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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).
The only definitive diagnostic test in the early acute stage is a bone scan, which will show heterotopic ossification 7 – 10 days earlier than an x-ray. The three-phase bone scan may be the most sensitive method of detecting early heterotopic bone formation.
The initiation of endochondral ossification starts by proliferation and condensation of mesenchymal cells in the area where the bone will eventually be formed. Subsequently, these mesenchymal progenitor cells differentiate into chondroblasts, which actively synthesize cartilage matrix components.
The Haversian canals surround blood vessels and nerve cells throughout bones and communicate with osteocytes (contained in spaces within the dense bone matrix called lacunae) through connections called canaliculi. This unique arrangement is conducive to mineral salt deposits and storage which gives bone tissue its strength.
It is synonymous with bone tissue formation. [1] There are two processes resulting in the formation of normal, healthy bone tissue : [ 2 ] Intramembranous ossification is the direct laying down of bone into the primitive connective tissue ( mesenchyme ), while endochondral ossification involves cartilage as a precursor.
Within adults and developing adults, most chondroblasts are located in the perichondrium. This is a thin layer of connective tissue which protects cartilage and is where chondroblasts help to expand cartilage size whenever prompted to by hormones such as GH, TH, and glycosaminoglycans. [2]
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The first step in the process is the formation of bone spicules which eventually fuse with each other and become trabeculae. The periosteum is formed and bone growth continues at the surface of trabeculae. Much like spicules, the increasing growth of trabeculae result in interconnection and this network is called woven bone.