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. . CHEST
CHEST Special Features
Special Features
-- --
Spontaneous Hemothorax*
Spontaneous Hemothorax*
A Comprehensive
A Comprehensive Review
Review
Hakim Azfar
Hakim Azfar Ali,
Ali, MD;
MD; Michael Lippmann, MD,
Michael Lippmann, MD, FCCP;
FCCP; Uday
Uday Mundathaje,
Mundathaje, MD;
MD;
and Ghulam Khaleeq, MD
and Ghulam Khaleeq, MD

(CHEST 2008;
(CHEST 2008; 134:4.&56-1065)
134:4.&56-1065) malignancies, tuberculosis,
malignancies, tuberculosis, uremia,
uremia, and
and vascular
vascular pro-
pro-
cesses such
cesses such as pulmonary infarction.
as pulmonary infarction.
Key words: coagulopathy;
Key words: coagulopathy; endometriosis;
endometriosis; exostoses;
exostoses; fibrothorax;
fibrothorax; A true
A true hemothorax,
hemothorax, however,
however, usually
usually signals
signals aa
hemothorax; neoplasia;
hemothorax; neoplasia; spontaneous
spontaneous pneumothorax
pneumothorax
more blatant
more blatant compromise
compromise of of the
the vascular
vascular wall.
wall. The
The
involved vessel
involved vessel could
could either
either bebe aa normal
normal vessel
vessel
Abbreviations: AVM =
Abbreviations: AVM = arteriovenous
arteriovenous malformation; EDS =
malformation; EDS = Ehlers-
Ehlers- undergoing an
undergOing an abnormal
abnormal stress
stress or
or an
an abnormal
abnormal vessel
vessel
Danlos syndrome;
Danlos syndrome; HCC
HCC = = hepatocellular
hepatocellularcarcinoma;
~inoma; HHTHHT =. heret,l-
=. heret,l-
itary
italy hemmorhagic
hemmorhagic telangiectasia; IPFT =
telangiectasia; IPFT = intrapleural
mtrapleural fibrm?lytic
fibrm?lytic rupturing without
rupturing without precipitating
precipitating factors.
factors.
therapy;
therapy; SHP spontaneous hemopneumothorax;
SHP == spontaneous hemopneumothorax; VATS
VATS == Vldeo-
video- Most of
Most of the
the data
data about
about spontaneous
spontaneous hemothorax
hemothorax
assisted thoracic
assisted thoracic surgery
surgery are in
are in the
the form
form of
of scattered
scattered case
case reports
reports and
and case
case
series. 11 Table
series. Table 11 gives
gives aa summary
summary of of the
the diagnoses
diagnoses
associated with
with nonpneumothorax-related
nonpneumothorax-related spontane-
spontane-
H fluid
emothorax by definition
definition is extraction of
of pleural
pleural associated
H emothorax by
fluid with
with aa hematocrit
hematocrit >
is extraction
> 50% ofof the
the blood
blood
50% ous
ous hemothoraces.
hemothoraces. The
discussed.
discussed.
The major
major etiologies
etiolOgies will
will be
be
hematocrit. Most
hematocrit. Most cases
cases of
of hemothorax
hemothorax are are related
related toto
open or
open or closed
closed chest
chest trauma
trauma oror procedures
procedures such such as
as
central lines,
central lines, thoracentesis,
thoracentesis, pleural
pleural biopsy,
biopsy, or or cathe-
cathe-
terization. Spontaneous
terization. Spontaneous hemothorax
hemothorax is is much
much lessless SPONTANEOUS HEMOPNEUMOTHORAX
SPONTANEOUS HEMOPNEUMOTHORAX
common, and
common, and the
the causes
causes include
include malignancies,
malignancies, anti- anti- Spontaneous pneumothorax
Spontaneous pneumothorax is is by
by far
far the
the most
most
coagulant medications,
coagulant medications, vascular
vascular ruptures
ruptures (aortic
(aortic dis-
dis- common cause
common cause of of hemothorax.
hemothorax. Our Our review
review found
found
section arteriovenous
section, arteriovenous malformations
malformations [AVMs]),
[AVMs]), en- en- hemothorax to
hemothorax to bebe aa complication
complication in in 33 to
to 7% of of cases
cases
dometriosis, pulmonary
dometriosis, pulmonary infarctions,
infarctions, adhesions
adhesions with with of
of spontaneous
spontaneous pneumothorax-
pneumothorax2 Spontaneous
Spontaneous hemo- hemo-
pneumothorax, and
pneumothorax, and hematologic
hematologic abnormalities
abnormalities such such pneumothorax
pneumothorax (SHP) (SHP) occurs
occurs mostly
mostly in in young
young pa-pa-
as hemophilia.
as hemophilia. tients
tients (mean
(mean age,age, 22.1 to to 34.0 years).
years).
Pleural fluid
Pleural fluid imparts
imparts aa red
red color
color indistinguishable
indistinguishable The
The earliest
earliest reports
reports of of the
the condition
condition have have been
been
from blood
from blood at at aa hematocrit>
hematocrit> 5%. It It isis therefore
therefore traced
traced to to Laennec
Laennec (1828), Whittaker
Whittaker (1876),3 and and Pitt
Pitt
always important
always important to to measure
measure the
the hematocrit
hematocrit of of any
any (1900). Approximately
(1900). Approximately 300 cases cases have
have been
been reported
reported in in
hemorrhagic
hemorrhagic pleural
pleural effusion.
effusion. The
The hematocrit
hematocrit may may the literature
the literature in the form
in the fonn ofof cases
cases andand case series.44 -- 12
case series. 12
be
be reduced
reduced duedue to secondary dilution
to secondary dilution toto less
less than
than the
the SHP is
SHP defined by
is defined by Ohmori
Ohmori and and colleagues 13
colleagues'? as as the
the
required
required 50% that of blood
that of blood within
within aa few few days,
days, accumulation of
accumulation of >> 400 mL mL of blood in
of blood in the
the pleural
pleural
making
making it it appear
appear as as aa hemorrhagic
hemorrhagic exudate.
exudate. The The cavity
cavity in in association
association with
with spontaneous
spontaneous pneumotho-
pneumotho-
definition
definition of of hemothorax
hemothorax has has therefore
therefore included
included rax.
rax. Sometimes
Sometimes the the patients
patients can can present
present in in shock
shock
hematocrits
hematocrits ranging
ranging from
from 25 toto 50% thatthat of of blood.
blood. with
with blood loss> 600 mL
blood loss> mL and
and the
the mechanical
mechanical effects
effects
Hemorrhagic
Hemorrhagic effusions
effusions have been associated
have been associated with with of aa pneumothorax.
of pneumothorax. There There are are said
said toto bebe three
three
mechanisms of
mechanisms of bleeding
bleeding in hemopneumothorax.l"
in hemopneumothorax. 14
*From the
*From the Albert
Albert Einstein
Einstein Medical
Medical Center,
Center, Philadelphia
Philadelphia PA.
PA.
The authors
The authors have
have nono conflicts
conflicts of
of interest
interest to
to disclose.
disclose. First, bleeding
First, bleeding can can result
result from
from aa tom tom adhesion
adhesion
Manuscript
Manuscript received
received March
March 14, 14, 2008;
2008; revision
revision accepted
accepted May
May 6,6, between the
between the parietal
parietal and
and visceral pleura.>2 At
visceral pleura. At autopsy,
autopsy,
2008.
2008. adhesions
adhesions have have been
been shown
shown to be present
to be present in in 50% of of
Reproduction
~eproduction ofof this
this article
article is
is prohibited written per;nission
prohibited ~~out written per;nission
from
from the
the American
American College
College ofof Chest
Chest Physicians
PhysICIans (www.chestjoumal.
(www.chestjoumal. patients> 20 years
patients> years ofof age
age who
who have
have hadhad nono history
history of of
orgimiscireprints.shtml).
orgimisclreprints.shtml). pulmonary
pulmonary disease.
disease. According
According to to Barry
Barry et et al,15
al,15 the
the
Correspondence to:
Correspondence Hakim Azjar
to: Hakim Azfar Ali,
Ali, MD, Division ojPulmonary
MD, Division ofPulmonary site
site ofof bleeding
bleeding is usually aa small
is usually small noncontractile
noncontractile
and Critical
and Critical Care,
Care, Albert
Albert Einstein
Einstein Medical
Medical Center,
Center, 5401
5401 Old
Old York
York
Rd, Philadelphia PA
Rd, Philadelphia 19141; e-mail:
PA 19141; e-mail: azjarali1@gmail.com
azfarali1@gmail.com vessel
vessel on on the
the parietal
parietal pleura,
pleura, where
where vascular
vascular adhe-
adhe-
DOl: 1O.1378/chest.08-0725
DOl: 1O.1378/chest.08-0725 sions
sions exist.
exist. Second,
Second, bleeding
bleeding may may result
result from
from thethe

