The surgical treatment of fibrous dysplasia. With emphasis on recent contributions from cranio-maxillo-facial surgery.

Abstract

Fibrous dysplasia is a congenital, metabolic, nonfamilial disturbance that occurs in one or more bones, at times in association with skin pigmentations or endocrine abnormalities. The authors report on a large personal series of 23 patients with fibrous dysplasia involving the craniofacial skeleton. The etiology, clinical findings, pathology, and differential diagnosis of this condition are reviewed and a working hypothesis is offered for the pathophysiology of this disorder. Approximately one-third of patients with fibrous dysplasia have involvement of the cranial or facial bones. The authors describe how new techniques in craniofacial surgery have opened up additional options for this group of patients. Deformity, diplopia, proptosis, sinus infection, deafness, and loss of vision, are some of the clinical features that may require early surgical management. Evidence is given to support more complete resection of bony lesions with immediate reconstruction by several techniques. The removal, remodeling, and replacement of the dysplastic bone is advanced as a promising new method for the management of these complex problems. Successful use of this technique in four patients is reported. In a separate group of patients, continuing good experience is reported with cranio-orbital reconstruction by means of large methyl-methacrylate implants. Both of these surgical approaches eliminate donor site morbidity that results from the grafting of large amounts of autogenous bone. Both techniques also avoid the problems associated with postoperative absorption of bone grafting. Several patients are reported in whom serious disturbances in visual function appear to have been prevented or reversed by early treatment. Factors leading to malignant change in patients with fibrous dysplasia are reviewed.

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