Fibrous Dysplasia of the Orbit in an Infant

Joseph, Elizabeth; Kachhara, Rajneesh; Bhattacharya, R. N.; Radhakrishnan, Venkatraman; Balachandran, Krishna

doi:10.1159/000028935

Cited by 8 publications

(3 citation statements)

References 7 publications

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“…The variable presentation and clinical course of fibrous dysplasia has been extensively reported in the literature [10, 11, 12, 13, 14, 15, 16, 17, 18, 19]. Although fibrous dysplasia is often thought of as a pediatric disease, it is increasingly recognized that a significant number of cases may not present until adulthood [27, 28].…”

Section: Discussionmentioning

confidence: 99%

Surgical Treatment of Fibrous Dysplasia of the Skull in Children

et al. 2002

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Introduction: We evaluate the role of surgery in the treatment of fibrous dysplasia of the skull in children. Methods: We identified 48 consecutive cases of fibrous dysplasia of the skull that were surgically treated at a single institution over a 23-year interval. The 28 patients that initially presented during the first two decades of life were selected for further analysis. Presenting symptoms, signs, surgical treatment, surgical outcome and the state of the disease at the extended follow-up interval were recorded. Results: Fibrous dysplasia of the skull in children most often involved the frontal, sphenoid and ethmoid bones. Most patients presented with facial asymmetry or proptosis. A gross total resection was achieved in 7 patients, subtotal resection in 17 patients, and 4 patients underwent biopsy alone. Over the follow-up interval, 7 patients had symptomatic progression of subtotally resected fibrous dysplasia and 3 patients had asymptomatic progression. The extent of resection was correlated with recurrence risk. Conclusions: In order to prevent progression of disease, an attempt at gross total resection is indicated in cases of fibrous dysplasia of the skull in childhood where the risk of neurologic morbidity is low and cosmetic results will be acceptable.

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Section: Discussionmentioning

confidence: 99%

Surgical Treatment of Fibrous Dysplasia of the Skull in Children

et al. 2002

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“…12 The woven bone is superimposed on a background of moderately cellular fibrous connective tissue. 22,25,41 The histological pattern appears to stablilize over time, and no difference is evident between that observed in adults and children. 20 …”

Section: Pathological Featuresmentioning

confidence: 95%

Cranioorbital fibrous dysplasia: with emphasis on visual impairment and current surgical management

Dumont

Boulos²,

Jane³

et al. 2001

FOC

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Fibrous dysplasia is a benign but slowly progressive disorder of bone in which normal cancellous bone is replaced by immature woven bone and fibrous tissue. Significant deformity and both acute and chronic visual impairment can result. A contemporary understanding of fibrous dysplasia, emphasizing the origins of visual impairment, indications for decompressive surgery, and the techniques for correction of the cosmetic deformity are presented. In their experience and review of the literature, the authors found the most frequent clinical presentations to be exophthalmos, displacement of the globe, abnormalities of extraocular motility, cosmetic deformity, and visual impairment. Although traditionally the cause of visual impairment has been ascribed to impingement of the optic canal on the optic nerve, the authors' experience is that the most common cause of visual loss is cystic degeneration of the tumor, particularly with those involving the anterior clinoid process. Exophthalmos and optic canal stenosis are less common causes of visual impairment. Indications for surgical intervention include acute and/or serially radiographically documented and relentless visual impairment and significant cosmetic deformity. Individualized management strategies are also discussed.

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“…Skorek et al, described an 8 year old boy with inferior orbital wall destruction secondary to fibrous dysplasia [16]. Joseph et al, discussed a case of gradually progressive unilateral exophthalmos and inferolateral displacement of the right globe in a 9 month old infant whom was found to have an extraconal lesion in the superomedial portion of the right orbit with simultaneous destruction of the superomedial orbital wall and roof, histologically confirmed to be fibrous dysplasia [17].…”

Section: Discussionmentioning

confidence: 99%

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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Fibrous Dysplasia of the Orbit in an Infant

Cited by 8 publications

References 7 publications

Surgical Treatment of Fibrous Dysplasia of the Skull in Children

Surgical Treatment of Fibrous Dysplasia of the Skull in Children

Cranioorbital fibrous dysplasia: with emphasis on visual impairment and current surgical management

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

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