Purpose: The aim of the present study was to describe a case oforbital fibrous dysplasia in a very young child.Case report: A 5-year-old girl presented with unilateral, painless,slowly progressive proptosis of the left eye and inferotemporalglobe displacement from about 1 year ago. The visual acuity inboth eyes was 20/20. The general physical examination was normal.Computed tomography (CT scan) revealed thickening and deformityof the orbital walls. Lateral orbitotomy and biopsy were performedand were compatible with fibrous dysplasia.Conclusion:   Craniofacial fibrous dysplasia usually occurs in thesecond decade of life, but it can also rarely present in the earlyyears of life. Therefore, fibrous dysplasia should be considered asa differential diagnosis of proptosis or globe displacement in theearly years of life.Keywords: Fibrous; Dysplasia; Orbit; Child; Iran

Sadeghi Tari, Ali

Torabi, Hamidreza

English

Journals Portal, Shahid Beheshti University of Medical Sciences

40 Journal of Ophthalmic and Optometric Sciences.Volume 1, Number 2, Winter 2017.Case ReportFibrous Dysplasia of the Orbit in a 5-Year-Old GirlHamidreza Torabi *1, MD; Ali Sadeghi Tari 2, MD1. Ophthalmology Department, Baqiyatallah University of Medical Sciences, Tehran, Iran.2. Ophthalmology Department, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran.*Corresponding Author: Hamidreza TorabiE-mail: dr_hamidrezatorabi@yahoo.comHow to cite this article: Torabi H, Sadeghi Tari A. Fibrous Dysplasia of the Orbit in a 5-Year-Old Girl: A Case Report. Journal of ophthalmologic and Optometric Sciences. 2017;1(2): 40-4.Article Notes:Received: Oct. 8, 2016Received in revised form: Oct. 29, 2016Accepted: Nov. 21, 2016Available Online: Jan. 30, 2017Keywords:FibrousDysplasiaOrbitChildIranAbstractPurpose: The aim of the present study was to describe a case of orbital fibrous dysplasia in a very young child.Case report: A 5-year-old girl presented with unilateral, painless, slowly progressive proptosis of the left eye and inferotemporal globe displacement from about 1 year ago. The visual acuity in both eyes was 20/20. The general physical examination was normal. Computed tomography (CT scan) revealed thickening and deformity of the orbital walls. Lateral orbitotomy and biopsy were performed and were compatible with fibrous dysplasia.Conclusion: Craniofacial fibrous dysplasia usually occurs in the second decade of life, but it can also rarely present in the early years of life. Therefore, fibrous dysplasia should be considered as a differential diagnosis of proptosis or globe displacement in the early years of life.Fibrous Dysplasia of the OrbitTorabi et al.41Journal of Ophthalmic and Optometric Sciences.Volume 1, Number 2, Winter 2017.IntroductionFibrous dysplasia (FD) is a benign slowly pro-gressive disorder of bone, where normal can-cellous bone is replaced by fibrous tissue and immature woven bone. This entity constitutes about 2.5 % of all bone tumors 1.Two clinical forms of FD are recognized: polyostotic and monostotic variants. Polyostotic lesions com-bined with cutaneous pigmentation, precocious puberty and endocrine dysfunction is called Albright’s syndrome 2. FD has a predilection to involve membranous bones such as maxil-la, mandible, frontal and parietal bones. When FD occurs in the craniofacial region, orbits are commonly involved 3. Patients with orbital FD usually present with classical manifestations of proptosis, diplopia and periorbital facial asym-metry 4. Other ocular complications have been reported including exposure keratitis, displace-ment of the globe and refractive errors second-ary to compression and distortion of globe. If the sphenoid bone is involved, the reduced di-ameter of the optic canal can lead to optic nerve damage and visual loss 5. In this manuscript we report a 5-year-old child with orbital fibrous dysplasia.Case reportThe present report was approved by the ethics committee of Baqiyatallah University of Med-ical Sciences and informed consent was given by patient’s parents for reporting this case. A 5-year-old girl presented with unilateral, painless, gradually progressive proptosis of the left eye from about 1 year ago. The visual acuity of both eyes was 20/20.The globe was displaced inferotem-porally, but there was no ocular motility restriction or diplopia (Figure 1).The general physical exam-ination was normal.Computed tomography (CT-Scan) of the or-bit revealed deformity of the orbital walls and thickening of the large wing of the sphenoid, squamous part of the temporal bone, zygo-matic arc and frontal bone. The left optic canal and optic nerve were normal in configuration. Paranasal sinuses had normal configuration and pneumatization (Figure 2).Lateral orbitotomy and biopsy were performed. The surgical specimen was sent for routine his-topathological examination. The postoperative period was uneventful.Grossly, the specimens consisted of multiple irregular creamy-gray tissue with bony consist-ency. Histologically, the lesion was composed of fragments of trabecular bone with reactive change, beside small pieces of neoplastic tissue composed of woven and trabeculae bone in fi-brous stroma. These histopathological findings were compatible with fibrous dysplasia.DiscussionVon Recklinghausen described a number of cases whose normal bones were replaced by immature bone and fibrous tissue in 1891, and the term fibrous dysplasia was first used in 1938 by Li-chtenstein 5. FD is a benign fibro-osseous lesion of unknown etiology involving skeletal bone and can occur as monostotic or polyostotic forms. Monostotic FD is about 4 times more frequentthan its polyostotic form 6.  