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Isolated aglossia congenita: A rare case of oromandibular limb hypogenesis syndrome type I B.
[oligodontia]
Aglossia
congenita
(
AC
)
,
congenital
total
absence
of
the
tongue
,
is
a
very
rare
midline
developmental
anomaly
,
hypothesized
to
be
associated
with
vascular
disruption
between
the
fourth
and
eighth
week
of
gestation
.
It
was
classified
by
Hall
(
1971
)
as
part
of
oromandibular
limb
hypogenesis
syndrome
(
OLHS
)
type
I
B
.
Most
of
the
cases
reported
with
OLHS
are
actually
hypoglossia
with
limb
abnormalities
whereas
isolated
aglossia
is
an
extremely
rare
entity
.
A
case
of
isolated
AC
is
presented
in
a
28
-
year
-old
Indian
male
.
He
had
long
narrow
face
,
tapering
chin
,
low
set
ears
,
and
microstomia
.
Intraorally
,
he
had
narrow
palatal
vault
,
constricted
oropharyngeal
isthmus
,
oligodontia
,
and
maxillo-
mandibular
hypoplasia
.
Interestingly
,
the
patient
showed
a
median
palatal
groove
,
which
has
not
been
reported
before
.
He
also
had
an
unusual
acquired
adaptive
mechanism
to
compensate
for
aglossia
.
This
report
presents
the
manifestations
of
this
rare
syndrome
,
its
complications
,
differential
diagnosis
,
and
rehabilitation
strategies
.
Diseases
Validation
Diseases presenting
"long narrow face"
symptom
oligodontia
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