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Hemangioblastoma of the optic nerve producing bilateral optic tract edema in a patient with von Hippel-Lindau disease.
[von hippel-lindau disease]
The
authors
present
a
novel
case
of
a
hemangioblastoma
of
the
optic
nerve
producing
bilateral
optic
tract
edema
in
a
patient
with
von
Hippel-
Lindau
disease
(
VHL
)
.
This
is
the
only
case
in
the
literature
documenting
optic
tract
edema
secondary
to
a
hemangioblastoma
of
the
optic
nerve
.
The
patient
was
a
34
-
year
-old
female
in
whom
this
lesion
was
causing
retro-orbital
pain
and
proptosis
.
She
had
previously
lost
vision
in
the
symptomatic
eye
secondary
to
a
retinal
hemangioblastoma
.
The
optic
nerve
lesion
was
excised
by
sectioning
the
optic
nerve
both
proximally
and
distally
to
the
lesion
.
There
were
no
complications
and
patient
's
symptoms
resolved
postoperatively
.
A
follow-up
magnetic
resonance
imaging
(
MRI
)
scan
revealed
complete
excision
of
the
mass
and
resolution
of
the
optic
tract
edema
.
Optic
nerve
hemangioblastomas
in
patients
with
VHL
are
rare
,
but
are
manageable
with
meticulous
microneurosurgery
and
with
appropriate
patient
expectations
.
This
is
the
first
known
case
of
an
optic
nerve
hemangioblastoma
producing
bilateral
optic
tract
edema
,
which
resolved
after
resection
of
the
prechiasmal
tumor
.
Hemangioblastoma
should
remain
in
the
differential
diagnosis
of
optic
nerve
tumors
,
especially
in
the
setting
of
VHL
.
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