Cherubism and its orbital manifestations.
Cherubism is a rare, fibroosseous lesion of the jaws that may have orbital manifestations of proptosis, lower eyelid retraction, superior globe displacement, and visual loss. Management of the orbital tumors in two cases from the same family is described. The original description of the disorder is reviewed. The inheritance pattern is autosomal dominant. Pathology findings show multinucleated giant cells in a vascular fibrous stroma. Teeth are often missing or displaced. Radiographic studies show symmetric cystic lesions in the jaws and sometimes involvement of the orbital floor and lateral orbital wall. Fibrous dysplasia is in the differential diagnosis, but can be distinguished by clinical characteristics. Treatment may involve curettement or recontouring of the bony lesions.
Hawes
M J
MJ
University of Colorado Medical Center, Denver.
D000293:Adolescent; D002636:Cherubism / Q000235:genetics / Q000473:pathology*; D005260:Female; D006801:Humans; D008875:Middle Aged; D009916:Orbital Diseases / Q000235:genetics / Q000473:pathology*; D010375:Pedigree