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Concomitant achondroplasia and Chiari II malformation: A double-hit at the cervicomedullary junction.
[achondroplasia]
We
report
the
first
case
of
a
neonate
with
concurrent
Chiari
II
malformation
and
achondroplasia
.
Although
rare
,
both
these
conditions
contribute
to
several
deleterious
anatomical
changes
at
the
cervicomedullary
junction
and
thus
predispose
to
acute
hydrocephalus
.
Although
our
patient
was
initially
asymptomatic
,
hydrocephalus
ensued
several
weeks
after
birth
and
required
cerebral
spinal
fluid
diversion
.
We
discuss
the
potential
links
between
the
two
conditions
,
the
pathophysiology
,
and
the
important
clinical
implications
for
the
management
of
the
increased
risk
of
hydrocephalus
.
Diseases
Validation
Diseases presenting
"hydrocephalus"
symptom
achondroplasia
alexander disease
canavan disease
congenital toxoplasmosis
harlequin ichthyosis
hirschsprung disease
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
krabbe disease
monosomy 21
proteus syndrome
severe combined immunodeficiency
sneddon syndrome
von hippel-lindau disease
wiskott-aldrich syndrome
This symptom has already been validated