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Fibrous dysplasia of the cranial bones: a case report and review of the literature.

Fibrous dysplasia (FD) is a relatively uncommon disorder that affects primarily the cranial region; its occurrence in the cranial base in combination with hindbrain herniation and aneurysmal bone cyst (ABC) constitutes an extremely rare condition. We report a case of polyostotic fibrous dysplasia wi...

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Detalles Bibliográficos
Autores principales: Iseri, Pervin K., Efendi, Husnu, Demirci, Ali, Komsuoglu, Sezer
Formato: Texto
Lenguaje:English
Publicado: Yale Journal of Biology and Medicine 2005
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2259144/
https://www.ncbi.nlm.nih.gov/pubmed/16464312
Descripción
Sumario:Fibrous dysplasia (FD) is a relatively uncommon disorder that affects primarily the cranial region; its occurrence in the cranial base in combination with hindbrain herniation and aneurysmal bone cyst (ABC) constitutes an extremely rare condition. We report a case of polyostotic fibrous dysplasia with progressive occipital, temporal, and clival involvement. Clinical findings and differential diagnosis with special emphasis on the imaging features were discussed. A small posterior fossa volume has been thought to lead to hind brain herniation. The resultant obstruction to the CSF pathways at the level of the foramen magnum has been implicated in the development and subsequent progression of syringobulbia.