A seven-year-old male visited a dentist for an initial exam and checkup. Radiographic examination revealed bilateral lesions in the posterior mandible and ascending rami.
Joen Iannucci Haring, DDS, MS
The patient`s parents denied any history of signs or symptoms associated with this region. The patient appeared to be in a general good state of health with no significant medical history. At the time of the dental appointment, the patient was not taking medications of any kind.
The patient`s vital signs were all found to be within normal limits. Examination of the head and neck region revealed no enlarged or palpable lymph nodes. Extraoral examination revealed a plump-cheeked facial appearance.
Intraoral examination revealed a bilateral bony expansion of the posterior mandible and ascending rami. No soft tissue abnormalities were noted.
After a thorough clinical examination and a review of the patient`s medical and dental histories, a panoramic radiograph and two bite-wings were ordered.
Examination of the panoramic radiograph revealed large bilateral, well-defined multilocular radiolucencies in the posterior mandible and ascending right and left rami (see radiograph).
Based on the clinical and radiographic examination available, which one of the following is the most likely diagnosis?
- fibrous dysplasia
- Paget`s disease of bone
- central giant cell granuloma
- ondotogenic keratocysts
Cherubism is a rare, developmental fibro-osseous condition. It is inherited as an autosomal dominant trait or occurs as a mutation. This benign condition only occurs in the jaws.
The term cherubism refers to the cherubic-like appearance of children with this condition. Affected patients are said to have an angelic appearance and resemble the cherubs (plump-cheeked little angels) depicted in Renaissance paintings.
The bony lesions of cherubism occur in childhood. The mean age for detection is seven years, although some cases are identified as late as 12 years. Males are affected more frequently than females. The lesions begin early in life as slow-growing expansions of the mandible or maxilla. These expansions are painless and occur in a buccal-lingual direction; the ascending rami exhibit expansion anteriorly as well. It is this symmetric bilateral expansion of bone that is responsible for the chubby-cheeked, cherubic appearance. The lesions continue to enlarge until puberty - at which time the size stabilizes.
The vast majority of cherubism cases exhibit involvement of the posterior mandible and ascending rami in a bilateral symmetric manner. In severe cases, most of the mandible may be involved.
The maxilla is less often involved. In such cases, the skin of the upper face may be stretched which results in the appearance of "upturned eyes." Developing teeth are often displaced and fail to erupt as the result of this condition.
The lesions of cherubism appear as large, multilocular, and expansile radiolucencies. The radiographic findings are particularly striking and highly diagnostic as the result of the bilateral pattern. Unerupted teeth are often displaced by the large lesions and appear to be floating with the cystic areas.
It is generally accepted that the lesions of cherubism are self-limited and undergo spontaneous regression at puberty, with relatively good resolution by age 30. Surgical intervention may be required in order to improve function or to improve the esthetic appearance of the patient. When necessary, a conservative curettage of the lesion with bony recontouring surgery is postponed until after puberty. The prognosis for patients with this condition is relatively good, especially if only the mandible is involved.
The decision to treat or simply observe a patient with cherubism is a difficult one. In some cases, early surgical intervention has provided excellent results. In other instances, early surgical intervention has resulted in a rapid regrowth of the lesions and a worsened facial deformity. In contrast, some untreated lesions regress and show varying degrees of remission after puberty, while others persist and continue to grow into adulthood.
Joen Iannucci Haring, DDS, MS, is an associate professor of clinical dentistry, Section of Primary Care, The Ohio State University College of Dentistry.