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Erdheim-Chester disease: The role of video-assisted thoracoscopic surgery in diagnosing and treating cardiac involvement.
[erdheim-chester disease]
Erdheim-
Chester
disease
is
a
rare
,
non-
Langerhans
histiocytosis
in
which
pericardial
involvement
is
diagnosed
with
increasing
frequency
and
is
associated
with
high
mortality
rates
.
A
53
-
year
-old
woman
presented
with
progressive
exertional
dyspnea
and
pericardial
effusion
was
discovered
.
Further
investigations
revealed
the
presence
of
a
diffuse
,
infiltrating
process
and
a
diagnosis
of
Erdheim-
Chester
disease
was
made
.
An
emergent
pericardiocentesis
by
subxiphoid
approach
was
completed
but
recurrent
drainage
obviated
removal
of
the
pigtail
catheter
.
A
pleuro-
pericardial
window
was
placed
using
video-assisted
thoracoscopic
surgery
(
VATS
)
and
analysis
of
the
resected
specimen
confirmed
pericardial
involvement
.
In
this
case
,
high
pericardial
fluid
output
demanded
definitive
treatment
of
the
pericardial
effusion
.
Traditionally
this
would
be
completed
via
thoracotomy
.
VATS
is
a
minimally
invasive
alternative
which
permits
exploration
of
the
thoracic
cavity
and
the
creation
of
a
pleuropericardial
window
.
We
describe
,
for
the
first
time
,
the
successful
use
of
VATS
for
both
diagnostic
confirmation
and
therapeutic
relief
of
recurrent
pericardial
fluid
drainage
due
to
pericardial
involvement
by
Erdheim-
Chester
disease
.
Diseases
Validation
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"first time"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
adrenomyeloneuropathy
alpha-thalassemia
aniridia
aromatase deficiency
canavan disease
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cowden syndrome
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dentin dysplasia
dentinogenesis imperfecta
dracunculiasis
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
fabry disease
familial mediterranean fever
gm1 gangliosidosis
harlequin ichthyosis
heparin-induced thrombocytopenia
hirschsprung disease
hodgkin lymphoma, classical
holt-oram syndrome
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malignant atrophic papulosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
monosomy 21
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oculocutaneous albinism
oligodontia
omenn syndrome
oral submucous fibrosis
papillon-lefèvre syndrome
pendred syndrome
phenylketonuria
primary effusion lymphoma
primary hyperoxaluria type 1
severe combined immunodeficiency
sneddon syndrome
triple a syndrome
trochlear dysplasia
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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