Diffuse Fibrous Dysplasia of the Lateral Skull Base

Objective: Fibrous dysplasia (FD) of bone is a benign, slowly progressive fibro-osseous tissue disease of unknown etiology. Surgery, mostly conservative, should be reserved for patients with functional impairment or cosmetic deformity. Design: Case report. Patient: A 47-year-old woman with previously diagnosed diffuse FD of the lateral skull and skull base was referred to our center due to untreatable pain of the ipsilateral side of the head. The patient used a combination of NSAIDs, opioids, and adjuvant analgesics (anticonvulsants and antidepressants), as well as bisphosphonates, without appropriate clinical response. Nonserviceable hearing and hypoesthesia of the trigeminal nerve were present on the ipsilateral side. CT demonstrated a lesion involving the pyramid, the middle fossa, the temporal squama, and the occipital bone bilaterally. Subtotal petrosectomy via transotic approach without facial nerve transposition was performed together with radical resection of the involved cranium. The large defect was reconstructed by two custom-made 3D microporous polyethylene implants (Medpor, Porex-Stryker). Results: Postoperative course was uneventful. Postoperative facial nerve palsy as well as the trigeminal hypoesthesia resolved completely within 6 months. The VAS score declined from 8 preoperatively to 2 postoperatively, with only irregular administration of tramadol as an SOS medication. The cosmetic result was excellent. There is no sign of recurrence 18 months after surgery. Conclusion: Despite the benign nature of the disease, radical resection of diffuse FD of the lateral skull base and reconstruction of the resultant defect is a challenging procedure simply because of the extent of surgery required.