Presentation
Patient presented with bilateral leg pain on examination and imaging showed bilateral tibia lytic lesion. [ncbi.nlm.nih.gov]
Degree of confidence Plain radiographs are highly specific when characteristic features are present in a lesion. [emedicine.com]
Entire Body System
- Recurrent Sinusitis
Patients typically complain of nasal congestion (>34% of those with symptoms and sinus involvement), headaches or facial pain, recurrent sinusitis, and hyposmia. [doi.org]
Course
- Severe Clinical Course
Also, in our series, subjects with McCune-Albright syndrome tended to experience a more severe clinical course. [doi.org]
Jaw & Teeth
- Tooth Loss
Periodontal and occlusal changes may also be present, and this may even result in tooth loss. 2 Changes in the development of the jawbone usually affect dental occlusion. [elsevier.pt]
Eyes
- Scotoma
Optic nerve dysfunction was defined as the presence of a characteristic scotoma (or field deficit), or an abnormal result on two of four other tests: visual acuity worse than 6/12, correct identification of fewer than 10 of the 14 Ishihara colour plates [nature.com]
Because there is no definitive test for optic neuropathy, abnormalities suggestive of optic neuropathy resulting from FD were defined as either an abnormal result on the visual field test (such as scotoma or field deficit) or an abnormal result on two [doi.org]
- Anterior Uveitis
Clinical symptoms comprised headache (n=3) and sensorineural disorder: recurrent anterior uveitis (n=1), visual acuity loss, epiphora and vestibular syndrome (n=1), and hearing loss (n=1). All cases were monostotic. [ncbi.nlm.nih.gov]
Workup
A 43-year-old woman with breast cancer underwent Tc-hydroxymethylene diphosphonate bone scintigraphy for preoperative workup. [ncbi.nlm.nih.gov]
X-Ray
- Pleural Mass
Chest radiograph shows expansion of multiple ribs involved by fibrous dysplasia that mimics pleural masses. Spine Spinal involvement is common in polyostotic disease and rare in monostotic disease. [emedicine.com]
Treatment
In patients with severe symptoms medical treatment can be implemented, but safety of this treatment in children remain controversial. [ncbi.nlm.nih.gov]
Prognosis
PROGNOSIS Biological Behavior • Benign tumor • Monostotic lesions have good prognosis • Polyostotic lesions tend to be more active or aggresive • Recurrence following curettage is common particularly when bone grafting the defect with autologous bone [tumorsurgery.org]
The prognosis may be difficult to establish because of the wide spectrum of disease severity. We have analyzed the data from the French National Reference center for FD. We have established a database from standardized medical records. [ncbi.nlm.nih.gov]
The prognosis generally is good, although poor outcomes are more frequent in younger patients and in those with polyostotic forms of the disease. The risk of malignant transformation is low. Dr. [journals.lww.com]
Microscopic Fibrous Dysplasia : Microscopic Fibrous Dysplasia : Microscopic Fibrous Dysplasia : Microscopic Fibrous Dysplasia : Microscopic Fibrous Dysplasia : Molecular Genetics Fibrous Dysplasia Fibrous Dysplasia Fibrous Dysplasia : Treatment & Prognosis [webpathology.com]
Etiology
Fibrous dysplasia is a nonneoplastic, developmental disease of the bone that begins in childhood with obscure etiology. Clinically, it is presented as a continuously growing, painless mass at late childhood. [ncbi.nlm.nih.gov]
Epidemiology
Updated Molecular and Epidemiologic diagrams added to all new chapters. [books.google.de]
The authors review the biological, epidemiologic, clinical, genetic, and radiographic characteristics of adult FD, with an emphasis on surgical management of FD. [ncbi.nlm.nih.gov]
Home About Us Acknowledgements Feedback Contact Us Site Map High Quality Pathology Images of Benign and Malignant Neoplasms and Related Entities Fibrous Dysplasia Home Orthopedic Tumor-like Lesions of Bone Fibrous Dysplasia Fibrous Dysplasia : Intro & Epidemiology [webpathology.com]
Johanna Benhamou, Deborah Gensburger, Claude Messiaen and Roland Chapurlat, Prognostic Factors From an Epidemiologic Evaluation of Fibrous Dysplasia of Bone in a Modern Cohort: The FRANCEDYS Study, Journal of Bone and Mineral Research, 31, 12, (2167-2172 [doi.org]
Pathophysiology
This review summarizes the pathophysiology, clinical findings, and treatment of FD, with an emphasis on the craniofacial and oral manifestations of the disease. Published 2016. This article is a U.S. [ncbi.nlm.nih.gov]
Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am. 2005;87:1848-1864. http://www.ncbi.nlm.nih.gov/pubmed/16085630 Lietman SA, Ding C, Levine MA. [rarediseases.org]
The goal was to better define the natural history of FD encasing the optic nerve and to identify the pathophysiological mechanisms contributing to the development of optic neuropathy. [doi.org]
Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am. 2005 Aug. 87 (8):1848-64. [Medline]. Marie P. Dysplasie fibreuse: aspects tissulaires, cellulaires et moléculaires. Revue du Rhumatisme. 2003. 7:681-6. [emedicine.medscape.com]
Prevention
There is no known way to prevent fibrous dysplasia. Treatment aims to prevent complications, such as recurrent bone fractures, to help make the condition less severe. [nlm.nih.gov]
En bloc resection and bone graft cannot prevent recurrence in skeletally immature patients with monostotic expansile fibrous dysplasia, and cannot alter for the natural history of the disease. [ncbi.nlm.nih.gov]