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==[[Hemothorax natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
==[[Hemothorax natural history, complications and prognosis|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 result in respiratory distress, lung entrapment with impaired pulmonary function, retained clot, chronic fibrothorax, empyema and extended hospitalization if untreated.


==Diagnosis==
==Diagnosis==

Revision as of 20:19, 8 March 2018


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

Synonyms and keywords: Hematothorax; haemothorax

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

Classification

Spontaneous haemothorax (SH) is a subcategory of haemothorax.

Pathophysiology

Pathogenesis

Three mechanisms of bleeding in haemothorax:

  • a 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

  • EDS forms part of a spectrum of genetically based connective tissue disorders that includes osteogenesis imperfect. 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.
  • 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.
  • 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.

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 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, mediastinal or pulmonary malignancy, metastatic renal carcinoma, Abrikossoff tumor, pulmonary angiosarcoma, osterochondroma, Kaposiform endodermal sinus tumour, hemangioendothelioma, epithelioid hemangioendothelioma, hemangioma, hemangiopericytoma fibrous tumor of the pleura, hepatocellular carcinoma, periosteal chondroma, chondroblastoma of the rib, synovial sarcoma, osteosarcoma, Ewig sarcoma, neurofibrosarcoma, thymoma, mediastinal meningioma, thoracic neuroblastoma, pleural mesothelioma, chronic myeloid leukaemia, neurofibromatosis type I (Morbus von Recklinghause), chondrosarcomas, ectopic meningioma, germ cells tumors.
  • Connective tissue disorders:Vascular Ehlers–Danlos syndrome (Ehlers–Danlos type IV, EDS IV), Marfan syndrome, Loeys–Dietz syndrome, familial thoracic aortic aneurysm syndrome, Shprintzen–Goldberg syndrome, Type I neurofibromatosis (NF-1) or Von recklinghausen disease (VRD).
  • Pleural disorders: spontaneous pneumothorax, Spontaneous pneumohemothorax (the accumulation of >400 mL of blood in the pleural cavity in association with spontaneous pneumothorax), 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: 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: 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, anticoagulants administration, Haemothorax has been also reported in the setting of plasminogen activator user for venous thrombosis in patient with pneumonia.
  • Miscellaneous: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), Extramedullary haematopoiesis (EMH).

Iatrogenous haemothorax

consequence of 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 and translumbar aortography.

Differentiating Hemothorax from other Diseases

Epidemiology and Demographics

Risk Factors

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 result in respiratory distress, lung entrapment with impaired pulmonary function, retained clot, chronic fibrothorax, empyema and extended hospitalization if untreated.

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | CT | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

Related Chapters

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