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scientific articles

Spontaneous Carotid Cavernous Fistula in Ehlers Danlos Syndrome

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source: Journal of Neurology, Neurosurgery, and Psychiatry

year: 1988

authors: Fox R, Pope FM, Narcisi P, Nicholls AC, Kendall BE, Hourihan MD, Compston DA


A patient is described with Ehlers Danlos syndrome presenting with spontaneous carotid cavernous fistula, in whom there was biochemical evidence for defective type III collagen synthesis. Despite the risks associated with arterial manipulation, the fistula was successfully closed by interventional neuroradiology and the patient has since remained well. This outcome is in contrast with the results in previous reports.

We describe a case of Ehlers Danlos syndrome type IV, presenting with a spontaneous carotid cavernous fistula successfully treated by interventional neuroradiology, in whom type III collagen deficiency was demonstrated.

organization: University of Wales College of Medicine

DOI: 10.1136/jnnp.51.7.984

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