Key result
Emergency surgery successfully manages contained ascending aortic rupture in type IV Ehlers-Danlos syndrome.
Case Report (n=1)
Demonstrates successful surgical management of an ascending aorta impending rupture in a high-risk patient with type IV Ehlers-Danlos syndrome.
May support emergency repair feasibility in vEDS aortic rupture; leaves open need for larger series before practice change.
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.
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Yilmaz et al. (2010) conducted a case report in Type IV Ehlers-Danlos syndrome with contained rupture of the ascending aorta (n=1). Emergency surgical intervention was evaluated on Successful surgical management. Emergency surgical intervention was successfully performed in a patient with type IV Ehlers-Danlos syndrome and a contained rupture of the ascending aorta.
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