FIBRO-OSSEOUS LESIONS OF THE MAXILLO-ETHMOID COMPLEX WITH ORBITAL INVOLVEMENT

D. Jha j, S. B a h a d u r 2, A. T h a k a r 3, A . C h a v a 4, A B S T R A C T : Fibro-osseous lesions involving the maxillo.ethmoid complex are rare. Extensive
lesions leading to proptosis are rarer still. We report here 22 cases of fibro.osseous lesions with orbital involvement. CT scan was the mainstay of radiological investigation. Transfucial excision was done in all cases, except three which necessitated a cranio.focial resection. Surgery resulted in significant reduction of proptosis and improved cosmesis in all cases. In three instances, a sliver of the tumor tissue was left behind due to its proximity to the dura or optic nerve. Two of these eases however went on to develop subsequent visual loss secondary to disease progression. Six cases neeessi~ated revision surgery for residual or recurrent lesion. Dise~se extension to the proximity of vital structures should be excised as continuing tumor growth may prove unsafe.

INTRODUCTION
The term f i b r e - o s s e o u s lesion e n c o m p a s s e s fibrous dysplasia, ossifying fibroma and cementifying fibroma. Fibrous dysplasia has been described as a congenital, metabolic, non-familial disturbance tha! produces 2.5</, of all bony tumors and over 7(A of all non-malignant tumors of the bone (Edgerton et al, 1985). Cemenlitying fibromas and ossifying fibromas are variants of the same disease arising in the periodontal ligamem (Hamner et al, 1968: Waldron, i 0 7 3 : C o h e n et al, 1982). They' are commonly found in Ihe .laY, bones. Orbital involvement is c o m m o n with proptosis, diplopia and loss of visual acuity. (Commins et al. 1998). The present paper retrospectively reviews twent>-two cases of fibre-osseous lesions which presented with orbital involvement. Ap, attempt i> made to a~sess the role of surgery in improvin.~ cosnlesis, reducing proptosi, and preventing visual los,,.

years with a mean of two years (Figl). In all cases, the protrusion of the eye ball was lateral and outwards. Four cases had an additional deviation upwards. Five cases in the study group had visual loss. However, none of the cases had chemosis. Epiphora was a problem in three patients. Besides a clinical work up to delineate the extent of lesion, general exainination was done to see for any evidence of polyostotic fibrous dysplasia or Albright's syndrome. The serum calcium, phosphorus and alkaline phosphatase levels were normal in all instances. Radiological assessment of the extent of lesion was obtained by a contrast enhanced CT scan. The maxillary and ethmoid bone were universally involved. Two cases had an associated secondary fronto ethmoid mucocele in addition to the primary lesion.

PATIENTS AND METHODS

Twenty-two cases of fibre-osseous lesions of the maxilloethmoid complex presenting with orbital inw~l\,cment between 1987-1997 constituted the study group. The demographic profile is described in Table 1. The presenting complaints are listed in Table 2. The majority of the patients were in the second decade. The duration of proptosis ranged from one month lo ten

Fig. 1 :

Massive fibrous dysplasia producing gross proptosis.

~Senior Research k:,,oc,,uc Pr~,fc~,,,or. Assi-,tanl Profcsso~. Senior Resident, Department of Otolarynuoloek. Head and Neck Surgery, All India Institute of 'vlcdic:~i %cicnccs IMIMSI. N\~ Delhi

226
"Fable

Fibro-Osseous Lesions of the Maxillo-Ethmoid

Complex with Orbital Involvement

I : A g e and sex D i s t r i b u t i o n T o t a l n u m b e r ,- Cases; 22

of

Table II : Clinical

Features

Fibrous Ossifying Cementifying fibroma Dysplasia Fibroma (4) (17) (1) Sex M:F Age 0-10
11-20

Total (22) 13:9 " Nil

15

1. 2. 3.

10:7 Nil 13 4 Nil

O:l Nil
1

3:1 Nil
l

4. 5. 6.

Proptosis Nasal obstruction Visual Loss Total (PL negative) Partial(6/24) Cheek swelling Telecanthus Palatal bulge

22 9 4 1 3 2 1

21-30 >30

Nil Nil

2
1

6
1

~GE.RY , ~ery was undertaken for (a) a significant cosmetic , ::gurem.ent (proptosis a n d / o r facial asymmetry) or (b) :~aml~st or hnpending functional impairment (visual loss). Un0%general anaesthesia ,trans-facial surgery ( lateral rhinptq~y and fronto-ethmoidectomy) was carried out in all cases except one ,who underwent a craniofacial resection in addition(Fig. II). Even though the margins of the lesio, were sometimes not clearly delineated, the sqrg~al attempt was to excise all grossly evident tumor. In three instances however, some residual tumor was deliberately left behind due to its proximity to the dura and optic nerve. Two of these three necessitated subsequent cranio-facial resection at an interval of thirteen and fifteen months respectively from the initial surgery. The approach in the craniofacial resection remained extradural(Fig.III). The dura however had to breached at the olfactory nerves and this was repaired with a pericranial galeal flap. Though the vision had been initially preserved in these patients, both of these subsequently became completely blind as a result of continuing tumor growth. The CT scan of the one patient with partial visual loss (6/24) demonstrated the tumor in close proximity to the optic nerve. This was the only patient where a craniofacial approach was taken at the first surgery itself. In addition,

