Spontaneous direct carotid-cavernous fistula in Ehlers-Danlos syndrome type IV: two case reports and a review of the literature.

@article{Chuman2002SpontaneousDC,
  title={Spontaneous direct carotid-cavernous fistula in Ehlers-Danlos syndrome type IV: two case reports and a review of the literature.},
  author={Hideki Chuman and Jonathan D. Trobe and Elizabeth M. Petty and Ulrike Schwarze and Melanie G Pepin and Peter H Byers and John P. Deveikis},
  journal={Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society},
  year={2002},
  volume={22 2},
  pages={75-81}
}
Two unrelated adults with Ehlers-Danlos syndrome type IV developed acute unilateral ophthalmoplegia and ipsilateral headache as a consequence of spontaneous (nontraumatic) direct carotid-cavernous fistulas. Because the interventional radiologist suspected the diagnosis of Ehlers-Danlos syndrome type IV, the carotid-cavernous fistulas were closed via the venous rather than the more standard arterial route in an attempt to avoid arterial dissection or rupture. In any patient presenting with a… CONTINUE READING

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