Crouzon's syndrome: clinical picture and treatment

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2018-03-22 20:44:09

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Crouzon Syndrome is a rare genetic disease.

crouzon syndrome

General feature

The Disease is characterized by facial deformities that occur during fetal development. The Gill arch of the human embryo is a transitional education, which later formed the vital organs, including the jaw. Impaired development of the branchial arches and leads to disease, which got its named after the French physician, first described it, Octave Crouzon. He first noted that the disease is genetic in nature: his patients were mother and daughter. This maxillo-facial disorder characterized by a number of characteristics. Naturally, the problem lies in the bones of the skull: for some reason the child they can be formed properly. For example, the Crouzon syndrome may manifest itself in the fusion of metopic, coronal, or sagittal suture, premature contraction of the coronal suture or of all seams.

crouzon syndrome photo

Symptoms

The Syndrome of Crouzon different pronounced symptoms. Young patients differ, first and foremost, too low set ears. Usually that's what caused all the problems with hearing arising in the future. In severe cases, the disease transformirovalsya in Meniere's disease. Among the characteristic signs include the brachycephalic, that is, short and broad neck, exophthalmos, is the bulging of the eye (this is due to the too small eyes), hypertelorism, that is too large, the distance between the eyes, small nose, resembling the beak. Common, but not obligatory symptoms are strabismus and underdevelopment of the maxilla. Underdevelopment of the jaw leads to the fact that the chin protrudes sharply. All these features characterize the syndrome of Crouzon (photos of patients you see in the article). For still unknown reasons, most patients have disproportionately narrow hips and shoulders.

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Diagnosis

As a rule, immediately after the appearance of the baby born can be diagnosed with the syndrome of Crouzon. The causes of the disease are not known. To confirm the presence of the disease, you will need to run some tests: x-rays, magnetic resonance imaging, fundus examination, genetic testing and scanning of the cerebral cortex.

Frequency

It is Worth noting that this disease is very rare: according to statistical studies, 25 of thousands of healthy people have one patient, and with high probability the family was already people suffering from the disease.

crouzon syndrome causes

Treatment

In General, the prognosis depends on how much deformation of the skull affected the condition of the tissues of the brain. In the first year of life on the skull of the child can be undertaken reconstructing surgery. As the baby grows, you may need a new operation: bones, jaws, soft tissues of the face. In some cases after surgery, the patient has to wear a specially made helmet. The main problem lies in the fact that the disease is often accompanied by psychomotor retardation and seizures.


Article in other languages:

AR: https://www.tostpost.com/ar/health/5425-crouzon-s-syndrome-clinical-picture-and-treatment.html

BE: https://www.tostpost.com/be/zdaro-e/9624-s-ndrom-kruzona-kl-n-chnaya-karc-na-lyachenne.html

DE: https://www.tostpost.com/de/gesundheit/9622-kruzona-syndrom-das-krankheitsbild-und-die-behandlung.html

ES: https://www.tostpost.com/es/la-salud/9630-el-s-ndrome-de-kruzona-cuadro-cl-nico-y-el-tratamiento-de.html

HI: https://www.tostpost.com/hi/health/5428-crouzon.html

JA: https://www.tostpost.com/ja/health/5426-crouzon.html

KK: https://www.tostpost.com/kk/densauly/9626-sindromy-kruzona-klinikaly-suret-zh-ne-emdeu.html

PL: https://www.tostpost.com/pl/zdrowie/9628-zesp-kruzona-obraz-kliniczny-i-leczenie.html

PT: https://www.tostpost.com/pt/sa-de/9624-a-s-ndrome-de-kruzona-quadro-cl-nico-e-tratamento.html

TR: https://www.tostpost.com/tr/sa-l-k/9630-kruzona-sendromu-klinik-tablo-ve-tedavi.html

UK: https://www.tostpost.com/uk/zdorov-ya/9628-sindrom-kruzona-kl-n-chna-kartina-l-kuvannya.html

ZH: https://www.tostpost.com/zh/health/5914-crouzon-s-syndrome-clinical-picture-and-treatment.html






Alin Trodden - author of the article, editor
"Hi, I'm Alin Trodden. I write texts, read books, and look for impressions. And I'm not bad at telling you about it. I am always happy to participate in interesting projects."

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