Transconjunctival Orbital Decompression in Cherubism.

Abstract

Cherubism is a rare fibro-osseous disease characterized by the progressive expansion of the mandible and maxilla during childhood. Orbital involvement occurs in a subset of patients and is clinically manifested as upward displacement of the affected eye. The bony changes tend to spontaneously diminish or even regress after puberty. The authors report a case of a young adult female who underwent craniofacial correction of the maxilla and mandible changes but continued to have unilateral scleral show. CT scans of the orbits revealed a residual mass in the right orbit, associated with superior displacement of the inferior orbital rim. The abnormal relationship between the eye and eyelid margin was fully corrected through mass debulking combined with inferomedial orbital decompression.