Casting an Eye on Fibrous Dysplasia of the Orbit

INTRODUCTION

Fibrous dysplasia (FD) is a benign fibro-osseous tumor of growing bone in which fibrous tissue replaces normal medullary bone. [1]

FD is attributed to a somatic mutation in the GNAS protein that results in proliferation of undifferentiated skeletal progenitor cells. The resultant bone in prone to overgrowth, deformation, and encroachment.

The symptoms resulting from craniofacial FD are dependent on the amount and location of disease. Amongst the most feared complications is optic nerve compression causing optic neuropathy and threatening visual deterioration and blindness.

Surgical intervention for FD of the orbit (FDO) (Fig. 1) involves orbital unroofing and optic nerve decompression, yet controversy has surrounded the timing and indication for this procedure (prophylactic vs. therapeutic treatment). [2,3]

Given the rarity of FD and the heterogeneity of its treatment course (Fig. 2), there is a paucity of data describing the progression of FDO and comparing the long-term outcomes of different surgical approaches.

OBJECTIVES

The presence of FDO threatens compressive optic neuropathy and irreversible vision loss. The purpose of this study is to retrospectively characterize the natural history of FDO and describe the longitudinal outcomes of 36 patients who underwent stratified surgical management (Fig. 3) at a single institution.

METHODS

Population