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

Mitsuhashi, Takashi; Miyajima, Masakazu; Saitoh, Rikizou; Nakao, Yasuaki; Hishii, Makoto; Arai, Hajime

doi:10.2176/nmc.44.548

Cited by 26 publications

(11 citation statements)

References 23 publications

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“…Direct fistulas are usually post-traumatic, caused by motor vehicle accidents or penetrating injuries; however, spontaneous CCFs account for approximately 25 % of all CCFs [4], and can be caused by ICA, ruptured cavernous-located aneurysms, collagen vascular disease, such as Ehlers-Danlos syndrome, or other causes [5][6][7]. The flow rates and venous drainage in type A fistulas are variable, and depend on the size of the arteriovenous connection.…”

Section: Introductionmentioning

confidence: 99%

Ophthalmologic outcome of direct and indirect carotid cavernous fistulas

et al. 2012

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Carotid cavernous fistulas (CCFs) can be classified as direct and indirect, depending on their flow rates and their etiology. Both forms can cause the same characteristic ophthalmological symptoms and signs. We analyzed these ocular characteristics and determined the prognostics factors associated with treatment outcome. Forty-seven patients with an angiographically confirmed diagnosis of CCF, a preoperative ophthalmic evaluation and at least one ophthalmic sign or symptom at the initial presentation were retrospectively evaluated. The patients were followed-up ophthalmically until the end of treatment, and the complications and the remaining ophthalmological signs and symptoms were then recorded. The patients' ages ranged from 13 to 89 years, with an average of 55.78 (±20.73) years, and a predominance of 28 female (57.8 %) patients. The patients with a direct CCF had a lower average age (p = 0.02). The most common symptoms were blurred vision in 17 (36.2 %) and proptosis in 37 (78.7 %) patients. Elevated intraocular pressure (IOP) was more prevalent in patients with an indirect CCF (p = 0.02). Thrill was more prevalent in patients with direct CCF (p = 0.01). The presence of an initial decrease of visual acuity at the first ophthalmic evaluation was significantly associated with the persistence of ocular symptoms after fistula treatment (odds ratio 3.33). In conclusion our study shows a slight difference in ophthalmic symptoms among patients with different types of fistula. Elevated IOP was significantly associated with indirect fistulas, whereas thrill was significantly associated with direct fistulas. The presence of an initial decrease of visual acuity was significantly associated with a worse ophthalmic prognosis.

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Section: Introductionmentioning

confidence: 99%

Ophthalmologic outcome of direct and indirect carotid cavernous fistulas

et al. 2012

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“…While rare in childhood, these complications affect 25% of the patients before the age of 20 and 80% by the age of 40. Arterial ruptures result in the majority of deaths with a median death at age 50 [3].…”

Section: Discussionmentioning

confidence: 99%

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

Mebust

2013

J Med Cases

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We describe a case of a 30-year-old female with a history of the Vascular form of Ehlers-Danlos Syndrome who presented to a community Emergency Department with complaints of eye pain, blurred vision, and diplopia. On examination, she was noted to have proptosis, scleral injection and an ocular bruit. Imaging studies demonstrated dissection of the carotid artery and a carotid cavernous sinus fistula. Patient underwent endovascular coil embolization of the lesion with resolution of symptoms.

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“…Since his original presentation, he has had only one sequelae: he presented 2 years after the initial event with headaches and a left fourth nerve palsy secondary to a left carotid cavernous fistula, which was treated endovascularly. Carotid cavernous fistulae are well described in the setting of vEDS 13,14 and while intracranial hemorrhage from a fistula could provide a substrate for seizures, there was no evidence for intracranial hemorrhage at the index event. At his second presentation, the patient had a lumbar puncture positive for subarachnoid hemorrhage in the setting of a negative CT head scan.…”

Section: Rheumatologymentioning

confidence: 99%