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 Table of Contents  
Year : 2018  |  Volume : 30  |  Issue : 3  |  Page : 228-229

Monostotic fibrous dysplasia of the orbital plate of ethmoid: A rare cause of retro-orbital pain

Department of Radiology, Malabar Medical College, Kozhikode, Kerala, India

Date of Web Publication17-Dec-2018

Correspondence Address:
Reddy Ravikanth
Department of Radiology, Malabar Medical College, Kozhikode, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/kjo.kjo_78_18

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How to cite this article:
Ravikanth R. Monostotic fibrous dysplasia of the orbital plate of ethmoid: A rare cause of retro-orbital pain. Kerala J Ophthalmol 2018;30:228-9

How to cite this URL:
Ravikanth R. Monostotic fibrous dysplasia of the orbital plate of ethmoid: A rare cause of retro-orbital pain. Kerala J Ophthalmol [serial online] 2018 [cited 2022 Oct 7];30:228-9. Available from: http://www.kjophthal.com/text.asp?2018/30/3/228/247593

Dear Sir,

A 20-year-old female patient presented to the Department of Ophthalmology due to gradually progressive left-sided retro-orbital pain associated with headache for 6 months. There was no facial asymmetry and ophthalmological examination revealed no diplopia or proptosis or dimness of vision [Figure 1]. Visual acuity was 6/6 in both eyes. A normal visual field measuring 90° temporally, 50° superiorly and nasally, and 60° inferiorly was noted in both eyes. Fundus examination showed no abnormalities in either eye. Pupillary response to direct and consensual light was normal in both eyes. There were no findings after the anterior rhinoscopic examination performed on the patient. During the endoscopic nasal examination, a nontender mass with a hard consistence and a smooth surface medializing the left middle concha was noted. On the paranasal computed tomography scan, a lobular mass-sized approximately 2.4 cm × 1.6 cm × 2.4 cm (AP × ML × CC) with the same density as the bone tissue was observed which covered the left orbital plate of ethmoid and ethmoid air cells, extending medially to the septum and laterally to the lamina papyracea and was characterized by ground-glass appearance [Figure 2]. There was obliteration of the left ethmoid sinuses with bulging into the left orbit producing some proptosis and local deformity of the eyeball and deviating the left optic nerve. The patient underwent left endoscopic sinus surgery and mass removed with the aid of curettage and drilling. During the histopathological examination of the material, it was observed that an irregular osseous trabeculae, which had no osteoblastic activity, dispersed inside the fibrous stroma [Figure 3]. The patient is clinically asymptomatic, is under follow-up every 6 months.
Figure 1: Clinical image of a 20-year-old female patient showing no obvious facial asymmetry or proptosis

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Figure 2: Axial computed tomography image in bone window demonstrating ground-glass appearance of monostotic fibrous dysplasia of the left ethmoidal plate and sinus (circle) bulging into the left orbit producing some proptosis and compression of the optic nerve

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Figure 3: Low-power view demonstrating osteoid trabeculae that are narrow and irregularly-shaped arranged haphazardly in a background of the dense fibroblastic stroma. The bony trabeculae are immature, inadequately mineralized and lack osteoblastic rimming (H and E, ×100)

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Fibrous dysplasia is a fibro-osseous lesion that is benign in nature, and the involvement of ethmoid bone is rarely reported.[1] Monostotic fibrous dysplasia of the ethmoid sinus is one of the rarest lesions. It was originally described in 1938 by Lichtenstein.[2] Monostotic and polyostotic forms are defined, depending on whether one or more bones are affected, which are noted in 30% and 70% of patients, respectively.[3] The most common location for monostotic form of fibrous dysplasia are ribs, proximal femur, and craniofacial bones. Among the craniofacial localizations of fibrous dysplasia, the maxillozygomatic complex is most frequent affected. Ethmoid, sphenoid, temporal and frontal bones are rarely involved. Malignant transformation is rare (0.5%) and is usually seen only in polyostotic cases.[4] The diagnosis of fibrous dysplasia is made on a combination of clinical, radiological, and pathological criteria.[5] Radiologically, fibrous dysplasia presents as a fine, granular, homogenous, appearance, likened to that of a ground glass. Open surgical technique and endoscopic removal – both are commonly employed to remove the lesion.[6] Fibrous dysplasia is of particular interest to the ophthalmologist because it can affect the craniofacial bones either as a monostotic fibrous dysplasia or as a part of polyostotic fibrous dysplasia which is sometimes associated with skin pigmentation and endocrine disorders and is known as Albright's syndrome. When the craniofacial bones are affected, encroachment on the paranasal sinuses, orbit, and foramina of the skull may lead to visual loss, proptosis, diplopia, and epiphora. In cases of fibrous dysplasia affecting the paranasal sinuses, the decrease of visual acuity is progressive, produced by bone proliferation and compression of the optic nerve. Differential diagnosis of fibrous dysplasia of ethmoid sinus should be considered in patients presenting before the age 30 with chronic retro-orbital pain.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Feldman MD, Rao VM, Lowry LD, Kelly M. Fibrous dysplasia of the paranasal sinuses. Otolaryngol Head Neck Surg 1986;95:222-5.  Back to cited text no. 1
Katz BJ, Nerad JA. Ophthalmic manifestations of fibrous dysplasia: A disease of children and adults. Ophthalmology 1998;105:2207-15.  Back to cited text no. 2
Kransdorf MJ, Moser RP Jr., Gilkey FW. Fibrous dysplasia. Radiographics 1990;10:519-37.  Back to cited text no. 3
Osguthorpe JD, Gudeman SK. Orbital complications of fibrous dysplasia. Otolaryngol Head Neck Surg 1987;97:403-5.  Back to cited text no. 4
Bland LI, Marchese MJ, McDonald JV. Acute monocular blindness secondary to fibrous dysplasia of the skull: A case report. Ann Ophthalmol 1992;24:263-6.  Back to cited text no. 5
Van Rompaey D, Schmelzer B, Verstraete W, Cammaert T. Fibrous dysplasia in the frontoethmoidal complex: Diagnosis and surgical aspects. Acta Otorhinolaryngol Belg 1994;48:37-40.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3]


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