|Year : 1985 | Volume
| Issue : 5 | Page : 317-319
Fibrous dysplasia of orbit
A Panda, Y Dayal, S Vasistha, NK Patnaik
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, New Delhi, India
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, New Delhi 110029
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Panda A, Dayal Y, Vasistha S, Patnaik N K. Fibrous dysplasia of orbit. Indian J Ophthalmol 1985;33:317-9
Fibrous dysplasia is an uncommon skeletal developmental anomaly of unknown origin without familial or hereditary tendency,. Most of these lesions begin in childhood and in some instances, cases do progress after puberty,,. There does not seem to be any sex predilection for the orbital form of disorder. However, Desmukh et al commented that it is thrice more common in females than in males. The purpose of this paper being to document 4 cases of proved fibrous dysplasia of orbit.
| Material and methods|| |
The details of 4 cases are tabulated [Table - 1].
| Discussion|| |
Fibrous dysplasia has been characterised as a pyramid of` diseases. The apex of the pyramid is the relatively rare Albright's syndrome, and its broad, in-definite base is a multitude of mono-osotic lesion.
The lesions of mono-ostotic fibrous dysplasia usually are discovered by accident, but they may present themselves because of pain, a pathological fracture, or a noticeable mass. In this type, the commonly affected bones are femur, tibia, rib and facial bones. The skull is not the usual, site of affection. The report of its occurrence in the orbit is very scanty in the literature.
Diffuse thickening of the bones with encroachment of the paranasal air sinuses, orbit and foramina of the skull can produce a variety of symptoms including visual loss, proptosis, diplopia, hearing loss, anosmia, nasal obstruction and epithora.
The disorder is most active during the second and third decades which is also true in our cases. After this period the dysplasia usually slows down.
Owing to the slow growing nature of the lesion some patients may become totally blind before a diagnosis is made. As the radiological findings are always characteristics in this disease an early radiological evaluation should be performed in all cases of unilateral proptosis not having any definite diagnosis. All of our cases could be diagnosed after reviewing the X-rays, Similarly CAT Scan has a value to know the areas of extension. In our third case it was possible to detect the intracranial extension by CAT scan and therefore, the case was referred to the neurologists for proper management.
The nature of fibrous dysplasia whether it is truly neoplastic or dysplastic hamartomatous mass, still debatable. The theory suggested by Lichtensteinr that it is the result of perverted activity of specific bone forming mesenchyma may be apparent. The theory of vallsla that it is secondary to a healing process due to trauma could be disputed as in three of our cases there was no history of trauma. However, our series is very small to comment for a definite theory.
There is no specific treatment of fibrous dysplasia. When it is well localised to supraorbital plate, surgical excision may be indicated. Cosmetic consideration may at times justify surgical therapy.
| Summary|| |
Four cases of fibrous dysplasia involving the orbit are being presented. The lateral orbitotomy was performed in one and the patient was asymptomitic for three years. In the second case the surgery was advised but the patient refused. The third case had an intracranical extension for which she was referred to the neurology for management.
| References|| |
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]
[Table - 1]