[Computed tomographic aspects of skull development in a child with cherubism].
The clinical picture of cherubism is similar to that of fibrous dysplasia. The initial clinical appearance involves the deformation of the maxillofacial area with orthodontic disorder. Usually it is found in the mandible giving the child a chubby-faced appearance, and it often occurs together with symmetric submandibular lymph node enlargement. This appearance reminds one of the cherubs seen in art. Only histological evidence for cherubism is inconclusive. The presence of multi-nucleated giant cells resembles fibrous dysplasia. A combination of clinical, radiographical and histological findings eventually leads to the correct diagnosis. An example is given of a patient displaying the typical disease process. Over a period of 12 years, we observed the progression of the disease from its initial appearance in a young child, through the full and characteristic display of a cherubic youth, and finally its regression. In conclusion, we advise restraint in planning surgical intervention. The diseases etiology is not entirely dear. The latest research points to genetic defects that lead to failure in the expression of matrix proteins.
Schleier
P
P
Klinik und Poliklinik für Mund-, Kiefer- und Gesichtschirurgie/Plastische Chirurgie der Friedrich-Schiller-Universität Jena. Peter.Schleier@med.uni-jena.de
Neumann
U
U
Müller
A
A
Hyckel
P
P
D000293:Adolescent; D002636:Cherubism / Q000000981:diagnostic imaging* / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D019465:Craniofacial Abnormalities / Q000000981:diagnostic imaging* / Q000235:genetics; D003937:Diagnosis, Differential; D018450:Disease Progression; D005500:Follow-Up Studies; D006801:Humans; D007091:Image Processing, Computer-Assisted; D021621:Imaging, Three-Dimensional; D008297:Male; D008437:Maxilla / Q000000981:diagnostic imaging; D011862:Radiography, Panoramic; D012886:Skull / Q000000981:diagnostic imaging*; D014057:Tomography, X-Ray Computed; D014071:Tooth Abnormalities / Q000000981:diagnostic imaging / Q000235:genetics