Orbital involvement in cherubism.
OBJECTIVE
To demonstrate the clinical, radiologic, and histopathologic features of a patient with orbital involvement in cherubism that prompted surgical treatment.


METHODS
Single interventional case report.


METHODS
Findings of the ophthalmic evaluation, computed tomography (CT) scans, intraoperative examination, and light microscopy of the specimens were analyzed.


METHODS
Globe displacement, orbital bony lesions detected on CT scans, histopathology, and postoperative results were assessed.


RESULTS
A 27-year-old female was seen with a slowly progressive superonasal globe displacement and a temporal orbital mass bilaterally of 6 years' duration. She had a history of cherubism, but her cheeks and jaws had a normal appearance instead of the bilateral fullness of the lower half of the face typical of the disease. CT scans demonstrated multicystic bony lesions arising from the orbital floors bilaterally. The masses were excised using an anterior transcutaneous transseptal orbitotomy. Histopathology demonstrated numerous giant cells in a fibrovascular stroma, confirming the clinical diagnosis of cherubism. Postoperative recovery was complete.


CONCLUSIONS
Orbital involvement in cherubism may develop beyond puberty, after stabilization or regression of the lesions in the jaws. Patients with cherubism should be routinely evaluated by an ophthalmologist.
Colombo
F
F
Department of Ophthalmology and Eye Hospital, University of Erlangen-Nürnberg, Erlangen, Germany.
Cursiefen
C
C
Neukam
F W
FW
Holbach
L M
LM
D000328:Adult; D002636:Cherubism / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D005260:Female; D006801:Humans; D009916:Orbital Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D014057:Tomography, X-Ray Computed