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Pierre-Robena's syndrome


Pierre Roben's syndrome represents set of three clinical signs: the inborn isolated sky crevice (usually, rather wide and extended), microgenias (reduction of the sizes of a mandible) and a glossoptosia (language root shift in the direction back).

Symptoms of the Syndrome of Pierre-Robena:

The main problem of the newborn with a syndrome Pierre Roben are episodes of an apnoea (apnoea), as a rule, arising in a dorsal decubitus. Besides, from the moment of the birth of the child and for the first 2 months of his life you can meet difficulties with feeding  and threat of aspiration (hit) of contents of an oral cavity in respiratory tracts with development of life-threatening aspiration complications of the kid (an apnoea, an acute bronchitis, pneumonia). For this reason newborns and children of the first months of life with Roben's syndrome need especially fixed observation and performance of a number of recommendations. Observing the recommendations submitted further, you will be able to avoid all possible problems.

Reasons of the Syndrome of Pierre-Robena:

The disease has genetically determined character. The probability of the birth of the child with this pathology averages 1 on 2000-8500 newborns.

Treatment of the Syndrome of Pierre-Robena:

Situation: optimum and vital position of the kid with a syndrome Roben is edgewise position or on a stomach with raised (or even fixed in such situation) the head. It allows to avoid obstruction (overshoot) of respiratory tracts the language root displaced back.

Feeding. At the satisfactory somatic status, despite objective difficulties, does not follow it is long to use probe food. Nevertheless, adaptation to other ways of feeding has to be carried out gradually, without being the reason of critical decrease in body weight of the kid. Optimum: to feed the child in edgewise position, semi-vertical, and sometimes practically in vertical, by means of a spoon (see the section the First steps) – at first with the smallest. From a spoon milk or mix has to get on droplets on mucous cheeks or language. At the same time control of the deglutitory movements and breath of the child has to be exercised. It is not necessary to fill in the large volume of mix (milk) in an oral cavity! Quite often, at the first stage invaluable help is given by the usual removable long latex nipples fixed on usual glass small bottles. Changing quantity and diameter of openings on a nipple, you can pick up a food flow, optimum for your child.

Pay attention: before feeding you have to be convinced of passability of the nasal courses, they should be cleared of the remains of food and crusts by means of the wadded turundochek impregnated with sterile oil. Important: When feeding the child together with food the considerable volume of air therefore it is reasonable to do breaks in feeding gets into a stomach and to keep the kid in vertical position before receiving an eructation air.

It is a little about nipples: in breaks between feedings surely offer the kid a baby's dummy and insist on its suction – it will allow to stimulate growth of a mandible.
Tactics of conservative treatment

The first two months of life of your kid can be rather difficult for you, however, further the situation will change to the best and you will forget about the problems connected with disturbances of breath and feedings. By the end of the first year of life, the mandible "will practically "catch up" with the normal parameters. And the sky plastics, also as well as at other types of crevices of the sky, will be executed in two stages: a staphyloplasty in 6-8 months, a palatoplasty in 12-14 months.

Dispensary observation of the child with a syndrome Pierre Roben within the first year of life is carried out by the maxillofacial surgeon, the pediatrician, the neurologist, the ENT specialist, and after 3-yokh years – the surgeon, the orthodontist (with performing orthodontic treatment - if necessary) and the logopedist (with carrying out logopedic training – if necessary). Frequency of dynamic surveys for the purpose of control of results of treatment is carried out not less than 1 time in 6 months – 1 year up to 18 years.

Pay attention: within the first 2 months of life of your kid it is especially important not to disregard it! Be attentive, being with the kid at home! At any changes in behavior or health of the kid – ask for medical care!

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