1056
1056 Special Features
Special Features
Table I-Etiology
Table I-Etiology of
of Hemothorax *
Hemothorax * In aa review
In review by by Hsu
Hsu et al 16
et al 16 of
of 488
488 patients
patients with
with
spontaneous
spontaneous pneumothorax
pneumothorax over over aa 6-year
6-year period,
period, 27 27
Category, --
Category.. - - Specific Etiology
Specific Etiology
patients
patients (5.5%)
(5.5%) had had an an associated
associated hemothorax
hemothorax (25 (25
Pneumothorax
Pneumothorax SHP
SHP men; mean
men; mean age,age, 22.3
22.3 years).
years). The The amount
amount of of blood
blood
Coagulopathy
Coagulopathy Congenital diseases
Congenital diseases
Hemophilia, Glanzman thrombasthenia
that was
that was drained
drained ranged
ranged fromfrom 400400 to 1,700 mL,
to 1,700 mL, with
with
Hemophilia, Glanzman thrombasthenia
Acquired
Acquired aa third
third of of the
the patients
patients in in shock.
shock. Twenty-one
Twenty-one patients
patients
Drug related
Drug related underwent
underwent VATS VATS withinwithin 1 1 day
day after
after admission,
admission, and and 6 6
Vascular
Vascular AVM (OWR
AVM (OWR disease)
disease) patients
patients were were treated
treated conservatively
conservatively with with tube
tube tho-
tho-
VRD
VRD racostomy
racostomy alone alone withwith no no recurrence
recurrence of of hemopneu-
hemopneu-
Aneurysms
Aneurysms
EDS type
type IVIV
mothorax.
mothorax.
EDS
Connective tissue
Connective tissue disease
disease Kakaris et
Kakaris al 21
et al 21 reviewed
reviewed 3,4893,489 patients
patients with
with spon-
spon-
Neoplasia
NeoplaSia Bone/soft tissue
Bone/soft tissue taneous pneumothorax
taneous pneumothorax between between 1970 1970 and and 2000.
2000.
Schwannoma (VRD)
Schwannoma (VRD) Seventy-one patients
Seventy-one patients (2%) (2%) [63 [63 menmen and and 8 8 women
women
Thymic growths
Thymic growths aged from
aged from 1313 to
to 8080 years]
years] presented
presented with with hemopneu-
hemopneu-
Vascular tumors
Vascular tumors
mothorax. Conservative
mothorax. Conservative treatment
treatment alone alone was
was effec-
effec-
Germ cell
Germ tl1'nl!lirrs
cell tl1'nl!lirrs
Hepatocellular
Hepatocellular tive in
tive in 1616 cases
cases (22.5%).
(22.5%). Among
Among the the other
other 5555 patients
patients
Lung cancer
Lung cancer (77.5%),
(77.5%), 28 28 patients (39.4%) were
patients (39.4%) operated on
were operated on anan
Mesothelioma
Mesothelioma emergency basis
emergency basis because
because they they were
were in in shock,
shock, while
while
PNET
PNET 27 patients
27 patients (38%)(38%) had had elective
elective surgery
surgery for clot
for clot
Miscellaneous
Miscellaneous Exostoses
Exostoses
evacuation, resection
evacuation, resection of of aa bulla,
bulla, ligation
ligation of of adhesions
adhesions
Extramedullary hematopoiesis
Extramedullary hematopoiesis
Endometriosis
Endometriosis between the
between the parietal
parietal and and visceral
visceral pleura,
pleura, andand irriga-
irriga-
Pulmonary sequestration
Pulmonary sequestration tion of the
tion of the pleural cavity.r!
pleural cavity.21
GI surgery
GI surgery A retrospective
A retrospective review reviews" 27 of
of 2424 patients with SHP
patients with SHP
'OWR == Osler-Weber-Rendu
'OWR Osler-Weber-Rendu disease;
disease; VRD
VRD == von
von Recklinghausen
Recklinghausen that compared
that compared 11 11 patients
patients treated
treated with with early
early VATS
VATS
disease; PNET
disease; PNET == primitive
primitive neuroectodermal tumor.
neuroectodermal tumor. and 13
and 13 treated
treated with with initial
initial conservative treatment
conservative treatment
and subsequent
and subsequent surgery surgery when
when needed
needed demonstrated
demonstrated
that
that the early VATS
the early VATS group group had had longer operating
longer operating
rupture of
rupture of vascularized
vascularized bullae
bullae and and underlying
underlying lung lung time, less
time, less preoperative
preoperative blood blood loss,
loss, less
less blood
blood trans-
trans-
parenchyma.v'-"
parenchyma,l6,17 Third, Third, it it may result from
may result from torntorn fusion
fusion (465
(465 mLmL vs vs 1,044
1,044 mL),mL), shorter
shorter period
period ofof chest
chest
congenitally aberrant
congenitally aberrant vessels
vessels between
between the the parietal
parietal tube drainage
tube drainage (4 days vs
(4 days vs 77 days),
days), andand shorter
shorter length
length
bullae. 3,17 In their series of patients with
pleura and bullae.v'" of stay
of stay (all
(all pp values
values < < 0.05),27
0.05),27
SHP, Tatebe
SHP, Tatebe and and coworkers
coworkers-s 18 reported
reported the the mucoid
mucoid In summary,
In summary, although
although therethere is still some
is still debate
some debate
degeneration and
degeneration and sclerotic
sclerotic changes
changes in in the
the arterial
arterial about the
about the management
management of of the
the patient
patient who who stabilizes
stabilizes
wall
wall ofof aberrant
aberrant vessels.
vessels. The factors that
The factors that disrupt
disrupt after aa chest
after chest tube
tube insertion without further
insertion without further blood
blood
normal homeostasis
normal homeostasis include
include the the fact
fact that
that bleeding
bleeding loss, the
loss, the predominant
predominant opinion opinion favors
favors prompt
prompt surgical
surgical
from
from the
the parietal
parietal pleura
pleura is not subject
is not subject to to control
control by by management for
management for most
most of of the
the patients
patients withwith SHP,28
SHP,28
collapse of
collapse of the
the lung
lung and
and that
that systemic
systemic BP BP is about six
is about six particularly
particularly in in aa patient
patient whowho is in shock,
is in shock, has rebleed-
has rebleed-
times that
times that inin the
the pulmonary
pulmonary arterialarterial circulation.
circulation. ing after
ing after lung
lung expansion,
expansion, or or fails
fails conservative
conservative man- man-
Systemic arteries
Systemic arteries are
are likely
likely the
the source
source of of bleeding
bleeding in in agement after
agement after 2424 h.h.
the more
the more massive
massive hemopneumothoraces.
hemopneumothoraces.
Treatment of
Treatment of hemopneumothorax,
hemopneumothorax, in in addition
addition
to
to fluid
fluid resuscitation
resuscitation and and blood
blood transfusion,
transfusion, consists
consists COAGULOPATHY
COAGULOPATHY
of
of the
the insertion
insertion of of an
an intercostal
intercostal tube,tube, followed
followed by by
surgical
surgical intervention,
intervention, via via either
either video-assisted
video-assisted tho- tho- Hemothorax associated
Hemothorax associated with
with aa coagulopathy
coagulopathy is is pre-
pre-
racic
racic surgery
surgery (VATS)
(VATS) or or open
open thoracotomy.
thoracotomy. This This is is dominantly
dOminantly aa result of anticoagulants
result of anticoagulants administered
administered in in
widely
widely considered
considered to to bebe aa surgical
surgical emergency
emergency be- be- the
the setting
setting of
of thromboembolic disease. 29
thromboembolic disease. 29,3o There
There are
are
cause
cause of the capability
of the capability of of the
the vessels
vessels to to bleed
bleed freely
freely 20 well-documented
20 well-documented cases
cases ofof anticoagulant-associated
anticoagulant-associated
as mentioned
as mentioned above.above. hemothorax. The
hemothorax. The coagulation
coagulation parameters
parameters were su-
were su-
We found
We found 10 10 series
series of
of patients
patients described
described in literature
in literature pratherapeutic in
pratherapeutic in six cases, and
six cases, and heparin
heparin was
was admin-
admin-
with
with aa total of 240
total of 240 patients,
patients, close
close toto 200
200 of of whom
whom istered in
istered in intermittent
intermittent bolus
bolus form
form in eight patients.
in eight patients.
were treated
were treated surgically
surgically (> 50% with
(> 50% VATS),16-25 and
with VATS),16-25 and There
There were
were equal number of
equal number of men
men andand women.
women. MostMost
the recurrence
the recurrence rate rate ofof bleeding
bleeding was was < < 1%1% in in all
all of the
of the cases
cases occurred
occurred inin the
the setting
setting ofof treatment
treatment with
with
groupS.20
groupS.20 Haciibrahimoglu
Haciibrahimoglu et a1 26
et a1 26 found
found an an incidence
incidence heparin and warfarin,
heparin and warfarin, and
and there
there waswas atat least
least one
one
of 3%
of 3% (9 cases out
(9 cases out ofof 291 spontaneous pneumotho-
291 spontaneous pneumotho- well-documented case
well-documented case with
with enoxparin.
enoxparin.P 31
races), and conservative
races), and conservative treatment
treatment was was adequate
adequate in in Overall, hemothorax
Overall, hemothorax is is very
very rare
rare in
in the
the setting
setting ofof
seven patients.
seven patients. anticoagulation, and
anticoagulation, and most
most cases
cases occur
occur within
within the
the first
first

