Cranial fibrous dysplasia: An institutional experience and review of the literature

Abstract

Background: Cranial or craniofacial fibrous dysplasia (CFD) is a rare entity which most often presents with either incidental finding or with pain/cosmetic disfigurement or visual/hearing problems. Multidisciplinary treatment with close follow-up or medical management/surgery is options. Management of these lesions can often give satisfying results. There is a dearth of neurosurgical literature on this subject matter. Our objective was to review the clinical symptomatology and outcome of CFD patients managed in our institution. Methods: This is a retrospective observational study of CFD patients managed in our institution over a period of 5 years. Clinical and radiological data were collected from departmental database. Outcomes were evaluated immediately and on 1–4 years follow-up. Results: A total of 21 patients were managed over a period of 5 years with age ranging from 12 to 55 years and symptoms of cosmetic issues or visual disturbance. Preoperative computed tomography scan with 3D reconstruction with bone window was done in all patients. In most of the patients (16/20), immediate reconstruction was done following excision. Five patients were managed conservatively. Follow-up was obtained over a period ranging from 1 to 4 years and all (except one) patients are doing well. Only one patient had permanent visual impairment in spite of early intervention. Conclusion: Craniofacial dysplasia has various modalities of management. Careful selection of patients for surgical or conservative management is feasible with good results both in short and long term.