Prophylactic optic nerve decompression in pediatric craniofacial fibrous dysplasia: To do or not to do?

Fibrous dysplasia (FD) is a pathological condition in which normal bone is replaced by abnormal fibro-connective tissue. Although characterized as a benign condition, it can be large and invasive, causing considerable disfigurement and dysfunction. When faced with such a destructive process in a child, the obvious questions raised are what to do about it and when. We present here a case of craniofacial FD in an 11-year-old boy, in whom the growth in the right orbit had caused swelling, epiphora, proptosis, and pain. The patient was treated with prophylactic optic nerve decompression with excellent results. While deteriorating vision is a definitive indication for optic nerve decompression, the need for a prophylactic treatment is a controversial one. The rate of irreparable loss of vision is well-documented, and the resulting morbidity is one which must be considered in children. We have singularized this case of prophylactic optic nerve to review the various clinical and treatment aspects of craniofacial FD and to specifically debate the viability of prophylactic optic nerve decompression in pediatric craniofacial FD.