Pediatric Fibrous Dysplasia of the Skull Base: Update on Management and Treatment.

BACKGROUND

Fibrous dysplasia (FD) is often difficult for skull base surgeons to address. FD arises due to the abnormal proliferation of fibroblasts, ultimately resulting in immature osseous tissue replacing normal cancellous bone. When the skull base is involved, it can result in cranial nerve compression. FD affecting the optic canal and optic nerve is the most concerning as new onset of vision loss is considered a surgical emergency. The prevalence of FD is approximately 3.6 per 1,000,000. The most severe implications of this disease are neurological deficits due to cranial nerve compression, cosmetic appearance, and high recurrence rates even in the setting of surgical and medical therapy interventions.

METHODS

A PubMed search of "pediatric fibrous dysplasia management" using MESH Terms was conducted. Articles were excluded for non-English languages, inaccessibility, and events/erratum/letters to the editor. Included articles were in English, as well as encompassed pediatric FD case reports or comprehensive reviews of FD that discussed pediatric presentations.

RESULTS

A total of 109 articles were reviewed, and 44 were included in the final review. Most articles were case reports. There is a clear need for guidelines regarding surgical intervention, especially in the pediatric population, where hormonal fluctuation can influence rates of recurrence and bony deformity. Overall, most surgeons recommend close observation with biomarkers and radiographic imaging for asymptomatic patients until at least the age of 16 years old. Conservative methods, such as RANK-L inhibitors, can be utilized to decrease growth with some success, especially in older adolescents.

CONCLUSION

This review is an update on this disease and its presentations, imaging findings, and treatment options. The current literature lacks clear guidance on management, especially in regard to surgical intervention or recurrence monitoring algorithms.