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Cherubism is a skeletal dysplasia limited to the maxilla and mandible, affecting children and adolescents. A 13-year-old male patient was referred to the Oxford University Hospitals Oral and Maxillofacial Surgery Department, by his orthodontist, following an incidental finding of bilateral multilocular radiolucencies of the mandible. This led to multiple clinical, radiographic, histopathological, genetic and biochemical investigations involving various medical and dental specialities. The multidisciplinary approach led to the successful management of this patient. Even though cherubism is self-limiting, long-term radiographic and clinical surveillance is required.
CPD/Clinical Relevance:
Dentists should understand the clinico-radiographic presentation of cherubism and have an awareness of how the possible functional and aesthetic complications associated with the condition are managed.
Article
Cherubism is a rare and non-neoplastic condition, limited to the maxilla and mandible. It is a type of skeletal dysplasia characterized by multilocular, bilateral and symmetrical fibro-osseous lesions, causing painless expansions of the jaws. The pathogenesis is commonly attributed to a mutation in the gene encoding for SH3-binding protein 2 (SH3BP2), which leads to the localized degradation and replacement of bone with fibrous tissue.
Cherubism has a childhood onset, with lesions first appearing between the ages of 2 and 7 years, and progressing until puberty. It is self-limiting, and there is regression and involution of the bone lesions during pubertal bone remodelling.2 The phenotypic symptoms range from a normal appearance to gross bilateral maxillomandibular deformities. These can lead to a rounded facial appearance, retraction of the lower eyelids leading to increased scleral show, drawing resemblance to a cherub.3 There are a plethora of differential diagnoses for cherubism. These include but are not limited to odontogenic or non-odontogenic cysts of the jaw, odontogenic tumours and giant-cell lesions.2
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