Spontaneous Haemomediastinum and Fatal Haemoperitoneum in woman with Vascular Ehlers-Danlos Syndrome
Journal Title: Archives of Pulmonology and Respiratory Care - Year 2017, Vol 3, Issue 2
Abstract
Vascular Ehlers-Danlos Syndrome (EDS) presents with fragility of blood vessels, with high incidence of fatal hemorrhages in middle-age adults. We present a 38-year old female with vascular EDS presented to the emergency unit with spontaneous hemomediastinum and hemothorax. A selective arteriography showed a bronchial artery aneurysm that could be embolized. Coagulated hemothorax was cleared up using intrapleural instillation of urokinase. Acute massive hemoperitoneum happened a few hours later, due to spontaneous disruption of a splenic artery aneurysm. It could not be managed and led to the decease of the patient. Multifocal bleeding is a frequent threatening event in vascular EDS.
Authors and Affiliations
Cal Isabel, Fernández Elena, Méndez Jose V, Jarabo Jose R
Keywords
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- EP ID EP340842
- DOI 10.17352/aprc.000024
- Views 67
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