Osteodysplastic

Osteodysplastic - pathological state of one or many of the bones caused by congenital disorder of their development. Osteodysplastic has several forms, depending on what phase has been breached development.
If osteodysplastic dice, excessive development of bone substance, or instead of bone has a cartilage or fibrous tissue. As a consequence, developed deformations of bones, which can lead to impaired function.
Osteodysplastic detected in the first years of life, adolescence and less frequently in older age. Recognition osteodysplastic is based on the data of x-ray studies. On radiographs depending on the form of osteodysplastic visible areas osteosclerosis (see) and the thickness of the cortical layer, single or multiple cysts, thinning of the cortical layer, as well as areas devoid of bone structure. In case of osteodysplastic, unlike fibrous osteodystrophies (see), there is no osteoporosis related defeat the plots of the bone.

Osteodysplastic (from the Greek. osteon - bone + dysplasia) is a pathological condition of the skeleton, due to a congenital process osteogenesis.
Osteodysplastic clinically can be observed in the first years of life or much later - in adolescence and young adulthood, depending on the degree of destruction of the skeleton and mainly from those strains that accompany it. Changes in the skeleton when Acting can be localized within one, two or many bones; they can be generalized. With the defeat of many bones changes are usually unilateral or predominantly one-sided. Can be selectively affected bones only upper or lower limbs.
Olkhon has many forms, depending on which phase of osteogenesis have any violations. If these violations are related to the phase of bone formation, there malarials, osteopenia, the so-called Hesse the osteosclerosis, generalized giperostos, or disease reliable community (with all these forms is excessive development of normal formed bone tissue), imperfect osteogenesis (osteogenesis imperfecta) or so-called increased fractures of bones, marble disease and osteomielite (in which the broken relationship in the formation of bone and blood-forming tissues) and a number of other diseases.
Violation of osteogenesis if osteodysplastic can occur on a phase of formation of cartilage, and then any disease, the merged group chondrodysplasia skeleton (multiple cartilage exostosis, enchondroma, the disease Ollie). Finally, violation of osteogenesis may occur in the phase of formation of fibrous tissue, and then developing fibrotic O. Fibrous dysplasia as an independent nosological form allocated Liechtenstein [Lihtensteina (L. Licbtenstein)] and then the Jaffa [jeffy (N. Jaffe)]. Before that it was described under the names osteitis fibrosa, fibrochistica disease, fibroadenoma.
Fibrous osteodysplastic has a clear clinical-radiological characteristics and patterns of flow.
Pathological anatomy. There are two forms of fibrous osteodysplastic - monasteriou (the defeat of a bone) and Polisario (injury of several bones), often one-sided. In the tubular bones process is developing in metafizik and diatezah, rarely in the epiphysis.
The affected bone is very easy sawn. Sawing on the cortical layer it thinned. Advanced medullar channel contains whitish-pink fibrous tissue types (Fig. 1). During a finger over the surface of a cut in the tissue of the defined fine bone inclusion. In affected areas can meet single or multiple smooth cysts containing serous or hemorrhagic fluid. Less frequent are the inclusion of the cartilage, which are usually located close to the epiphyseal plate. Due to thinning of the cortical layer and the proliferation of fibrous tissue affected areas bones become bloated, deformed. Thinning of the cortical layer reduces the strength of the bones and to pathological fractures. Flat bones in affected areas overblown, sawing on the cortical plate their thin, bone substance spongy layer is replaced by fibrous tissue.
Microscopically find the proliferation of cellular-fibrous tissue foci formation in it of bone structures. Osteogenic fabric may consist mainly of immature elongated cells, among which there are few bone bulochki (Fig. 2). Such multicellular areas are forced sometimes to differentiate the process sarcoma. In other cases, dominated by collagen fibers (Fig. 3)located in the form of intersecting beams, sometimes galinadiamond. Bone formations represent different stages in the development of the bone - from primitive fibrous and osteogenic beams to a more Mature bone structures. For bone beams characterized by disorderly nonfunctioning location. Sometimes bone beams form whimsical covers, which resembles the structure of the spongy osteomas (Fig. 4). With the defeat of the skull and especially the jaw bone bone inclusion can be in the form of a spherical stratified formations resembling semanticly. Often in the pathological tissues meet centers, consisting of single-core and giant multi-core cells, which origin Century A. Rusakov explains the excitement of osteoplastic tissue. Enable cartilage microscopically can resemble chondroma or have a structure Mature hyaline cartilage.

Fig. 1. Tumorous lesion foci of tissue in diaphysis tubular bones. (The Drug T. F. Angelevich.)
Fig. 2. Osteogenic fabric with primitive bone Balochi.
Fig. 3. Network gentle collagen fibers in fibrous tissue in osteodysplastic.
Fig. 4. Areas with a large number of bone beams.

