Spontaneous thrombosis of an orbital arteriovenous malformation revealing hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber disease). A case report.

Détails

ID Serval
serval:BIB_513C324C7187
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Etude de cas (case report): rapporte une observation et la commente brièvement.
Collection
Publications
Titre
Spontaneous thrombosis of an orbital arteriovenous malformation revealing hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber disease). A case report.
Périodique
Interventional neuroradiology : journal of peritherapeutic neuroradiology, surgical procedures and related neurosciences
Auteur(s)
Van Went C., Ozanne A., Saliou G., Dethorey G., De Monchy I., Krings T., Ducreux D., Labetoulle M.
ISSN
1591-0199 (Print)
ISSN-L
1591-0199
Statut éditorial
Publié
Date de publication
12/2011
Peer-reviewed
Oui
Volume
17
Numéro
4
Pages
466-471
Langue
anglais
Notes
Van Went, Charles
Ozanne, A
Saliou, G
Dethorey, G
De Monchy, I
Krings, T
Ducreux, D
Labetoulle, M
eng
Case Reports
2011/12/24 06:00
Interv Neuroradiol. 2011 Dec;17(4):466-71. Epub 2011 Dec 16.
Publication types: Case Reports ; Journal Article
Publication Status: ppublish

Résumé
Hereditary Haemorrhagic Telangiectasia (HHT) is a genetic disorder responsible for cutaneous or mucosal telangiectasia and arteriovenous malformations (AVMs). The most frequent locations are lung and brain. In contrast, orbital AVMs are very rare. We describe a case of symptomatic orbital arteriovenous malformation due to spontaneous thrombosis. A 65-year-old woman was referred for chronic right eye proptosis associated with dilation of conjunctival vessels with a jellyfish pattern. Right visual acuity was 20/40 and intraocular pressure was 40 mmHg. Personal and familial history of recurrent epistaxis, associated with multiple telangiectasia within lips and palate, led to the diagnosis of HHT. Magnetic resonance imaging (MRI) completed with cerebral angiography found a giant and occluded AVM within the right orbit. Other AVMs were also found in brain and chest, confirming the diagnosis. Antiglaucomatous eyedrops were added to reduce intraocular pressure and a steroid therapy was begun. Two months later, visual acuity decreased in the right eye, due to a central retinal vein thrombosis. In conclusion, Most brain or pulmonary AVM can be treated by embolization. By contrast, this treatment in case of orbital location can lead to central retinal artery and/or central retinal vein occlusion, which may also appear as a spontaneous complication of the orbital AVM. Therapeutic management of orbital AVM is thus not standardized, and the balance between spontaneous and iatrogenic risk of visual loss has to be taken into account.

Mots-clé
Aged, Antihypertensive Agents/therapeutic use, Arteriovenous Malformations/diagnosis, Arteriovenous Malformations/drug therapy, Cerebral Angiography, Diagnosis, Differential, Female, Glucocorticoids/therapeutic use, Humans, Magnetic Resonance Imaging, Orbit/blood supply, Telangiectasia, Hereditary Hemorrhagic/diagnosis, Telangiectasia, Hereditary Hemorrhagic/drug therapy, Visual Acuity
Pubmed
Création de la notice
20/01/2017 16:30
Dernière modification de la notice
03/03/2018 17:10
Données d'usage