Emergency repair of type A aortic dissection in type IV Ehlers-Danlos syndrome.
Ehlers-Danlos syndrome type IV is a distinctive syndrome in which thin and fragile skin, premature ageing, bruising and scarring are combined with lethal or life-threatening arterial weakness. Aortic rupture either at the aortic root and arch, or sometimes lower down the artery, are particularly characteristic. Even quite minor injury can produce dangerous vascular tearing and damage. Technical difficulties encountered in arterial repair or venous ligature are particularly worrying. The authors report the treatment of a ruptured type A aortic dissection associated with Ehlers-Danlos syndrome where the extreme fragility of the tissues and tendency to bleed posed a difficult task for the surgeon.
Ascione
R
R
Bristol Heart Institute, Bristol Royal Infirmary, UK.
Gomes
W J
WJ
Bates
M
M
Shannon
J L
JL
Pope
F M
FM
Angelini
G D
GD
D000328:Adult; D000784:Aortic Dissection / Q000209:etiology* / Q000473:pathology / Q000601:surgery*; D001019:Aortic Rupture / Q000209:etiology* / Q000473:pathology / Q000601:surgery*; D001158:Arteries / Q000473:pathology; D002198:Capillary Fragility; D003094:Collagen / Q000648:ultrastructure; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D014656:Vascular Surgical Procedures / Q000379:methods; D014693:Ventricular Fibrillation / Q000150:complications
D003094:Collagen