Patient presented with bilateral leg pain on examination and imaging showed bilateral tibia lytic lesion. [ncbi.nlm.nih.gov]

• Developmental Disorder

  Fibrous dysplasia is benign developmental disorders of bone where normal lamellar cancellous bone is replaced with immature fibro-osseus tissue resulting in poorly formed trabeculae of immature woven bone. [ncbi.nlm.nih.gov]

  Fibrous dysplasia, rare congenital developmental disorder beginning in childhood and characterized by replacement of solid calcified bone with fibrous tissue, often only on one side of the body and primarily in the long bones and pelvis. [britannica.com]

  Articles Abstract Author Information Authors Fibrous dysplasia is a developmental disorder of bone that can present in a monostotic or polyostotic form. Primarily affecting adolescents and young adults, it accounts for 7% of benign bone tumors. [journals.lww.com]

  GENERAL INFORMATION Fibrous dysplasia is not considered a tumor; it is a developmental disorder of the bone. It appears similar to a tumor on radiological studies. [tumorsurgery.org]

• Falling

  One patient sustained a refracture after surgery resulting from an in-hospital fall. The fracture was treated without further surgery, and it healed. [ncbi.nlm.nih.gov]

• Localized Pain

  BACKGROUND AND OBJECTIVE: Fibrous dysplasia (FD) is a benign bone lesion manifested by local pain, swelling and deformity change. [ncbi.nlm.nih.gov]

• Diplopia

  Fibrous dysplasia of the zygomatic bone may cause orbital dystopia, diplopia, proptosis, loss of visual acuity, swelling, mass formation, or facial asymmetry. We present 1 case of fibrous dysplasia with isolated zygomatic bone involvement. [ncbi.nlm.nih.gov]

  The most common presenting features included atypical facial pain and headache, complaints referable to the sinuses, proptosis and diplopia, hearing loss, and facial numbness. [jamanetwork.com]

  The searches were conducted using the Medical Subject Heading (MeSH) terms (fibrous dysplasia) AND (orbit OR vision OR visual OR optic OR exophthalmos OR diplopia OR proptosis) and limited to “Human”. [doi.org]

  Postoperative complications occurred in 15 (50%) patients, including infection, binocular diplopia, and cranial nerve palsies in 3 (10%) patients each; pain in 2 (13.3%); epistaxis, hypertrophic scar, anaemia, and ectropion in 1 (6.7%) each. [nature.com]

• Hyperpigmentation

  He had never had hyperpigmentation of the skin or endocrine dysfunction indicative of the McCune–Albright syndrome, and no other family members had skeletal lesions. [nejm.org]

  It is characterized by deformities and fractures of the bones, endocrine abnormalities including early puberty, and skin hyperpigmentation Fibrous dysplasia of bone affecting several bones. [icd9data.com]

  We report here on a child with McCune-Albright syndrome (FD in addition to hyperfunctioning endocrinopathies and skin hyperpigmentation) treated with cyclical intravenous infusions of pamidronate in which conventional radiography, contact microradiography [ncbi.nlm.nih.gov]

  The less frequent polyostotic type may be associated with endocrine abnormalities, skin hyperpigmentation and soft tissue myxoma (so-called Albright syndrome). [atlasgeneticsoncology.org]

  A specific form is called In both cases presented, there was a contiguous McCune-Albright's syndrome, consists of the polyostotic affection restricted to the craniofacial bones and type followed by cutaneous hyperpigmentation and early corresponding to [arquivosdeorl.org.br]

• Cafe-Au-Lait Spots

  When melanotic pigmentation (cafe-au-lait spots) and multiple endocrine hyperfunction are additionally associated it is referred to as albright syndrome ICD-9-CM Volume 2 Index entries containing back-references to 756.54 : [icd9data.com]

  McCune-Albright syndrome, another form of fibrous dysplasia, includes different symptoms, such as early onset of puberty and skin spots, called cafe-au-lait spots. [stanfordhealthcare.org]

  FD can also be associated with birth marks (cafe-au-lait spots), and a number of endocrine problems such as precocious puberty, hyperthyroidism, low blood phosphorus, and excess growth hormone. [milliondollarbikeride.org]

• Coxa Vara

  RESULTS: The 227 femurs were classified into five reproducible types: type 1 (33 %), normal bone strength without angular deformity; type 2 (30 %), decreased bone strength without angular deformity; type 3 (12 %), isolated coxa vara with neck-shaft angle [ncbi.nlm.nih.gov]

  Ernesto Ippolito, Pasquale Farsetti, Matteo Benedetti Valentini and Vito Potenza, Two-Stage Surgical Treatment of Complex Femoral Deformities with Severe Coxa Vara in Polyostotic Fibrous Dysplasia, The Journal of Bone and Joint Surgery, 10.2106/JBJS.N [doi.org]

• Facial Pain

  The most common presenting features included atypical facial pain and headache, complaints referable to the sinuses, proptosis and diplopia, hearing loss, and facial numbness. [jamanetwork.com]

  Signs and symptoms can include facial pain, headache, cranial asymmetry, facial deformity, tooth displacement, and visual or auditory impairment (figures 1 and 2 ) [ 4, 8 ]. [head-face-med.biomedcentral.com]

  Patients typically complain of nasal congestion (>34% of those with symptoms and sinus involvement), headaches or facial pain, recurrent sinusitis, and hyposmia. [doi.org]

• Headache

  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]

• Aura

  The authors experienced a patient of fibrous dysplasia originating from the ethmoid bone which presented with severe headache with some features suggestive of menstrual migraine without aura. [ncbi.nlm.nih.gov]

A 43-year-old woman with breast cancer underwent Tc-hydroxymethylene diphosphonate bone scintigraphy for preoperative workup. [ncbi.nlm.nih.gov]

In patients with severe symptoms medical treatment can be implemented, but safety of this treatment in children remain controversial. [ncbi.nlm.nih.gov]

A, Proximal femur of patient 13 at the start of pamidronate treatment. B, After 2.0 yr of treatment. [doi.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]

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]

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 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]

Fibrous dysplasia is considered to be a developmental, tumor-like (hamartomatous), fibro-osseous disease of unknown etiology. There is a maxillary predominance when craniofacial FD occurs in the jaws and the maxillary sinus is commonly involved. [ncbi.nlm.nih.gov]

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]

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]

Fibrous Dysplasia: Pathophysiology, evaluation and treatment. JB&JS [Internet]. Agust 2015;87-A(8). [revistamedicasinergia.com]

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]

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]

In some cases, bracing may be used to prevent fracture in weakened bones. However, bracing has not been shown to be effective in preventing progression of deformity. [orthoinfo.aaos.org]