Abstract
Cherubism is a rare disease characterized by significant loss of medullary bone which is replaced by excessive amounts of fibrous tissue growth within the mandible and maxilla. We present a case of a 4-year-old boy with a rapidly enlarging mandible and maxilla, causing significant change in the facial contour, malocclusion and phonation difficulties. He was treated with aggressive tumor curettage, lateral mandibular cortex osteotomies with medial repositioning. This allowed obliteration of the enlarged medullary space and restoration of the normal mandibular anatomy. At 12 months postoperatively, the patient had significant improvement in facial contour, normal outward appearance, and stable dentition.
Highlights
Cherubism is a rare, autosomal-dominant, non-neoplastic fibro-osseous condition predominantly affecting the mandible and maxillary bones
There is no accepted approach for the majority of patients who fall into the “grey zone” between these two extremes of presentations, as was our patient’s case
Our report further strengthens this group of patients who have good outcomes with arrested tumor growth and excellent facial contouring following early surgical intervention
Summary
Autosomal-dominant, non-neoplastic fibro-osseous condition predominantly affecting the mandible and maxillary bones. It was first described as “familial multilocular cystic disease of the jaws” by William Jones in 1933[1]. The histopathology of the tissue involved in relation to cherubism demonstrates multinucleated osteoclast-like giant cells near bone and within the soft fibrous stroma. We present a cherubism case of an 4-year-old boy with moderate but rapidly expanding burden of disease. He underwent surgical management with curettage of the tumor, lateral cortex mandibular osteotomies and repositioning with an excellent outcome. Repeat CT scan examination showed normalization of the mandibular anatomy and significant ossification around the molars providing dental stability at the site of the mandibular bone repositioning [Figure 2C and D]
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