Spontaneous Haemomediastinum and Fatal Haemoperitoneum in woman with Vascular Ehlers-Danlos Syndrome

Isabel Cal, Elena Fernández, Jose V Méndez and Jose R Jarabo*

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.

Keywords: Ehlers-Danlos syndrome; Bronchial aneurysm; Haemomediastinum; Haemoperitoneum

Published on: May 29, 2017 Pages: 48-49

Full Text PDF Full Text HTML DOI: 10.17352/aprc.000024
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