Fibrous dysplasia of bones: tibia, skull, and other

Fibrous dysplasia of bone: a modern treatment of severe pathology

People can walk, write and control your body on their own, thanks to the musculoskeletal system. The skeleton plays an important role in the human body was sturdy and reliable.

Therefore, it is important to know about diseases associated with the skeletal system.

What is the fibrous osteodysplasia?

Despite the fact that this is a fairly rare disease, it was studied by scientists from around the world. After hearing from one doctor diagnosis of fibrous osteodysplasia – disease Braitseva of Liechtenstein, the third – pathology Jaffe-Lichtenstein, you know, they are talking about one and the same disease.

The essence of the pathology is the failure in bone formation due to impaired development of epiphyseal plate during bone growth. Lost the ability of osteoblastic conversion of mesenchyme into bone tissue.

Patients bone is converted into a sort of fibrous substance. The simplest bone structure is radically changed, they contain a cartilage on or osteoid substance. Formed unusual bone with fibrotic marrow.

In the affected portions of the skeleton instead of bone appears dense, elastic, especially education. Loose weight containing, instead of the lamellar structure of spindle-shaped cells, provokes a kind of Islands of cartilage.

In some cases, the presence of separate huge cells of osteoclast type. Fibrous tissue can migrate from the epiphyseal plate to spread and cause osteoid tissue, forming a bone with incomplete type of bone formation.

The causes of the disease

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The question of the origin of fibrous dysplasia of bone is still a matter of debate in the medical scientific community.

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Despite the variety of interpretations, most scientists loitering in the direction of the theory of the pathogenesis of the disease. Seeing it as a congenital malformation of bone tissue.

Most modern and common believe the doctrine of the dysplastic nature of the development of fibrous osteodysplasia, considering the probability of conversion of immature bone tissue in a tumor.

Violation of the endocrine, Central nervous system, or the system: hypophysis – adrenal cortex, as shown by clinical observations, are a major cause of the disease.

Violations result from a natural but extremely distorted and delayed, abnormal development of embryonic osteoblastic mesenchyme.

Cells lose the ability to transform into normal osteoblasts.

The process of ossification of the skeleton, occurring at the time of embryonic formation, is extremely anomalous. To hurt the child begins in the womb, during the initial formation of bone bodies, although symptoms may not be manifested immediately after birth.

Classification of the disease

Prognosis and treatment largely depend on the number of affected bones.

There are four forms of the disease according to the number of affected bones:

  1. Monoscale form – struck one bone. Occurs in people of all ages. Particularly vulnerable to the pathology of the elderly, more common in men than women.
  2. Oligosaline form – the defeat of two or three bones. As a rule, it affects certain adjacent areas of the skeleton (for example, adjacent the ribs or the bones of the skull).
  3. Polisario shape – lose more than three bones. In this notice, often large nekotrye changes. See also the absence of extraskeletal lesions.
  4. Albright’s syndrome – the bone lesions are accompanied by skin pigmentation and endocrine disorders. In some cases, in girls, there is early puberty.

Often, patients are unaware of their pathologies. In the initial stage of the disease develops without subjective symptoms or objective clinical signs.

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The main symptoms

To signs of pathology include:

  1. Deformation of bones. The curvature can be barely noticeable or significant (up to changes of the whole limb segment). Thickening or bone lesions depends on how long the disease and its localization.In some cases, this symptom is not accompanied by pain. In other appears with it, or much later.

  2. Pathological fractures. In the intergrowth of the bones are formed of degenerative zone adjustment. Frequent pain. May be a cracked bone, intraosseous hemorrhage, occurring even in the absence of overload.
  3. Lameness. Is caused by the change of limb length (lengthening or shortening). Observed pain in the affected area.
  4. Hormonal disturbances. Underthebridge or freckled spots of brown coloring on the skin of buttocks, neck nape, etc. Often there is an adenoma of the thyroid gland, hyperthyroidism. For girls, early development of sexual characteristics, premature menstruation. Boys – excessively fast growth, early coarsening of voice, etc.

The paucity of clinical symptoms complicates diagnosis of the disease in the early stages of development. In this case the laboratory tests will not show abnormalities as the metabolic processes are not violated.

Fibrous dysplasia of the bones detected by x-rays. In the picture the doctors can see the area of the diffuse enlightenment of bone tissue. Diagnosis must be timely, as well as treatment, as this disease is classed as precancerous processes.

Therapy

Treatment of pathological bone formation is the use of standard orthopedic measures: strengthening the musculoskeletal system, prevention of fractures, strengthening of the skeleton, etc.

Recommend to consume a lot of vitamins, regularly massage, sea bathing, conduct therapeutic exercises.

To resort to operative interference should be only if extensive deformations when drug treatment is not effective.

Prosthetics is appointed by the pronounced pathology.

The consequences of running pathology

Untimely appeal to the doctor provokes the following complications:

  1. Malignant transformation of the tumor, chondrosarcoma, osteosarcoma, cm.
  2. Against the background of developing the disease and related pathology – hearing, sight, appear headaches, develops rickets, disturbed heart rhythm.
  3. Gigantism and acromegaly – increased growth hormone activity.

The doctors ‘ prognosis is often disappointing. Although, in most cases, this is not a fatal disease, but often leads to disability.

On the background of frequent fractures disrupted the natural fixation of the skeleton, disrupted the overall proportion of the body structure.

At the same time, with a single or small foci of fibrous dysplasia of the bones, the Outlook is positive. It is not dangerous and is not a threat.

Accounting for about 5.5% of all bone diseases and 9.5% of benign bone tumors (data provided by M. V. Volkov) disease, however, deserves more attention. Every year it hurts more and more children and elderly.

Malformed bone bodies lower support function of the skeleton. This anomaly is accompanied by not only the deformation bones, but also frequent fractures. When multiple localization can lead to disability.