Cherubism: Rare fibro-osseous dysplasia
Cherubism is a rare, genetic, fibro-osseous dysplasia which presents with progressive bilateral upper and lower jaw enlargement, with typical facial characteristics. First described by Jones in 1933, there are about 300 reported cases in the literature, the facial features bearing a passing resemblance to a biblical cherub.Features include remodelling of normal bone to fibrous tissue with cyst formation, leading to early tooth loss, mandibular and maxillary enlargement. Orbital involvement is very rare, tending to present in advanced cases, leading to globe dislocation, ophthalmoplegia, optic nerve compression and macular striae and scarring. Where patients present with significant globe displacement and/or optic neuropathy, mass debulking of the lesion can be successful, as described in this case.