Roben's syndrome

Description:

Roben's syndrome — the inborn defect of maxillofacial area which is characterized by three main clinical signs: mandible underdevelopment (lower micrognatia), glossoptosia (underdevelopment and sticking of language) and existence of a crevice of the sky. Robena a syndrome meets on each 10 — 30 thousand newborns.

Symptoms of the Syndrome of Roben:

At once after the birth there is a sharp disturbance of breath connected with retraction of language. The child is uneasy, cyanosis is expressed. Because of disturbance of the act of swallowing during feeding of the child there can come suffocation. Outward of the newborn — the so-called "bird's person" arising in connection with a mandible underdevelopment is characteristic (from poorly expressed underdevelopment to its total absence). Along with a typical triad (the lower micrognatia, a glossoptosia and a crevice of the sky) patients with Roben's syndrome have also other malformations (an inborn cataract, a myopia, heart diseases, urinogenital system, anomaly of development of a breast and a backbone, and also a polydactylia (presence of excess fingers) and inborn lack of extremities). The intellectual underdevelopment is noted approximately at 20% of patients. The diagnosis is based on clinical manifestations of this syndrome.

Reasons of the Syndrome of Roben:

Factors of emergence and the mechanism of development are not found out. There are instructions on the fact that of a syndrome the disturbances of embryonic development caused by various pathology of the prenatal period are the cornerstone of Roben.

Treatment of the Syndrome of Roben:

Treatment has to be carried out practically right after the birth. In mild cases for prevention of disturbance of breath it is enough to keep the newborn in a ventral decubitus or vertically, having inclined the head to a breast. When feeding it is impossible to hold the child horizontally because of danger of hit of food in respiratory tracts and development of aspiration pneumonia with the subsequent death.

At sharply expressed lower micrognatia (an underdevelopment of a mandible and the considerable shift of language of a kzada) removal of language forward in physiological situation is required in the operational way. It is recommended to sew language to an under lip, cheeks or a mandible in several points for a period of 1 — 2 month. In hard cases the gastrostomy is imposed.

At more advanced age eliminate a sky crevice, carry out orthopedic and operational treatment of the lower micrognatia. Occupations with the logopedist for establishment of the correct speech are shown. Prevention consists in an exception of all adverse factors in the prenatal period of development of the child.

There is an alternative of a distraktsionny method of treatment. The essence of a method is an installation of the payment which is individually made of plastic which fastens on prosthetic glue on an upper jaw. This payment presses on a language root, thereby pushing a jaw forward, and also closes a crevice, solving problems with food, breath and all other difficulties.