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Dental and maxillofacial characteristics of six Japanese individuals with ectrodactyly-ectodermal dysplasia-clefting syndrome.
[oligodontia]
Objective
:
Ectrodactyly
-
ectodermal
dysplasia
-clefting
syndrome
is
a
congenital
anomaly
characterized
by
ectodermal
dysplasia
,
ectrodactyly
,
cleft
lip
and
palate
,
and
lacrimal
duct
anomalies
.
Because
this
syndrome
is
frequently
accompanied
by
a
congenital
lack
of
teeth
,
narrow
palate
,
and
malocclusion
,
comprehensive
orthodontic
intervention
is
required
.
Design
:
To
highlight
the
specific
dental
and
maxillofacial
characteristics
of
ectrodactyly
-
ectodermal
dysplasia
-clefting
syndrome
,
six
Japanese
individuals
diagnosed
with
the
syndrome
are
described
here
.
Patients
:
The
subjects
consisted
of
two
boys
and
four
girls
(
age
range
,
6
.
0
to
13
.
9
years
)
diagnosed
with
ectrodactyly
-
ectodermal
dysplasia
-clefting
syndrome
by
medical
and
dental
specialists
.
Their
conditions
included
ectodermal
dysplasia
(
hypodontia
,
microdontia
,
enamel
hypoplasia
,
and
abnormalities
in
hair
and
nails
)
,
cleft
lip
and
/
or
palate
,
and
ectrodactyly
.
Cephalograms
,
panoramic
x-rays
,
and
dental
casts
were
taken
;
systemic
complications
were
recorded
at
the
first
visit
to
our
dental
hospital
.
Results
:
All
individuals
had
severe
oligodontia
with
9
to
18
missing
teeth
.
The
missing
teeth
were
mainly
maxillary
and
mandibular
incisors
and
second
bicuspids
,
arranged
in
a
symmetrical
manner
.
Cephalometric
analysis
showed
retruded
and
short
maxilla
due
to
cleft
lip
and
/
or
palate
.
It
is
interesting
that
all
individuals
showed
a
characteristically
shaped
mandibular
symphysis
with
a
retruded
point
B
.
It
is
likely
that
this
unusual
symphyseal
morphology
is
due
to
the
lack
of
mandibular
incisors
.
Conclusions
:
This
study
demonstrates
the
presence
of
severe
oligodontia
in
the
incisal
and
premolar
regions
and
describes
a
characteristic
maxillary
and
mandibular
structure
in
Japanese
individuals
with
ectrodactyly
-
ectodermal
dysplasia
-clefting
syndrome
.
Diseases
Validation
Diseases presenting
"shaped mandibular symphysis with a retruded point"
symptom
oligodontia
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