Long-Term Outcome of Fibrous Dysplasia: Reconstruction With Dysplastic Bone -Case Report-

Abstract

A 10-year-old girl presented with facial asymmetry associated with bulging of the right fronto-orbital region with no symptoms. Computed tomography demonstrated enlargement of the right zygomatic, frontal, ethmoid, and sphenoid bones. Abnormal proliferation of the bone had obliterated the sphenoid, right frontal sinus, and right ethmoid sinuses. These radiological findings suggested right optic nerve compression due to fibrous dysplasia. Right optic canal decompression was performed. In preparation for recurrence, the resulting bone defect in the right orbital roof was reconstructed using the outer table of the split lesion bone. The removed frontal bone was divided into intact and lesioned parts, and the intact part was returned. The lesioned part was split and the outer table graft used to reconstruct the frontal region. A temporal musculopericranial flap was used to form a barrier between the opened ethmoid sinus and cranial cavity. A protrusion appeared on the left forehead 10 years later, and was shaved to improve the aesthetic appearance. The patient was followed up for a total of 23 years. The use of dysplastic bone involves the risk of recurrence, but the period of recurrence is delayed and the progression stops after adolescence, so the second operation involved only shaving for aesthetic appearance. This procedure is one of the treatments of choice because of easy reconstruction, easy revision, and good results.

Craniofacial Fibrous Dysplasia Involving the Orbit

“…10 The reported recurrence rate of FD lesions is variable, mostly between 25% and 55%. 16,23 In a series of 18 patients who underwent optic nerve decompression for orbital FD, Cutler et al 11 reported full encasement of the optic nerve on postoperative cranial CT scans in 15 (83%) of them, but only 2 of the 15 patients experienced symptoms of optic neuropathy. However, it was not known whether this was due to incomplete decompression or regrowth.…”