What harm can micrognathia syndrome have?

summary

Micrognathia syndrome is a syndrome characterized by congenital micrognathia, glossoptosis, cleft palate and inspiratory airway obstruction in infants, also known as cleft palate micrognathia glossoptosis syndrome, micrognathia glossoptosis syndrome, micrognathia megaglossia syndrome, inspiratory airway obstruction syndrome, Robin syndrome, Pierre Robin syndrome, etc. Respiratory tract obstruction caused by this disease can cause death, the mortality rate is as high as 30% - 65%, accounting for about 1 / 5 of the newborn.

What harm can micrognathia syndrome have?

Tongue droop, respiratory tract obstruction, the root of the tongue under normal circumstances depends on the mandibular lingual muscle traction support of the mandibular chin joint, so it can be in the anterior position. When the mandible is moved backward deformity, the root of the tongue loses its support, and the oropharyngeal isthmus shrinks and is blocked, causing airway obstruction. Due to airway obstruction and breast-feeding disorder, the children may have compensatory inspiratory action and sucking force, which will increase the negative pressure in the hypopharynx, chest and esophagus and force the base of tongue to hang back. At the same time, a lot of air into the stomach, can cause nausea. Vomit volume is inhaled into the lower respiratory tract, resulting in aspiration pneumonia or atelectasis. The degree of obstruction of respiratory tract is very different in this disease. In mild cases, there is inspiratory wheezing only in supine position, while in sober or crying, the airway is basically unobstructed, the respiratory obstruction is mostly silent, and the wheezing is different from laryngogenic wheezing.

It may be accompanied by eye defects, skeletal deformities, auricle deformities, deafness caused by structural abnormalities of middle and inner ear, hypertrophy of proliferator, congenital heart disease and mental retardation. Typical cases have inspiratory airway obstruction since birth, sometimes accompanied by wheezing, cyanosis, aspiration depression under ribs and sternum, which is caused by hypoplasia of mandible, cleft palate, large tongue space and backward drooping displacement. Due to the more serious symptoms in supine position, such children often have feeding difficulties, not easy to suck, swallow, easy to cough, resulting in malnutrition, weight gain, slow growth. As a result of cleft palate, food is easy to choke into the trachea and eustachian tube, easy to complicated with aspiration pneumonia and otitis media.

The results showed that the leukocyte count and neutrophil count of peripheral blood increased significantly in patients with infection; When hypoxemia occurred, PaO2 decreased and PaCO2 increased. X-ray, echocardiography and ECG were selected according to the clinical examination.

matters needing attention

The prognosis was poor. The children died early due to feeding difficulties, malnutrition, respiratory distress, pulmonary infection and cardiovascular malformations. Preventive measures are the same as other birth defects. In order to reduce and reverse the incidence of birth defects, prevention should be carried out from pre pregnancy to prenatal.