21
Volume1 Issue1
|
N
ews and
E
vents
Diagnosis
“One of the challenges in diagnosing ED
is that is it not always a simple, consist-
ent, predictable clinical presentation,”
explains Dr. Kevin Butterfield, division
chief of dentistry and oral andmaxillofa-
cial surgeryat theOttawaHospital.
ED refers to more than 170 rare genetic
disorders that cause abnormal ectoderm
development. ThemainEDdefectscanbe
divided into 4 types of dysplasia—dental,
hair,nail andsweatglands.Dentistscanbe
instrumental indiagnosingEDas theycan
observe first-hand dental defects caused
by thevariousdisorders, including:
•
Anodontiaor hypodontia
•
Taurodontismof deciduousmolars
•
Permanent dentitionoften limited
to: central incisors, firstmolars
andcanines (maxilla); canines,
first premolars andfirstmolars
(mandible)
•
Deficient alveolar ridges
(associatedwithhypodontia)
•
Underdeveloped jaws
•
Malformed teeth
•
Cleft lipor palate
•
Absenceof lingual frenulum
When Jacob’s dentist believed that the
toddler likely had ED, he referred him to
a pediatric dentist who practises at the
Children’s Hospital of Eastern Ontario
(CHEO). “Wewere so relievedandgrateful
tomeet with a specialist experienced in
treating patients with ED,” remembers
Ms. Howard.
Treatment
PatientswithEDareusuallygoodcandidates
for implant therapy, and the procedure
success rate compares to that of normal
patients. However, they often have to
undergoboneaugmentation inpreparation
for implant placement. In October 2013,
Dr. Butterfield performed dental implant
placementonJacob,who isnow5yearsold.
“While the varying clinical presentations
can cause difficulties in the diagnostic
process, theprostheticmanagement has
dramatically improvedaswearenowable
to provide patients with a predictable
functional result,” saysDr. Butterfield.
Unlikemany patients who struggle to find
specialists inanyneededareaof treatment,
Jacob isfollowedbyamultidisciplinaryteam
ofhealthprofessionals. “Ourteamofexperts
includes Dr. Butterfield, a prosthodontist,
a pediatric dentist, a geneticist and a
pediatrician,” explains Ms. Howard. “We
havehoweverbeenunsuccessful infinding
adermatologistwithexperience in treating
patients with ED, so Jacob’s eczema is not
undercontrol at thispoint.”
Outlook
Prognosis for peoplewith ED is generally
verygood. Theyhaveanormal lifeexpec-
tancy, andmost disorders donot lead to
developmental delays.
Jacob’s resilience to his condition never
ceases to impress his mother. “He
absolutely refuses to let ED slow him
down. He could teachmany of us what
courage reallymeans,” saysMs.Howard.
a
“Jacobabsolutely refuses to letED slowhimdown.
He could teachmany of uswhat courage reallymeans...”
TheCanadianEctodermal
DysplasiaSyndromes
Association (CEDSA)
WhenJacobwasfirstdiagnosed,
therewasnoCanadianorganization
dedicatedtohelpingpatientswithED.
Tohelpfill thatgap inservice,Meghan
Howard foundedtheCanadian
EctodermalDysplasiaSyndromes
Association (CEDSA) in2010.
CEDSAsupports familiesthrough
teleseminarswithmaxillofacial
surgeons,acomprehensivewebsite,
regularnewsletters,asupport fund
tohelpcoverthe importantdental
andmedicalcostsassociatedwith
treatment,andthecreationofa
securemedicalanddentalexpert
database.
TolearnmoreaboutCEDSA
activitiesandservices,
visit
Sources
1
CanadaEctodermalDysplasiaSyndromesAssociationwebsite[accessed2013Oct21].Available:www.ectodermaldysplasia.ca.
2
JonesJ,HollandMetal.Ectodermaldysplasia[accessed2013Oct21].Available:radiopaedia.org/articles/ectodermal-dysplasia.
3
VanSickelsJE,RaybouldTP,HicksEP. Interdisciplinarymanagementofpatientswithectodermaldysplasia.
JOral
Implantol
.2010;36(3):239-45.
4
LiD,LuiY,MaW,SongY.Reviewofectodermaldysplasia:casereportontreatmentplanningandsurgicalmanagementof
oligodontiawith implantrestorations.
ImplantDent
.2011Oct;20(5):328-30.
