(8), 1712–1721. https://doi.org/10.1002/ajmg.a.32957 

You may also be interested in: Emanuel Syndrome Guide for Families & Caregivers