Fibrous dysplasia is a congenital, non-inherited, benign intramedullary bone lesion in which the normal bone marrow is replaced by abnormal fibro-osseous tissue. The disorder can be monostotic (involving a single bone) or polyostotic (involving multiple bones). As the abnormal fibro-osseous tissue compromises the mechanical strength of bone, it can result in pain, deformity, fractures, or abnormalities in bone mechanics with inappropriate bone alignment. This narrative review attempts to summarize more than 20 years of observations of patients with FD to help pediatric orthopedists establish a care framework that can improve its identification, understand the impact that endocrinopathies can have on its clinical presentation, and optimize the management of bone disorders. Our focus is specifically on orthopedic manifestations of FD and modern management alternatives. The past 20 years have provided major advances in understanding of fibrous dysplasia (FD), and it is clear that the pediatric orthopedist's role remains highly relevant in the management of all types of FD. Surgical treatment remains appropriate when pain is unresponsive to other medical treatments, when a pathological fracture is impending or has happened, when a deformity is worsening or has formed, or when there is a suspicion of malignant transformation. The pediatric orthopedist must be aware, therefore, of the particularities of the different bones on which they may be called to intervene, and they should give very careful consideration to their operative strategy, which must be adjusted to the biological and static characteristics of the bone.
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http://dx.doi.org/10.3389/fped.2024.1502262 | DOI Listing |
Indian J Otolaryngol Head Neck Surg
January 2025
Department of Otorhinolaryngology, Saveetha Medical College and Hospital, SIMATS, Saveetha Nagar, Thandalam, Chennai, Tamil Nadu 602105 India.
Fibrous dysplasia, a rare benign condition with an uncertain cause, is characterized by substituting normal medullary bone with abnormal and weak fibrous and osseous tissue. This primary bone disorder is non-neoplastic and involves a deficiency in osteoblastic differentiation and maturation, which begins in the bone's mesenchymal precursor. A 20-year-old female attended ENT OPD with complaints of headache for 3 years, A diagnostic nasal endoscopy was done which showed no significant abnormalities, and CT PNS was done which showed Extensive sclerotic bony expansion with a ground glass appearance involving the clivus, right greater wing of sphenoid, pterygoid processes - Likely fibrous dysplasia since she had no other signs or symptoms, she was kept under observation.
View Article and Find Full Text PDFBMC Cardiovasc Disord
March 2025
Department of Cardiovascular Surgery, The First Affiliated Hospital of Xi'an Jiaotong University, No. 277 Yanta West Road, Xi'an, 710061, China.
Background: Arrhythmogenic cardiomyopathy (ACM) is a genetic myocardial disease characterized by progressive myocyte loss and fibrofatty (fibrous and adipose) tissue replacement to predispose these patients to fatal ventricular arrhythmias and impairment of ventricular systolic function. The relationship of ACM and myocarditis has gained significant attention.
Case Presentation: This case presented a 28-year-old female who was admitted to the hospital with complaints of recurrent lower limb edema and palpitations for 6 months.
J Med Case Rep
February 2025
Department of Radiology, Stanford University School of Medicine, Stanford, CA, USA.
Background: Fibrous dysplasia is an uncommon bone disorder in which medullary bone is replaced by disorganized fibro-osseous tissue. Fibrous dysplasia typically exhibits slow growth that stabilizes with skeletal maturity. We report a case in which rapid progression of a clival lesion otherwise typical for fibrous dysplasia in an adult male led to concern for a malignant rather than a benign lesion.
View Article and Find Full Text PDFFront Pediatr
January 2025
Pediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospitals, Geneva, Switzerland.
Fibrous dysplasia is a congenital, non-inherited, benign intramedullary bone lesion in which the normal bone marrow is replaced by abnormal fibro-osseous tissue. The disorder can be monostotic (involving a single bone) or polyostotic (involving multiple bones). As the abnormal fibro-osseous tissue compromises the mechanical strength of bone, it can result in pain, deformity, fractures, or abnormalities in bone mechanics with inappropriate bone alignment.
View Article and Find Full Text PDFIndian J Thorac Cardiovasc Surg
March 2025
Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.
Osteofibrous dysplasia (OFD) is a rare benign bone lesion primarily affecting the long bones' anterior cortex, commonly observed in adolescents. Typically found in the tibia and fibula, it represents less than 1% of primary bone tumors. While OFD is predominantly seen in the lower extremities, occurrences in ribs, particularly in adult females, are exceptionally rare.
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