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 rad iographic features of cherubism. Study design. Nonparametric statistics were used in a long-term follow-up o f 18 patients through 2 generations from 6 Danish families. Results, The radiographic grade of cherubism was significantly related to s ex, maximal buccal bone expansion, course of cherubism, and number of aplas ia or ectopic impacted teeth, but it was not related to families. Normal de ntition in nonaffected regions was present or was obtained in 14 of 14 pati ents (age, > 14 years). Surgical treatment did not provoke growth of lesion al tissue in 22 of 22 cases. Radiographically, the bone structure in the le sional areas was related to age in all grades of cherubism: new bone format ion in radiolucent areas (age, > 20 years), normal bone structure with muit ilocular sketches (age, 32 to 39 years), and completely normal bone structu re (age, > 41 years), also found in 7 of 7 carriers of cherubism (age, > 32 years). Conclusions, This group analysis verifies the knowledge of cherubism previo usly based on cumulative reviews of findings in single-family and case repo rts.