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CHEST /134/5/ NOVEMBER, 2008 1057
1057
week of
week of therapy
therapy and and onon the
the samesame side side as
as the
the initial
initial all women
all women with with HHTHHT should
should be be screened
screened for for pulmo-
pulmo-
symptoms.v-
symptoms. 32 Hemothorax
Hemothorax with with anticoagulation
anticoagulation in in the
the nary AVMs
nary AVMs beforebefore becoming
becoming pregnant.pregnant.
setting of
setting of pulmonary
pulmonary embolism
embolism has has aa high
high morbidity
morbidity Ehlers-Danlos syndrome
Ehlers-Danlos syndrome (EDS) (EDS) type type IV IV hashas been
been
and mortality,
and mortality, with
with 44 patients
patients dying dying of of the
the 20
20 patients
patients associated with
associated with hemothorax
hemothorax in in the
the setting
setting of of internal
internal
reported. Rupture
reported. Rupture of of aa pulmonary
pulmonary infarct infarct should
should bebe mammary artery
mammary rupture:52
artery rupture: 52,.53 This form
,.53 This form of of the
the EDS,
EDS,
suspected when
suspected when aa patient
patient with
with aa proven
proven or or suspected
suspected also known
also known as as vascular
vascular type,type, results
results from
from deficient
deficient or or
pulmonary thromboembolic
pulmonary thromboembolic disease disease has has abrupt
abrupt cir-
cir- defective type
defective type III III collagen.
collagen. Arterial
Arterial tears tears are are thethe
culatory and
culatory and respiratory
respiratory insufficiency
insufficiency in in association
association most serious
most serious complication,
complication, and and pulmonary
pulmonary manifes- manifes-
with aa rapidly
with rapidly accumulating
accumulating unilateralunilateral pleural
pleural effu-
effu- tations besides
tations besides hemothorax
hemothorax include include hemoptySiS
hemoptysis from from
sion 7 to
sion to 10
10 days
days out,
out, compared
compared to to aa slowly
slowly develop-
develop- pulmonary artery
pulmonary artery rupture
rupture or or tears
tears in in lung
lung paren-
paren-
ing effusion
ing effusion in in aa purely
purely anticoagulant-related
anticoagulant-related hemo- hemo- chyma, thick-walled
chyma, thick-walled cavities
cavities resulting
resulting from from previous
previous
thorax.P
thorax. 33 AA rupture
rupture of of an
an infarct
infarct is is aa rare
rare complication
complication lung rupture, pneumothoraces,
lung rupture, pneumothoraces, bullous bullous lung lung disease,
disease,
of thromboembolic
of thromboembolic disease disease because
because only only 10%
10% areare panacinar emphysema,
panacinar emphysema, pulmonary pulmonary cysts, cysts, and and bron-
bron-
complicated by
complicated infarcts.34
by infarcts. 34.3.5
.3.5 chiectasis.v'
chiectasis: 54 Hemothorax
Hemothorax has has beenbeen reported
reported with with
In our
In our review,
review, twotwo cases
cases of of hemothorax
hemothorax.:.~:th antico-
with antico- EDS in
EDS in the
the setting
setting of of pneumothorax
pneumothorax also..5.5 also..5.5
agulation in
agulation the nonpulmonary
in the nonpulmonary embolic embolic setting
setting were
were Another frequent
Another frequent cause cause of of bleeding
bleeding is is von
von Reck-Reck-
reported: one
reported: one case
case was
was after
after angioplasty,36
angioplasty;" and and the
the linghausens disease,
linghausens disease, or or neurofibromatosis.
neurofibromatosis. Bleeding Bleeding
other case
other case was
was inin the
the setting
setting of of anticoagulation
anticoagulation for for an
an in the
in the abdominal
abdominal cavity, cavity, retroperitoneum,
retroperitoneum, and and softsoft
artificial heart
artificial heart valve.
valve.!l Hemothorax
Hemothorax has has been
been reported
reported tissues has
tissues been reported
has been reported as as aa result
result of of rupture
rupture of of
in the setting
in the setting of of tissue
tissue plasminogen
plasminogen activator activator use
use for
for friable vasculature
friable vasculature by by either
either vascular
vascular invasion
invasion by by
venous thrombosis
venous thrombosis in in aa patient
patient with with pneumonia.
pneumonia.F 37 neurofibromas or
neurofibromas or arterial
arterial dysplasia..56
dysplasia..56 In In this
this disease,
disease,
Congenital hemorrhagic
Congenital hemorrhagic disorders
disorders are are another
another cause
cause bleeding occurred
bleeding occurred eithereither by by the
the rupture
rupture of of associated
associated
for aa spontaneous
for spontaneous hemothorax.
hemothorax. Hemophilia Hemophilia was was vascular anomalies.57
vascular anomalies-? or or by
by direct
direct rupture
rupture of of aa schwa-
schwa-
associated in
associated in eight
eight cases,
cases, mostly
mostly in in men,8.38-41
men,8.38-41 andand nomma.P"
nomma: 58 The
The incidence
incidence of of vascular
vascular lesions
lesions in in neu-
neu-
Glanzmans thrombasthenia
Glanzmans thrombasthenia in one. 42
in one. 42 rofibromatosis type
rofibromatosis type 1 1 have
have beenbeen reported
reported to to bebe only
only
Treatment of
Treatment of anticoagulation-induced
anticoagulation-induced hemothorax hemothorax 3.6%:59 Two
3.6%:59 Two types
types of of vascular
vascular involvement
involvement have have beenbeen
should focus
should focus on on the
the correction
correction of of the
the coagulopathy.
coagulopathy. described: (1)
described: (1) stenotic
stenotic or or aneurysmal
aneurysmal alterationsalterations in in
If rupture
If rupture of of aa pulmonaIY
pulmonary infarct infarct is is suspected,
suspected, aa large vessels
large vessels suchsuch as as the
the aorta
aorta and and itsits branches
branches like like
surgical consultation
surgical consultation should
should be be obtained
obtained for for possible
possible the intercostal
the intercostal artery,artery, subclavian,60,61
subclavian,60,61 and and internal
internal
thoracotomy and
thoracotomy and resection.
resection.F 33 thoracic, which
thoracic, which may may rupture
rupture or or dissect
dissect62 62,63; and (2)
,63; and (2)
dysplastic features
dysplaStiC features in in smaller
smaller vessels.
vessels.
Treatment consists
Treatment consists of of thoracotomy
thoracotomy and and surgical
surgical
ligation of
ligation of the
the bleeding
bleeding vessels,
vessels, primarily
primarily indicated
indicated in in
VASCULAR
the presence
the presence of of active
active bleeding
bleeding with with associated
associated
Aortic dissection
Aortic dissection or or rupture
rupture is is aa major
major reported
reported cause
cause hemodynamic compromise.
hemodynamic compromise. A A more
more conservative
conservative ap- ap-
of hemothorax.
of hemothorax. It It occurs
occurs mostly
mostly on on left
left side,
side, with
with proach with
proach with endovascular
endovascular embolization
embolization or or nonopera-
nonopera-
hundreds of
hundreds of cases
cases having
having been
been reported.
reported. We We will
will not
not tive management
tive management can can bebe taken
taken in in case
case of of hemody-
hemody-
discuss aortic
discuss aortic dissection
dissection in in detail
detail in in this
this review.
review. namic stability;
namic stability; however,
however, more more frequent
frequent postprocedural
postprocedural
We found
We found 32 32 reported
reported casescases of of hemothorax
hemothorax asso- asso- monitoring is
monitoring is required.
required. PollakPollak et et alal64
64 reviewed 155
reviewed 155
ciated with
ciated with pulmonary
pulmonary vascular
vascular malformations.
malformations. In In patients (148
patients (148 patients
patients with with HHT) HHT) with with pulmonary
pulmonary
about half
about half the
the cases,
cases, thethe patients
patients had had documented
documented AVMs who
AVMs who underwent
underwent embolization
embolization dUring during aa periodperiod
Osler-Weber-Rendu disease.
Osler-Weber-Rendu disease. AVMs
AVMs are are found
found in in 15
15 of 33 years.
of years. TheThe symptoms
symptoms during during follow-up
follow-up consisted
consisted
to 33%
to 33% of of cases
cases in
in this
this disease.
disease. 43 43-46
- 46 of respiratory
of respiratory manifestations
manifestations (n (n = = 13),
13), cerebral
cerebral isch- isch-
Hemoptysis is
Hemoptysis more common
is more common than than hemothorax
hemothorax in in emia (n
emia (n = = 4),
4), brain
brain abscess
abscess (n (n = = 5),5), hemoptysis
hemoptysis
this disease.
this disease.t" 47 In
In aa review
review ofof 143
143 patients
patients with
with heredi-
heredi- (n =
(n = 3),
3), andand seizure
seizure (n (n == 1).1). The
The problems
problems were were
tary hemmorhagic
tary hemmorhagic telangiectasia
telangiectasia (HHT)-associated
(HHT)-associated pul- pul- related to
related to growth
growth of of AVMs,
AVMs, recanalization,
recanalization, residual residual
monary AVMs,
monary AVMs, 66 had had hemothorax.
hemothorax." 48 Contrast
Contrast echo-
echo- AVMs, and
AVMs, and collateral
collateral or or accessory
accessory feeding feeding vessels.
vessels.
cardiography, blood
cardiography, blood gasgas analysis
analysis on on 100%
100% oxygen,49
oxygen,49 Clinical and
Clinical and anatomic
anatomic evaluation
evaluation after after pulmonary
pulmonary
and CT
and CT angiography.5°
angiography.5° are are the
the screening
screening measures
measures AVM embolization
AVM embolization is is important
important in in order
order to to detect
detect
shown to
shown to be
be useful
useful to to diagnose
diagnose pulmonary
pulmonary AVMs. AVMs. these lesions.
these lesions. Patients
Patients withwith persistent,
persistent, reperfused,
reperfused, or or
An interesting
An interesting reported
reported association
association was was that
that 30
30 to
to enlarging lesions
enlarging lesions often
often have
have symptoms,
symptoms, but but aa signif-
signif-
50% patients
50% patients with
with AVMAVM bleeding
bleeding were pregnant:51
were pregnant: 51 icant minority
icant minority of of patients
patients are are asymptomatic.
asymptomatic. More More
In all
In all cases,
cases, pulmonary
pulmonary hemorrhage
hemorrhage occurred occurred in in the
the frequent assessment
frequent assessment may may improve
improve detection
detection before before
second or
second or third
third trimesters,
trimesters, suggesting
suggesting that that the
the risk
risk of
of the onset
the onset of of symptoms.
symptoms.s- 64

