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scientific articles

Ehlers-Danlos Syndrome

key information

source: British Medical Journal

year: 1967

authors: Bannerman RM, Graf CJ, Upson JF

summary/abstract:

SIR, Ehlers-Danlos syndrome may have a wide variety of manifestations. It is often difficult to be sure of the diagnosis, particularly in mildly affected individuals, since the microscopic anatomy is in ” a disputed state,” and the basic defect is unknown. Thus Mr. A. P. Barabas’s suggested clinical subdivision of cases (3 June, p. 612) is most helpful in elucidating the probable heterogeneity of the syndromne, and particularly in pointing out the existence of an ” arterial ” type which may cause fatal haemorrhage in early, adult life.

As Mr. Barabas is aware, intracranial arterial aneurysm has been reported as a complication, and one of us recently described three cases of spontaneous carotidcavernous fistula in Ehlers-Danlos syndrome.’ Two of the patients are siblings, a sister and brother, and the sister has since died after suffering a succession of further arterial complications. A brief report of this case may therefore be of interest.

organisation: Buffalo General Hospital

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