Fibrous dysplasia of the skull in children

Early diagnosis of skull fibrous dysplasia in children is relatively easy, based on a history of painless progressive bony bulging. The therapeutic approach is still controversial, due to the benignity of the lesion. However, the clinical course may be unpredictable, with sudden appearance of symptoms, some of which can be important and irreversible. In pediatric patients, the possibility that an early surgical correction might positively interfere with the natural history of the lesion has to be evaluated by taking into account the obvious difficulties that will be encountered in reconstructing the skull after a wide excision of the pathologic bone. In the present report, we describe our personal experience on the surgical treatment of 9 children, ranging in age between 7 and 14 years. The patients were subdivided into two groups, according to the localization and extent of the disease. Patients in group 1 presented an involvement of the hair-covered cranium and/or the fronto-orbital region. Group 2 patients presented with a multizonal involvement of the skull, including the central cranial base (pterygoid, sphenoid, petrous and mastoid bone). The different surgical options, chosen for the two groups of patients, are discussed, together with the technical methodologies utilized, the cosmetic results and the long-term follow-up.