spontaneous rupture
spontaneous rupture increases
increases as as blood
blood volume
volume and and Coil embolization
Coil embolization is is certainly
certainly effective
effective for for reduc-
reduc-
cardiac output
cardiac output increase.
increase. SomeSome authors
authors-" suggest that
48 suggest that ing right-to-Ieft
ing right-to-Ieft shunt,shunt, improving
improving arterial arterial hypoxemia,
hypoxemia,

1058
1058 Special Features
Special Features
and increasing
and increasing exercise
exercise capacity
capacity inin patients
patients with
with et al
et 77 reviewed
al77 reviewed 10 10 patients
patients with
with hemothorax
hemothorax and and
large or
large or numerous
numerous pulmonary
pulmonary AVMs.65
AVMs.65 InIn the
the setting
setting HCC; 55 patients
HCC; patients had
had drainage
drainage of hemothorax, 33 pa-
of hemothorax, pa-
of aa hemothorax,
of hemothorax, particularly
particularly with
with hemodynamic
hemodynamic tients were
tients were managed
managed without
without chest
chest drainage,
drainage, 11 patient
patient
instability or
instability or recurrence,
recurrence, aa surgical
surgical approach
approach should
should had transcatheter
had transcatheter arterial
arterial embolization,
embolization, while
while an-
an-
be considered.
be considered. other patient
other patient had
had transcatheter
transcatheter arterial
arterial injection
injection of
of
alcohol. All
alcohol. All patients
patients in
in this
this series
series died within 33 months
died within months
of the
of the diagnosiS
diagnosis of
of hemothorax.
hemothorax. Specific
Specific treatment
treatment ofof
NEOPLASIA
NEOPLASIA
neoplasia-associated hemothorax
neoplasia-associated hemothorax depends
depends onon the
the tu-
tu-
mor, but
mor, but the
the prognosis
prognosis for all malignancy-associated
for all malignancy-associated
The most
The most common
common neoplasias
neoplasias associated
associated with with hemo-
hemo- hemothoraces remains
hemothoraces remains poor.
poor.
thorax include
thorax include schwanommas
schwanommas of of von
von Recklinghausen
Recklinghausen
disease (discussed
disease (discussed above) above) and and soft-tissue
soft-tissue tumors
tumors such such
as sarcomas,
as sarcomas, angiosarcomas,
angiosarcomas, and and hepatocellular
hepatocellular car- car- MISCELLANEOUS
MISCELLANEOUS
cinomas. Lung
cinomas. Lung cancercancer is is aa distinctly
distinctly uncommon
uncommon
cause of hemothorax even in iIllJ.1e
the setting of pleural Exostoses
Exostoses
extension. Other
extension. Other associated
associated causes causes are are rupture
rupture of of Hemothorax is
Hemothorax is aa rare
rare but
but well-documented
well-documented com- com-
thymomas'"
thymomas 66 or
or thymic
thymic cysts,
cysts, liver
liver metastasis,
metastasis, pericy-
pericy- plication of
plication of exostoses
exostoses andand has
has only
only been
been described
described in in
tomas.f" and
tomas,67 and some
some germgerm cell cell tumors,68.69
tumors,68.69 like like "cystic"
"cystic" about 16
about 16 patients
patients (6 (6 with
with hereditary
hereditary multiple
multiple exos-exos-
chondroblastoma."?
chondroblastoma. 7o toses, and
toses, and two two solitary
solitary exostoses)
exostoses) in in the
the English-
English-
Angiosarcoma is
Angiosarcoma is aa rare
rare tumor
tumor of of endothelial
endothelial cells cells 82-84
literature. 82
language literature.
language - 84
that originates
that originates in in small
small bloodblood vessels.
vessels. It It can
can affect
affect The etiological
The etiological mechanisms
mechanisms proposed
proposed by by most
most
any organ,
any organ, but but it it usually
usually occurs
occurs in in skin,
skin, soft
soft tissue,
tissue, authors are
authors are the
the shearing
shearing of of the
the pleura
pleura or or diaphragm
diaphragm
breast, and visceral
breast, and visceral organs.7
organs.7 11,72 The most
,72 The most common
common by the
by the relatively
relatively sharp
sharp margins
margins of of the
the intrathoracic
intrathoracic
metastatic site
metastatic site forfor angiosarcoma
angiosarcoma is is the
the lung;
lung; there-
there- exostosis or
exostosis or erosion
erosion of of an
an adjacent
adjacent vessel
vessel thatthat can
can be
be
fore, pulmonary
fore, pulmonary complications
complications such such as as hemoptysis,
hemoptysis, dilated because
dilated because of of chronic
chronic frictional
frictional irritation 85,86
irritation85 and
,86 and
pneumothorax, hemothorax,
pneumothorax, hemothorax, atelectaSis,
atelectasis, and and postob-
postob- inflammation. Most
inflammation. Most of of the
the cases
cases have
have been
been reported
reported
structive pneumonia
structive pneumonia have have been been reported
reported in in the
the in the
in first 33 decades
the first decades of of life,
life, with
with only
only aa couple
couple of of
literature both
literature both fromfrom primary
primary thoracic
thoracic and and metastatic
metastatic cases in
cases in elderly
elderly men.
men. Exostoses
Exostoses are are hard
hard to to detect
detect onon
angiosarcomas. 73 73-75
- 75
angiosarcomas. aa plain
plain radiograph.
radiograph. A A CT
CT with
with bone
bone windows
windows is is better
better
The prognosis
The prognosis of of patients
patients with with angiosarcoma-
angiosarcoma- delineating the
in delineating
in the location
location andand angulation
angulation of of exostoses.
exostoses.
associated hemothorax
associated hemothorax is is poor,
poor, and and we we found
found aa 6- 6- to
to Exostoses are
Exostoses are usually solitaryS7,88 but
usually solitary87,88 but may
may be be multiple
multiple
8-month mortality
8-month mortality rate> rate> 80% 80% in in our
our review.
review. In In aa as part
as part of
of hereditary
hereditary multiple exostoses.89
multiple exostoses. 89-92
- 92 A
A surgical
surgical
recent review,
recent review, MoriyaMoriya et et aF6
aF6 reported
reported 17 17 patients
patients approach by
approach by VATS
VATS or or aa thoracotomy
thoracotomy is is needed
needed to to resect
resect
with bloody
with bloody effusion
effusion or or bloody
bloody pleUrisy
pleurisy withwith vascular
vascular the exostoses
the exostoses andand prevent
prevent recurrence.
recurrence.
tumors. Most
tumors. Most patients
patients in in the
the series
series presented
presented with with
dyspnea, hemoptysis,
