Abstract
IntroductionFibrous dysplasia is a benign tumour of the bones and is a disease of unknown aetiology. This report discusses a case of proptosis and visual deterioration with associated bony mass involving the right orbit.Case presentationA 32-year-old Nigerian man of Yoruba ethnic origin presented to the eye clinic of our hospital with right-eye proptosis and visual deterioration of 7-year duration. Presentation was preceded by a history of trauma. Proptosis was preceded by trauma but was non-pulsatile with no thrill or bruit but was associated with bony orbital mass. The patient reported no weight loss. Examination of his right eye showed visual acuity of 6/60 with relative afferent pupillary defect. Fundal examination revealed optic atrophy. Computed tomography showed an expansile bony mass involving all the walls of the orbit. The bony orbital mass was diagnosed histologically as fibrous dysplasia. Treatment included orbital exploration and orbital shaping to create room for the globe and relieve pressure on the optic nerve.ConclusionFibrous dysplasia should be considered in the differential diagnosis of slowly developing proptosis with associated visual loss in young adults.
Highlights
Fibrous dysplasia is a benign tumour of the bones and is a disease of unknown aetiology
Journal of Medical Case Reports 2009, 3:14 http://www.jmedicalcasereports.com/content/3/1/14 only involves the craniofacial skeleton in about 10% of cases, having a predilection for the ribs and femur [3]
Fibrous dysplasia results from a defect in osteoblastic differentiation affecting the final maturation of the bone [11]
Summary
Fibrous dysplasia is a benign, slowly growing disorder of bone in which the normal cancellous bone is replaced by immature woven bone and fibrous tissue [1]. Initial exploration of the right orbit performed at the referring hospital revealed a bony hard mass involving both the lateral and medial orbital wall. The right ocular examination revealed a visual acuity of 6/60 with a proptosis of 17 mm (Hertel exophthalmometer). Cranial computed tomography scan (Figures 1 and 2) showed a right expansile bony mass involving 1) the orbital roof and especially the lesser wing of the sphenoid; and 2) the medial and lateral walls of the orbit, especially the greater wing of the sphenoid. At the time of writing, his postoperative visual acuity remains at 6/60 and he is receiving follow-up in the outpatient clinic
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