Fronto-orbital fibrous dysplasia.
Fibrous dysplasia, which is characterized by a disorganized mixture of fibrous and osseous elements in the affected bones, is a non-neoplastic developmental anomaly caused by an activating mutation. Despite an identical histological pattern, the clinical behavior varies according to the site of involvement. Fronto-orbital lesions behave more aggressively and generally continue to grow during adulthood. During the last 20 years, 32 cases of fronto-orbital fibrous dysplasia have been treated and the results of treatment have been assessed. The indications for surgery were mainly visual deterioration due to optic nerve compression, globe malposition and widespread disease causing gross facial deformity. The treatment of these patients consisted of radical resection of the fibro-osseous tissue, decompression of the optic nerve canal and reconstruction of the resected fronto-orbital areas with either noninvolved bone grafts or with dysplastic bone which was contoured down or, in one case, heated in the sterilizer. Recently, Pamidronate, a bone resorption inhibitor, has been added to the treatment protocol. During follow-up there have been two recurrences, repeat optic nerve decompression has not been required and overall the esthetic results are satisfactory. Fronto-orbital fibrous dysplasia can have a devastating outcome with high recurrence rates and blindness, especially following incomplete resection. A radical and extensive approach to treatment is recommended; this seems to be the best choice as witnessed by the good results achieved in this series.
Yavuzer
Reha
R
Institute for Craniofacial and Reconstructive Surgery, Providence Hospital, Southfield, Michigan, United States of America
Bone
Henry
H
Jackson
Ian T.
IT