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December 1965

Spontaneous Carotid-Cavernous Fistula: Ehlers-Danlos Syndrome and Related Conditions

Author Affiliations

From the Department of Neurological Surgery, Buffalo General Hospital.

Arch Neurol. 1965;13(6):662-672. doi:10.1001/archneur.1965.00470060098011

THE PROTEAN manifestations of Ehlers-Danlos syndrome, a rare disease of congenital nature involving body structures of mesodermal origin, are well known. Considered as a disturbance of the connective tissue, the abnormality concerns mainly the elastic and more particularly the collagen tissue throughout the body. This disorder is characterized by hyperelasticity of the skin, hyperextensibility of the joints, and histories of "easy bruising" with cutaneous hemorrhage and prolonged poorly controlled bleeding with surgical procedures. More dramatic manifestations of the disorder in other organ systems occur, eg, spontaneous rupture of the heart and lung, ectasia of the viscera, cardiac anomalies, diaphragmatic hernia, and gastrointestinal diverticula.

Recently, Rubenstein and Cohen13 described as unique a patient with Ehlers-Danlos syndrome associated with multiple intracranial aneurysms and spontaneous subarachnoid hemorrhage.

Aneurysm of the larger vessels (aorta) has been recorded by McKusick12 in this syndrome. There appears to be only one reference in the

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