Hemothorax

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Hematothorax; haemothorax

Hemothorax

Overview

Hemothorax as a clinico-pathological entity can be defined in two ways. Morphologically, it is a pathologic collection of blood within the pleural cavity, between the lung surface and inner chest wall. Clinically , hemothorax is defined as a pleural fluid with a hematocrit ranging from at least 25–50% of peripheral blood. In cases of long standing haemothorax due to haemodilution, hemothorax can appear with lower levels of hematocrit. massive hemothorax is defined as the drainage of more than 1500 cc of blood upon chest tube insertion.

Historical Perspective

Haemothorax has been detailed in numerous medical writings dating back to ancient times. In 1794, the first intercostal incision was developed by John Hunter to treat and drainage of the hemothorax.

Classification

Spontaneous haemothorax (SH) is a subcategory of haemothorax.

Pathophysiology

Pathogenesis

Three mechanisms of bleeding in haemothorax:

• torn adhesion between the parietal and visceral pleurae.
• Rupture of neovascularized bullae as a complication of subpleural emphysematous blebs.
• Torn congenital aberrant vessels branching from the cupola and distributed in and around the bulla in the apex of the lung.

Genetics

• Ehlers-Danlos syndrome (EDS) forms part of a spectrum of genetically based connective tissue disorders that includes osteogenesis imperfecta. Vascular Ehlers-Danlos syndrome (EDS IV) is characterized by an alteration in the COL3A1 gene. This gene encodes type III collagen and The alteration of this type of collagen produces aneurisms and ruptures of vessels and organs than can lead to haemothorax.

• Osler–Weber–Rendu disease, also known as hereditary hemorrhagic telangiectasia (HHT), is an autosomal dominant disease characterized by multiple cutaneous, systemic, and/or pulmonary arteriovenous malformations (AVMs). Bleeding from pulmonary lesions usually occur as haemoptysis and rarely as Spontaneous Haemothorax.

• Costal exostosis or Osteochondroma is an autosomal dominant hereditary abnormality and the most common benign thoracic bone tumor. It often presents singly or in multiple sites that can cause laceration of the lung and hemothorax.

• Hemophilia A is a X-linked hereditary disorder of blood clotting that caused by the development of an inhibitor against coagulation factor VIII (FVIII). Hemophilia A manifests with early muscle and subcutaneous bleeding and rarely with haemothorax.

• Glanzmann thromboastenia is an autosomal-recessive bleeding disorder characterized by a lifelong bleeding tendency due to abnormalities of the platelet integrin αΠbβ3 [[[glycoprotein]] (GP) IIb; CD41/IIIa; CD61]. Glanzmann thromboastenia usually presents with mucocutaneous bleeding such as easy bruising, purpura, gingival bleeding, epistaxis, menorrhagia, haemarthrosis, haematuria, intracranial and visceral hemorrhage are rare but even rarer is Spontaneous Haemothorax.

• Type I neurofibromatosis (NF-1) or Von recklinghausen disease (VRD) is an autosomal dominant disease. This entity can affect any organ system, and is characterized by skin tumors and abnormal cutaneous pigmentation. Pathogenetic mechanisms for vasculopathy associated with VRD are: (I) direct vascular invasion from adjacent tumors; and (II) vascular dysplasia with thickening and concomitant reduced strength of the vessel wall and aneurysm formation.
• There are other hereditary entities such as Loeys–Dietz syndrome, familial thoracic aortic aneurysm syndrome, or Shprintzen–Goldberg syndrome that are predisposed to aortic dissection and haemothorax.

Causes

Traumatic haemothorax

• Blunt force injury cases such as those that occur in vehicular collisions and following falls or jumps from heights.
• Penetrating thoracic injuries produced by stab or gunshot wounds.

Spontaneous or non-traumatic haemothorax

Spontaneous haemothorax is a rare clinical condition in the absence of trauma or iatrogenic causes. Bilateral spontaneous haemothorax is a very rare entity and the main cause of it, is primary or metastatic pleural angiosarcoma.

