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Anaesthesia for the patient with neonatal adrenoleukodystrophy.
[neonatal adrenoleukodystrophy]
The
authors
present
and
discuss
the
care
of
a
nine
-
month
-old
with
neonatal
adrenoleukodystrophy
who
required
general
anaesthesia
for
gastrointestinal
endoscopy
.
Neonatal
adrenoleukodystrophy
is
an
inherited
disorder
of
peroxisomal
enzymes
.
Anaesthetic
care
may
be
affected
by
the
presence
of
hypotonia
,
liver
function
abnormalities
,
gastroesophageal
reflux
,
and
impaired
adrenocortical
function
.
Preoperative
sedation
is
contraindicated
because
of
the
risk
of
precipitating
airway
obstruction
due
to
pre-existing
hypotonia
.
Anaesthetic
induction
and
tracheal
intubation
should
be
performed
to
minimize
the
risk
for
aspiration
of
gastric
contents
.
The
choice
of
muscle
relaxant
should
take
into
account
the
pre-existing
hypotonia
as
well
as
the
possibility
of
hyperkalaemia
in
response
to
succinylcholine
.
Anaesthetic
agents
known
to
decrease
the
seizure
threshold
should
be
avoided
in
patients
with
a
seizure
disorder
.
In
addition
,
anaesthetic
agents
that
rely
on
the
liver
for
metabolism
should
be
used
with
caution
in
patients
with
cirrhosis
.
When
time
permits
,
these
patients
should
be
screened
for
adrenocortical
insufficiency
before
surgery
,
and
perioperative
steroid
coverage
is
advisable
when
preoperative
testing
of
adrenocortical
function
is
not
feasible
.
While
these
patients
eventually
die
after
progressive
deterioration
,
full
recovery
from
the
effects
of
anaesthesia
and
surgery
can
be
achieved
with
attention
to
neurological
,
metabolic
,
and
physical
problems
.
