Traumatic clavicle distos (dysplasia syndrome Sheithauer-Marie-Santana): symptoms and treatment

Clavicular-cranial desotos: characteristic symptoms and treatment of dysplasia

Traumatic discos clavicle (clavicular dysplasia-cranial syndrome Sheithauer-Marie-Santana) – a disease that is related to mixed forms of diseases of the skeleton.

The problem is hereditary, and is transmitted in an autosomal dominant pattern. The disease is characterized by complete or partial absence of the clavicle, and also problems in the development of the skull bones.

The complete absence of clavicles in the medical practice occurs quite rarely – in 10% of cases. Most often the pathology is manifested as the absence of the clavicle, acromion of the division and reduced in size the blades.

The cause of the disease is disorders occurring in one of the genes on the sixth chromosome.

How it looks and manifests

Primarily, the clavicular-cranial desotos is characterized by variations of the clavicles, hypoplasia or complete absence of one.

In this disease the person can move clavicle to one another in such a way that it can each other touch.

Changes occur in the structure of the skull: the brain is significantly increased and reduced part of the facial. The Fontanelle may not close throughout life.

In view of these external characteristics, the person turns out to be very small, and the forehead and crown very large. Considering that this is a genetic disease and get sick it is usually whole families, such people often the entire family was engaged in circus activities.

The upper jaw is also changing: the hard palate shortened, the jaw is underdeveloped, shorter in size. Development permanent teeth can be delayed: for example, baby teeth can last up to 25-30 years of age.

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As a result, deformation of the clavicle, may be a pinched nerve plexus, which in turn can cause the underdevelopment of the muscles and General weakness of the upper extremities.

But that’s not all, a person with a traumatic clavicular dysplasia can be:

  • growth retardation;
  • hypertelorism is excessively increased distance between paired organs (abnormally wide-set eyes);
  • underdevelopment of the bones of the pelvis;
  • cleft palate (or arch of the sky);
  • later eruption of primary teeth;
  • ultracompact teeth, increased tooth decay;
  • reduced the rib cage;
  • kyphosis;
  • osteosclerosis;
  • scoliosis;
  • the result is a reduced bone mineralization occurs their breakage;
  • in some cases, possible deafness;
  • asymmetric length of fingers;
  • brachycephalic – korotkovolnovoi;
  • mental retardation is not observed.

Immediately after the appearance of a baby into the world the presence of a disease among other symptoms can say:

  • on palpation the bones of the skull are soft;
  • significantly increased range of motion (shoulder joints) due to the complete or partial absence of the clavicles.

To six to eight years of age for patients with this disease require x-ray examination, since until that time, abnormalities in the bone system no.

On reaching six years, the bones of the skull close to normal, while the bones of the pelvis become more typical appearance of this disease.

Medical care

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Medical assistance under this syndrome includes several stages.

Dental care

Clavicular cranial desotos is a disease that is not treatable, so doctors in this case is to fight with the manifestations of the anomaly.

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In childhood the most significant is the problem of teething and further fix their position. For this reason, patients with pathology will certainly need to turn to an orthodontist who will help with the help of plates to put the teeth into place.

Further, the work of dentists is to keep the baby teeth for a more comfortable and rapid eruption of permanent teeth (if such does not happen naturally, the doctor exposes them artificially).

Surgery

If the clavicle is completely absent surgical intervention is not assigned due to inappropriate. In such cases, the only possible conservative treatment.

If at least shortened the bone fragments are present, surgery is the place to be. In this case, it will be urgent surgery to repair the collarbone.

Surgery – especially individual case: if the patient (or patient’s parents) wish to resolve the problem, surgery will be scheduled, if not, the patient will be offered a conservative treatment.

Exercise therapy for treatment and recovery

For patients with traumatic clavicular dysplasia physiotherapy are imperative, as is weakened by anomalies of the upper limb must be strengthened.

Movement and exercise should be fully painted by individual program depending on the characteristics of the disease, and those areas of the body that should be pursued.

Vitaminization

Vitamins for patients is extremely important, especially for those who are involved in sports. Thus, strengthening the bone tissue and the whole organism.

Since the immunity of people and this kind of dysplasia is low, it is necessary to support special medicines and hardening, especially for the prevention of colds.

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Prevention of osteoporosis

From a very early age, the patient is required in the prevention of osteoporosis. You first need to increase the intake of calcium and vitamin D in the body. Mandatory sunbathing, and eating liver, sour cream, butter and sea fish.

If untreated, symptoms may appear more clearly, but time passed and repeated therapy will be key to the normal functioning of the limbs.

When choosing a career it is important to exclude the possibility of a heavy load on the shoulder girdle.