• Vascular disorders causing spontaneous hemothroax include aortic aneurysm rupture, rupture of thoracic aortic dissection - dissection is due to arterial hypertension followed by atherosclerosis -, rupture of a saccular aortic aneurysm and traumatic rupture of the pericardial sac during cardiopulmonary resuscitation in individuals with hemopericardium, fatal spontaneous dissection of supra-aortic vessels without any evidence of aortic disease during pregnancy and early puerperium, bronchial artery aneurysm rupture, aneurysmatic internal thoracic artery, intercostal vessels, internal mammary artery aneurysm, or pulmonary congenital aberrant vessels, ruptured mycotic aneurysms, innominate truncal dissection, using neck veins for mainlining and rupture of a subclavian artery aneurysm, pulmonary arteriovenous malformations (AVMs), fatal and non-fatal AVM-associated massive hemothorax is often linked to Osler–Weber–Rendu disease, associated with congenital heart disease such as rupture of a patent ductus arteriosus, Eisenmenger syndrome, aortic coarctation and bicuspid aortic valve disease.

• Malignancies causing spontaneous hemothroax include lymphangiosarcoma and vascular mediastinal schwannoma, schwanommas of von Recklinghausen disease, lymphangioma, mediastinal teratoma, metastatic choriocarcinoma, metastatic renal carcinoma, Abrikossoff tumor, pulmonary angiosarcoma, osteochondroma, Kaposiform endodermal sinus tumour, hemangioendothelioma, hemangioma, hemangiopericytoma fibrous tumor of the pleura, hepatocellular carcinoma, periosteal chondroma, chondroblastoma of the rib, synovial sarcoma, osteosarcoma, Ewing sarcoma, neurofibrosarcoma, thymoma, mediastinal meningioma, thoracic neuroblastoma, pleural mesothelioma, chronic myeloid leukaemia, neurofibromatosis type I (Morbus von Recklinghause), chondrosarcomas, ectopic meningioma and germ cells tumors.

• Connective tissue disorders causing spontaneous hemothroax include Vascular Ehlers–Danlos syndrome (Ehlers–Danlos type IV, EDS IV), Marfan syndrome, Loeys–Dietz syndrome, familial thoracic aortic aneurysm syndrome, Shprintzen–Goldberg syndrome and Type I neurofibromatosis (NF-1) or Von recklinghausen disease (VRD).

• Pleural disorders causing spontaneous hemothroax include spontaneous pneumothorax, spontaneous pneumohemothorax (the accumulation of >400 mL of blood in the pleural cavity in association with spontaneous pneumothorax) and pleural metastasis.

• Costal exostoses or osteochondroma occurs either sporadically or as a manifestation of a genetic disorder known as hereditary multiple exostoses (HME). Lesions mainly occur in infants and children and their complications include haemothorax, pneumothorax, diaphragmatic or pericardial lacerations and visceral pleural injury.

• Gynecological disorders causing spontaneous hemothroax include Intrathoracic implantation of ectopic endometrial tissue occurs as a result of migration of endometrial tissue through the diaphragm. Spontaneous haemothorax may be a response to cyclical hormonal changes in menstruating women.

• Hematological disorders causing spontaneous hemothroax include hemophilia, immune-mediated platelet destruction due to Many drugs such as sedatives, tranquilizers, anticonvulsants, and heparin, anticoagulant-associated hemothorax, specifically, as a result of thrombembolic disease treatment, hematology-related hemothorax include Glanzmann thrombasthenia, thrombotic thrombocytopenic purpura, and intrathoracic extramedullary hematopoiesis, intrathoracic extramedullary hematopoiesis due to a secondary process, such as myeloproliferative disorders, hemolytic anemia, hereditary spherocytosis, chronic asthma, and Gaucher disease, beta thalassemia, rupture of extramedullary hematopoietic pulmonary nodules, Haemothorax has been also reported in the setting of plasminogen activator user for venous thrombosis in patient with pneumonia.

• Miscellaneous causing spontaneous hemothroax include tuberculosis, necrotizing lung infection, uremia, spontaneous hemothorax secondary to a ruptured parasitic hydatid (Echinococcal) cyst of pulmonary parenchyma, Malaria is another rare parasitic etiology for spontaneous hemothorax, amyloidosis-induced spontaneous mediastinal hemorrhage with hemothorax due to perivascular and vascular wall involvement, systemic diseases like systemic lupus erythematosus (SLE) and Henoch-Schönlein purpura, pulmonary emboli, ectopic pregnancy, Extralobar pulmonary sequestration (EPS).

Iatrogenous haemothorax

Iatrogenous haemothorax may be caused by either intrathoracic vessel cannulation, chest drain insertion, needle thoracocentesis, pleural or lung biopsies, closed-chest cardiopulmonary resuscitation, placement of subclavian- or jugular-catheters, endoscopic thoracic interventions, cardiopulmonary surgery, sclerotherapy of oesophageal varices, rupture of pulmonary arteries after placement of Schwann–Ganz catheters, thoracic sympathectomy or translumbar aortography.