dyspnea, hemoptysis, and and chest
chest pain pain TheThe notable
notable
Endometriosis
Endometriosis
finding in
finding in thethe series
series was was occurrence
occurrence of of bilateral
bilateral
spontaneous hemothorax
spontaneous hemothorax in in 99 of of the
the 17 17 patients.
patients. Endometriosis is
Endometriosis is another
another etiology
etiology for
for spontaneous
spontaneous
However the
However the pleural
pleural fluidfluid hematocrit
hematocrit in in this
this review
review hemothorax or
hemothorax or hemopneumothorax.
hemopneumothorax. This This isis seen
seen in
in
ranged from
ranged from 25 25 toto 50%,
50%, and and therefore
therefore only only aa fewfew menstruating women
menstruating women and and may
may present
present as as symptoms,
symptoms,
patients had
patients had true
true hemothorax
hemothorax by by definition
definition but but the
the signs, and
signs, and radiographic
radiographic changes
changes that
that recur
recur in in the
the
mortality was
mortality was similar
similar to to aa true
true hemothorax
hemothorax (90% (90% at at chest concurrent
chest concurrent withwith menses.
menses. SuchSuch phenomena,
phenomena,
10 months).
10 months). termed catamenial
termed catamenial by by Dr.
Dr. Lilington
Lilington in in 1972,
1972, maymay
Hepatocellular carcinoma
Hepatocellular carcinoma (HCC) (HCC) is is another
another tumortumor take on
take on aa number
number of of clinical
clinical manifestations,
manifestations, includ-
includ-
that can
that can present
present as as aa hemothorax,
hemothorax, particularly
particularly when when ing pneumothorax,
ing pneumothorax, hemothorax,
hemothorax, hemoptysis,
hemoptysis, pulmo-
pulmo-
it is
it is metastatic
metastatic to to the
the chest.
chest. ThisThis is is aa very
very rare
rare compli-
compli- nary nodules,
nary nodules, chest
chest pain,
pain, dyspnea,
dyspnea, and
and others,93
others.F' Two
Two
cation, with
cation, with high high mortality
mortality secondary
secondary to to uncontrolla-
uncontrolla- thirds of
thirds of these
these patients
patients have
have aa patholOgiC
pathologic diagnosis
diagnosis of of
ble hemorrhage."?
ble hemorrhage. Most of
77 Most of the
the cases
cases of of hemothorax
hemothorax thoracic endometriosis
thoracic endometriosis on on surgery,
surgery, pOSSibly
possibly due due toto
are reported
are reported from from Asia Asia and and sub-Saharan
sub-Saharan Mrica, Africa, cyclical changes
cyclical changes in in pathology
pathology withwith menstruation.
menstruation.
where the
where the cancer
cancer is is prevalent.
prevalent. The The frequent
frequent sites sites of
of Catamenial hemothorax
Catamenial hemothorax represents
represents aa common
common man- man-
metastasis for
metastasis for HCCHCC include
include the the lung,
lung, lymph
lymph node,node, ifestation of
ifestation of thoracic
thoracic endometriosis
endometriosis syndrome,
syndrome, oc- oc-
adrenal glands,
adrenal glands, and and bones.
bones. Metastatic
Metastatic HCCs, HCCs, like like the
the curring in
curring in 14%
14% ofof known
known cases,
cases, and
and concomitant
concomitant
primary tumor,
primary tumor, are are usually
usually highly
highly vascular
vascular and and cancan pelvic endometriosis
pelviC endometriosis was was found
found in
in 100%
100% of of cases.
cases.v' It
94 It
lead to
lead to spontaneous
spontaneous or or secondary
secondary hemothorax.
hemothorax" 78 The
The is unilateral
is unilateral and
and affects
affects the
the right
right side
side >> 80%
80% of of the
the
most common
most common symptoms symptoms reported reported in in different
different case case time; however,
time; however, bilateral
bilateral hemothorax
hemothorax from from thoracic
thoracic
79-- 81
reports 79
reports 81included
included dyspnea
dyspnea and and chest
chest pain.
pain. Sohara
Sohara endometriosis has
endometriosis been reported.
has been reported.v" The majority
95 The majority of of

www.chestjournal.org
www.chestjournal.org CHEST /134/5/
CHEST /134/5/ NOVEMBER,
NOVEMBER, 2008
2008 1059
1059
these
these patients
patients are
are young,
young, nulliparous,
nulliparous, blackblack women.w
women. 96
Patients
Patients may may be hemodynamically stable
be h~~ynamically stable with
with an an
effusion.
effusion. TheThe etiology
etiology of of thoracic
thoracic endometriosis
endometriosis isis
not
not clear
clear but
but includes
includes possible
possible transdiaphragmatic
transdiaphragmatic
migration
migration of of endometrial
endometrial tissue
tissue via
via naturally
naturally occur-
occur-
ring
ring fenestrations
fenestrations and and occurring
occurring moremore frequently
frequently on on
the
the right
right side.
side. Hematogenous
Hematogenous or or lymphatic
lymphatic spread
spread
after
after uterine
uterine manipulation
manipulation from from pelvic
pelvic procedures
procedures isis
another
another possibility.
possibility. Diagnosis
Diagnosis isis often
often delayed
delayed for for
months
months or or even
even years,
years, and
and aa high
high index
index of of suspicion
suspicion
isis necessary.
necessary.
The
The goal
goal ofof medical
medical therapy
therapy forfor the
the thoracic
thoracic endo-
endo-
metriosis
metriosis syndrome
syndrome isis to to minimize
minimize estrogen
estrogen secre-
secre-
tion.
tion. AA number
number of of hormonal
hormonal agents
agents are are utilized
utilized to to
achieve
achieve this
this objective.
objective. Danazol,
Danazol, aa derivativ.t0f
derivative of 17-a-17-a.-
ethinyltestostyerone,
ethinyltestostyerone, minimizesminimizes the the midcycle
midcycle surgesurge
ofof leutinizing
leutinizing hormone,
hormone, thus thus mimicking
mimicking the the chronic
chronic
anovulatory
anovulatory state.
state. Progestational
Progestational agentsagents havehave been
been
given
given to to induce
induce atrophy
atrophy of of endometrial
endometrial tissue. tissue.
Gonadotropin-releasing
Gonadotropin-releasing hormone hormone agonists
agonists minimize
minimize
follicle-stimulating
follicle-stimulating hormone
hormone and and leutinizing
leutinizing hormone
hormone FIGURE 1.
FIGURE 1. CT
CT ofofthe
the chest
chest showing
showing pleural
pleural effusion
effusion and
and paren-
paren-
secretion,
secretion, inducing
inducing hypogonadotropic
hypogonadotropic gonadism.gonadism. OralOral chymal
chymal nodules.
nodules.

