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Fibrous dysplasia is a very rare [2] nonhereditary genetic disorder where normal bone and marrow is replaced with fibrous tissue, resulting in formation of bone that is weak and prone to expansion. As a result, most complications result from fracture , deformity, functional impairment, pain, and the impingement of nerves. [ 3 ]
Fibrodysplasia ossificans progressiva (/ ˌ f aɪ b r oʊ d ɪ ˈ s p l eɪ ʒ (i) ə ɒ ˈ s ɪ f ɪ k æ n z p r ə ˈ ɡ r ɛ s ɪ v ə /; [1] abbr. FOP), also called Münchmeyer disease or formerly myositis ossificans progressiva, is an extremely rare connective tissue disease in which fibrous connective tissue such as muscle, tendons, and ligaments turn into bone tissue (ossification).
Monostotic fibrous dysplasia occurs with equal frequency in both sexes and normally develops early in life, with lesions frequently identified late in the first and early second decades. Most patients are asymptomatic, with the diagnosis often established after an incidental finding or with pain, swelling, or fracture.
Fibrous dysplasia is a rare congenital disease of the bone that often affects the cranial facial structures, Dr. Netanel Ben-Shalom, a nuerosurgeon, at Northwell Lenox Hill Hospital, tells TODAY ...
Radiation therapy has been associated with malignant transformation of skull base fibrous dysplasia, and should be avoided in all but the most dire cases. [ 6 ] Cushing syndrome is a rare but potentially fatal complication that can occur in the first year of life.
Osteoclastic and osteoblastic remodeling contributes to the change of normal bone to fibrous tissue and cyst formation. As noted by the name, the patient's face becomes enlarged and disproportionate due to the fibrous tissue and atypical bone formation. The sponge-like bone formations lead to early tooth loss and permanent tooth eruption problems.
Dysplasia is any of various types of abnormal growth or development of cells (microscopic scale) or organs (macroscopic scale), and the abnormal histology or anatomical structure(s) resulting from such growth. [1] Dysplasias on a mainly microscopic scale include epithelial dysplasia and fibrous dysplasia of bone.
Fibrodysplasia ossificans progressiva, a rare disease in which fibrous tissue becomes ossified; Fibromuscular dysplasia, a disease characterized by the fibrous thickening of the renal artery; Fibrous dysplasia, a disease that causes growths or lesions in one or more bones of the human body