Single-stage complex reconstruction for fibrous dysplasia: A case report

Objective and Importance: Fibrous dysplasia is a benign skeletal condition which can involve the craniofacial skeleton. Surgery is indicated for correction of deformity and neurovascular decompression. Traditional surgery involves harvesting remote bone or calvarial grafts, requiring prolonged, multisite procedures. Custom implants may eliminate the need for bone harvesting, but usually require staged procedures with interim bony defects. We report the use of preoperative cranial modeling and implant design, allowing for a single reconstructive procedure. Clinical Presentation: A 32-year-old woman with McCune-Albright syndrome was referred to Neurosurgery and Ophthalmology for right facial pain, deformity, and eye pressure. Examination revealed a 6-mm right eye proptosis and decreased sensation and severe pain in the right V2 distribution. Imaging demonstrated extensive fibrous dysplasia, with right orbital mass effect and nerve compression. Intervention: A single procedure was planned to remove and replace the involved bone. High-resolution CT was used to create a scale model of the patient's cranium. The planned resection was performed on the model, allowing for preoperative custom implant design. We then performed single-stage resection and reconstruction. The right superior and lateral orbital rims, portions of the left superior and medial orbital rims, and the nasion were resected. The custom implant was contoured to fit and remaining small defects were filled with Fast Set Norian. No complications occurred. At follow-up, the patient reported pain and pressure, relief, and examination showed resolution of proptosis, improved sensation, and excellent cosmesis. Conclusion: Preoperative cranial modeling and implant design allows for a shorter, one-step resection and reconstruction procedure without compromising structural or cosmetic outcome.