Polyostotic fibrous dysplasia with cranial hyperostosis: new entity or most severe form of polyostotic fibrous dysplasia?
Polyostotic fibrous (McCune-Albright) dysplasia is an uncommon nonhereditable disorder characterized by localized or widespread cystic changes in the skeleton. The limb bones are predominantly affected; craniofacial involvement is rare. We have encountered a severely affected man, with the additional manifestation of massive craniofacial hyperostosis. It is questionable whether this condition is an autonomous entity or represents the end of the spectrum of severity of polyostotic fibrous dysplasia.
Viljoen
D L
DL
Department of Human Genetics, Medical School, University of Cape Town, South Africa.
Versfeld
G A
GA
Losken
W
W
Beighton
P
P
D000328:Adult; D005357:Fibrous Dysplasia of Bone / Q000145:classification*; D005359:Fibrous Dysplasia, Polyostotic / Q000145:classification* / Q000175:diagnosis / Q000000981:diagnostic imaging; D006801:Humans; D006957:Hyperostosis Frontalis Interna / Q000145:classification* / Q000175:diagnosis / Q000000981:diagnostic imaging; D008297:Male; D012886:Skull / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed