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Lawrence R. Lustig, MD; Michael J. Holliday, MD; Edward F. McCarthy; George T. Nager, MD

Arch Otolaryngol Head Neck Surg. 2001;127:1239-1247.

Objective  To gain a broader appreciation of the clinical presentation, operative treatment, and outcome of patients with fibrous dysplasia involving the skull base.

Design  Retrospective review of a clinical case series.

Setting  A single tertiary academic medical center.

Patients  Twenty-one patients with histopathologically confirmed fibrous dysplasia involving the skull base cared for over a 15-year-period (1983-1998).

Main Outcome Measures  Clinical and radiographic location of the fibrous dysplasia lesions within the skull base, clinical presentation, surgical intervention, and clinical outcome were tabulated for each patient.

Results  The ethmoids were most commonly involved (71%), followed by the sphenoid (43%), frontal (33%), maxilla (29%), temporal (24%), parietal (14%), and occipital (5%) bones. The most common presenting features included atypical facial pain and headache, complaints referable to the sinuses, proptosis and diplopia, hearing loss, and facial numbness. Surgical treatment, guided by clinical presentation, ranged from simple biopsy with conservative follow-up to craniofacial resection.

Conclusions  Fibrous dysplasia can present in myriad ways within the skull base. Modern imaging modalities and histopathologic analysis have made diagnosis relatively straightforward. Surgery, particularly in such a challenging region as the skull base, should be reserved for patients with functional impairment or a cosmetic deformity. Because of the benign nature of the condition, the surgery itself should be relatively conservative, with the primary goal being preservation of existing function.

From the Departments of Otolaryngology–Head and Neck Surgery (Drs Lustig, Holliday, and Nager) and Pathology (Mr McCarthy), The Johns Hopkins University, Baltimore, Md.

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