Hemothorax pathophysiology: Difference between revisions
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* [[Ehlers-Danlos syndrome]] (EDS) forms part of a spectrum of genetically based [[connective tissue disorders]] that includes [[osteogenesis imperfecta]]. [[Ehlers-Danlos type IV syndrome|Vascular Ehlers-Danlos syndrome (EDS IV)]] is characterized by an alteration in the [[Ehlers-Danlos syndrome|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. | * [[Ehlers-Danlos syndrome]] (EDS) forms part of a spectrum of genetically based [[connective tissue disorders]] that includes [[osteogenesis imperfecta]]. [[Ehlers-Danlos type IV syndrome|Vascular Ehlers-Danlos syndrome (EDS IV)]] is characterized by an alteration in the [[Ehlers-Danlos syndrome|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. | ||
* [[Hereditary hemorrhagic telangiectasia|Osler–Weber–Rendu disease]], also known as [[hereditary hemorrhagic telangiectasia]] (HHT), is an [[Genetic Disorders|autosomal dominant disease]] characterized by multiple [[Skin|cutaneous]], systemic, and/or [[Lung|pulmonary]] [[arteriovenous malformations]] (AVMs). Bleeding from pulmonary lesions usually occur as [[Hemoptysis|haemoptysis]] and rarely as Spontaneous Haemothorax. | * [[Hereditary hemorrhagic telangiectasia|Osler–Weber–Rendu disease]], also known as [[hereditary hemorrhagic telangiectasia]] (HHT), is an [[Genetic Disorders|autosomal dominant disease]] characterized by multiple [[Skin|cutaneous]], systemic, and/or [[Lung|pulmonary]] [[arteriovenous malformations]] (AVMs). Bleeding from pulmonary lesions usually occur as [[Hemoptysis|haemoptysis]] and rarely as Spontaneous Haemothorax. There is an association between Osler-Weber-Rendu disease and bilateral heterochronic spontaneous hemothorax. | ||
* [[Rib|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. | * [[Rib|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. | ||
Revision as of 20:19, 16 March 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Haemothorax is a pathologic collection of blood within the pleural cavity, between the lung surface and inner chest wall. Three mechanisms of bleeding in haemothorax include torn adhesion between the parietal and visceral pleurae, rupture of neovascularized bullae as a complication of subpleural emphysematous blebs, and torn congenital aberrant vessels branching from the cupola and distributed in and around the bulla in the apex of the lung. There are some genetic disorder that are predisposed to 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. There is an association between Osler-Weber-Rendu disease and bilateral heterochronic spontaneous hemothorax.
- 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.