Pathophysiology of carotid-cavernous fistulas in vascular Ehlers-Danlos syndrome: a retrospective cohort and comprehensive review.

Date publication

juin 2018

Journal

Orphanet journal of rare diseases

Auteurs

Membres identifiés du Cancéropôle Est :
Dr ALBUISSON Juliette

Tous les auteurs :
Adham S, Trystram D, Albuisson J, Domigo V, Legrand A, Jeunemaitre X, Frank M

Lien Pubmed

https://www.ncbi.nlm.nih.gov/pubmed/29940997

Résumé

Vascular Ehlers-Danlos syndrome (vEDS) is a rare condition characterized by connective tissue fragility. Direct spontaneous carotid-cavernous fistula (sCCF) is reportedly pathognomonic of vEDS. We conducted this study to understand the possible mechanisms of occurrence of sCCF in this subset of patients.

Mots clés

Carotid-cavernous fistula, Genetics, Neurology, Neuroophthalmology, Pathophysiology, Rare vascular disease, Vascular Ehlers-Danlos syndrome

Référence

Orphanet J Rare Dis. 2018 06 25;13(1):100