Cherubism (Subscribe)  

* hereditary form of polyostotic fibrous dysplasia
* involves mandible and maxillae
* may regress in early adulthood
* autosomal dominant

Cherubism aka/or Familial fibrous dysplasia of jaw Definition - "A fibro-osseous hereditary disease of the jaws. The swollen jaws and raised eyes give a cherubic appearance; multiple radiolucencies are evident upon radiographic examination." may cause or feature - Symptoms and Signs Cervical lymphadenopathy Hypertelorism, ocular Congenital conditions Hypodontia Prognathia

cherubism can be caused by mutations in the SH3BP2 gene. Swelling of the lower face begins around the third or fourth year of life and progresses until the late teens. The enlargement may be exaggerated by enlargement of submandibular lymph nodes. X-ray reveals multilocular cystic changes in the mandible and maxilla...

Axial CT scan through facial bones at level of hard palate of 5-year-old boy with cherubism shows typical expansile lesions involving base of maxilla and mandibular rami. Additional images from this study (not shown) revealed uniform symmetric involvement of entire maxilla and mandible, typical of cherubism.

Search string "cherubism"[majr] yields 207 articles Dec 2007 URL - http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=PureSearch&db=pubmed&details_term=%22cherubism%22%5Bmajr%5D