Orbital fibrous dysplasia

R Gogi; K Nath; Gopal Krishna

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Year : 1976 | Volume : 24 | Issue : 3 | Page : 18-21

Orbital fibrous dysplasia

R Gogi, K Nath, Gopal Krishna
M. U. Institute of Ophthalmology, Aligarh, India

Correspondence Address:
R Gogi
M. U. Institute of Ophthalmology, Aligarh
India

Source of Support: None, Conflict of Interest: None

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How to cite this article:
Gogi R, Nath K, Krishna G. Orbital fibrous dysplasia. Indian J Ophthalmol 1976;24:18-21

How to cite this URL:
Gogi R, Nath K, Krishna G. Orbital fibrous dysplasia. Indian J Ophthalmol [serial online] 1976 [cited 2023 Nov 28];24:18-21. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?1976/24/3/18/31292

Table 1

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Fibrous dysplasia is an unusual bony growth, the exact etiology of which is not known and generally there are associated extra skeletal abnormalities.[12]

In 1938, Lichtenstein[22] separated this entity from other osteo-dystrophies under the name of "Polyostotic fibrous dysplasia." Lichtenstein and Jaffe[21] recognised that there are monostotic and polyostotic types and named this clinical complex as fibrous dysplasia. Different names have been given by various workers to this condition as shown in [Table - 1]. The polyostotic type may form part of Albright's disease, in which patchy skin pigmentation and precocious puberty are present amongest young females.

It is a rare orbital lesion and its reports in literature are scanty. Nath and Gogi[17] have not recorded any such case amongest 120 primary orbital tumours. However, there are few reports describing this orbital lesion, which are shown in [Table 2].

In the present case, fibrous dysplasia of medial wall and roof of the orbit is being reported.

Case Report

A young man of 25 years, was examined in April, 1974, at M. U. Institute of ophthalmology, Aligarh. He complained of painful gradual protrusion of left eye for the last four years. The pain also radiated to the frontal region and left eyebrow. There was no history of any trauma or any inflammation. Visual acuity was 6/6 in both the eyes. Examination of the left eye showed (7.0 mm), outwards (8.0 mm), and downwards (2.0 mm) proptosis [Figure - 1]. Due to the mechanical pressure of the tumour, the elevation, dextroversion and dextroelevation were restricted. A hard, smooth almond size tumour fixed to the bone was palpable in the upper inner quadrant of the orbit. X-rays of the orbit showed, a radio-opaque shadow at the junction of medial wall and roof of the orbit. The tumour showed irregular areas of increased density with clear cut margins [Figure - 2][Figure - 3]. Venogram showed displacement of superior ophthalmic vein [Figure 4], while the needle biopsy showed poorly formed bone with fibrous tissue proliferation in between. On the basis of these findings a presumptive diagnosis of fibrous dysplasia was made. There was no other general bony deformity and other systems were normal. Right eye was normal.

On 16. 4. 75, lateral cum anterior orbitotomy was done, the growth was resected and chiselled out. Patient was discharged on 6. 5. 75, with restricted !abduction and adduction and 1 mm of proptosis in the forward direction.

On gross examination growth measured 2.5 x 2.0 x 1.5 cm. It was firm with small bony spicules projecting on the surface. It was cut with a gritty sensation. Microscopic examination revealed foci of osseous tissue with fibroblasts in between [Figure - 5]. There was no giant cell reaction or areas of haemorrhages. Thus it was diagnosed as a case of fibrous dysplasia.

On 6. 9. 75, patient was readmitted with the recurrence of the proptosis in the left eye. Vision in this eye had deteriorated to 6/36. There was marked limitation of ocular movements of left eye in dextroversion, dextrodepresion, and direct elevation. The left eye was proptosed forward (14mm) and outwards (22mm). There was an alpable swelling in the upper inner quadrant of the left orbit, which was firm, nodular and posterior limit of the growth could not be defined. Growth was fixed to the adjacent orbital tissues. Fundus showed slight hyperemia with blurring of the disc margins and slight congestion of the veins. X-ray examination confirmed the recurrence of the growth. On 21. 9. 75, the growth was again removed through the same approach taking care that it was removed completely. Gross appearance and histopathology was similar to the previous mass.

Comments

In the present case on clinical grounds a probable diagnosis of osteoma was made. However, histopathological examination of needle biopsy and of the tumour mass established it to be fibrous dysplasia. Orbital roof is the commonest site for fibrous dysplasia.[4],[6],[8],[11],[14],[15],[18],[20],[24]. Rarely it may affect medial wall or floor of the orbit.[19] The present case was involoving both the medial wall and the roof of the orbit. We could trace only one such case from the English literature.

Radiologically fibrous dysplasia may manifest as a simple ballooning of the bone with slight to moderate degree of bony sclerosis[20] or depending upon the dominance of osseous element and thus a considerable degree of "ground glass" appearance or even intense radio-opacity may be present.[26] The present case showed ground glass opacity with well demarcated margin [Figure - 2][Figure - 3].

Gross pathology of the tumour showes expanded bone, and thined out cortex. The distension of the bone is due to the erosion of the original cortical bone by overgrowth of the tissues into the medullary cavity. The lime salts are responsible for the grittiness.[12]

Histopathological examination may show tiny cysts, areas of haemorrhages, giant cell reaction apart from the fibrous tissue and foci of osseous tissue.[1] The proportion of the fibrous and osseous tissue may vary from case to case, but essential tissues tend to show osseous element.[12] Frequently one may find small specules or spherules of bony tissue, osteoid with one or more foci resembling psammoma bodies which may cause difficulty in differentiation from psammomamatous meningiomas[20].

Recurrence in the present case is obviously due to incomplete removal of the growth on the first operation.[12],[32] It is rare feature [5],[12],[28],[32] and X-ray therapy in small doses has been advocated in such cases.[28] In inoperable lesion with recurrence, the use of testosterone has been described although the rationale of this therapy is not clear.[5]

The nature of fibrous dysplasia is still unknown whether truely neoplastic or dysplastic harmartomatous mass is debatable.[2],[25],[27] Lichtenstein[27] suggested that it is apparently the result of perverted activity of specific bone forming mesenchyme, while Valls[29] suggested that it is secondary to a healing process due to trauma, which appears most unlikely in the majority of cases.

Summary

A case of orbital fibrous dysplasia affecting the medial wall and roof of the orbit with the recurrence has been reported. Radiological, gross pathology and microscopic changes have been described. Disputed nomenclature of the lesion has been reviewed.

Acknowledgements

We are thankful to Mr. U. C. Gupta, senior phototechnician and Mr. A. Y. Khan, senior technician of Pathology labs. of M. U. I. O. for their help in preparing this manuscript.

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