Hemorrhagic Fibrous Dysplasia with Acute Neurological Decline: Case Report and Review of the Literature.

Abstract

Fibrous dysplasia is a rare, benign fibro-osseous malformation whose occurrence in the craniofacial area can result in optic nerve compression, a cerebral mass effect, and cosmetic deformity. Most lesions will progress slowly, and the risk of malignant progression is rare. We present the case of a 21-year-old woman who had presented with acute worsening visual loss secondary to hemorrhagic fibrous dysplasia with ensuing optic nerve compression. Emergent surgical decompression resulted in rapid improvement of her visual dysfunction. The pathological features demonstrated a mixed pattern of woven bone in a fibrous background and secondary aneurysmal bone cyst-like changes. Hemorrhagic transformation of craniofacial FD remains rare but can present with acute neurologic deterioration. Rapid diagnosis and treatment can allow reversal of patient morbidity. We have also included Supplementary Video 1 to illustrate the surgical principles, and we review the reported data of similar cases.