Symptomatic cranial fibrous dysplasias: clinico-radiological analysis in a series of eight operative cases with follow-up results

Abstract

Cranial fibrous dysplasias are rare and comprise less than one percent of all primary bone lesions. These painless progressively expanding destructive bone swellings produce cosmetic deformities (commonest), sino-orbital and auditive complications (less common), peripheral compressive cranial neuropathies (uncommon) and compressive central neurological manifestations (rarest). Until recently some of the primary dysplastic skull base lesions were either treated expectantly or excised incompletely. In a significant percentage of cases, these lesions were considered inaccessible. However, with the development of modern neuro-imaging (CT and MRI) and the applications of current skull base approaches, the more aggressive and radical surgical procedures are being undertaken, with a significant improvement in overall outcome. We present a series of 8 cases of fibrous dysplasia in the age group of 10-33 years with a mean age of 19.75 years. Four cases each had monostotic type and polyostotic type. All patients presented with mild to moderate headaches, and seven cases with some form of cosmetic deformity. In 4 patients there was a unilateral proptosis, while only two had visual deterioration. One patient had anosmia and one hearing impairment. In one case fibrous dysplasia was hemicranial, and in another case the disease was pancranial. Neuro-imaging studies have well delineated the extent of the lesions. In two patients with unilateral fronto-orbital fibrous dysplasia, the removal of dysplastic bone, autoclaving and remodeling and replacement were performed. In two patients of sphenoidal wing fibrous dysplasia a total excision was possible. In the remaining four patients, a partial excision was performed. Overall outcome was good. There was no mortality. One patient had recurrent post-operative extradural haematomas. The authors present the results of an analysis of eight surgically treated cases of symptomatic cranial fibrous dysplasias along with their long-term outcome and the lessons learnt from this series.