Abstract
We report a case of a 34-year-old female with type IV Ehlers-Danlos syndrome diagnosed with a carotid cavernous fistula presenting with progressive proptosis. Endovascular embolization using balloons or coils carries a high risk of complications in this group of patients, owing to the extreme fragility of the blood vessels. Initial treatment was conservative until an intracerebral haemorrhage occurred. To avoid transfemoral angiography, the ipsilateral carotid arteries and the internal jugular vein were surgically exposed for insertion of two endovascular sheaths. The patient was transferred from theatre to the angiography suite and the sheaths were used for embolization access. The fistula was closed, with preservation of the carotid artery, using Guglielmi detachable coils deployed in the cavernous sinus from the arterial and venous sides. Rapid resolution of symptoms and signs followed, which was sustained at 6-month follow-up. This technique offers alternative access for endovascular treatment, which may reduce the high incidence of mortality associated with catheter angiography in this condition.
Similar content being viewed by others
References
Debrun GM, Aletich VA, Miller NR, DeKeiser RJW (1996) Three cases of spontaneous direct carotid cavernous fistulas associated with Ehlers-Danlos syndrome type IV. Surg Neurol 46:247–252
Pollack JS, Custer PL, Hart WM, Smith ME, Fitzpatrick MM (1997) Ocular complications in Ehlers-Danlos syndrome type IV. Arch Ophthalmol 115(3):416–419
Farley MK, Clark RD, Fallor MK, Geggel HS, Heckenlively JR (1983) Spontaneous carotid-cavernous fistula and the Ehlers-Danlos syndromes. Ophthalmology 90(11):1337–1342
Kanner AA, Maimon S, Rappaport ZH (2000) Treatment of spontaneous carotid-cavernous fistula in Ehlers-Danlos syndrome by transvenous occlusion with Guglielmi detachable coils. J Neurosurg 93:689–692
Schievink WI, Piepgras DG, Earnest F, Gordon H (1991) Spontaneous carotid-cavernous fistulae in Ehlers-Danlos syndrome type IV. J Neurosurg 74:991–998
Burrows NP (1999) The molecular genetics of the Ehlers-Danlos syndrome. Clin Exp Dermatol 24:99–106
Beighton P, De Paepe A, Steinmann B, Tsipouras P, Wenstrup RJ (1998) Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997. Ehlers-Danlos National Foundation (USA) and Ehlers-Danlos Support Group (UK). Am J Med Genet 77:31–37
Horowitz MB, Purdy PD, Valentine RJ, Morrill K (2000) Remote vascular catastrophes after neurovascular interventional therapy for type IV Ehlers-Danlos syndrome. AJNR Am J Neuroradiol 21:974–976
North KN, Whiteman DAH, Pepin MG, Byers PH (1995) Cerebrovascular complications in Ehlers-Danlos syndrome type IV. Ann Neurol 38:960–964
Forlodou P, de Kersaint-Gilly A, Pizzanelli J, Viarouge MP, Auffray-Calvier E (1996) Ehlers-Danlos syndrome with a spontaneous caroticocavernous fistula occluded by detachable balloon: case report and review of literature. Neuroradiology 38(6):595–597
Pope FM, Kendall BE, Slapak GI, Kapoor R, McDonald WI, Compston DAS, Mitchell R, Hope DT, Miller-Craig MW, Dean JCS, Johnston AW, Lynch PG, Sarathchandra P, Narcisi P, Nicholls AC, Richards AJ, Mackenzie JL (1991) Type III collagen mutations cause fragile cerebral arteries. Br J Neurosurg 5:551–574
Pool JL (1977) The development of modern intracranial surgery. Neurosurgery 1(3):233–237
Serbinenko FA (1971) Balloon occlusion of cavernous portion of the carotid artery as a method of treating carotid cavernous fistulae. Zh Vohr Neirokhil 6:3–9
Guglielmi G, Vinuela F, Dion J, Duckwiler (1991) Electrothrombosis of saccular aneurysms via endovascular approach. Part 2: Preliminary clinical experience. J Neurosurg 75:8–14
Francois PW, Woillez M, Maillet WP (1955) Maladie d’Ehlers-Danlos avec aneurysm arterio-veineux intra-cranien. Bull Soc Ophtalmol Fr 5:392–395
Graf CJ (1965) Spontaneous carotid-cavernous fistula. Ehlers-Danlos syndrome and related conditions. Arch Neurol 13:662–672
Fox R, Pope FM, Narcisi P, Nicholls AC, Kendall BE, Hourihan MD, Compston DAS (1988) Spontaneous carotid cavernous fistula in Ehlers Danlos syndrome. J Neurol Neurosurg Psychiatry 51:984–986
Kashiwagi S, Tsuchida E, Goto K, Shiroyama Y, Yamashita T, Takahasi M, Ito H (1993) Balloon occlusion of a spontaneous carotid-cavernous fistula in Ehlers-Danlos syndrome type IV. Surg Neurol 39:187–190
Jansen O, Dorfler A, Forsting M, Hartmann M, von Kummer R, Tronnier V, Sartor K (1999) Endovascular therapy of arteriovenous fistulae with electrolytically detachable coils. Neuroradiology 41:951–957
Halbach VV, Higashida RT, Hieshima GB, Hardin CW, Yang PJ (1988) Transvenous embolization of direct carotid cavernous fistulas. AJNR Am J Neuroradiol 9:741–747
Lach B, Nair SG, Russell NA, Benoit BG (1987) Spontaneous carotid-cavernous fistula and multiple arterial dissections in type IV Ehlers-Danlos syndrome. J Neurosurg 66:462–467
Conflict of interest statement
We declare that we have no conflict of interest.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Hollands, J.K., Santarius, T., Kirkpatrick, P.J. et al. Treatment of a direct carotid-cavernous fistula in a patient with type IV Ehlers-Danlos syndrome: a novel approach. Neuroradiology 48, 491–494 (2006). https://doi.org/10.1007/s00234-006-0084-1
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00234-006-0084-1