Spontaneous carotid-cavernous fistulae in Ehlers-Danlos syndrome Type IV

Case report

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✓ Spontaneous bilateral carotid-cavernous fistulae and cervical artery dissection is reported in a 20-year-old woman with Ehlers-Danlos syndrome Type IV. The clinical features of 16 previously published cases of spontaneous carotid-cavernous fistulae associated with Ehlers-Danlos syndrome Type IV are reviewed, for a total of 17 cases. The mean age of the 14 women and three men was 31.6 years. Only direct fistulae were encountered. Diagnostic neuroangiography carried morbidity and mortality rates of 36% and 12%, respectively; neuroradiological treatment resulted in death in one of six patients. The possible value of desmopressin in the management of these patients is discussed. In view of the risks of arterial puncture and surgery, the authors emphasize the importance of early recognition of Ehlers-Danlos syndrome.

Article Information

Address reprint requests to: Wouter I. Schievink, M.D., Department of Neurologic Surgery, Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905.

© AANS, except where prohibited by US copyright law.

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Figures

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    Preoperative right carotid subtraction arteriograms, anteroposterior (A) and lateral (B and C) views, demonstrating marked ectasia and tortuosity of the extracranial internal carotid artery. A and B: A subcranial aneurysm measuring approximately 15 mm in length arises just proximal to the carotid canal (arrows) most likely due to previous carotid artery dissection. Shunting of contrast material into the cavernous sinus and draining through the ophthalmic vein can also be observed. C: Detail of cavernous sinus region demonstrating shunting of contrast material through the fistula (arrow) into the cavernous sinus.

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    Preoperative left vertebral subtraction arteriograms, anteroposterior (left) and lateral (center and right) views, demonstrating an ectatic extracranial vertebral artery with an aneurysm at the C-1 vertebral level (left and center, arrows) and multiple wall irregularities, including an intimal flap (right, arrow) indicating arterial dissection. Intracranial filling is through the vertebrobasilar system. Also note collateral filling of the left external carotid artery.

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