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

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.