6 genetic disorders you may not know affect the face and mouth

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It’s National Craniofacial Awareness Month this month.  Children with craniofacial syndromes experience significant alterations to their facial features and bony structures of the skull and jaw. Many children have to endure multiple surgeries to manage these various conditions and improve their quality of life.  It can be especially daunting for these children, who grow up feeling different from other kids, along with their families who may feel overwhelmed and exhausted with numerous medical procedures.  We can’t even remotely imagine the many challenges these families may have to face. Since life may be hectic enough, we have taken the time to compile a list of resources that can provide some information and support networks to those who live with these syndromes and their families.

Here are some general websites where you can find more information about a particular syndrome. For some of the more common craniofacial syndromes, we have highlighted the dental implications that may be associated.

http://www.diseaseinfosearch.org/site/index

http://rarediseases.info.nih.gov/

 

  • Cleft Lip/Palate

http://www.cleftsmile.org/

http://www.cleftline.org/

Dental Implications

-altered number, size, shape, and position of baby or permanent teeth; primarily affected are the lateral incisors

-teeth can be displaced and erupt into abnormal positions

-teeth can be poorly formed with an abnormally shaped crown or root.

  • Hemifacial Microsomia (Goldenhar Syndrome)

http://www.faces-cranio.org/Disord/Golden.htm

Dental Implications

-weakness in moving the side of the face that is smaller

-soft palate may move to the unaffected side of the face

-fusions in the bones in the neck

-tongue may be smaller on the affected side of the face

  • Treacher Collin Syndrome

http://www.ccakids.com/treacher-collins-syndrome.html

Dental Implications

-shortened jaw

-cleft palate

-midface hypoplasia/malocclusion  (changes in the bite)

-open bite

-shortening of the soft palate

  • Crouzon Syndrome

http://www.faces-cranio.org/Disord/Crouzon.htm

http://rarediseases.info.nih.gov/gard/6206/crouzon-syndrome/resources/1

http://www.ccakids.com/crouzon-syndrome.html

Dental Implications

-abnormally shaped head

-cleft palate

-receded upper jaw

-crowding of the teeth

-speech impairment

-narrow/high-arched palate

-posterior bilateral crossbite

-hypodontia (fewer teeth)

-crowding of teeth

  • Apert Syndrome

http://ghr.nlm.nih.gov/condition/apert-syndrome

http://www.faces-cranio.org/Disord/Apert.htm

Dental Implications

-underdeveloped jaw

-crowded teeth

-arch shaped palate

-cleft soft palate

-trapezoidal shaped mouth

-severely delayed tooth eruption

-shovel shaped incisors

-severe crowding of teeth

-mandibular overjet (overbite)

-anterior and posterior crossbites

  • Trisomy 21 (Down Syndrome)

http://www.ds-int.org/

http://www.globaldownsyndrome.org/

Dental Implications

-protruding tongue

-narrow small palate

-high arched palate

-delayed and unusual patterns of eruption (varying orders)

-missing primary and permanent teeth

-microdontia

-variations in tooth morphology and number

-over retained primary teeth

-peg lateral teeth

-shovel shaped incisor teeth

Copyright 2014 The Children’s Oral Care Centre

Red Hot “Burning” Lips – Are they really as sexy?

Sun Safety – Part 1

The lips are vulnerable sensitive areas on the body that receive much sunlight and yet are often missed or overlooked for sun safety.  Initial sun-induced damage is short-term and acute, resulting in sunburn, blistering or peeling.  However, lip cancer develops over time and it is related to accumulated exposure to the sun and repeated damage to your lips from sunburn.   You can significantly reduce the risk of lip cancer if you start sun protection for your lips at an early age.

Who is more susceptible to sun damage?

Those who have a cumulative exposure to ultraviolet radiation (UVR) are most susceptible.  Repeat exposure leads to chronic changes such as, freckling, loss of skin elasticity, red spider veins and actinic cheilosis, the drying and cracking of lips.  Another important factor that can give insight into risk for sun damage is skin phenotype or color.

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Actinic cheiloses occur more frequently in those with light-complected, skin types I and II, individuals compared to darker-complected individuals.  Research has also shown that squamous cell carcinoma of the lower lip occurs 30 times more frequently in light skinned individuals compared to darker skinned individuals.  This is because the vermilion, border where the lip meets the face, has more melanin in darker-complected individuals and this may give more protection from UVR.   This puts those at highest risk to be individuals who burn easily, do not tan and tend to form nonfacial along with facial freckles.  See the table below for the varying skin phenotypes and their response to UVR exposure to get a better idea of risk level.

Some other risk factors for sun damage and lip cancer include; having an outdoor occupation, living in a rural area, decreased immune system and tobacco habits.   Aging is one of the greatest risks due to the fact that chronic and repeated exposure is more likely as you age.  Men are affected more often than women due to differences in occupations and women may have more protective habits such as applying sun protector or lipstick to their lips.  Those who form early sun exposure habits are also at a higher risk for sun damage as they will ultimately have more sun exposure throughout their life.

Table 1. Skin Phenotypes

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Stay tuned for Sun Safety – Part 2 coming out in August, we will be highlighting ways you can protect yourself from the beautiful but harmful sun.  Do you know what is in sunscreen and how it actually protects? What do SPF, UV-A, UV-B mean and how are they related to one another? Check back in August!

Copyright 2014 The Children’s Oral Care Centre