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[Cherubism. Apropos of a major case]
J B Belloc1, M Divaris, G F Cancemi
1Service de Stomatologie et de Chirurgie Maxillo-faciale, Hôpital de la Pitié-Salpêtrire, Paris.
Revue De Stomatologie Et De Chirurgie Maxillo-Faciale
|January 1, 1993
Summary
Cherubism, a rare genetic disorder, typically resolves on its own. However, this case highlights a severe form requiring surgical intervention for a young girl.
Area of Science:
- Genetics
- Oral and Maxillofacial Surgery
- Pediatric Dentistry
Background:
- Cherubism is a rare, autosomal dominant genetic disorder characterized by benign, bilateral, symmetrical fibro-osseous lesions of the jaw.
- Typically presents in early childhood with painless swelling of the mandible and maxilla, progressing until puberty and often regressing spontaneously.
- While generally self-limiting, some cases may necessitate intervention due to severity or functional impairment.