Surgical Treatment of Fibrous Dysplasia of the Skull in Children

Abstract

Introduction: We evaluate the role of surgery in the treatment of fibrous dysplasia of the skull in children. Methods: We identified 48 consecutive cases of fibrous dysplasia of the skull that were surgically treated at a single institution over a 23-year interval. The 28 patients that initially presented during the first two decades of life were selected for further analysis. Presenting symptoms, signs, surgical treatment, surgical outcome and the state of the disease at the extended follow-up interval were recorded. Results: Fibrous dysplasia of the skull in children most often involved the frontal, sphenoid and ethmoid bones. Most patients presented with facial asymmetry or proptosis. A gross total resection was achieved in 7 patients, subtotal resection in 17 patients, and 4 patients underwent biopsy alone. Over the follow-up interval, 7 patients had symptomatic progression of subtotally resected fibrous dysplasia and 3 patients had asymptomatic progression. The extent of resection was correlated with recurrence risk. Conclusions: In order to prevent progression of disease, an attempt at gross total resection is indicated in cases of fibrous dysplasia of the skull in childhood where the risk of neurologic morbidity is low and cosmetic results will be acceptable.

Surgical Management of Polyostotic Craniofacial Fibrous Dysplasia: Long-Term Outcomes and Predictors for Postoperative Regrowth

“…15–17 This is in keeping with the natural history of fibrous dysplasia, where lesions appear to expand during childhood and reach final disease burden around the time of skeletal maturity. Fibrous dysplasia lesion activity is thought to decrease with age because of apoptosis of mutation-bearing mesenchymal cells, leading to histologic improvement 18 and a decrease in fracture rate in older patients.…”

Surgical management of maxillofacial fibrous dysplasia under navigational guidance

“…7 All our subjects were adults with a long history of no active change over recent years, and their major complaint was facial asymmetry. We therefore decided to go ahead with conservative shaving, by which we focused on the cosmetic indication.…”

A Case Report-Fibrous Dysplasia of Temporal Bone

“…Surgery is done usually for cosmetic or functional reasons [22]. With surgical treatment offering only hope of cure although decision to operate in areas commonly affected such as the skull base requires a careful comparison of the morbidity caused by surgical resection and that caused by the disease itself [23]. Pathologic bone is spongy and soft and is easily curetted away, although recurrences are common.…”