Fig. III : Same case as (1), one week postoperatively showing initial reduction of proptosis. Proptosis continued to improve till about six months postoperatively.

a trans-sphenoethmoidal optic nerve decompression was also undertaken. No deterioration or improvement of vision was noted after surgery. The extent of improvement in proptosis was assessed subjectively. Post-operatively, a marked improvement in proptosis was noted in all cases. However, complete normal appearance was not achieved. In no case did surgery result in either improvement or deterioration of vision. Most of this improvement was apparent within two months of the surgery, though some improvement continued till six months. Six cases (27%) necessitated a revision surgery for residual or extensive recurrent pathology producing cosmetic deformity and significant proptosis. Two were treated by the cranio-facial approach and four by the transfacial approach. The interval between the first operation and the second one ranged from five to seven years. In all three patients in whom a craniofacial resection was undertaken, a prophylactic pericranial galeal flap was used at surgery for repair and augmentation of the basal dura.

Fig. II :

Tumor exposed in the course of cranio-facial resection.

Indian Journal of Otolaryngology and Head and Neck Surge~ Vol. 53 No. 3, July - September 2001

Fibre, O~se,m.~ Lcsiotls ~!{ the Maxillo-EthmoM

Complex with Orhital hlvolvement

227

Continuing follow-up of these cases has ranged from five months to nine years with a mean of 2.6 years.

DISCUSSION The differentiation between fibrous dysplasia, ossifying fibroma and cementifying fibroma is not always clearcut. Such a differentiation depends on clinical, radiological Surgical t h e r a p y f o r m s the m a i n s t a y of treatmeJlt. and histological features taken in unison. Radiotherapy should be avoided since it is both ineffective Fibrous dysplasia may be monostotic !7(Y7, ) or polyostotic and appears to increase the incidence of malignant (30%). The polyostotic form associated with endocrine degeneration (Edgerton et al, 1985). Indications for surgery disturbances (Albright's syndrome) represents onl~ 3~7c include correction of the cosmetic deformity and the threat of all reported c a s e s O s s i f y i n g f i b r o m a is atwavs to vision secondary to encroachment of the optic canal. monostotic. It is commoner in women qWaldron. 1073). The decision regarding the timing and extent of surgery, Fibrous dysplasia in contrast occurs equally in both sexes. can h o w e v e r be difficuit. Proponenl~ ot minima It also presents at an earlier age than ossit}'ing fibnmm. conservative surgery cite the generally slow pr%~ress of the disease and the belief that these lesions ceas? Plain films and computed tomography aid m differentiating progress or regress by the end of the second d e c between fibrous dysplasia and ossit:ving fibroma i Shields (Edgerton et al 1985). Others {Lund and Harrison i98~,. et al, 1983). Ossifying fib!omas characteristically have aelt however tavour a more radical approach and fa',o:'r defined margins and are round or ovoid, monostotic lesions complete excision of the lesmn on the basis tea t ~.'.fis producing expansion of the inw)lved bone. The cortex minimises subsequent involvement of the optic nerve by of the bone is frequently thinned to an egg-shell rim which residual tumor. Surgery in the present study wa, carried is usually intact. The matrix of the lesion is fairly out to achieve reduction in proptosis, thereby improving homogenous and of a ground glass quality. In some the cosmetic appearance and to prevent deterioratioh i:" instances, however solid or patchy sclerosis may be vision. present in the matrix of the lesion. In the,e cases, it may be confused with fibrous dysplasia (Schwarz, 1064). Visual loss results from involvement of the sphenoid bone Fibrous dysplasia of the skull is usually sclerotic and and compression of the nerve in its bony canal. Bilateral expands the bone throughout its length rather than in a involvement has been described (Calderon and Brady Iocalised fashion. Typicall?, its borders are poorl~ defined and merge with normal bone. in contrast to the sharply marginated borders of ossifying fibroma.(Fig IV-VII.
Histopathologically, ossifying fibroma is composed of fibrous stroma rich in fibroblasts with rather flat nuclei and ill defined cytoplasm. Isolated spicules of lamellar bone are scattered throughout the connective tissue stroma.
Fig. V : Coronal CT scan showing intracranial extension of fibrous dysplasia.

Many of the spicules are surrounded by a delicate rim of osteoid, r i m m e d in turn by o s t e o b l a s t s I f"u and Perzin, 19741. The appearance differs from immature w o v e n bone, usually without o s t e o b l a s t s ,xl~icn characterises fibrous dysplasia.

Fig. IV : Fibrous dysplasia impinging on the optic nerve causing blindness (axial CT scant.

Fig. VI:

Cementifying fibroma of the maxilla (axial CT).