contraceptive
contraceptive pills,pills, which
which cause
cause amenorrhea,
amenorrhea, have have
also
also been used. 97 In
beenused.?? In general,
general, medical
medical therapy
therapyalone
alone isis
insufficient
insufficient forfor the
the treatment
treatment of ofthe
the pleural
pleural manifes-
manifes- CD34
CD34 (Fig
(Fig 3),
3), and factor VIII
and factor VIII antigen.
antigen. The
The patient
patient
tations
tations of
ofthoracic endometriosis. 93 Recurrence
thoracic endometriosis.s' Recurrence often often survived
survived only
only 33 months.
months. Primary
Primary or metastatic I05
or metastatic-P"
necessitates
necessitates pleural
pleural inspection,
inspection, with with resection
resection of of pleural
pleural angiosarcoma
angiosarcoma should
should be
be considered
considered aa cause
cause
endometrial
endometrial implants.
implants. Repair
Repair of of diaphragmatic
diaphragmatic fen- fen- of
of spontaneous
spontaneous hemothorax,
hemothorax, especially
especially in
in patients
patients
estrations
estrations hashas also
also been suggested.98
been suggested. 98 Hysterectomy
Hysterectomy with
with bilateral
bilateral or
or intractable hemothorax. 107
intractable hemothorax.t??
and
and oopherectomy
oopherectomy have have also
also been
been performed
performed as as aa
surrogate
surrogate for
for hormonal
hormonal therapy,
therapy, withwith varied
varied results.
results.
DIAGNOSIS
DIAGNOSIS AND
AND MANAGEMENT
MANAGEMENT
Idiopathic
Idiopathic
Once
Once aa bloody
bloody effusion
effusion isis obtained,
obtained, thethe first
first step
step
AA definite
definite cause
cause for
for spontaneous
spontaneous hemothorax
hemothorax isis should
should be
be toto check
check the
the hematocrit
hematocrit to to confirm
confirm aa
usually identifiable with investigation. If
usually identifiable with investigation. Ifpreliminary
preliminary hemothorax.
hemothorax. A A hemorrhagic
hemorrhagic effusion
effusion should
should prompt
prompt
investigations remain non diagnostic, the case
investigations remain nondiagnostic, the case maymay suspicion
suspicion of
of aa malignancy,
malignancy, infections
infections like
like tuberculo-
tuberculo-
come to a thoracotomy or an autopsy to identify aa
come to a thoracotomy or an autopsy to identify
definite etiology.99 It
definite etiology.99 It isis very
very rare
rare to
to fail
fail to
to identify
identify aa
cause for hemothorax with only three to four cases
cause for hemothorax with only three to four cases in in
the literature, which remained truly idiopathic. -
the literature, which remained truly idiopathic. 1OO 103
1OO -103

Bilateral
Bilateral Spontaneous
Spontaneous Hemothorax
Hemothorax
This
This isis aa very
very rare
rare entity.
entity. We
We found
found eight
eight cases
cases in
in
the
the literature
literature of of spontaneous
spontaneous bilateral
bilateral hemothorax
hemothorax
reported
reported over over the
the last
last 50
50 years.
years. Four
Four ofof them
them were
were
related
related to to vascular
vascular tumors,
tumors, specifically
specifically angiosarco-
angiosarco-
mas.7575,76,104,
mas. 105 One
,76,104,105 One each
each was
was related
related toto aa subclavian
subclavian
artery
arteryaneurysm,
aneurysm, endometriosis,
endometriosis, an an iatrogenic
iatrogenic coagu-
coagu-
lopathy,106 and
Iopathy.t?" and oneone was
was idiopathic.
idiopathic. The
The ninth
ninth case
case isis
that
that ofof aa 56-year-old
56-year-old womanwoman we we recently
recently encoun-
encoun-
tered
tered whowho presented
presented with with bilateral
bilateral spontaneous
spontaneous he- he-
mothorax
mothorax and and lung
lung nodules
nodules (Fig
(Fig 1).
1). Pathology
Pathology re- re-
vealed
vealed an an epitheloid
epitheloid angiosarcoma,
angiosarcoma, aa highly
highly vascular
vascular
tumor
tumor bothboth in in the
the pleura
pleura and
and the
the lung
lung (Fig
(Fig 2),
2), with
with FIGURE 2.
FIGURE 2. Lung
Lung nodule
nodule showing
showing clusters
clusters of
ofepitheloid
epitheloid cells
cells with
with
immunochemistry
immunochemistry revealing revealing positivity
positivity forfor CD31,
CD31, sinusoidal
sinusoidal spaces
spaces (hematoxylin-eosin, original XX 200).
(hematoxylin-eosin, original 200).

1060
1060 Special Features
Special Features
fistula,
fistula, vascular tumors, coagulopathies,
vascular tumors, coagulopathies, neurofibro-
neurofibro-
matosis, or
matosis, or exostoses.
exostoses. It It is important to
is important to realize
realize that
that
even aa hemothorax
even hemothorax can can appear
appear to to be
be like
like aa hemor-
hemor-
rhagic
rhagic effusion
effusion with
with aa lower hematocrit after
lower hematocrit after aa few
few
days
days because
because there
there isis significant
significant dilution
dilution in in 33 to
to 44
days.
days. This
This maymay bebe aa problem
problem because
because the the presenta-
presenta-
tion
tion with
with aa hemothorax
hemothorax is is not
not always
always acute,
acute, particu-
particu-
larly
larly in
in aa young
young adult
adult with
with no no underlying
underlying respiratory
respiratory
compromise.
compromise. The The flowchart
flowchart in Figure 44 shows
in Figure shows an an
algorithmic approach
algorithmic approach to to the
the various
various steps
steps in the diag-
in the diag-
nosis. Besides
nosis. Besides the
the coagulation
coagulation parameters,
parameters, aa contrast-
contrast-
enhanced CT
enhanced CT of
of the
the chest
chest yields
yields helpful
helpful information
information
about
about the
the etiology.
etiology. An echocardiogram with
An echocardiogram with bubble
bubble
study points
study points toward
toward aa pulmonary
pulmonary AVM AVM if if there
there is is aa
delayed
delayed appearance
appearance of of bubbles
bubbles in in the
the right
right heart
heart
FIGURE 3.
FIGURE 3. Immunostaining
Immunostaining showing
showing strong
strong positivity
positivitywith vimentin
with vimentin chambers.
chambers. The The yield
yield from
from pleural
pleural cytology
cytology is is vari-
vari-
(original X 100).
(original X 1(0).
able. It is
able. It is hard
hard to
to make
make an an unequivocal
unequivocal diagnosis
diagnosis of of
the
the usual
usual neoplastic
neoplastic etiologies
etiologies suchsuch asas angiosarcomas
angiosarcomas
sis,
sis, or pulmonary infarction.
or pulmonary infarction. A
A hemothorax
hemothorax identified
identified solely
solely on
on the
the basis
basis of
of cytology
cytology of of the
the pleural
pleural fluid,
fluid, but
but
by aa hematocrit
by hematocrit of
of 25
25 to
to 50%
50% that
that of
of blood
blood should
should immunochemical
immunochemical markers markers may may increase
increase the the yield
yield and
and
lead
lead one
one to
to consider
consider workup
workup forfor aa vascular
vascular etiology
etiology degree
degree of of confidence.
confidence. In In case
case ofof endometriosis,
endometriosis, the the
either
either aa dissecting
dissecting aneurysm,
aneurysm, AVM/arteriovenous
AVM/arteriovenous findings
findings usually
usually include
include nonspecific
nonspecific hemosiderin-
hemosiderin-

Pleural Effusion
Pleural Effusion II
II Hematocrit(Hct)
Hematocrit(Hct)

