[Vascular Ehlers-Danlos syndrome: a clinical syndrome to be known by every anaesthesiologist and intensive care physician].
We report the case of a 29-year-old female who presented with a series of major vascular complications in rapid succession: haemothorax following rupture of a mammary artery aneurysm, pulmonary embolism, anterior myocardial infarction secondary to spontaneous dissection of the left anterior descending artery and rupture of a false aneurysm of the splenic artery. A diagnosis of Ehlers-Danlos syndrome (vascular variant) was considered the most likely in this context. Characterized by an extreme vascular fragility, this rare disease poses important clinical management issues for the anaesthetist and intensive care physician.
Fèvre
M-C
MC
Service de réanimation chirurgicale et polyvalente, CHU Albert-Michallon, B.P. 217, 380432 La Tronche cedex 09, France.
Falcon
D
D
Jouk
P-S
PS
Seinturier
C
C
Jacquot
C
C
D000328:Adult; D000784:Aortic Dissection / Q000209:etiology*; D017541:Aneurysm, False / Q000209:etiology; D017542:Aneurysm, Ruptured / Q000209:etiology*; D015897:Comorbidity; D003323:Coronary Aneurysm / Q000209:etiology*; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000175:diagnosis; D004630:Emergencies; D005260:Female; D020022:Genetic Predisposition to Disease; D006491:Hemothorax / Q000209:etiology*; D006801:Humans; D008323:Mammary Arteries / Q000473:pathology; D009203:Myocardial Infarction / Q000209:etiology*; D011655:Pulmonary Embolism / Q000209:etiology*; D012422:Rupture, Spontaneous; D013157:Splenic Artery / Q000473:pathology