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Long-term results after proton beam therapy for retinal papillary capillary hemangioma.
[von hippel-lindau disease]
To
evaluate
the
potential
benefit
and
risk
of
proton
beam
therapy
in
the
treatment
of
symptomatic
retinal
papillary
capillary
hemangioma
.
Retrospective
interventional
case
series
.
This
study
included
patients
presenting
with
symptomatic
exudative
retinal
papillary
capillary
hemangioma
with
or
without
association
with
von
Hippel-
Lindau
disease
.
All
patients
were
treated
either
as
a
first
or
a
secondary
treatment
option
by
proton
beam
therapy
between
2001
and
2009
.
The
minimum
follow-up
was
30
months
.
Eight
eyes
of
8
patients
(
3
male
and
5
female
,
with
a
mean
age
of
36
years
[
range
22
-
80
years
]
)
were
treated
for
symptomatic
papillary
retinal
hemangioma
.
The
median
interval
between
onset
of
macular
edema
and
proton
beam
therapy
was
1
.
7
months
(
range
0
.
5
-
3
.
3
months
)
.
The
median
follow-up
period
was
84
months
(
range
32
-
106
months
)
between
proton
beam
treatment
and
last
follow-up
.
Exudation
completely
resolved
in
all
but
1
patient
after
4
.
2
months
on
average
(
range
2
.
8
-
7
.
2
months
)
.
Mean
visual
acuity
prior
to
proton
beam
irradiation
was
0
.
7
logMAR
(
0
.
2
DIN
(
DIN
58220
norm
)
)
(
range
2
-
0
.
3
logMAR
)
and
declined
to
0
.
8
logMAR
(
0
.
16
DIN
;
range
2
-
0
.
1
logMAR
)
at
last
follow-up
examination
(
no
statistical
significance
,
P
=
.
071
)
.
The
anatomic
outcome
after
proton
beam
therapy
for
retinal
papillary
hemangioma
is
convincing
,
whereas
functional
outcome
may
be
compromised
because
of
tumor
location
,
long
-persisting
macular
edema
,
extensive
exudation
,
and
poor
initial
visual
acuity
.
In
patients
with
extended
retinal
detachment
surgical
intervention
was
still
necessary
.
Although
proton
beam
therapy
is
proven
to
be
a
therapeutic
option
,
treatment
will
remain
challenging
.
Diseases
Validation
Diseases presenting
"edema"
symptom
acute rheumatic fever
adrenomyeloneuropathy
aniridia
cadasil
canavan disease
classical phenylketonuria
coats disease
congenital toxoplasmosis
cushing syndrome
cutaneous mastocytosis
erythropoietic protoporphyria
esophageal carcinoma
fabry disease
familial mediterranean fever
focal myositis
harlequin ichthyosis
homocystinuria without methylmalonic aciduria
inclusion body myositis
lamellar ichthyosis
liposarcoma
lymphangioleiomyomatosis
malignant atrophic papulosis
pleomorphic liposarcoma
systemic capillary leak syndrome
trochlear dysplasia
von hippel-lindau disease
waldenström macroglobulinemia
This symptom has already been validated