Fibrous osteodysplastic can be combined with vaselene lesions. Described the combination of its multiple fibromiome soft tissues, neurofibromatosis skin and Sometimes other patients fibrous osteodysplastic celebrate areas of abnormal skin pigmentation and premature puberty. This triad of symptoms called Albright syndrome. More often Albright syndrome occurs when polyscale sided form of fibrous O. skin Pigmentation appears as landkarten or freckled spots brown color on the skin of the head, neck, back, waist, buttocks, thighs. The location of pigmented spots may not match the affected side of the bones. Age spots are marked at birth or appear in the first years of life. Microscopically deposition of melanin found in the basal layer of the epidermis. Premature puberty when Albright syndrome is mostly girls, manifesting itself in the form of early menstruation and early development of secondary sexual characteristics. In boys, it is rare and is the rapid growth, early brutalization vote, premature owolosenie of pubic hair. Often there are other hormonal disturbances (persistent thymus gland, thyroid adenoma, gipertireoidizmom).
The clinical picture. Fibrous osteodysplastic flows benign prognosis is good (ossification slowly, much later than usual, but still occurs). The disease is diagnosed most often in childhood or adolescence, sometimes at a later age, developing very slowly and stabilizing upon termination of growth of the skeleton. Common in women in 3-4 times more often than men.
In case of osteodysplastic can be affected bone cartilage and connective tissue of origin. Dominated by the defeat bones of the lower extremity (femur and tibia), and from flat - pelvic bones. There are often some degree of pain, usually occurring after injury or heavy loads. Sometimes, the first manifestation is a pathological fracture with further consolidation. At high concentration, particularly in peripheral bones, reveals a certain size swelling, painless at a palpation.
It is also possible violations of growth: the true LLD, accompanied her arched curves. With the involvement of the epiphyseal region occurs shortening of the bones. It is possible restrictions of movement, fatigue. Significant deformation can be defined on the eyes, especially strain thigh, causing lameness.
Any changes on the part of other organs and systems, including endocrine, in case of osteodysplastic not revealed, except Albright syndrome (see above). The content of calcium in the serum is not raised. Known only a few cases zlokacestvennoe fibrous O.


X-ray picture of fibrous osteodysplastic characterized by several features. In places of destruction of the cortical layer thinning on the inside, but never interrupted. In some places, on the contrary, it can be compensatory thickened. The inner contour of its scalloped. The bone structure is represented rarefied diffuse or focal type, under the form reminding cystic changes (Fig. 5). On the background of depression can be of different types of bone crossbar - from barely coming up rough, creating trabecular or a honeycomb pattern (Fig. 6). Are mainly affected diaphyseal departments of the long bones. Changes in the structure of the epiphyses and epifizov having independent kernel ossification, are rare. Even with the defeat of the femur, where the predominant localization is the proximal Department, head and Vartely remain unchanged. Zone germ cartilage is how the borders of the affected area. During the transition process for the epiphyseal growth line may vary articular surface of the bone.
Along with the structural changes affected departments of the bones in one degree or another vzduchu, marked and variously pronounced curvature. With the defeat of the thigh that is typical coxa vara (Fig. 7). On top of distortions can occur zone adjustment. Morphological substrate radiographically detected diffuse or focal racemose of prosvetlenie are the same or diffuse nodular accumulation of dense fibrous tissue (not true cysts filled with liquid).

Fig. 5. Single listopadovka foci of depression in diatezah long tubular bones. Nerezko expressed swellings on the level of those sites.
Fig. 6. The coarse and fine, the structure for a considerable length of divisov long bones without deformation and with large arched deformation.
Fig. 7. Focal lesion of the right half of the pelvis and femur with arched her curving and reduction cervico-diaphyseal angle (coxa vara).

Observed changes in the skull, which is affected by itself or in combination with other bones. In most cases, bone growths in the brain and facial departments of the skull are one-way, causing more or less pronounced asymmetry head (elefantiasis cranii). At the same time as the secondary effects are the compression of the brain and its meninges, exophthalmos (due to the deformation of the eye). With the defeat of the upper jaw is obliteration of air cavities. The localization of the changes in the base of the skull may come a narrowing of holes with compression passing through them, vessels and nerves. However, such complications are rare.
Differential diagnosis of fibrous osteodysplastic in advanced lesions of the skeleton does not represent any difficulties. X-ray picture allows to exclude giperparatireoidizm the osteodystrophy on the basis of a lack of systemic osteoporosis, availability unaffected bones and plots with unmodified bone structure in the affected bones, not to mention completely different clinical manifestations of this disease. To distinguish fibrous osteodysplastic from chondromatosis bones, and in the absence of deformation from multiple eosinophilic granuloma often possible only after the biopsy. If odnorodnyh defeats the likely significant differential diagnostic difficulties, requiring also histological studies (puncture, biopsy).
The dynamics of the process for radiological monitoring even after long periods of time is very slow. Respiratornye changes are reduced to burnetii and robotreplay the rebuilding of bone (Fig. 8).

Fig. 8. Respiratornye changes in the form of filling the pockets of depression, burnetii and robotreplay rebuilding of bone.

Described cases of fibrous osteodysplastic, accompanied by some nekotnei malformations (underdevelopment or absence of one kidney, coarctation of the aorta, arteriovenous aneurysm, etc. and also a few cases of a combination of fibrous osteodysplastic with multiple mikromumy.
For fibrous O. also include Albright syndrome (see the Albright syndrome).
Treatment of fibrous osteodysplastic in close severe deformation is reduced to a conservative orthopedic activities. To active intervention should be treated with caution, as extensive resection and curettage proceed with sluggish bone regeneration (fibrous tissue is replaced not bone, and fibrous cloth).