Short communication
Cherubism: long-term follow-up of 2 patients in whom it regressed without treatment

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Abstract

Cherubism is a rare hereditary, self-limiting fibrous dysplasia characterised by painless enlargement of the jaws in childhood. Although, it is accepted that the lesions of cherubism are eventually replaced by bone, there have been few long-term follow-up reports with clinicoradiographic documentation of spontaneous remission of the disease, without treatment. We report two cases of cherubic boys who were followed for 17 and 19 years. Clinicoradiographic examination during this period showed regression of the disease without surgical correction.

Introduction

Cherubism is a rare, non-neoplastic, self-limiting fibro-osseous disease, characterised by painless expansion of the mandible or maxilla, or both. Children are normal at birth and the expanding jaw is noticed within the first years of life, becoming progressively larger until the beginning of adolescence.1, 2 It is genetically inherited,3, 4 although many non-familial cases have been reported.2, 5, 6, 7

Although, regression of the lesion is expected during puberty, documented reports with long-term follow-up are rare.1, 2, 6 The aim of this paper is to report two patients who were followed for 17 and 19 years. Clinicoradiographic observation showed that the cherubic characteristics regressed, emphasising the importance of close long-term follow-up.

Section snippets

Case 1

A 6-year-old boy was brought by his parents for evaluation of a painless growth on his face. A panoramic radiograph (Fig. 1A), and an incisional biopsy confirmed the diagnosis of cherubism and it was planned to follow him up. By the age of 12, the growth had stabilised. At the age of 21, he returned for extraction of ectopic and unerupted teeth and swelling was seen only in the maxilla (Fig. 1B). At 23 years of age his remaining teeth required extraction and at this time he had only minor

Discussion

The current knowledge about the clinical behaviour of cherubism is based on the study by Jones et al.,1 on a few series of cases,2, 5, 8, 9, 10 on single case reports,6, 7 and on reviews of published cases. Although, almost every paper states that the disease progresses until puberty, stabilises and then regresses, only a few studies have followed untreated patients for a long period showing documented regression of the lesions. Katz et al.,6 who reported regression in a patient with an

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