Congenital malformation of bones in children Chondrodystrophy

The term "chondrodystrophy" refers to a systemic disease that is congenital in nature and is associated with impaired development of the cartilage system of the embryo.

The formation of a blemish occurs already with 3-4 weeks of life of the embryo, and some of the fruits die even during intrauterine development.

Preterm infants with this disease are viable enough. If the pathology is detected before delivery, a cesarean section is performed to prevent complex birth injuries.

Species of the pathology of

Chondrodystrophy is considered one of the most common congenital disorders of skeletal development.

As a result of the development of this pathology, the patients are characterized by disproportionate dwarfism.

The essence of this disease was described by Kaufman, who studied this pathology in detail.

In particular, the scientist has established several forms of chondrodystrophy:

• hypoplastic - in this case there is insufficient development of cartilaginous tissue, as well as a decrease in epiphyses;
• hyperplastic - it is characterized by rapid disordered growth of epiphyseal cartilage, as well as an increase in epiphyses;
• malation - gelatin-like softening of the cartilage is observed in this situation.

In most cases, the hypoplastic form is diagnosed.

Sometimes three types of cartilage tissue change are observed at once.

In recent years, there are quite a few atypical forms of pathology, which have little common signs with the classic kind of disease.

What happens - let's understand

The essence of chondrodystrophy is the violation of the endochronic development of bones.

When the pathological process develops, the integrity of the bones that develops from the chondroblastic system is affected.

In this case, the development of bone tissue, which differs from connective tissue origin, remains normal.

In the photo a child with a diagnosis of chondrodystrophy

This means that when the anomaly develops, the bones of the extremities and the base of the cranium are affected. In this case, periosteal and endosteal growth remains normal.

From the point of view of histology, an abnormality can be defined as the abnormal location of cells of the germ cell cartilage. There is also a different degree of degeneration.

In this case, the cartilage cells are not formed. There are no zones of preparatory calcification. Therefore, the cartilaginous nodes penetrate into the medullary tissue, and the bone marrow and vessels exit into the bone tissue of the epiphyses.

Where is the primary source of chondrodystrophy?

This pathology is rare.

The onset of the disease occurs at an early stage of embryo development. However, his etiology remains unknown.

Individual experts claim that the cause of chondrodystrophy is the intoxication of the body.

To development of pathology can lead nicotinism or alcoholism of future parents. Also, the cause can be hidden in the infectious pathologies of the mother during the child's bearing. Sometimes it leads to syphilis and endocrine pathologies.

Jansen believes that pathology is the result of disharmony in the formation of the fetus and amnion. According to observations of some specialists, it was possible to establish the fact that the disease can be transmitted by inheritance.

Sporadic cases of pathology can also be observed. There is a theory according to which the cause of the disease lies in diencephalo-hormonal disorders.

Clinical picture

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

They also have an impressive head size and are characterized by protruding frontal and parietal tubercles. They hang over a face that has a relatively small size.

In addition, there is a saddle and fairly wide nose.

Symmetric changes usually occur in the bone tissue. With the distorted development of bone tissue, the epiphyses deform along the epiphyseal plate. Because of this, there are varus and valgus deformities of the legs.

Symptoms are especially worse after the baby starts walking.

This disease is curved and curled inside the hip bone. On the spine there are no normal bends, which causes the development of an absolutely flat back.

Sometimes the physiological lordosis in the lower back is increased. The brushes are square in shape, since the fingers have the same length.

Muscles develop without deviations, and therefore for patients with chondrodystrophy often characterized by a great physical strength.

Diagnostic approach

Diagnosis of pathology is not particularly difficult, since the diagnosis can easily be made based on the characteristic

. Square brushes are a common occurrence in the anomaly of

changes in the appearance of a 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 young children, chondrodystrophy should be distinguished from rickets. On the basis of a limb shortening, radiography data and the failure of vitamin D, an accurate diagnosis can be made.

Aims and methods of treatment

The main goal of therapy is to prevent and eliminate deformities of the hands and feet. At the same time there are no radical methods of fighting this disease.

Orthopedic treatment of chondrodystrophy consists in the use of orthopedic devices. Osteotomy is also performed,

Osteotomy is one of the ways of treating the

disease which allows to correct bends.

With general physical weakness and backlog in bone formation, the treatment consists of sea bathing, finding on the seashore, using sun baths. Also, iron preparations and vitamin complexes can be prescribed.

In each case of pathology, an attempt should be made to stimulate the growth of bones. This is done by administering the hormone of the anterior lobe of the pituitary gland, called the somatotropin .However, there are no long-term observations regarding the action of this hormone.

Stimulating growth through the use of bone pins that are introduced into the metaphyses of long bones does not yield tangible results.

Leg deformities in chondrodystrophy are eliminated by all kinds of osteotomies. This can be done only after the process of bone growth has been completed.

This can be judged by the state of epiphyseal ossification. With pronounced kyphosis, prolonged reclamation is applied in the plaster bed. To eliminate clubfoot, conventional methods are used.

If painful arthrosis of large joints is observed in adult patients with impairment, temporary rest is indicated. Thermal procedures are also carried out. Mud treatment is especially useful.

What determines the success of the treatment?

Provided adequate therapy for this disease and prevention of possible complications, the prognosis for life of patients with chondrodystrophy is favorable.

A rare congenital disease, the exact causes of which have not yet been established. To prevent the development of complications and maintain the normal quality of life of such patients, they must be constantly monitored by a doctor.