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

Abstract

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.

Carotid-cavernous fistulas

“…Cavernous-carotid fistulas are reported in anywhere from 3% to 24% of patients with cavernous carotid aneurysms. 38,41,45,80 In addition to cavernous aneurysms, genetic conditions such as fibromuscular dysplasia, 29,39 Ehlers-Danlos syndrome, 8,15,69 and pseudoxanthoma elasticum 67 are known to predispose patients to spontaneous CCFs. It is thought that arterial wall defects in these patients predispose them to CCF formation after minor stress, such as coughing or Valsalva maneuver.…”

Proptosis, Diplopia, and an Ocular Bruit in a Patient With Ehlers-Danlos Syndrome

“…This leads to the symptoms of tinnitus, ocular pain, chemosis, opthalmoplegia, diplopia and proptosis. In addition, reflux of arterialized blood into cerebral cortical veins can lead to rupture and CNS hemorrhage [5].…”

Spontaneous Carotid-Cavernous Fistula in a Patient With Ehlers-Danlos Syndrome Type IV-Case Report-

“…17,26,27) The morbidity was 36% and the mortality was 12% for 25 angiographic procedures in patients with EDS type IV. 25) Diagnostic procedures requiring arterial punctures should be avoided, except in extreme circumstances. 13) Transvenous angiography or MR imaging are advocated as the initial investigative method.…”

“…2,[5][6][7]9,11,13,14,16,17,21,23,25,26) The age at which the syndrome manifested ranged from 17 to 52 years (mean 31.8 years). The patients were predominantly female (male:female ratio of 1:5.4).…”