Poster électronique

Neurofibromatose type 1 de la tête au pied
F. Benchakroun, R. Dafiri - Rabat - Maroc
Congrès 2011
Résumé
Objectifs
Rappeler et illustrer les différents aspects en imagerie rencontrées lors de la neurofibromatose type 1. Souligner le rôle des différentes explorations.
Matériels et méthodes
Ce poster didactique est basé sur notre expérience concernant les neurofibromatoses. L'exploration radiologique comprend selon la topographie lésionnelle des radiographies standard, TDM et/ou IRM.
Résultats
Nous avons constaté une prépondérance des atteintes neurologiques : lésions hamartomateuses, gliomes des nerfs optiques, dysplasies crânio-orbitaires et plus rarement le schwannome kystique et l'épendymome. Les atteintes rachidiennes les plus fréquentes sont les scolioses idiopathiques. Les scolioses dystrophiques, la luxation atlonto-axiale sont rares. L'atteinte osseuse la plus caractéristique est la pseudarthrose tibiale. L'hémorragie souspériostée, l'asymétrie des membres sont inhabituels. Les masses des parties molles sont dominées par les neurofibromes et les tumeurs malignes des gaines nerveuses. De rares cas d'atteinte vésicale ont été rapportés. Des tumeurs abdominopelviennes ont été notéés : tumeur rectale, pancréatique, neuroblastome, phéochromocytome.
Conclusion
La neurofibromatose type 1 est une pathologie multisystémique avec un polymorphisme clinique et radiologique. L’apport de l’imagerie dans cette pathologie est capital. L'IRM est actuellement la technique de choix. Elle apporte des arguments décisifs aussi bien pour le diagnostic positif, l'évaluation du pronostic et le suivi évolutif des lésions.
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2018-05-13 09.10 :
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2017-04-12 13.20 :
Shana Phillips

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2017-04-12 03.14 :
Ann Perez

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