Hct<50%of
Hct<50%of
blood
blood II 1
1 II blood
Hct>50%of
Hct>50%of
blood

II II II
~
~
~ ~
1
1
II Pleural Fluid
Pleural Fluid CT chest
CT chest after
after Pleural fluid
Pleural fluid culture!
culture!
II Chest
Chest Tube
Tube
Drainage
Drainage
Cytology
Cytology drainage
drainage AFB/Serum BUN/Cr
AFB/Serum BUN/Cr
II
II II 1
1
~
Bronchogeniccancer
Bronchogenic cancer 1
1 11
TB
TB
Pulmonary infarction
Pulmonary infarction
II medication
PTlPTTlPlatelet count!
PTlPTTlPlatelet count!
medication history
history
~
CT with
CT with contrast
drainage
drainage
contrast after
after
f---
f---
Echo with
Echo with bubble
study (shunt
study
bubble
(shunt study).
study).
Uremia
Uremia If vascular
If vascular
Mesotheliomas

II
Mesotheliomas anomaliessuspected
anomalies suspected

1
Coagulopathies
Coagulopathies

Congenital or
or Acquired
1
!!
Congenital Acquired
coagulopathy
coagulopathy

II Vascular
Vascular II Non-Vascular I
Non-Vascular I
Anomalies
Anomalies
II
!! ~ Bony-
Bony-
AVM's
AVM's Soft tissue
Soft tissue anomalies
anomalies
(±OWR syndrome)
(±OWR syndrome) masses
masses Exostoses
Exostoses
Neurofibromatosis
Neurofibromatosis
Aoeurysms(intercostal •• internal
Aoeurysms(intercostal internal
mammary)
mammary)
(±EDS,VRD)
(±EDS,VRD) Biposy
Biposy
Needle or
Needle or
Open±
Open±
Auid
Fluid
Aogiosarcomas
Aogiosarcomas cytology
cytology
Endometriosis
Endometriosis
Thymoma/thymic cyst
Thymoma/thymic cyst
Pleuralmetastases
Pleural metastases
Germ cell
Germ tumors
cell tumors

FIGURE 4.
FIGURE Flowchart showing
4. Flowchart showing thethe steps
steps inin the
the evaluation
evaluation of of aa spontaneous
spontaneous hemothorax;
hemothorax;
echocardiography; TB
echo == echocardiography;
echo tuberculosis; AFB
TB == tuberculosis; acid-fast bacilli;
AFB == acid-fast bacilli; Cr
Cr == creatinine; PI' == prothrombin
creatinine; PI' prothrombin time;
time;
PIT == partial
PIT partial thromboplastin time. See
thromboplastin time. Table 1
See Table 1 footnote
footnote for
for expansion
expansion of abbreviations.
of abbreviations.

www.chestjournal.org
www.chestjournal.org CHEST/134/5/ NOVEMBER, 2008
CHEST /134/5/ NOVEMBER, 2008 1061
1061
Table
Table 2-History
2-History and
and Physical
Physical Examination
Examination Clues to
Clues to pleura, setting the
pleura, setting the stage
stage for for aa trapped
trapped lung. lung. In In its
its
Etiology in
Etiology in Hemothorax
Hemothorax early
early development,
development, this this thinthin membrane
membrane has has little
little
Age
Age
substance
substance and and is is attached
attached very very loosely
loosely to the under-
to the under-
Systemic symptoms
Systemic symptoms (weight
(weight loss)
loss) lying pleural
lying pleural surface.
surface. By By thethe seventh
seventh day, day, there
there is is an
an
Hemoptysis
Hemoptysis angioblastic
angioblastic and and fibroblastic
fibroblastic proliferation.
proliferation. The The mem-mem-
Trauma
Trauma oror surgery
surgery brane continues
brane continues to to thicken
thicken by by progressive
progressive deposition
deposition
Medication
Medication history
history (antiplatelets,
(antiplatelets, anticoagulants)
anticoagulants)
and
and organization
organization of of the
the coagulum
coagulum within within the the cavity.
cavity.
Bleeding
Bleeding tendencies
tendencies (epistaxis,
(epistaxis, hematuria,
hematuria, joint
joint effusions)
effusions)
Periodicity of effusions
Periodicity of effusions (association
(association with
with menstrual
menstrual cycles)
cycles)
An
An understanding
understanding of of the the pathologic
pathologic featuresfeatures of of aa
Cafe au
Cafe au last spots or
lait spots or neurofibromas
neurofibromas clotted hemothorax
clotted hemothorax makes makes it it clear
clear that,
that, if if possible,
possible,
Telangiectasias
Telangiectasias the
the clotted
clotted hemothorax
hemothorax should should be be evacuated
evacuated within within 7 7
Myasthenia
Myasthenia to 10
to 10 days
days of of injury.
injury. If, If, however,
however, the the hemothorax
hemothorax is is
Hyperextensible
Hyperextensible joints
joints not evacuated
evacuated by by the the tenth
tenth day, day, thethe clotted
clotted blood
not blood
Increased
Increased skin
skin laxity
laxity
cannot be
cannot be easily
easily removed
removed and and aa decortication
decortication is is
usually
usually required.
required. 109. This complication
109. 110 This complication can can occur
occur
in as
in as many
many as as 55 toto 30%
30% of of patients,
patients, 108 and up
108 and up toto 40%
40%
laden histiocytes,
histiocytes, but but rarely
rarely endometrial
endometrial epithelial of these
of patients will
these patients will require
require further
further surgical
surgical inter-
inter-
laden epithelial
cells vention for
vention for nonresolving,
nonresolving, complicated complicated intrapleural
intrapleural
cells may
may be identified. A
be identified. A good
good history and physical
history and physical
examination can can yield collections, empyema,
collections, empyema, or or fibrothorax.
fibrothorax. The The placement
placement
examination yield valuable
valuable cluesclues as
as to the etiol-
to the etiol-
ogy of aa hemothorax
hemothorax (Table of additional
of additional chest chest tubes tubes to to treat
treat aa retained
retained hemo- hemo-
ogy of (Table 2). 2).
thorax
thorax is ineffective because
is ineffective because of of the
the presence
presence of of
As far
As far as
as management
management is is concerned,
concerned, it it depends
depends on on
clotted blood
clotted blood and and loculationsJl1
loculationsJl1
the cause
the cause and and is discussed under
is discussed under the
the respective
respective
subheadings. Thoracostomy
Thoracostomy drainagedrainage is is usually
usually thethe VATS
VATS is believed to
is believed to be the best
be the best available
available modality
modality
subheadings.
first step
step inin aa stable
stable patient.
patient. In In aa hemodynamically for
for the management of
the management of clotted
clotted hemothorax,
hemothorax, with with
first hemodynamically
unstable patient
patient or or if
if the
the rate
rate of of bleeding
bleeding is is >
> 500
500 efficacy
efficacy rates upwards of
rates upwards of 80%.
80%. VATS VATS is is an
an accurate,
accurate,
unstable
mUh in
mUh in the
the first
first hour
hour oror > 200 to
> 200 to 300 mUh subse-
300 mUh subse- safe,
safe, and
and reliable
reliable operative
operative therapy therapy for for retained
retained post- post-
quently, an an early
early surgical
surgical approach
approach with with ongoing
ongoing resus-
resus- traumatic
traumatic pleural pleural collections,
collections, even even in in patients
patients present-
present-
quently,
citation is favored. Correction
Correction of of the coagulopathy ing later
ing later thanthan the conventionally accepted
the conventionally accepted 3- 3- toto 5-day
5-day
citation is favored. the coagulopathy
may be be all
all that
that is
is needed
needed in in cases
cases ofof anticoagulant-
anticoagulant- postevent window.U''
postevent window. 1l2
may
induced VATS
VATS was was foundfound to to be superior to
be superior to intrapleural
intrapleural
induced bleeding.
bleeding. Imaging
Imaging is is important
important if the suspi-
if the suspi-
cion is high forfor an fibrinolytic therapy
fibrinolytic therapy (IPFT), (IPFT), both both in in terms
terms of of de-de-
cion is high an AVM/arteriovenous
AVM/arteriovenous fistula. This
fistula. This
may include
include aa CT CT angiogram creased hospital
creased hospital stay stay and and needneed for for thoracotomy.108
thoracotomy.l'"
may angiogram or or rarely
rarely an an arterio-
arterio-
gram. However, fibrinolysis
However, fibrinolysis with with streptokinase
streptokinase is an effec-
is an effec-
gram. Embolization
Embolization remainsremains an an option
option in in treatment
treatment
of vascular
vascular anomalies.
anomalies. The management of of residual tive adjunctive
tive adjunctive treatment treatment in in hemothorax
hemothorax as shown by
as shown by
of The management residual
blood multiple case
multiple case reports
reports and reviews. In
and reviews. In aa multicenter
multicenter
blood is is controversial,
controversial, withwith aa growing number favor-
growing number favor-
ing early
early VATS
VATS particularly if aa significant
significant amount
amount of of trial 113
trial of 48
11 3 of 48 patients
patients with with loculated
loculated effusions
effusions includ-
includ-
ing particularly if
clot is present to to prevent
prevent fibrothorax
fibrothorax and ing
ing 1414 patients
patients with with nonmalignant
nonmalignant hemothorax hemothorax and and 4 4
clot is present and restrictive
restrictive
patients with
patients with malignant
malignant hemothorax,hemothorax, the the authors
authors
physiology.
physiology.
found
found aa 92% 92% success
success rate rate for for fibrinolysis.
fibrinolysis. The The samesame
success
success rate rate has has beenbeen reported
reported by by other
other authors.
authors.U" 1I4
Untreated Herrwthorax
Untreated Herrwthorax
Kimbrell
Kimbrell et et al 1l5
1l5 studied 25
al studied 25 patients
patients with>with> 300 300 mL mL
Drainage of
Drainage of hemothorax
hemothorax > > 200
200 to
to 300
300 mL mL of of of hemothorax
of hemothorax after after 33 days days of of tube
tube thoracostomy
thoracostomy by by
blood is
blood necessary for
is necessary for symptomatic
symptomatic management
management as as administration of
administration of 250,000
250,000 IV IV of streptokinase or
of streptokinase or
well as
well as the
the prevention
prevention of of long-term
long-term complications
complications of of 100,000 IV
100,000 IV of of urokinase
urokinase mixed mixed in in 5050 mLmL of of sterile
sterile
leaving
leaving itit untreated.
untreated. A A retained
retained hemothorax
hemothorax can can lead
lead saline solution
saline solution with with dwelldwell time time of of 44 hh for
for 66 days,
days, andand
to infection,
to infection, chronic
chronic fibrothorax,
fibrothorax, lung
lung entrapment,
entrapment, achieved resolution
achieved resolution in in 2323 patients
patients (90%)(90%) without without
and impaired
and impaired pulmonary
pulmonary function.
function. If If the
the pleural
pleural bleeding complications.
bleeding complications.
effusion
effusion persists
persists despite
despite tube
tube thoracostomy,
thoracostomy, aa diag-
diag- IPFT has
IPFT has beenbeen found found to to be be safe
safe without
without major major
nosis
nosis of clotted or
of clotted or retained
retained hemothorax
hemothorax can can bebe made,
made, concern
concern for for systemic
systemic fibrinolysis,
fibrinolysis, although
although some some side side
which
which in the trauma
in the trauma literature
literature is defined as
is defined an absence
as an absence effects like
effects like nausea,
nausea, fever, fever, and and myalgias,
myalgias, and and one one case
case
of radiologic
of radiologic improvement
improvement despite
despite tube
tube thoracostomy
thoracostomy of hemorrhage
of hemorrhage have have been been reported.
reported.U" 116 As proposed
As proposed
on the
on the second
second or or third
third day
day after
after the
the event.
event.l'"
108 by some
by some authors,116
authors.U" aa reasonable reasonable approachapproach in in aa stable
stable
Once the
Once the clot
clot has been allowed
has been allowed to organize, it
to organize, it will
will patient
patient can can be be toto first
first utilize
utilize IPFT IPFT and, and, ifif unsuccess-
unsuccess-
adhere to
adhere to the
the lung
lung and
and pleura,
pleura, making
making itit difficult
difficult to
to ful, can
ful, can proceed
proceed to to surgical
surgical therapy;
therapy; however,
however, dura- dura-
remove. The
remove. The agitation
agitation of
of cardiac
cardiac and
and respiratory
respiratory move-
move- tion of
tion of hospital
hospital stay stay may may be be aa deterrent
deterrent to to its
its use.
use.
ment rapidly
ment rapidly defibrinates
defibrinates the
the blood,
blood, and
and aa fibrin
fibrin clot
clot IPFT is
IPFT is not
not an an option
option in in cases
cases in in which
which the the hemo-
hemo-
thus formed
thus formed is is deposited
deposited onon the
the visceral
visceral and
and parietal
parietal thorax is
thorax related to
is related to aa coagulopathy
coagulopathy or or vascular
vascular lesions
lesions

