Polyostotic fibrous dysplasia with craniofacial localization presenting with frontal lobe compression in a...

We describe a rare case of polyostotic fibrous dysplasia with craniofacial localization associated with involvement of the spine and extremities. A 14-year-old girl presented with progressive headache, left frontal swelling, exophthalmos of the left eye, deformity and palpable mass in the left frontal area. Cranial computerized tomography revealed extensive involvement of all cranial bones except right frontal and right parietal bones. Most of the facial bones were invaded. Especially, there was the involvement of ethmoidal air sinuses and frontal sinus also. Computerized tomography showed left frontal lobe compression. In addition, the spine and bones of the extremities were involved in the patient. Craniofacial approach was planned. Cranial surgery was performed by an extradural frontal approach. Firstly, the frontal cyst was excised. For left frontal lobe decompression, we removed successfully all the abnormal bones causing mass effect and frontal deformity. Secondly, facial surgery was performed with external approach. An attempt to remove all of the involved bone is necessary, as the lesion may recur and grow if a portion of dysplastic bone is left in place. However, the present case suggests that removal of all the involved bones in the polyostotic fibrous dysplasia may be possible in spite of extensive involvement. In this situation, it may be removal of only abnormal bones responsible for compression of affected neural elements is indicated.