Fibrous dysplasia of the orbit.

Bibby, K; McFadzean, Robert M.

doi:10.1136/bjo.78.4.266

Cited by 71 publications

(31 citation statements)

References 26 publications

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“…FD was first described by von Recklinghausen in 1891 in McCune-Albright Syndrome (MAS) with genetically linked with the GNAS1 gene somatic mutation, however in 1938 Lichtenstein coined the term fibrous dysplasia in its monostotic and polyostotic character [1][2][3]. FD comprises 2.5% of all bony tumors and 7.5% of benign bony lesions [7].…”

Section: Discussionmentioning

confidence: 99%

“…FD typically involves the skull, long bones, and ribs in which there is replacement of normal bone marrow with fibro-osseous tissue manifesting as a painless asymmetric bony enlargement [1,3,6]. FD is monostotic in 70-80% of cases, while 20-30% are polyostotic, and only 10% of overall cases are found to be in the craniofacial region, most commonly in the maxilla and frontal bone [1,2,6]. The monostotic variant usually presents in the craniofacial bones, femur, tibia and ribs, while the polyostotic form typically presents in the femur, tibia and pelvis with common complaints of pain associated with recurrent fractures [8].…”

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“…FD exhibits a "ground glass" appearance due to the woven bone and fibrous component with areas of sclerosis, centered primarily in the medullary canal with cortical expansion [6][7][8]. Occasionally, there can be areas of cartilaginous differentiation with ring or punctate calcifications, and if prominent, the diagnosis should be fibrocartilaginous dysplasia [8].Fibrous dysplasia management includes observation if symptoms are minimal to assess for tumor growth, conservative debulking, or radical bony excision and reconstruction [2]. Goisis et al, states that non-surgical approaches results in poor outcomes and surgical treatment is determined by substantial functional decline [3].…”

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An Exophthalmic Clinical Presentation of Cementomatous Fibrous Dysplasia in an Orbital Roof

Yanoff¹

2017

OCR

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or polyostotic in nature [1][2][3]. When presenting in the craniomaxillofacial region, specifically the orbit, it can cause painless bony enlargement producing ocular manifestations of exophthalmos, deformity, and visual problems. Neuroimaging is essential to identify the extent of bony involvement. Fibrous dysplasia should be high on the differential for a pediatric ophthalmologist as it typically presents in the first 3 decades of life. Malignant transformation is unlikely in children, but FD can cause visual loss especially when involving compression of the optic nerve. There have been no previously reported findings of a cytogenetic or genetic mutation associated with monostotic fibrous dysplasia lesions. Clinical Presentation and Diagnostic ImagingA 10-year-old boy presented to the St. Christopher's Hospital for Children Ophthalmology Department in Philadelphia, PA in October 2015 as a new patient. The chief complaint was a protruding right eye noted by the patient and his parents. The right exophthalmos had been occurring over a 3-month period. Upon arrival to the ophthalmology department, the patient was immediately sent to the Neurosurgery department with notification that the patient had slowly progressing proptosis of his right eye in need of Neuroimaging (Figures 1 and 2). Brown A, et al., J Ophthalmic Clin Res 2017, 4 AbstractA 10-year-old boy with no prior ocular complaints presented to the St. Christopher's Hospital for Children in Philadelphia with slowly progressing right eye proptosis. Computed tomography and magnetic resonance imaging of the head and brain showed a right orbital roof intraosseous mass displacing the superior rectus muscle, causing right exophthalmos. Debulking of the lesion was performed. Pathology diagnosed cementomatous variant of fibrous dysplasia with a genetic component of loss of chromosomal segments at XQ and 2Q, specifically 2q33.1 and Xq26.3q27.1. Chromosomal losses at Xq26 and 2q33 were described in three cases of cemento-ossifying fibromas by Sawyer et al., in 1995. We provide supporting evidence for a possible cytogenetic relationship between Xq26 and 2q33 chromosomal losses and the occurrence of fibro-osseous lesions. Based on the patient's history, ocular and histological examination, exophthalmos occurred in this patient due to orbital roof cementomatous variant of fibrous dysplasia. To the best of our knowledge, there have been no previously reported cases or cytogenetic findings associated with a cementomatous variant of fibrous dysplasia in the orbital roof. Figures 2A & 2B: MRI described the mass with similar measurements to have a 1.5 cm multi-septated, cystic-fluid filled, necrotic center with mass effect on the inferior frontal lobe while flattening and inferiorly displacing the superior rectus muscle of the right globe, causing the noted proptosis. The right globe and optic nerve were intact in both CT and MRI. Differential diagnosis based on imaging was fibrous dysplasia, rhabdomyosarcoma, Langerhans cell histocytosis and Lymphoma/Leukemia. The patient...

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Section: Discussionmentioning

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An Exophthalmic Clinical Presentation of Cementomatous Fibrous Dysplasia in an Orbital Roof

Yanoff¹

2017

OCR

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“…Criteria for study population inclusion were histopathologic diagnosis of CFD, radiologically confirmed optic canal narrowing, pre-and post-treatment visual status (based on visual fields and visual acuity), and .4 months of follow-up. The included studies are listed in Tables S1, S2, S3 (supplementary material) [8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33]. Follow-up included periodic (6-12 months) radiological imaging with CT and MRI as well as ophthalmologic exams.…”

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confidence: 99%

Surgery versus Watchful Waiting in Patients with Craniofacial Fibrous Dysplasia: A Meta-Analysis

Amit¹,

Collins²,

FitzGibbon³

et al. 2012

J Neurol Surg B

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Background: Fibrous dysplasia (FD) is a benign bone tumor which most commonly involves the craniofacial skeleton. The most devastating consequence of craniofacial FD (CFD) is loss of vision due to optic nerve compression (ONC). Radiological evidence of ONC is common, however the management of this condition is not well established. Our objective was to compare the long-term outcome of patients with optic nerve compression (ONC) due to craniofacial fibrous dysplasia (CFD) who either underwent surgery or were managed expectantly.

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Orbital Tumors

Dutton¹,

Sines²,

Elner³

2011

Smith and Nesi’s Ophthalmic Plastic and Reconstructive Surgery

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Fibrous dysplasia of the orbit.

Abstract: Twelve patients with fibrous dysplasia of the orbit are reviewed and the ophthalmic findings described. Three case histories are presented in detail. Six patients were managed conserva-

Cited by 71 publications

References 26 publications

An Exophthalmic Clinical Presentation of Cementomatous Fibrous Dysplasia in an Orbital Roof

An Exophthalmic Clinical Presentation of Cementomatous Fibrous Dysplasia in an Orbital Roof

Surgery versus Watchful Waiting in Patients with Craniofacial Fibrous Dysplasia: A Meta-Analysis

Orbital Tumors

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