(2008) Azfar H Et Al - Spontaneous Hemothorax
(2008) Azfar H Et Al - Spontaneous Hemothorax
(2008) Azfar H Et Al - Spontaneous Hemothorax
Special Features
Spontaneous Hemothorax*
A Comprehensive Review
Hakim Azfar Ali, MD; Michael Lippmann, MD, FCCP; Uday Mundathaje, MD;
and Ghulam Khaleeq, MD
Specific Etiology
Pneumothorax
Coagulopathy
SHP
Congenital diseases
Hemophilia, Glanzman thrombasthenia
Acquired
Drug related
AVM (OWR disease)
VRD
Aneurysms
EDS type IV
Connective tissue disease
Bone/soft tissue
Schwannoma (VRD)
Thymic growths
Vascular tumors
Germ cell tumors
Hepatocellular
Lung cancer
Mesothelioma
PNET
Exostoses
Extramedullary hematopoiesis
Endometriosis
Pulmonary sequestration
GI surgery
Vascular
Neoplasia
Miscellaneous
Coagulopathy
Hemothorax associated with a coagulopathy is predominantly a result of anticoagulants administered in
the setting of thromboembolic disease.29,30 There are
20 well-documented cases of anticoagulant-associated
hemothorax. The coagulation parameters were supratherapeutic in six cases, and heparin was administered in intermittent bolus form in eight patients.
There were equal number of men and women. Most
of the cases occurred in the setting of treatment with
heparin and warfarin, and there was at least one
well-documented case with enoxparin.31
Overall, hemothorax is very rare in the setting of
anticoagulation, and most cases occur within the first
CHEST / 134 / 5 / NOVEMBER, 2008
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Vascular
Aortic dissection or rupture is a major reported cause
of hemothorax. It occurs mostly on left side, with
hundreds of cases having been reported. We will not
discuss aortic dissection in detail in this review.
We found 32 reported cases of hemothorax associated with pulmonary vascular malformations. In
about half the cases, the patients had documented
Osler-Weber-Rendu disease. AVMs are found in 15
to 33% of cases in this disease.43 46
Hemoptysis is more common than hemothorax in
this disease.47 In a review of 143 patients with hereditary hemmorhagic telangiectasia (HHT)-associated pulmonary AVMs, 6 had hemothorax.48 Contrast echocardiography, blood gas analysis on 100% oxygen,49
and CT angiography50 are the screening measures
shown to be useful to diagnose pulmonary AVMs.
An interesting reported association was that 30 to
50% patients with AVM bleeding were pregnant.51
In all cases, pulmonary hemorrhage occurred in the
second or third trimesters, suggesting that the risk of
spontaneous rupture increases as blood volume and
cardiac output increase. Some authors48 suggest that
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all women with HHT should be screened for pulmonary AVMs before becoming pregnant.
Ehlers-Danlos syndrome (EDS) type IV has been
associated with hemothorax in the setting of internal
mammary artery rupture.52,53 This form of the EDS,
also known as vascular type, results from deficient or
defective type III collagen. Arterial tears are the
most serious complication, and pulmonary manifestations besides hemothorax include hemoptysis from
pulmonary artery rupture or tears in lung parenchyma, thick-walled cavities resulting from previous
lung rupture, pneumothoraces, bullous lung disease,
panacinar emphysema, pulmonary cysts, and bronchiectasis.54 Hemothorax has been reported with
EDS in the setting of pneumothorax also.55
Another frequent cause of bleeding is von Recklinghausens disease, or neurofibromatosis. Bleeding
in the abdominal cavity, retroperitoneum, and soft
tissues has been reported as a result of rupture of
friable vasculature by either vascular invasion by
neurofibromas or arterial dysplasia.56 In this disease,
bleeding occurred either by the rupture of associated
vascular anomalies57 or by direct rupture of a schwanomma.58 The incidence of vascular lesions in neurofibromatosis type 1 have been reported to be only
3.6%.59 Two types of vascular involvement have been
described: (1) stenotic or aneurysmal alterations in
large vessels such as the aorta and its branches like
the intercostal artery, subclavian,60,61 and internal
thoracic, which may rupture or dissect62,63; and (2)
dysplastic features in smaller vessels.
Treatment consists of thoracotomy and surgical
ligation of the bleeding vessels, primarily indicated in
the presence of active bleeding with associated
hemodynamic compromise. A more conservative approach with endovascular embolization or nonoperative management can be taken in case of hemodynamic stability; however, more frequent postprocedural
monitoring is required. Pollak et al64 reviewed 155
patients (148 patients with HHT) with pulmonary
AVMs who underwent embolization during a period
of 3 years. The symptoms during follow-up consisted
of respiratory manifestations (n 13), cerebral ischemia (n 4), brain abscess (n 5), hemoptysis
(n 3), and seizure (n 1). The problems were
related to growth of AVMs, recanalization, residual
AVMs, and collateral or accessory feeding vessels.
