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Aniridic Glaucoma: Long-term Visual Outcomes and Phenotypic Associations.
[aniridia]
The
aim
of
this
study
was
to
evaluate
long
-term
visual
outcomes
in
patients
with
aniridic
glaucoma
.
Retrospective
noncomparative
observational
case
series
.
A
review
of
medical
records
of
128
eyes
of
64
consecutive
patients
with
aniridic
glaucoma
,
diagnosed
after
the
age
of
5
years
was
analyzed
.
The
parameters
studied
included
age
at
presentation
,
family
history
,
baseline
intraocular
pressure
(
IOP
)
,
type
and
the
number
of
surgical
interventions
,
and
associated
comorbidities
.
Best
corrected
visual
acuity
(
BCVA
)
in
the
better
eye
.
Mean
age
at
presentation
was
15
.
86
±
10
.
11
years
(
range
,
5
to
47
y
)
.
The
average
follow-up
was
7
.
69
±
4
.
98
years
(
range
,
1
to
17
y
)
.
At
the
final
follow
up
only
18
patients
had
BCVA
better
than
6
\
60
and
only
5
patients
had
BCVA
of
>
6
/
18
.
Seventeen
of
the
64
(
26
.
5
%
)
patients
developed
phthisis
in
1
eye
.
The
final
visual
outcomes
were
not
associated
with
age
at
presentation
(
P
=
0
.
64
)
or
sex
(
P
=
0
.
76
)
;
however
,
those
with
a
higher
baseline
IOP
(
P
=
0
.
017
)
,
those
with
familial
aniridia
(
P
=
0
.
037
)
,
and
those
who
underwent
more
number
of
surgical
interventions
had
poorer
visual
outcomes
(
P
=
0
.
004
)
.
Kaplan-
Meier
analysis
demonstrated
the
probability
of
bilateral
blindness
to
be
69
.
8
%
and
97
.
6
%
at
5
and
10
years
,
respectively
.
Long
-term
visual
outcomes
after
therapy
among
aniridic
glaucoma
patients
remain
poor
.
Higher
baseline
IOP
,
the
presence
of
familial
aniridia
,
and
a
history
of
greater
number
of
surgical
interventions
are
associated
with
poorer
long
-term
visual
outcomes
.
Diseases
Validation
Diseases presenting
"aniridia"
symptom
aniridia
kabuki syndrome
oculocutaneous albinism
This symptom has already been validated