Non-familial cherubism: A case report with its surgical management
An 11-year-old male patient of non-consanguineous parents came with the chief complaint of missing teeth in both upper and lower jaw. Patient gave a history of painless bilateral swelling in the mandibular region. Swelling arose at the age of approximately 3-4 years, which progressed rapidly until the age of 8 years. There was no history of a similar disease in any the parents or siblings. Prenatal and natal history revealed a normal full term pregnancy. There was no history of any drug therapy. Patient did not suffer from any serious illness, mental retardation or social inabilities. On clinical examination, it was noticed that swelling was more prominent on the right side, firm on palpation and non-tender. The extension of swelling was from the anterior part of ramus to mandibular premolar region on the right side and from the anterior part of ramus to mandibular molar region on the left side []. Both right and left submandibular lymph nodes were palpable. Gingiva was pink, firm, fibromatous, covering almost all the teeth []. Mandibular buccal vestibular fullness due to swelling in both right and left side was seen. Panoramic-radiograph [] showed large, multiloculated soap bubble-like cavities at the angle and ramus of the mandible as well as thin vestibular cortical bone without involving condyles. These lesions were accompanied by displacement and impaction of erupted teeth and dental follicles. Incisal one-third of mandibular incisors were visible in the oral cavity, all the other teeth were impacted.\nSerum alkaline phosphate, calcium and phosphorous levels were within the normal ranges. Histopathological evaluation of an incisional biopsy in right mandibular region showed highly vascular fibrous stroma with numerous multinucleated giant cells. The family, clinical, radiographic, laboratory and microscopic features suggested a diagnosis of non-familial cherubism. The treatment consisted of an external bevel gingivectomy in all quadrants using a scalpel and electro cautery. In between, gingivectomy was performed quadrant wise with adequate healing time [Figures and ]. Post-operatively healing was excellent with improvement in quality-of-life. Orthodontic treatment has been planned for the present case in the near future and follow-up visits are still in progress.