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CASE REPORT
Year : 1990  |  Volume : 38  |  Issue : 1  |  Page : 39-41

Orbital reconstruction in fibrous dysplasia-A case report


S.C.B. Medical College & Hospital, Cuttack, India

Correspondence Address:
Madhumati Misra
S.C.B. Medical College & Hospital, Cuttack-753 007
India
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Source of Support: None, Conflict of Interest: None


PMID: 2365440

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  Abstract 

A case of orbitofrontal fibrous dysplasia with optic canal involvement is reported. Surgical excision of the mass with unroofing of the optic canal followed by orbito-cranian reconstruction by methyl methacrylate gave excellent results.


How to cite this article:
Misra M, Mohanty AB, Rath S. Orbital reconstruction in fibrous dysplasia-A case report. Indian J Ophthalmol 1990;38:39-41

How to cite this URL:
Misra M, Mohanty AB, Rath S. Orbital reconstruction in fibrous dysplasia-A case report. Indian J Ophthalmol [serial online] 1990 [cited 2020 Dec 5];38:39-41. Available from: https://www.ijo.in/text.asp?1990/38/1/39/24545



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  Introduction Top


Fibrous dysplasia of bone is a disease process of unknown cause which presents itself in a variety of clinical manifestations. McCune and Albright in mid 1930's described the syndrome of multiple foci of fibrous dysplasia, irregular brown patchy skin pigmentation, and sexual precocity and labeled the disease as'osteitis fibrosa cystica'. In 1938 Lichenstein seperated the desease from other osteodystrophies under the name of 'Polyostotic fibrous dysplasia'. The disorder has been subsequently described under various terminologies [Table - 1].

In the monostatic form of the disease, only one focus is involved and skin/endocrinal manifestations are ab­sent [3],[4]. The skull bone/orbit is usually involved in mon­ostatic fibrous dysplasia. We report a case of fibrous dysplasia who developed visual loss due to encroach­ment of the mass on the optic canal [4],[5],[6].sub Optic canal de­compression followed by methyl methacrylate orbito­cranioplasty saved vision and restored physical appear­ance.


  Case report Top


A 40 year old man noted a painless bulge over the left eve brow since over 2 years which slowly increased in size. Examination revealed a diffuse 3cm x 3cm bony swelling over the left frontal area. The left eye was displaced downwards with mechanical limitation of ele­vation [Figure - 1]. Vision in the left eye was reduced to 6/24 and the left fundus appeared congested. The right eye was clinically normal.

X-ray revealed a hyperdense mass widening them left frontal bone and orbital roof. CT scan showed a hyper­dense frontal mass encroaching into the orbital cavity from above, producing down tilt proptosis [Figure - 2]. Wors­ening of proptosis, papilloedema and visual failure upto 3/60 was noted within 3 months. Excision of the mass lesion followed by orbital reconstruction was consid­ered.

A classical left frontal craniotomy skin incision was made. A hard, vascular mass was resected and chiselled out from the left frontal skull/orbital roof and unroofing of the left optic canal was done. A 3mm ledge was drilled around the cranial defect with a high speed burr to allow the edgeof the acrylic plate to be recessed and firmly fixed. Four drill holes were placed through the skull around the circumference of the defect. A 5mm. thick methyl methacrylate mould was made over the defect and allowed to solidify. Four drill holes were made on the plate opposite the holes in the skull and the plate was secured in position with 28-gauge stainless steel wire. The overlying scalp was stitched in layers. Histopathological examination revealed foci of osseous tissue with dense fibroblastic proliferation compatible with fibrous dysplasia of the bone. The postoperative orbital CT Scan showed a near normal orbital cavity [Figure - 3]. The patient was discharged after 15 days with the left eye vision improved to 6/12. He was asympto­matic till the last review [Figure - 4].


  Discussion Top


Fibrous dysplasia of the bone is a disease of unknown aetiology and without familial incidence or hereditary tendency. Although of congenital origin the disorder becomes apparent around the age of 10 years [3],[7],[8]. The disease rarely presents above fourty years of age as en­countered in the present case. This disorder has been described under various terminologies [Table - 1]. In fi­brous dysplasia, substantia gilationsa and bone marrow of medullary cavity is replaced by fibrous tissue, islands of poorly calcified bone and hyaline cartilage [4],[5],[8]. As a consequence, there is a widening of the affected bone, thinning of its cortex and replacement of marrow by solid fibrous tissue [3],[4],[5],[6],[7],[8],[12],[13],[14]. Histological examination may show areas of haemorrhage, tiny cysts, giant cell reac­tion apart from fibrous tissue and foci of osseous tissue resembling psammomatous meningioma [4].

