Oral and Maxillofacial Radiology
Cherubism: A 36-year long-term follow-up of 2 generations in different families and review of the literature*

https://doi.org/10.1067/moe.2000.108438Get rights and content

Abstract

Objective: To clarify the relationships between the varying clinical or radiographic features of cherubism. Study Design: 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. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;90:765-72)

Section snippets

Patients and methods

The patients consisted of the 18 children and young adults who fulfilled the following objective criteria of cherubism: (1) clinically, a bilateral (rarely unilateral) painless expansion of the buccal bone surface in the posterior parts of the mandible, combined with (2) radiographic findings of multilocular (rarely unilocular) radiolucencies in the lower molar regions and posteriorly up into the coronoid processes, and (3) the pathologic bone cavities filled in by a tissue similar to the gross

Clinical findings

All 18 patients showed bilateral lesions without changes in the shape of the lingual bone surfaces of the jaws. Only the 2 patients of 3° showed exposure of the inferior part of the sclerae.

The grade of cherubism, based on the radiographic findings of the location and extension of the lesions at the age of 7 years, did not change during the progressive course of the disease. A significant sex difference in the distribution of grades was demonstrated (P <.05) with the highest median grade of 2+°

Discussion

Several family reports from different countries have clarified that cherubism is a hereditary bone disease1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14 without relation to race.6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22 However, single case reports have shown that cherubism may occur in a patient apparently without hereditary origin of the disease,17, 19, 23, 24, 25, 26, 27 as in 2 of the cases reported here.

In general, cherubism does not involve other parts of the skeleton2

Acknowledgements

The orthodontic evaluations of the patients were carried out by Professor Sven Kreiborg, Dr Odont, Department of Pediatric Dentistry, School of Dentistry, Faculty of Health Sciences, University of Copenhagen. The later clinical information and radiographs of the adult patient of 3° and his son were given by Dr Mogens Besserman, Chief of Department of Oral and Maxillofacial Surgery, Næstved County Hospital, Denmark. The help from Professor Kreiborg and Dr Besserman, as well as the help from

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      Citation Excerpt :

      Intraoral findings are associated with malocclusion and abnormal dentition with displaced, unformed, unerupted or absent teeth along with the early loss of deciduous teeth [8,13]. The radiographic findings consist of bilateral, multilocular and irregular radiolucent lesions of jaw bones [7,14]. The benign nature is shown by the non-disrupted thin cortical layer in the expanded bone [7,13].

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    *

    Reprint requests: Nina von Wowern, DDS, PhD, Dr Odont, Department of Oral and Maxillofacial Surgery, School of Dentistry, University of Copenhagen, Nørre Allé 20, DK-2200 Copenhagen N, Denmark

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