Clinical and anatomic evaluation after pulmonary
AVM embolization is important in order to detect
these lesions. Patients with persistent, reperfused, or
enlarging lesions often have symptoms, but a significant minority of patients are asymptomatic. More
frequent assessment may improve detection before
the onset of symptoms.64
Coil embolization is certainly effective for reducing right-to-left shunt, improving arterial hypoxemia,
Special Features
Neoplasia
The most common neoplasias associated with hemothorax include schwanommas of von Recklinghausen
disease (discussed above) and soft-tissue tumors such
as sarcomas, angiosarcomas, and hepatocellular carcinomas. Lung cancer is a distinctly uncommon
cause of hemothorax even in the setting of pleural
extension. Other associated causes are rupture of
thymomas66 or thymic cysts, liver metastasis, pericytomas,67 and some germ cell tumors,68,69 like cystic
chondroblastoma.70
Angiosarcoma is a rare tumor of endothelial cells
that originates in small blood vessels. It can affect
any organ, but it usually occurs in skin, soft tissue,
breast, and visceral organs.71,72 The most common
metastatic site for angiosarcoma is the lung; therefore, pulmonary complications such as hemoptysis,
pneumothorax, hemothorax, atelectasis, and postobstructive pneumonia have been reported in the
literature both from primary thoracic and metastatic
angiosarcomas.7375
The prognosis of patients with angiosarcomaassociated hemothorax is poor, and we found a 6- to
8-month mortality rate 80% in our review. In a
recent review, Moriya et al76 reported 17 patients
with bloody effusion or bloody pleurisy with vascular
tumors. Most patients in the series presented with
dyspnea, hemoptysis, and chest pain The notable
finding in the series was occurrence of bilateral
spontaneous hemothorax in 9 of the 17 patients.
However the pleural fluid hematocrit in this review
ranged from 25 to 50%, and therefore only a few
patients had true hemothorax by definition but the
mortality was similar to a true hemothorax (90% at
10 months).
Hepatocellular carcinoma (HCC) is another tumor
that can present as a hemothorax, particularly when
it is metastatic to the chest. This is a very rare complication, with high mortality secondary to uncontrollable hemorrhage.77 Most of the cases of hemothorax
are reported from Asia and sub-Saharan Africa,
where the cancer is prevalent. The frequent sites of
metastasis for HCC include the lung, lymph node,
adrenal glands, and bones. Metastatic HCCs, like the
primary tumor, are usually highly vascular and can
lead to spontaneous or secondary hemothorax.78 The
most common symptoms reported in different case
reports79 81 included dyspnea and chest pain. Sohara
www.chestjournal.org
Miscellaneous
Exostoses
Hemothorax is a rare but well-documented complication of exostoses and has only been described in
about 16 patients (6 with hereditary multiple exostoses, and two solitary exostoses) in the Englishlanguage literature.82 84
The etiological mechanisms proposed by most
authors are the shearing of the pleura or diaphragm
by the relatively sharp margins of the intrathoracic
exostosis or erosion of an adjacent vessel that can be
dilated because of chronic frictional irritation85,86 and
inflammation. Most of the cases have been reported
in the first 3 decades of life, with only a couple of
cases in elderly men. Exostoses are hard to detect on
a plain radiograph. A CT with bone windows is better
in delineating the location and angulation of exostoses.
Exostoses are usually solitary87,88 but may be multiple
as part of hereditary multiple exostoses.89 92 A surgical
approach by VATS or a thoracotomy is needed to resect
the exostoses and prevent recurrence.
Endometriosis
Endometriosis is another etiology for spontaneous
hemothorax or hemopneumothorax. This is seen in
menstruating women and may present as symptoms,
signs, and radiographic changes that recur in the
chest concurrent with menses. Such phenomena,
termed catamenial by Dr. Lilington in 1972, may
take on a number of clinical manifestations, including pneumothorax, hemothorax, hemoptysis, pulmonary nodules, chest pain, dyspnea, and others.93 Two
thirds of these patients have a pathologic diagnosis of
thoracic endometriosis on surgery, possibly due to
cyclical changes in pathology with menstruation.
Catamenial hemothorax represents a common manifestation of thoracic endometriosis syndrome, occurring in 14% of known cases, and concomitant
pelvic endometriosis was found in 100% of cases.94 It
is unilateral and affects the right side 80% of the
time; however, bilateral hemothorax from thoracic
endometriosis has been reported.95 The majority of
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