Mortality in Ehlers–Danlos Syndrome Due to Spontaneous Rupture of Large Arteries

Abstract
DISSECTING aneurysm of the aorta, although more commonly associated with Marian's syndrome, has been reported in several patients with Ehlers–Danlos syndrome.1 This complication has occurred in young persons and is presumably related to the generalized abnormality of connective tissue in this condition.The present report describes 2 boys with the syndrome who died after spontaneous rupture of large arteries — a popliteal artery in 1 and a subclavian artery in the other.Case ReportsCase 1. E.K., a 12-year-old boy, was renowned locally for the many lacerations and dislocations that he suffered as a result of ordinary trauma. He presented . . .