CASE REPORTS   

Italian Journal of Maxillofacial Surgery 2010 December;21(3):155-60

Copyright © 2011 EDIZIONI MINERVA MEDICA

language: English

Clinical and radiological characteristics of a severe form of cherubism: case report

Piombino P., Spinzia A., Parascandolo S., Abbate V., Califano L.

¹ Department of Maxillofacial Surgery, Federico II University, Naples, Italy 2 Department of Maxillofacial Surgery, A. Cardarelli Hospital of Naples, Italy

Cherubism is a rare, inherited condition characterized by fibro-osseous lesions of the maxilla and mandible. It has recently been localized in chromosome 4p16.3. The fullness of the lower half of the face and retraction of the lower lids gives the characteristic “eyes raised to heaven” cherubic appearance. A case report of a 11-year old boy with extensive orbital involvement of cherubism is presented. The patient has orbital manifestations including lower lid retraction, proptosis, diplopia and globe displacement. Taking into account the severity of the disease based on the modified Motamedi grading system, we decided to treat the orbit surgically and to place the patient under a post-surgery weekly follow-up to assess the spontaneous regression of the extra-ocular disease in accordance with the data reported in literature. We maintain that decompressive surgery on the orbit is desirable in severe cases of the disease to avert the risk of irreversible ocular lesions.