|Year : 1976 | Volume
| Issue : 2 | Page : 37-39
Unilateral proptosis due to fibrous dysplasia
Asst. Professor of Neuro-Ophthalmology, Madurai Medical College, Madurai, India
Asst. Professor of Neuro-Ophthalmology, Madurai Medical College, Madurai
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Natchiar G. Unilateral proptosis due to fibrous dysplasia. Indian J Ophthalmol 1976;24:37-9
Fibrous dysplasia of bone is comparatively an uncommon entity of unknown etiology which has a slow course with it symptomatology appearing during the first decade. It is characterised by accumulation of fibrous connective tissue which may replace the normal bone. The disease may affect a single bone (monostotic fibrous dysplasia). The ocular and orbital symptoms produced vary according to the bone that is affected. When the dysplasia involves the orbit, it usually does through one of the major bones contributing the orbital contour like maxillary, malar, ethmoid, frontal and sphenoid bones. The displacement of the eye is usually in the direction opposite to the source of expanding mass.
| Case report 1|| |
14 year old boy was referred to Neuro-Ophthalmic Clinic with the history of proptosis of the right eye of two years duration. The history revealed patient sustained a trivial injury over the right side of the forehead and according to the patient he developed prominence and bulging forward of right eye ball after the injury. Since four months he is ha\ing defective vision in the right eye and recurrent attacks of pain.
On examination he had a mild axial proptosis of right eye with full ocular movements. [Figure - 1],[Figure - 2]. There was a diffuse swelling over the supratemporal aspect of right orbit. On palpation there was a bony hard swelling about two inches away From the lateral third of the right eye-brow. There was no bony defect. The proptosis was neither reducible, nor increased in size during change of posture. Orbital margins felt normal. Finger could be insinuated into the orbit all around the globe and there was no soft mass felt in and around the orbit. The anterior segment of both eyes were quite normal. His vision in the right eye was 6/9 and in the left eye was 6/6. Fundus was normal in both eyes. Fields revealed concentric contraction for white and red test objects in the right eye and normal field in the left eye. There was no other positive neuroophthalmic or neurological finding. Clinically the possibility of osteoma was thought off. Blood counts were found to be normal.
X-ray skull lateral view revealed a dense sclerotic bony shadow involving the frontal, sphenoid, and ethmoid bones. In addition to the sclerotic lesion two spherical cystic lesions were seen over the frontal bone near the vault. X-ray optic foramen revealed a definite narrowing of right optic foramen and on the left side it was found to be normal. Radiologically, the findings were suggestive of fibrous dysplasia. Since his proptosis was very minimal and his vision was 6/9, no attempt was made for decompressing the optic nerve and we have advised him to have a follow-up once in two months so that if there is further decrease in visual acuity or further; reduction in the fields, exploration could be attempted.
| Case report 2|| |
Complete clinical history of this patient is not available at present, but, he was seen in the Ophthalmic Department, Erskine Hospital, Madurai in 1956 with the history of proptosis of the left eye. At that time his vision, fundus and ocular movements were normal. Radiologically condition was diagnosed as fibrous dysplasia involving the frontal, malar and maxillary and sphenoid bone.
He was asked to report periodically, but, he was not followed-up until eight years later he landed with severe degree of proptosis and gross deformity of left side of face. Advancing fibrous dysplasia was thought of clinically, but, radiologically and histopathologically condition was confirmed as osteogenic sarcoma and he was advised deep X-ray therapy.
This case history is a good example to note that sarcomatous changes can occur on fibrous dysplasia over long number of years.
| Discussion|| |
The condition is so slow growing that some children may become totally blind in one eye before a diagnosis is made. Usually, the deformity and pathological fracture may attract them for early medical aid. Fibrous dysplasia of the skull is often unassociated with bony changes elsewhere. The common ophthalmic findings are exophthalmos, optic atrophy, papilloedema and disorders of ocular motility. When the fibrous dysplasia affects the maxilla the eye ball is pushed upwards and when the roof of orbit is involved the eye ball is pushed downwards. When the sphenoid is affected proptosis is less severe and more straight forward. Malignant changes like sarcomatous changes have developed in cases of fibrous dysplasia and according to Schwartz and Alpert' malignancy develops in fibrous dysplasia in 0.5 per cent of cases.
| Summary|| |
Two cases of unilateral proptosis presenting with bony involvement in and around orbits with positive radiological findings are presented. Any young individual presenting with unilateral proptosis associated with irregular bony mass in and around the orbit must be subjected to careful radiological study including other long bones. Surgical interference is indicated whenever there is any threatening visual failure.
| References|| |
Schwartz, D. T., and Alpert, M., 1964, Amer. J. Med. Sci., 1,
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]