Ehlers-Danlos syndrome and "congenital" arteriovenous fistulae. A clinicopathologic study of a family

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Abstract
A family with Ehlers-Danlos syndrome (E-D) was studied. The proband died from rupture of a thoracic-aorta aneurysm. Autopsy showed a fistula between the aorta and the inferior vena cava and between the left femoral artery and the corresponding vein. Four of the proband''s 9 siblings and his father showed definite but variable external manifestations of E-D but did not show any internal manifestations. This is the first known case of E-D with arteriovenous fistulae. A hypothesis is advanced that these fistulae, as well as other so-called congenital defects found in this condition, may be secondary manifestations of the basic connective-tissue disorder.

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