Harlequin syndrome: symptoms, features and treatment

Newborn babies are very defenseless. Even in the womb, they can be influenced by various negative factors that cause pathologies of the development of crumbs. As a result, being born, the little one has many problems. One of the worst deviations is Harlequin syndrome. The disease was named after the character of the Italian comedy of masks, who wore a suit of multi-colored shreds of fabric.

Harlequin syndrome in a newborn

Doctors discover the problem immediately after the birth of the baby. According to them, Harlequin syndrome can be explained by the imperfection of the development of the autonomic system, which ceases to control the correct vascular tone. The disease can also be triggered by simple gravity. Why is this happening? Doctors cannot give a definite answer to an exciting question. The range of reasons is very wide: from a simple malfunction in the development of the fetus to the wrong lifestyle that a pregnant woman leads.

harlequin syndrome

The syndrome can occur both throughout the body and only in certain parts of the body. Such babies, doctors say, will constantly suffer from colds, since their thermoregulation is impaired. The peanut will sweat profusely at any time of the year, then become very cold, as a result of which he will be provided with constant viral infections. In addition, the syndrome causes a lot of discomfort, so the child often cries, he is restless and irritable.

Symptoms

According to some reports, the syndrome can be a consequence of fetal asphyxiation or an intracranial injury during delivery. It is also diagnosed in cases of damage to the hypothalamus or medulla oblongata. In any case, a neurologist is immediately called for a consultation. It is quite simple to detect the disease: its main symptom is redness of the skin, when the crumb lies on its side. In this case, the border of the color change passes exactly along the line of the spine.

harlequin syndrome photo

Unequal color of body parts occurs a few minutes after laying the baby. This is how Harlequin's syndrome manifests itself. Photos of young children in medical encyclopedias show an unsightly picture - the upper part of their body has a normal color, the underlying one becomes bright red, sometimes with a bluish tint. The phenomenon is noticeable for several minutes. Some experts say the syndrome may be a sign of prematurity.

Skin manifestations

If medical terms are used to describe the disease, then Harlequin syndrome manifests itself as compact keratinization of the skin. There is no so-called granular layer on it; instead, plaques are formed that form when the epidermis is cracked. These formations have a hexagonal shape, often they are separated by deep red cracks. The skin color is gray or icteric.

Children born with this syndrome often have other abnormalities: they may have deformity of the mouth (it resembles fish lips), eversion of the eyelids, improperly developed, too flat ears. In some cases, microcephaly is diagnosed when the baby has an underdeveloped cranium: as a result, he is born with a very small brain. Such children die in the neonatal period or when they are born.

Features of the disease

Kids do not always die if they have Harlequin pathology. Syndrome is not a sentence. The disease may not be pronounced, moreover, if it is quickly and correctly diagnosed, it is possible to alleviate the condition of the crumbs with special drugs. In such children, not only heat transfer is disturbed, but also the electrolyte balance. Keratinized skin makes breathing and movement difficult. In large cracks, infection can get and sepsis develop.

harlequin syndrome in a newborn

Fortunately, the disease is not very common. At the same time, the sex of the baby does not matter: both boys and girls are affected by pathology. Doctors say that the syndrome is often severe. Moreover, the prognosis in most cases is rather unfavorable. The etiology of the syndrome has not yet been established.

Therapy

The baby is prescribed a course of treatment, which is aimed at maintaining a normal body temperature and stabilizing the water-electrolyte balance. In addition, such a patient requires a normal diet, anti-inflammatory, antibacterial therapy. The use of retinoids in the form of ointments and oral capsules significantly increases the survival rate if the baby is diagnosed with Harlequin syndrome. Treatment involves the use of drugs where the active active ingredients are isotretinoin and acitretin. Despite this, defects remain on the skin until the end of days. The quality of life in such patients is significantly reduced.

harlequin syndrome treatment

If the syndrome is not pronounced, then you can do without medications. It is enough for parents to maintain a normal temperature in the children's room - in the region of +20 degrees. You need to accustom the baby to climatic changes: first he must adapt to the cold and only then to heat. Thus, you will train the autonomic system and the walls of the vessels of the baby. Remember: if the baby is sweating, change clothes immediately. Such procedures will certainly give a positive result.


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