Indian Journal cq' Otolarvngology and Head and Neck Surgery Vol. 53 No. 3, July - September 2001

228

Fibro-Osseous Lesions of the Maxillo-Ethmoid

Complex with Orbital bn'olvement

1969). In such cases, surgical decompression of the optic canal has been reported to preserve vision (Donso et al 1982). Sassin and Rosenberg (1968) have reported success in preventing further visual loss by surgically u~roofing the optic canal. Visual loss once manifest is u21ikely to be reversed. In two of our patients wherein ~ile tumor was very close to the optic nerve, residual tumor tissue was deliberately left behind on the premise that surgical removal had the potential of causing functional loss more damaging than the tumor itself. Both these cases however manifested continued tumor growth and became subsequently blind. In retrospect therefore, it is apparent ha! ~he patients would have been better off had we attempted complete tumor removal at the first operation. The one patient who had an optic nerve decompression did not have any visual loss or improvement because of the procedure, it h o w e v e r did prevent any further progression of the visual loss. Cessation of tumor growth may occur alter puberty. This factor however should not be relied upon in planning the surgical management. Craniofacial resection was carried out in two cases with recurrent tumor and in one case with intracranial extension. This afforded a wide exposure and satisfactory excision of the lesion. There is an increasing awareness in recent years that in certain extensive fibro-osseous lesions particularly those extending intracranially, craniofacial resection may be a justifiable procedure, both in terms of achieving greater reduction in proptosis as well as preserving vision (Lund et al 1988, Catalano et al 1994). CONCLUSION In this series, satisfactory results were obtained in terms of reduction of proptosis, cosmesis and vision preservation. Complete reduction of proptosis however may not be achieved. Surgical excision should aim at complete excision of all visible tumor. The aim should be to pick up early cases before vital functions are compromised. Close proximity of the tumor to the optic nerve warrants aggressive surgery. Early intervention holds the key to good patient management. REFERENCES
I. Calderon, M., Brady, H.R. (1969) : Fibrous dysplasia of bone with bilateral optic foramina involvement. American Journal of Ophthalmology 68(3):573.-515 Catalano,P.J., .Hecht,C.S.,Biller, H.E,Lawson,W., Post, K.D., Sachdev, V., Sen, C h a n d r a n a t h . , Urken, M.L. (1994).

Craniofacial resection. An analysis of 73 cases. Archives of Otolaryngology, Head and Neck Surgery. 120: 1203-1207. 3. Cohen, H.C., Macpherson, T.A., Barnes, L., Kennerdell, JOS (1982) Cemento-ossifying fibroma of the ethmoidal sinus manifesting as proptosis. Annals of Ophthalmology 14(2): 173175. Commins, D.J., Tolley, N.S., Milford, C.A. (1998). Fibrous dysplasia and ossifying fibroma of the paranasal sinuses. Journal of Laryngology and Otology.112:964-968. Donso, L.A., Magargal, L.E., Eiferman, R.A. (1982) : Fibrous dysplasia of the orbit with optic nerve decompression. Annals of Ophthalmology 14:80-83. Edgerton, M.T., Persing, J.A., Jane, A.J. (1985) : The Surgical treatment of fibrous dysplasia. Annals of Surgery 202(4):459479. Fu, Y.S., Perzin, K.H. (1974) : Non-epithelial tumors of the nasal cavity, paranasal sinuses and nasopharynx. A clinicopathologic Study II. Osseous and Fibrosseous lesions, including osteoma fibrous dysplasia, ossifying fibroma, osteoblastoma, giant cell tumor and osteosarcoma. Cancer 33(5):1289-1305. Hamner, J.E. lIl., Scofield, H.H., Cornyn, J (1968) : Benign fibrosseous jaw lesions of periodontal membrane origin. Cancer 22:861-87 Lund,V.J., Harrison,D..F.N.[1988]Craniofacial resection tbr tumors of the nasal cavity and paranasal sinuses. The American Journal of Surgery. 156: 187-190.

4.

5.

6.

7.

8.

9.

10. Lund, V.J., Howard, D.J., Wei, W.I., Cheesman, A.D. (1998). Cranio-facial resection for tumors of the nasal cavity and paranasal sinuses - a 17 year experience. Head and Neck 20 (2): 97-105.
1 1 . . S a s s i n , J . E , Rosenberg,R.N.(1968). Neurological complications

of fibrous dysplasia of the skull. Archives of Neurology. 18;363369 12. Schwarz, E. (1964). Ossifying fibroma of the face and skull. American Journal of Roentgenol Radium ther Nuclear Medicine. 91(5):1012-1015. 13. Shields, J., Nelson, L., Brown, J., Dolinskas, C. (1983) : Clinical, computed tomographic and histopathological characteristics of Juvenile ossifying fibroma with orbital involvemefit. American Journal of Ophthalmology 96(5):650-653 14. Waldron, C.A.(1973): Benign fibro-osseous lesions of the jaws. A clinical, radiologic, histologic review of 65 cases. Part ll. Benign fibro-osseous lesions of periodontal ligament origin. Oral surgery, Oral medicine, Oral pathology 35(3):340-350.

Address for Correspondence :

Prof. Sudhir Bahadur Department of ENT, AIIMS New Delhi - I l0 029.

2.

Indian Journal c~f Otolacvngology and Head and Neck Surgery' Vol. 53 No. 3. July - September 2001