Treatment of a direct carotid-cavernous fistula in a patient with type IV Ehlers-Danlos syndrome : a novel approach

• Published in: Neuroradiology Vol. 48; no. 7; pp. 491 - 494
• Main Authors: HOLLANDS, J. K, SANTARIUS, T, KIRKPATRICK, P. J, HIGGINS, J. N
• Format: Journal Article
• Language: English
• Published: Berlin Springer 01.07.2006; Springer Nature B.V
• Subjects: Adult; Angioplasty, Balloon - methods; Biological and medical sciences; Blood vessels; Carotid-Cavernous Sinus Fistula - complications; Carotid-Cavernous Sinus Fistula - diagnosis; Carotid-Cavernous Sinus Fistula - therapy; Case studies; Chromosome aberrations; Ehlers-Danlos Syndrome - complications; Embolization, Therapeutic - methods; Female; Genetic disorders; Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy; Humans; Investigative techniques, diagnostic techniques (general aspects); Medical genetics; Medical procedures; Medical sciences; Nervous system; Nervous system (semeiology, syndromes); Neurology; Radiodiagnosis. Nmr imagery. Nmr spectrometry; Human; Skin disease; Nervous system diseases; Ehlers-Danlos syndrome; Elastic tissue disease; Radiodiagnosis; Diseases of the osteoarticular system; Artery; Genetic disease; Treatment; Blood vessel; Carotid; Ehlers Danlos syndrome; Systemic disease; Carotid-cavernous fistula; Surgical approach; Circulatory system
• ISSN: 0028-3940; 1432-1920
• DOI: 10.1007/s00234-006-0084-1

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.