Epidemiology and Demographics

The exact incidence of haemothorax is not clear. Chest injuries occur in approximately 60% of all polytrauma cases and haemothorax is most frequently caused by chest trauma. The occurrence of haemothorax related to trauma in the United States is estimated to be 300,000 cases annually.

Natural History, Complications and Prognosis

Bleeding into the pleural space is exposed to the motion of the diaphragm, lungs, and other intrathoracic structures. The agitation of cardiac and respiratory movement defibrinates the blood, and a fibrin clot thus formed is deposited on the layers of pleura. Within several hours of cessation of bleeding, clot formation is inevitably and it will be difficult to remove. The membrane continues to thicken by progressive deposition, so the clotted haemothorax should be evacuated within a reasonable time after onset of bleeding. Chronic and retained hemothorax may progress to develop respiratory distress, lung entrapment with impaired pulmonary function, retained clot, chronic fibrothorax, empyema and extended hospitalization if left untreated.

Diagnosis

The most common symptoms of hemothorax include severe chest pain, and dyspnea that can be life threatening when hemodynamic instability and hypovolemic shock occurs. Some patients with hemothorax may have reduced concentrations of hemoglobin. On chest x-ray, hemothorax is characterized by meniscus of fluid blunting the costophrenic angle or diaphragmatic surface and tracking up the pleural margins of the chest wall. Ultrasonography may be helpful in the diagnosis of hemothorax. Computed Tomographic scan is not indicated in the initial trauma setting to diagnosis of hemothorax.

Treatment

Medical Therapy

The mainstay of medical therapy for hemothorax is, fluid resuscitation and blood transfusion. All patients, regardless of causes, require attention for fluid resuscitation and blood transfusion. Prophylactic use of antibiotics following haemothorax reduces the rate of infectious complications such as pneumonia and empyema during at least 24 hour after the start of chest tube drainage. Antibiotic treatment should be directed to Staphylococcus aureus and Streptococcus species and the use of first generation cephalosporins during the first 24 hour in patients treated with chest tube drainage is recommended. Intrapleural fibrinolytic therapy (IPFT) has been advocated as an alternative to evacuate residual blood clots and breakdown adhesions in low-resource settings where the relatively costly and sophisticated technique of VATS may not be available, feasible or applicable. Several studies report on IPFT with streptokinase, urokinase or tissue plasminogen activator (TPA). Duration of treatment with IPFT can vary between 2 and 9 days for streptokinase and 2–15 days for urokinase.

Surgical Therapy

The successful management of hemothorax depends on the severity of the blood loss and subsequent hemodynamic stability of the patient. The mainstay of therapy for hemothorax is intercostal chest drain (ICD) and oxygen therapy that significantly reduce the morbidity and mortality. Evacuation of haemothorax by chest tube does not succeed in all cases. The resultant retained intrapleural collections are referred to as Residual Haemothorax (RH).blood in the pleural cavity may organize and fibrose, resulting in a loss of lung volume and empyema if untreated. Video assisted thoracic surgery (VATS), minimally invasive surgery, has been found to be highly successful for treatment of these residual collections, especially when used early. VATS also can be used to treat patients with active blood loss but with stable haemodynamics, not only to stop the bleeding but also to evacuate blood clots and breakdown adhesions to prevent fibrothorax and restrictive physiology . An optimal period between the start of haemothorax and VATS of 48–72 h is repeatedly advocated and longer intervals lead to increased rates of complications, according to some authors. A longer time span increases the chance of intraoperative conversion to thoracotomy, prolongs postoperative drainage time and is associated with a higher incidence of hospital admissions. Thoracotomy with ongoing resuscitation is the procedure of choice for patients with haemodynamic instability due to massive haemothorax or active bleeding. The criteria for thoracotomy, are blood loss by chest tube 1.500 ml in 24 h or 200 ml per hour during several successive hours and the need for repeated blood transfusions to maintain haemodynamic stability. Surgical exploration allows control of the source of bleeding and evacuation of the intrathoracic blood; and also is required for adequate empyema drainage and/or decortication.

Prevention

There is no established method for prevention of hemothorax. However, early and adequate treatment which prevents of complication (suppuration) is necessary. Some factors which most frequently promote suppuration of the thoracic cavity, developing from traumatic haemothorax. so, attention is called to secure the necessary personal and material conditions to the preventive treatment.

Case Studies

Case #1

Related Chapters

• Cardiac Tamponade
• Hemopneumothorax
• Pleural Effusion
• Pneumothorax
• Tension Pneumothorax

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