A case of traumatic, direct, carotid cavernous fistula (CCF) associated with Ehlers — Danlos syndrome (EDS) Type IV is reported along with a review of the literature. Excluding the present case, three similar cases associated with EDS-TypeIV have already been reported by Gerard M. Debrun et Al1. Despite the risks associated with endovascular manipulation, the fistula was successfully closed by intravascular embolisation but the patient expired a few days later because of underlying disease-associated vascular and visceral complications.
DebrunGMAletichVA: Three cases of spontaneous direct carotid cavernous fistulas associated with Ehlers-Danlos syndrome type. Surg Neurol46: 247–252, 1996.
EhlersE: Cutix Laxa, Neigung Zu Haemorrhagien in der Haut, und lockerung mehrerer Artikulationen. Derm. Z.8: 173–174, 1901.
4.
DanlosM: Un cas de cutis laxa avec tumeurs par contusion chronique des coudes et des genoux. (Xanthome juvenile pseudo-diabetique de M.H. Hallopeau et Mace De Lépinay). Bull Soc Fr Derm Syph19: 70–72, 1908.
5.
SchievinkMIMichelsVVPiepgrasDG: Neurovascular manifestations of heritable connective tissue disorders — A review. Stroke25: 889–903, 1994.
6.
DoloresFCJudithHMDonaldS: Spontaneous arterial perforation. The Ehlers-Danlos specter. J Vas Surg5: 248–255, 1987.
7.
BarbarasAP: Vascular complication in the Ehlers-Danlos syndrome. J Cardiovasc Surg13: 160, 1972.
AllanMPeaceman: Ehlers-Danlos syndrome and pregnancy: Association of type IV disease with maternal death. Obstetrics & Gynecology69(3): 428–431, 1987.
10.
LachBNairSG: Spontaneous carotid-cavernous fistula and multiple arterial dissections in type IV Ehlers-Danlos syndrome. Case report. J Neurosurg66: 462–467, 1987.
11.
FarleyMKClarkRD: Spontaneous carotid-cavernous fistula and the Ehlers-Danlos syndromes. Ophthalmology90(11): 1337–1341, 1983.
12.
FoxFMNarcisiPP: Spontaneous carotid cavernous fistulas in Ehlers-Danlos syndrome. Journal of Neurology, Neurosurgery, and Psychiatry51: 984–986, 1988.
KreigTIhmeA: Molecular defects of collagen metabolism in the Ehlers-Danlos syndrome. Int J Dermatol20: 415–425, 1981.
21.
ByersPHHolbrookKAMcGillivrayB: Clinical and ultrastructural heterogeneity of type IV Ehlers-Danlos syndrome. Hum Genet47: 141–150, 1979.
22.
PopeFMNichollsAC: New autosomal dominant and recessive types. JR Soc Med73: 180–186, 1980.
23.
PyeritzREStolleCA: Ehlers-Danlos syndrome type IV due to a novel deficit in type III procollagen. Am J Med Genet19: 607–622, 1984.
24.
PopeFMNichollsAC: Molecular abnormalities of collagen: A review, JR Soc Med76: 1050–1082, 1983.
25.
RubySTKramerJCassidySB: Intracranial carotid artery aneurysm. A vascular manifestation of type IV Ehlers-Danlos syndrome. Conn Med53(3): 142–144, 1989.
26.
GrafCJ: Spontaneous carotid-cavernous fistula: Ehlers-Danlos syndrome and related conditions. Arch Neurol13: 662–672, 1987.