The affected bone on radiology appear balooned and sclerosed with thinned out cortex [3],[4],[5],[6],[7],[8],[12],[13],[15] CT examina­tion shows a very dense, expanded mass isodense with the cortical bone [Figure - 2]. The typical ocular involvement is that of unilateral exophthalmos which progresses in a downward fashion as seen in the present case. Optic/ visual failure indicate encorachment on the optic canal and warrants urgent unroofing of the optic-canal [4],[5],[6].In the present case, intracranial decompression of the optic nerve was also done for failing vision.

The tumour usually recurs after surgery if wide excision of the neighbouring bone is not done [4]. Such excision results in a large cranial defect. Any defect larger than 2 to 3 cm over the cerebral convexity should be repaired for restoration of cerebral protection, physical appear­ance and intracranial pressure relationships [6]. If such defects are not repaired, ventricular migration towards the defect, displacement of brain structures, hemispheric collapse, cerebrospinal fluid hydrodynamic changes may occur in such patients [6]. The' syndrome of the trephined' consisting of headaches, dizziness, intolerance to noise and vibration, irritability and fatigability due to the direct effect of atmospheric pressure on the brain may occurs.

Various materials can be used for cranioplasty such as autogenic bone (rib, ilium), metals and acrylic resins. Among them, acrylic resins provide favourable proper­ties including strength, inertness, availability of thermal nonconductivity and ease of applications. No report of carcinoma or sarcoma has followed the use of acrylic plastic in humans [6].

The neuro-ophthalmological complications of untreated fibrous dysplasia have been systematized by Liakos, Walker and Carruta (1979). Primary (Depending on the site of involvement)

1. Frontal bone-proptosis and swelling.

2.Skull base-extraocular muscle palsy.

3.Optic canal-visual loss, optic atrophy.

4.Body of sphenoid-chiasmal compression.

5.Maxilla-epiphora.

Secondary

1. Malignant transformation, sarcomatous charge.

2.Mucocele, by obstruction of ostia of sinuses.

3.Ossifying fibroma.

 
  References Top

1.
Mc. Cune. Albright quoted Sergent RA. In paediatric opthalmology. Ed. Harley RD. B. saunders company. London. P. 744. 1975.  Back to cited text no. 1
    
2.
Lichenstein L-Arch Surg. 36.814.1938.  Back to cited text no. 2
    
3.
Gogi R. Nath K, Krishna G-Ind. J. Ophthal. 21,18,1976.  Back to cited text no. 3
    
4.
Housespian EM, Trokel SL, Jakobiec FO, Hilal SK, Tumours of the Eye In youmans neurosurgery. Ed. Youmans Jr. Vol.5, Ed. 2, W.B. Saunders company, Londan P. 3056, 1982,  Back to cited text no. 4
    
5.
Liakos Gm. Walker CB, Carruta JAS- Br. J. Ophthal. 63, 611,1979.  Back to cited text no. 5
    
6.
Prolo DJ-Cranial defects and cranioplasty. In neurosurgery Vol.2, Ed. Wilkins RH, Rengachary SS., McGraw Hill book company, London. P. 1647-53, 1985.  Back to cited text no. 6
    
7.
Harpman JA- J laryng. 74, 497,1960.  Back to cited text no. 7
    
8.
Nath K, Gogi R- Ind. J. Ophthal, 24, 1, 1976.  Back to cited text no. 8
    
9.
Goldhamer S- Fortschir, Rontgenato, 49,456,1934.  Back to cited text no. 9
    
10.
Falconer MA, Cope CL-Ouart J. Med. N.S. 11, 21, 1942.   Back to cited text no. 10
    
11.
Webb. JRD. - Proc. Roy SOC. Med. 42, 557. 1948.   Back to cited text no. 11
    
12.
King RB., Hayes GJ- Arch Ophthal, 46,553, 1951.  Back to cited text no. 12
    
13.
Hobbs He- 1955, "Xvii concillium ophthalmology, 1954, Canda U.S.A., Acta, 13, 1956.  Back to cited text no. 13
    
14.
Dandy WE-Arch. Ophthal., 25, 191, 1941.  Back to cited text no. 14
    
15.
De Jean J- Bull. Soc. Ophthal. France. 6, 517, 1959.  Back to cited text no. 15
    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]
 
 
    Tables

  [Table - 1]



 

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