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Timing of surgery for feminizing genitoplasty in patients suffering from congenital adrenal hyperplasia.
[congenital adrenal hyperplasia]
This
chapter
refers
only
to
female
patients
with
congenital
adrenal
hyperplasia
(
CAH
)
.
CAH
represents
the
largest
subgroup
of
individuals
with
46
,
XX
disorders
of
sex
development
.
The
stimulation
of
the
androgen
production
leads
to
a
prenatal
virilization
among
these
girls
.
The
phenotype
is
influenced
by
the
severity
of
the
enzyme
defect
,
leading
to
a
virilization
of
the
external
genitalia
in
varying
degrees
.
On
the
other
hand
,
the
affected
girls
are
clearly
anatomically
female
with
regularly
developed
female
internal
genitalia
.
Female
puberty
and
potential
female
fertility
are
therefore
to
be
expected
.
The
operation
to
feminize
the
genitalia
includes
the
separation
of
the
sinus
urogenitalis
,
the
creation
of
a
functionally
wide
enough
vagina
,
the
remodeling
of
the
labioscrotal
folds
to
create
larger
labia
,
and
,
if
necessary
,
a
reduction
clitoroplasty
.
Considering
the
lack
of
scientific
evidence
,
it
is
not
possible
to
make
definitive
statements
regarding
the
timing
of
surgery
for
girls
with
CAH
.
There
are
no
studies
that
prove
whether
one
-
or
two
-
stage
surgery
provides
more
or
clearer
advantages
.
