The deforming actos

Actos deforming (ostosis deformans; synonym: octet deforming, osteitis deformans, deforming osteodystrophy, Paget's disease) - skeletal disease characterized by enhanced rebuild bones, accompanied by their deformations.
Actos deforming develops in older, sometimes in middle age, in both sexes, can be family and inheritance, captures one or more bones (mono - and polissena form), never generalized. Often the process affects the tibia, the bones of the skull, vertebrae.
Macroscopically long bones are wide, deformed (Fig. 1). With the defeat of one of the pair of bones (Fig. 2) she is intensively growing and bends (normal bone preventing its extension). Sawing on the cortical layer of bone wide, loses compact structure, "spongiosum"; visible areas of depression and osteosclerosis. Defeat is of a local nature or extended to the whole bone.

Fig. 1. Deformation of the femoral bone.
Fig. 2. Elongation and bending of the radial bone.
Fig. 3. Changing the facial and cerebral cranium.
Fig. 4. Mosaic structure of the bone.

Skull bones sharply thicken; circumference of the skull is increased, and the cavity is reduced. With the defeat of the bones of the face may be ugly, it is sometimes similar to syphilis (Fig. 3). The affected vertebrae first increase in size, then reduced in height; developing kyphosis leads to the shortening of the body.
Microscopically find typical rebuilding of bone. The process of resorption and bone substances are separated by time, therefore, on the border of the deposits of new bone formed pronounced line gluing, giving bone mosaic structure (Fig. 4). Resorption of bone structures expressed sharply and is accompanied by the appearance of a large number of osteoclasts. Between bone beams and in a vascular channel grows fibromyoma fabric, serving as a source of bone formation.
Clinical manifestations of deforming ostase depend on the localization process. The defeat of the long bones accompanied by pain and deformities. The defeat of the skull bones are first impacted by the increase in the size of the skull, in a further narrowing of the skull cavity leads to headaches, the fall of view, to cerebellar symptoms, with the defeat of the pyramid of the temporal bone come disorders of hearing. Rebuild bones is not a physiological nature: bones do not maintain functional load, there are pathological fractures. In blood serum report higher levels of alkaline phosphatase.
Deforming actos may be complicated by the development of sarcomas of different types. When polyscale form of the disease sarcoma can be multicenter.
Treatment of deforming ostase - quiet, high-calorie diet, vitamins a and D, hydrotherapy, to reduce pain applies radiotherapy.
X-ray picture of deforming ostase depends on the location, prevalence, duration and extent of the development process, the sequence changes. The dominant factor of morphological changes in the bones is the rebuilding of bone structure, resulting in apparent cases comes characteristic deformation of the affected bones.
The pathological process is shown, as a rule, a few, and sometimes in many bones, although the observed lesions and only one bone. Rebuilding of bone initially expressed by vacuum (resorption of bone usually in a limited area. Subsequently, on the background of this depression, there are signs of reparative process in the form of structural picture of the bone rungs or newly formed bone mass with amorphous structure. Bone substance is formed on the surface of the bone, and on the part of angosta. Cortical layer thickens, bone marrow same space respectively tapering. Cortical layer has longitudinally spongy structure, and the border between the changed cortical layer and bone marrow space becomes vague (Fig. 5).

Fig. 5. Thickening of the tibia with a thickening her cortical layer and the narrowing of bone marrow space. Razvlechenie patterns of cortical layer. Vague border between the cortical layer of bone marrow and space.
Fig. 6. Thickening of the skull, the rebuilding of bone formation flocculent amorphous areas.

The bones of the skull after the initial limited rarefaction (osteoporosis cranii circumscripta) thicken (sometimes several times), differentiation them on compact discs and the sponge-like material is lost. On the background of depression appear flocculent unstructured parts of the newly created bone substance as a reflection of the reparative process (Fig. 6). As a result of reconstruction the structure thick bones, including the facial skull, has shirokolisty figure.
In the vertebrae restructuring is expressed mild thickening of their bodies in the lateral and front-back lines, the rarefaction of bone in the Central area of the vertebral body and its thickening on the periphery. Creates a peculiar pattern of vertebra in the form of a so-called frame (Fig. 7). Gradually there may come a flattening of the vertebral bodies. Perestroika in the bones of the pelvis, in addition to the thickening, accompanied fan-shaped arrangement of newly formed bone spars.
Characteristic changes in the tubular bones of the limbs (Fig. 8). Together with thickening, arched curve and razvlecheniem cortical substance in the tibia arise Looser zone, perestroika (see). Sometimes there are pathological fractures large bones in the form of so-called banana fracture (transverse direction of the plane of fracture).

Fig. 7. Rebuilding of bone of the vertebral body, thickening him on the periphery in the form of a "frame".
Fig. 8. Lozanovskii zone of perestroika in the tibia with Paget's disease.