Retinal manifestations of xanthoma disseminatum (Non-Langerhans cell histiocytosis)

Détails

ID Serval
serval:BIB_3090865DA6E0
Type
Actes de conférence (partie): contribution originale à la littérature scientifique, publiée à l'occasion de conférences scientifiques, dans un ouvrage de compte-rendu (proceedings), ou dans l'édition spéciale d'un journal reconnu (conference proceedings).
Sous-type
Abstract (résumé de présentation): article court qui reprend les éléments essentiels présentés à l'occasion d'une conférence scientifique dans un poster ou lors d'une intervention orale.
Collection
Publications
Institution
Titre
Retinal manifestations of xanthoma disseminatum (Non-Langerhans cell histiocytosis)
Titre de la conférence
Ophta 2010
Auteur(s)
Konstantinidis  Lazaros
Organisation
103e Congrès annuel de la Société Suisse d’Ophtalmologie
Adresse
Montreux Music & Convention Center MMCC
Grand-Rue 95,
P.O. Box 1151,
CH-1820 Montreux
Statut éditorial
Publié
Date de publication
18/09/2010
Pages
S80
Langue
anglais
Résumé
Xanthoma disseminatum (XD) is a rare normolipemic histiocytic disorder of non-Langerhans cell origin. Apart from eye- lids involvement, only sporadic reports of ocular involvement have been described. As far as we are aware this is the first report of retinal manifestations related to XD. History and Signs A 57-year-old woman with known history of XD which occurred 25 years previ- ously (Montgomery syndrome), presented with blurred vision in her RE. She had inactive skin and pulmonary lesions. Visual acuity (VA) at presentation was 1.0. Multiple intraretinal yellow deposits were present with pigment epithelium alterations superior to the macula. Fluorescein angiography revealed ill-defined hyperfluo- rescence while the ultrasound revealed a thickening of the sclera. Fifteen months later the patient VA was 0.5 associated with shal- low subfoveal fluid accumulation and lipid infiltration. Therapy and Outcome Oral steroid treatment led to clinical, anatomical and functional improvement after a period of 6 months. VA improved to 1.0 at the last follow-up. Conclusions The present report illustrates a case that may represent an active chorioretinal disseminated XD. Oral steroids treatment may be an efficacious treatment.
Création de la notice
06/09/2019 13:22
Dernière modification de la notice
07/09/2019 6:26
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