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Fibrous dysplasia

  1. Reasons
  2. Symptoms
  3. Diagnosis
  4. Treatment


Fibrous dysplasia

this disruption in the formation of fabrics, development of internal organs or the parts of the body. Most often the disease was congenital, but may occur in more Mature age, from 10 to 30 years. Have dysplasia there are several varieties, such as livenote person cherubism and Albright’s syndrome.

Reasons

The reasons for the development of this disease remain a mystery in our days. There is a perception that fibrous dysplasia of bones develops due to a mutation in the genes. Facts that would prove that the disease is inherited, no. Basically, the disease shows itself, while the patient is in childhood, although there are rare cases where the first manifestation was diagnosed in older patients. There are also congenital cases when children had a number of abnormalities at birth or were in the womb.

Symptoms

Often, fibrous dysplasia has no pronounced symptoms. The patient may for a long time not know of the disease, as pain and no discomfort to a certain level of spread of the disease. The disease may affect one bone or affect the whole skeletal system. The feature of the damage to fibrous dysplasia, determined by the number of cellular-fibrous tissue, studded with blood vessels, and scattered bones around which is osteobrama fabric, in turn, surrounded by cells. It is possible to distinguish two types of growths in the bones of focal and diffuse. At first, focal lesions, the transformed part in isolation from the surrounding tissues and easily departs from the adjacent bone. The tissue affected by the transformation, becomes thick, but does not lose its elasticity fades, it is clearly visible bone blotches. In the second, a diffuse bone lesion, it is impossible to determine the size of the damaged tissue, due to the fact that the adjacent bone becomes porous and soft, the cortical layer becomes thinner. The porous substance of the bone is replaced with dense pale yellow substance. With both types it is possible to detect single cysts.

The clinical manifestations of solitary bone lesions unobtrusive, do not have clear symptoms. The infected area gradually becomes thicker, however, no painful sensations arise. The disease becomes noticeable after approximately 2 years from the beginning of the inflammatory process. Often asymmetry. When the manifestation of disease in multiple bones at the same time the symptoms remain the same, but the affected area is greatly increased. Frequent fractures are one of the most common signs, also possible skin pigmentation.

Diagnosis

To diagnose fibrous dysplasia is possible by means of x-ray machine and histological analysis. Histological verification note the continuity of the changes in the tissue during puberty, which often comes infected before they are due. During the examination x-rays reveal lesions filled with different density of bone tissue. In some cases, it becomes noticeable pigmentation of the skin over the affected area.

Treatment

In the vast majority of cases, if the deviation is not pronounced, and diagnostics carried out at an early stage if the disease is fibrous dysplasia, the treatment gives positive results. Some patients will be fairly standard therapy, and for treatment of other is necessary to apply the scraping and plasticity of grafts. The failures are because of resorption of graft and recurrence of the initially diagnosed by symptoms.

Fibrous dysplasia of the femur in a running state leads to a change called a shepherd’s stick. Upon such a violation, it is necessary to conduct a local surgery to remove the deformed area and replacing it with a suitable graft. In the absence of pathology and recurrence of treatment is successful.

Fibrous dysplasia of the skull in disrepair is transformed into a variation called livenote face. With such a localization of the disease like bone bulges. If discovered this fibrous dysplasia in children, shown lightweight surgical intervention to eliminate further deformation of the facial bones, while their subsequent growth and development the growth of the patient.

Fibrous dysplasia of the tibia externally manifested by lameness and gait disorders rarely notice the shortening of the limb in which the affected bone. The treatment also requires surgery, plastics and further observations. After surgery patients have to use crutches and orthopedic devices to full or partial recovery.

The extent, duration and intensity of therapy can set only a doctor specializing in the treatment of this disease. The method of treatment chosen for each patient individually exclusively. For children, due to the fragility and insufficient development of the skeletal system of a child organism, first used in therapy and only if it is not effective, surgery. In an adult patient diagnosed with the severity and the neglect of developing the disease. In most known examples the operation is performed immediately. With the defeat of one of her bones removed and replaced with graft. When multiple lesions require a more lengthy and serious treatment with application of high-tech devices.

In General, after the therapy, the operation and implementation of the transplant patient gets much better. But we cannot stop here. Need constant supervision of a specialist, which will reduce the likelihood of relapse, until complete recovery of the patient.