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Macular pigment optical density spatial distribution measured in a subject with oculocutaneous albinism.
[oculocutaneous albinism]
Previous
studies
of
macular
pigment
optical
density
(
MPOD
)
distribution
in
individuals
with
oculocutaneous
albinism
(
OCA
)
have
primarily
used
objective
measurement
techniques
including
fundus
reflectometry
and
autofluorescence
.
We
report
here
on
a
subject
with
OCA
and
their
corresponding
MPOD
distribution
assessed
through
heterochromatic
flicker
photometry
(
HFP
)
.
A
subject
with
a
history
of
OCA
presented
with
an
ocular
history
including
strabismus
surgery
of
the
LE
with
persistent
amblyopia
and
mild
,
latent
nystagmus
.
Best
corrected
visual
acuity
was
20
/
25
-
RE
and
20
/
40
-
LE
.
Spectral
domain
optical
coherence
tomography
(
SD
-
OCT
)
and
fundus
photography
were
also
obtained
.
Evaluation
of
MPOD
spatial
distribution
up
to
8
degrees
eccentricity
from
the
fovea
was
performed
using
HFP
.
SD
-
OCT
indicated
a
persistence
of
multiple
inner
retinal
layers
within
the
foveal
region
in
the
RE
and
LE
including
symmetric
foveal
thickening
consistent
with
foveal
hypoplasia
.
Fundus
photography
showed
mild
retinal
pigmented
epithelial
(
RPE
)
hypopigmentation
and
a
poorly
demarcated
macula
.
OriginPro
9
was
used
to
plot
MPOD
spatial
distribution
of
the
subject
and
a
33
-
subject
sample
.
The
OCA
subject
demonstrated
a
foveal
MPOD
of
0
.
10
with
undetectable
levels
at
6
degrees
eccentricity
.
The
study
sample
showed
a
mean
foveal
MPOD
of
0
.
34
and
mean
6
degree
eccentricity
values
of
0
.
03
.
Consistent
with
previous
macular
pigment
(
MP
)
studies
of
OCA
,
overall
MPOD
is
reduced
in
our
subject
.
Mild
phenotypic
expression
of
OCA
with
high
functional
visual
acuity
may
represent
a
Henle
fiber
layer
amenable
to
additional
MP
deposition
.
Further
study
of
MP
supplementation
in
OCA
patients
is
warranted
.
Diseases
Validation
Diseases presenting
"amblyopia"
symptom
aniridia
coats disease
oculocutaneous albinism
This symptom has already been validated