Ocular complications in craniofacial fibrous dysplasia.

Abstract

SUMMARY Three cases of craniofacial fibrous dysplasia are reported, all presenting to the ophthalmologist first. They all had ocular complications due either to direct involvement of the orbital bones or to secondary complications. Sphenoidal mucocele is added to the other complications previously reported, and accordingly a classification of the various ways that fibrous dysplasia can affect the ocular structures is proposed.

Cranioorbital fibrous dysplasia: with emphasis on visual impairment and current surgical management

“…41 Other explanations include exophthalmos-induced optic nerve traction, sinus mucocele formation, with increased intraorbital pressure, spontaneous hemorrhage, bone cyst formation, or rare vascular events. 4,7,9,10,14,22,28,30,35,39,40 More recently, our group and others have questioned whether the most common cause of visual loss in fibrous dysplasia is indeed bone overgrowth-induced optic canal stenosis. This traditionally held belief may not be supported after careful scrutiny and critical analysis, particularly of cases in which quality CT or MR imaging studies and careful neuroophthalmological data are available.…”

Surgery improves vision and cosmetic appearance of an adult patient with fibrous dysplasia of the frontal bone

“…4 Among FD of the entire body, cranio-facial FD is relatively rare, and it is known that ocular problems such as visual loss, diplopia and proptosis occur in 20-35% of patients with FD of this region. [5][6][7] Here we report a case of FD of left frontal bone that was successfully treated by two staged surgeries.…”

Fibrous dysplasia of the sphenoid associated with visual loss and optic disc oedema

“…We considered the possibility that the mucocele may have contributed to the visual loss as has been previously reported, [6][7][8]15,16 but rejected this as it was not thought to be in contact with the optic nerve.…”

“…There may be unilateral proptosis (with secondary exposure keratitis), trigeminal neuralgia and/or anaesthesia, extraocular muscle palsies, chiasmal compression, or maxillary sinus involvement with epiphora resulting from nasolacrimal duct compression. 7 Dull headache, facial dysmorphia, 11 and optic atrophy 1 may also be found in these patients. Fibrous dysplasia is thought to occur mainly in childhood, but growth may continue into adult life and because of this, earlier more radical surgery has been advocated attempting complete excision when possible.…”