1062
1062 Special Features
Special Features
such as
such as AVMs
AVMs and
and angiosarcomas because it
angiosarcomas because it may
may 16
16 Hsu Hsu NY,
NY, Hsieh
Hsieh NJ,NJ, Liu
Liu HP,
HP, etet aI.aI. Video-assisted
Video-assisted thoraco-
thoraco-
worsen t~,~leeding. In
worsen In such
such cases,
cases, aa surgical
surgical ap-
ap- scopic
scopic surgery
surgery for for spontaneous
spontaneous hemopneumothorax.
hemopneumothorax. World World
JJ SurgSurg 1998;
1998; 22:23-26
22:23-26
proach may
proach may be
be the
the only
only option.
option.
17
17 Luh Luh SP,SP, Tsao
Tsao TC-TC-Y.Y. Video-assisted
Video-assisted thoracicthoracic surgery
surgery forfor
spontaneous
spontaneous haemopneumothorax.
haemopneumothorax. Respirology Respirology 2007; 2007; 12:12:
443-447
443-447
SUMMARY
SUMMARY 18 Tatebe
18 Tatebe S, Kanazawa H,
S, Kanazawa H, Yamazaki
Yamazaki Y, Y, et aI. Spontaneous
et aI. Spontaneous
hemopneumothorax. Ann
hemopneumothorax. Ann Thorac
Thorac Surg Surg 1996;
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62:1011-1015
We have
We have reviewed
reviewed thethe major
major etiologies
etiologies ofof sponta-
sponta- 19
19 Chang
Chang IT, IT, Dai Dai ZK,
ZK, Kao
Kao EL.EL. Early
Early video-assisted
video-assisted thoracic
thoracic
neous (nontraumatic)
neous (nontraumatic) hemothorax.
hemothorax. The The major
major points
points surgery for
surgery for primary
primary spontaneous
spontaneous hemopneumothorax.
hemopneumothorax.
that were emphasized include the following:
that were emphasized include the following: (1) (1) World JJ Surg
World Surg 2007;
2007; 31:19-25
31:19-25
distinguishing hemorrhagic effusions from
distinguishing hemorrhagic effusions from true he- true he- 20
20 Hwong
Hwong TM, TM, Ng Ng CS,CS, Lee TW, et
Lee TW, et al. Video-assisted thoracic
al. Video-assisted thoracic
mothorax because
mothorax because these
these two
two entities
entities point
point toward
toward aa surgery for
surgery for primary
primary spontaneous
spontaneous hemopneumothorax.
hemopneumothorax. Eur Eur
JJ Cardiothorac
Cardiothorac Surg Surg 2004;
2004; 26:893-896
26:893-896
different set of differential diagnoses; (2) differential
different set of differential diagnoses; (2) differential 21 aI. Spontaneous
21 Kakaris
Kakaris S, S, Athanassiadi
Athanassiadi K, Vassilikos K,
K, Vassilikos K, etet aI. Spontaneous
diagnosis with
diagnosis with attention
attention toto major
major etiologies
etiologies and
and anan hemopneumothorax aa rare
hemopneumothorax rare but
but life-threatening
life-threatening entity. entity. Eur
Eur
algOrithmic approach to
algorithmic approach to the the diagnosis;
diagnosis; andand (3)
(3) gen-
gen- JJ Cardiothorac
Cardiothorac Surg Surg 2004; 25:856-858
2004; 25:856-858
eral management
eral management of of hemothociax
hemotboeax with conservative
with conservative 22 Hsiao
22 CW, Lee
Hsiao CW, Lee SC,SC, Tzao
Tzao C, C, et
et aI.aI. Minithoracotomy
Minithoracotomy with with
and surgical approaches with attention to
and surgical approaches with attention to etiology
etiology simultaneous video-assisted
simultaneous video-assisted thoracoscopic
thoracoscopic surgery surgery vs video-
vs video-
assisted thoracoscopic
assisted thoracoscopic surgery
surgery forfor spontaneous
spontaneous hemopneu-
hemopneu-
specific treatments.
specific treatments. mothorax.
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23 Wu
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video-assisted
thoracic surgery
thoracic surgery forfor spontaneous
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