Chondrodystrophy: causes, symptoms and treatment, photos of sick children

Congenital malformation of bones in children chondrodystrophy

The term «chondrodystrophy» understand a systemic disease, which is congenital in nature and is associated with impaired development of the cartilaginous system of the embryo.

The formation of defect occurs from as early as 3-4 weeks of the life of the embryo, and some fruits perish even during prenatal development.

Full-term babies with this disease are quite viable. If pathology is detected before birth, performed a cesarean section to prevent birth injuries difficult.

Varieties of pathology

One of the most common congenital disorders of skeletal development is chondrodystrophy.

The development of this pathology for patients characterized by disproportionate dwarfism.

The essence of this disease has been described by Kaufman, who has extensively studied this pathology.

In particular, he has noticed several forms of chondrodystrophy:

  • hypoplastic – in this case, there is a lack of development of cartilage, and reduction of the epiphyses;
  • hyperplastic – it is characterized by a rapid chaotic growth of the epiphyseal cartilage, and the increase of the epiphyses;
  • malacina – in this situation, there is topped gelatinous softening of the cartilage.

In most cases, is diagnosed hypoplastic form.

Sometimes there is just three types of changes of cartilage.

In recent years, there is quite a lot of atypical forms of pathology that have little common features with the classical form of the disease.

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The essence of chondrodystrophy is in violation encontrarnos development of bones.

With the development of the pathological process affects the integrity of bones, which develop from chondroblasts system.

The development of bone tissue, which is connective tissue origin, is normal.

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This means that during the development of the anomaly there is a loss of the bones of the limbs and the base of the skull. While periosteal and endosteally growth remains normal.

From the point of view of histology anomaly can be defined as the abnormal arrangement of cells in growing cartilage. Also there is a different degree of degeneration.

Is not formed columns of cartilage cells. No preparetomove areas of calcification. So cartilaginous nodes penetrate into the medullar tissue, and bone marrow and vessels leave the bone tissue of the epiphyses.

Where to find the source of chondrodystrophy?

This pathology develops rarely.

It should be noted that among the patients slightly more often women of color.

Its onset occurs at an early stage of development of the embryo. While its etiology remains unknown.

Some experts argue that the reason for chondrodystrophy is intoxication.

To the development of pathology can lead nicotinism or alcoholism of the parents. Also the reason may lie in the infectious pathology of the mother during pregnancy. Sometimes it causes syphilis and endocrine pathology.

Jansen believes that pathology is the result of disharmony in the formation of the fetus and amnion. According to the observations of some specialists were able to establish the fact that the disease can be inherited.

There may also be sporadic cases of disease. There is a theory that the cause of the disease lies in diencephalon-hormonal disorders.

The clinical picture

People with chondrodystrophy have a fairly typical appearance. They are inherent shortening of the upper and lower extremities. In this case, is dominated by the shortening of the hips and shoulders in the background of normal body size.

Also such people have a head is sizeable, and it is characterized by a protruding frontal and parietal hillocks. They hang over the face, which is relatively small.

In addition, there is saddle-shaped and rather broad nose.

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In the bone typically occur a symmetric change. If perverted the development of bone tissue at the epiphyseal plate epiphyses deformed. This raises varus and valgus leg deformities.

Symptoms particularly intensify after the child begins to walk.

In this disease are distorted and curl inside thigh bone. On the spine there are no normal curves, which causes the development of totally flat back.

Sometimes there is a physiological lordosis in the lumbar region. Brushes have a square shape, because the toes have the same length.

Muscles develop without deviation, and therefore for patients with chondrodystrophy is often characterized by a large physical force.

Diagnostic approach

Diagnosis of pathology does not cause much difficulty, since the diagnosis can easily be put on the basis of the characteristic

changes the appearance of the person.

Radiography can detect changes in the development of the bones of the hands and feet. They are characterized by a sharp shortening and thickening.

In children of early age the chondrodystrophy has to be distinguished from rickets. On the basis of shortening of the limb, x-ray data and the failure of the use of vitamin D cannot make an accurate diagnosis.

The purposes and methods of treatment

The main goal of the therapy is in the prevention and elimination of deformation of the hands and feet. While radical methods of fighting this disease is currently lacking.

Orthopedic treatment of chondrodystrophy is to use orthopedic devices. Also performed osteotomy,

which allows to correct the curvature.

When the total of physical weakness and delayed bone formation in the treatment is sea bathing, being on the sea shore, the use of sunbathing. Can also be prescribed iron supplements and vitamin complexes.

In each case, pathology must be an attempt to stimulate bone growth. This is done using the introduction of the hormone of the anterior pituitary, which is called somatotropin. However, long-term observations on the actions of this hormone are not available.

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Encourage growth by the use of bone pins which are introduced into metaphysi long bones, does not produce tangible results.

Foot deformities if chondrodystrophy removed by various osteotomies. This can be done only after the completion of bone growth.

About it is possible to judge the state of epiphyseal ossification. In severe kyphosis is used long reclinate in a plaster bed. To eliminate clubfoot used conventional methods.

If you experience painful arthrosis of large joints of adult patients suffering from a violation, shows a temporary rest. Also performed thermal treatments. It is particularly useful to mud.

During pregnancy, women suffering from disease, should be under medical supervision. It is important to remember the complexity of natural childbirth in this case. Therefore, the specialist must consider the need for caesarean section.

What determines the success of treatment?

With adequate therapy of this disease and prevent possible complications the prognosis in patients with chondrodystrophy is favorable.

Quite a rare congenital disease, the exact cause of which is still not installed. To prevent complications and to maintain a normal quality of life for these patients, they should always be under the supervision of a physician.