Cherubism: a 36-year long-term follow-up of 2 generations in different families and review of the literature.
OBJECTIVE
To clarify the relationships between the varying clinical or radiographic features of cherubism.


METHODS
Nonparametric statistics were used in a long-term follow-up of 18 patients through 2 generations from 6 Danish families.


RESULTS
The radiographic grade of cherubism was significantly related to sex, maximal buccal bone expansion, course of cherubism, and number of aplasia or ectopic impacted teeth, but it was not related to families. Normal dentition in nonaffected regions was present or was obtained in 14 of 14 patients (age, >14 years). Surgical treatment did not provoke growth of lesional tissue in 22 of 22 cases. Radiographically, the bone structure in the lesional areas was related to age in all grades of cherubism: new bone formation in radiolucent areas (age, >20 years), normal bone structure with multilocular sketches (age, 32 to 39 years), and completely normal bone structure (age, >41 years), also found in 7 of 7 carriers of cherubism (age, >32 years).


CONCLUSIONS
This group analysis verifies the knowledge of cherubism previously based on cumulative reviews of findings in single-family and case reports.
Von Wowern
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Department of Oral and Maxillofacial Surgery, School of Dentistry, University of Copenhagen, Denmark.
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000848:Anodontia / Q000209:etiology; D002636:Cherubism / Q000150:complications / Q000000981:diagnostic imaging* / Q000235:genetics / Q000473:pathology; D002648:Child; D003718:Denmark; D005192:Family Health; D005260:Female; D005500:Follow-Up Studies; D006579:Heterozygote; D006801:Humans; D008297:Male; D010375:Pedigree; D011859:Radiography; D012737:Sex Factors; D018709:Statistics, Nonparametric; D014079:Tooth Eruption, Ectopic / Q000000981:diagnostic imaging / Q000209:etiology / Q000601:surgery; D014095:Tooth, Impacted / Q000000981:diagnostic imaging / Q000209:etiology / Q000601:surgery