Fibrous dysplasia of the orbit in childhood. Clinical features and management.

In 16 children with orbitocranial fibrous dysplasia confirmed histopathologically, facial asymmetry, orbital dystopia, and unilateral proptosis were the main presenting problems. Five children had radiological evidence of narrowing of the optic canal but only two developed optic atrophy and visual loss. One patient had persistent epiphora due to involvement of the bony nasolacrimal duct. Sixteen patients were treated by excision of the dysplastic bone and 15 had immediate craniofacial reconstruction. A good cosmetic and functional result was achieved in the majority of cases although transient vertical diplopia and minor degrees of ptosis occurred postoperatively in about a third. Fibrous dysplasia of the orbit is progressive in childhood and is best managed by radical surgery and reconstruction in a specialized craniofacial unit.