Craniofacial bones  Figure1: Left eye proptosis and inferotemporal displacement of the globe, before biopsy (Left) and after biopsy (Right).Fibrous Dysplasia of the Orbit Torabi et al.42 Journal of Ophthalmic and Optometric Sciences.Volume 1, Number 2, Winter 2017.Figure 2: Deformity and thickening of the orbital walls and inferotemporal displacement of the globe (Coronal view) and mild proptosis (Axial view). may be involved in 20 % of monostotic and 40 - 60 % of polyostotic cases 7. FD generally occurs during the second decade of life and lesions may remain static in growth after puberty 8,9. In this study we reported an unusual case of FD in a 5 year-old-girl. Fadle et al., reported 22 cases with orbitocranial FD and the youngest patient in their study was 17-year-old (mean age was 29.5) 10. Leong et al., reported a 12 year-old-girl with craniofacial FD with orbital involvement, who developed progressive visual impairment 11.Visual manifestations are common in craniofa-cial FD and include proptosis, which is present in about 30 % of cases, globe displacement and strabismus, diplopia, extraocular muscle palsies, epiphora and visual loss due to optic nerve compression 5,6. The presenting symptoms in our case were mild proptosis and globe displace-ment that began in the 4th year of life. The radiological appearance of FD may range from very radiolucent lesions composed of mainly fibrous tissue to markedly sclerotic ex-panded bone made up of dense bony trabeculae. Any degree of appearance between these two extremes may be present 3.Treatment of FD includes surgical and non-surgical options, but the results of nonsurgicalFibrous Dysplasia of the OrbitTorabi et al.43Journal of Ophthalmic and Optometric Sciences.Volume 1, Number 2, Winter 2017.options are poor. Indications for surgery are both cosmetic and functional 6. Complete re-section of the involved area is the treatment of choice, but this invasive approach may contribute to functional impairments and sec-ondary deformities that are more damaging for the patient than the FD itself 12. There is lit-tle risk of malignant degeneration, with trans-formation of dysplastic bone to osteogenic sarcoma, fibrosarcoma and chondrosarcoma 5. The incidence of malignant transforma-tion in normal bone is 0.001 % but in FD it is 400 times greater 5. Radiotherapy may in-crease the risk of malignant transformation 2.ConclusionCraniofacial fibrous dysplasia usually occurs in the second decade of life, but it can also rarely present in the early years of life. There-fore, fibrous dysplasia should be considered as a differential diagnosis of proptosis or globe displacement in the early years of life.Fibrous Dysplasia of the Orbit Torabi et al.44 Journal of Ophthalmic and Optometric Sciences.Volume 1, Number 2, Winter 2017.References1. Feiring W, Feiring EH, Davidoff LM. Fibrous dys-plasia of the skull. J Neurosurg. 1951;8(4):377-97. 2. Bibby K, McFadzean R. Fibrous dysplasia of the orbit. Br J Ophthalmol. 1994;78(4):266-70.3. Joseph E, Kachhara R, Bhattacharya RN, Radhakr-ishnan VV, Balachandran K. Fibrous dysplasia of the orbit in an infant. Pediatr Neurosurg. 2000;32(4):205-8.4. Cruz AA, Constanzi M, de Castro FA, dos Santos AC. Apical involvement with fibrous dysplasia: im-plications for vision. Ophthal Plast Reconstr Surg. 2007;23(6):450-4.5. Goisis M, Biglioli F, Guareschi M, Frigerio A, Mortini P. Fibrous dysplasia of the orbital region: current clinical perspectives in ophthalmology and cranio-maxillofacial surgery. Ophthal Plast Reconstr Surg. 2006;22(5):383-7.6. Traibi A, El Oueriachi F, El Hammoumi M, Al Bouzidi A, Kabiri EH. Monostotic fibrous dysplasia of the ribs. Interactive Cardiovascular and Thoracic Surgery. 2012;14(1):41-3. 7. Demner LP. Fibro-osseous lesions of bone; in Ack-erman LU, Spjut HJ, Abell MR (eds): Bone and Joints, International Academy of Pathology, Monograph No 17. Baltimore, Williams & Wilkins, 1976,209-35.8. Hempel JM, Karkos PD, Issing WJ. Fibrous dysplasia of the frontal bone. Ear Nose Throat J. 2006;85(10):654, 656-7.9. Osguthorpe JD, Gudeman SK. Orbital complica-tions of fibrous dysplasia. Otolaryngol Head Neck Surg. 1987;97(4):403-5.10. Fadle KN, Hassanein AG, Kasim AK. Orbi-tocranial Fibrous Dysplasia: Outcome of Radical Resection and Immediate ReconstructionWith Tita-nium Mesh and Pericranial Flap. J Craniofac Surg. 2016;27(8):e719-e723. 11. Leong LT, Ming BJ. Craniofacial Fibrous Dys-plasia Involving the Orbit: A Case Report and Lit-erature Review. Asia Pac J Ophthalmol (Phila). 2015;4(3):151-4.12. Jaffe HL. Fibrous dysplasia. In: Tumors and tu-morous conditions of the bones and joints. Philadel-phia: Lea & Febiger; 1958:117-42.Footnotes and Financial DisclosuresConflict of Interest:The authors declare no conflict of interest with the subject matter of the present  manuscript. 

Fibrous Dysplasia of the Orbit in a 5-Year-Old Girl

https://journals.sbmu.ac.ir/basir/article/download/18489/13777

Fibrous Dysplasia of the Orbit in a 5-Year-Old Girl

Abstract

Similar works

Full text

Available Versions

Journals Portal, Shahid Beheshti University of Medical Sciences