title: Ascending aorta impending rupture: successful surgical management in a patient with type IV Ehlers-Danlos syndrome.
abstract: Ehlers-Danlos syndrome is an inherited disorder of the connective tissue characterized by increased elasticity of the skin, hypermobile joints and fragility of the vascular system. Vascular complications in these patients, requiring surgical repair, can be life-threatening with high perioperative morbidity and mortality. Reports of successful treatment of aortic ruptures or dissections in vascular Ehlers-Danlos syndrome patients are rare. We describe our surgical technique and precautions in an Ehlers-Danlos syndrome patient with a contained rupture of the ascending aorta, requiring emergency surgical intervention.
authors.affiliation: Department of Cardiothoracic Surgery, St Antonius Ziekenhuis, Nieuwegein, The Netherlands.
authors.forename: Aladdin
authors.initials: A
authors.lastname: Yilmaz
authors.forename: Thomas
authors.initials: T
authors.lastname: Dessing
authors.forename: Luca
authors.initials: L
authors.lastname: Botta
mesh_terms: D001014:Aortic Aneurysm / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D001019:Aortic Rupture / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D019917:Blood Vessel Prosthesis Implantation; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000473:pathology / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome