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Techniques for toddlers: linear band incision for harlequin ichthyosis with associated compartment syndrome.
[harlequin ichthyosis]
Harlequin
ichthyosis
(
HI
)
is
a
rare
autosomal
recessive
disorder
of
cornification
in
which
children
are
born
with
an
extremely
thick
stratum
corneum
that
becomes
a
restrictive
circumferential
encasement
around
the
orifices
,
limbs
,
chest
,
and
abdomen
,
resulting
in
limb
contractures
.
We
present
a
neonate
diagnosed
in
utero
with
HI
.
The
infant
was
born
with
encasing
bands
of
thickened
skin
creating
strictures
that
were
causing
digital
and
limb
cyanosis
(
compartment
syndrome
)
.
We
treated
the
child
using
a
new
technique
of
lysis
of
the
encasing
bands
that
we
call
linear
band
incision
,
using
a
new
escharotomy-like
procedure
while
the
infant
was
undergoing
a
3
-
week
course
oral
acitretin
therapy
.
The
technique
involved
linear
incision
and
lysis
of
encasements
that
resulted
in
reperfusion
of
the
injured
limbs
and
prevention
of
further
digital
necrosis
.
The
child
is
currently
a
healthy
8
-
year
-old
boy
with
skin
manifestations
resembling
congenital
ichthyosiform
erythroderma
.
He
has
use
of
all
of
the
limbs
that
were
released
in
the
procedures
and
is
maintained
on
frequent
application
of
bland
emollients
.
Linear
band
incision
is
a
potentially
life-
and
limb
-saving
technique
in
children
with
HI
.
