Polyostotic fibrous dysplasia is a rare disorder of bone development characterized by focal replacement of normal bone matrix with disorganized fibroosseous tissue. This leads to bone deformity, hypervascularity, impingement on surrounding structures, and fracture. Image 1A represents the preoperative magnetic resonance image of a 25-yr-old male with polyostotic fibrous dysplasia who underwent decompressive hemicraniectomy. The resected portion of skull (yellow box) is notable for atypical thickness and irregularity of the dysplastic bone. Image 1B shows this same area after magnetic resonance image contrast administration. The significant contrast uptake (white enhancement) demonstrates the highly vascular nature of the bone. This patient’s intraoperative course was complicated by severe, refractory hemorrhage. Image 2 represents the gross specimen of resected skull, again notable for its thickness and highly vascularized surface.

Patients with polyostotic fibrous dysplasia present two...

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