Poster électronique

L'atteinte rénale et rétropéritonéale de la maladie d'Erdheim-Chester : étude rétrospective de 7 cas
A. Iancu (1), D. Argatu (2), P. Puech (2), M. Lambert (2), X. Leroy (2), D. Launay (2), E. Hachulla (2), PY. Hatron (2), L. Lemaitre (2) - (1) Valenciennes - France, (2) Lille - France
Congrès 2009
Résumé
Objectifs
Etudier l'aspect de l’infiltration rétropéritonéale dans le cadre de la maladie d'Erdheim-Chester.
Matériels et méthodes
Etude rétrospective sur une période de 10 ans de 7 patients (4H/3F) âgés de 47 à 81 ans présentant une maladie d'Erdheim-Chester et développant une atteinte rénale et rétropéritonéale au cours de leur maladie. Au cours de l'évolution, les patients ont bénéficié d'examens IRM et/ou scanner qui nous ont permis de sélectionner les principaux critères sémiologiques évocateurs de la maladie.
Résultats
Il existait chez chaque patient une double infiltration à la fois périrénale et rétropéritonéale. L'atteinte rénale montre une disposition caractéristique : bilatérale, symétrique, périrénale, péripyélique avec extension au sinus et à la portion proximale de l'uretère. Tous les patients présentaient une hydronéphrose atypique, épargnant le pyélon. A la différence des autres fibroses rétropéritonéales, les portions lombaires basses et pelviennes des uretères n'étaient pas atteintes. L'atteinte périaortique était circonférentielle et régulière. Dans tous les cas l’infiltration etudieé presentait un hyposignal en sequences T2 simulant une fibrose.
Conclusion
Il existe un aspect caractéristique de l'atteinte rétropéritonéale dans la maladie d'Erdheim-Chester.
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2018-05-13 09.09 :
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