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Hemihyperplasia-multiple lipomatosis syndrome (HHML): a challenge in spinal care.
[proteus syndrome]
A
15
-
year
-old
girl
developed
a
progressive
paraparesis
over
a
period
of
six
months
,
secondary
to
spinal
cord
compression
by
a
lipomatous
mass
and
anomalies
of
the
vertebral
column
.
Clinically
,
a
right
hemihyperplasia
affecting
the
trunk
and
lower
limb
was
evident
,
as
well
as
a
right
convex
lumbar
scoliosis
.
CT
and
MRI
demonstrated
severe
spinal
cord
compression
resulting
from
intraspinal
lipomatosis
,
overgrowth
of
right
facet
joints
(
T
8
to
L
5
)
,
and
kyphoscoliosis
.
Surgical
decompression
was
undertaken
.
A
lumbar
scoliosis
of
48
degrees
was
partially
corrected
by
means
of
dual-rod
instrumentation
.
The
neurological
deficit
improved
significantly
,
and
ambulation
was
progressively
restored
.
The
patient
carried
the
diagnosis
of
Proteus
syndrome
for
several
years
,
but
reevaluation
of
clinical
features
prompted
the
diagnosis
of
Hemihyperplasia
Multiple
Lipomatosis
syndrome
(
HHML
)
.
This
rare
sporadic
disorder
is
often
confused
with
Proteus
syndrome
.
As
in
Proteus
syndrome
,
spinal
cord
compression
in
patients
with
HHML
can
result
from
lipomatous
infiltration
and
/
or
significant
spinal
abnormalities
including
kyphoscoliosis
and
overgrowth
.
HHML
and
Proteus
syndrome
are
discussed
and
compared
with
special
emphasis
on
spinal
and
orthopaedic
pathologies
.
