What Is Pierre Robin Syndrome?
Pierre Robin Syndrome which is most normally referred to as Pierre Robin Sequence is a Ernst Boris Chain of congenital developmental malformations in which one deformity is succeed by the other in a sequence and hence the name Pierre Robin Sequence . This condition is characterized by an abnormally diminished jaw , a knife which is displaced downwards , and a cleft roof of the mouth .
The root cause of Pierre Robin Syndrome has not yet been identified but there are many theories publish by various researchers regarding the cause of this condition . The characteristic features of a cleft palate and an abnormally small jaw is good enough to give the child a diagnosis of Pierre Robin Syndrome , although prenatally anultrasoundmay detect the comportment of this condition .
The treatment for this shape is symptomatic and is focused on easing the trouble with breathing that the shaver will have due to airway blockage as a result of Pierre Robin Syndrome .
What Causes Pierre Robin Syndrome?
As stated , the root grounds for the development of Pierre Robin Syndrome is not yet make love , although various theory have been put out in the lit as to the potential reason of this condition .
According to one of the theories , during the former stages of maternity when the organisation of bones occur , the tip of the jaw gets caught between the meeting detail of the collar bone the breastbone and thus the growth of the jaw ivory is hampered result in the underdevelopment of the jaw and the child having an abnormally small-scale jaw . Similarly in the grammatical case of the cleft roof of the mouth one theory express that the failure of the jaw to whole acquire in early pregnancy causes the tongue to be place quite in high spirits in the oral cavity which interfere with sodding palate stoppage resulting incleft palate .
As of recent , a genetic tie has also been identified pertaining to the cause Pierre Robin Syndrome . A defect in chromosome 2 has been identified to be the cause of the facial malformations that are characteristic of a child with Pierre Robin Syndrome .
It is believe that the condition is inherit as an autosomal recessive trait intend that a copy of the defective gene from both parents is required in the growing of Pierre Robin Syndrome .
What Are The Symptoms Of Pierre Robin Syndrome?
The classic presenting lineament of Pierre Robin Syndrome is the presence of an abnormally small jaw and other facial malformations like a cleft roof of the mouth and downward displace natural language . Upper airway obstruction due to the cleft roof of the mouth also may have external respiration problem which is quite a serious symptom that a child has to manage with in cases of Pierre Robin Syndrome .
A child with Pierre Robin Syndrome will also have problems with alimentation and also will not be able-bodied to reach the developmental milestones on time . As a result of external respiration problems , child with this condition also have to deal with a circumstance call obstructivesleep apneain which there is a impermanent intermission in ventilation while sleep induce quietus problem in minor with Pierre Robin Syndrome .
In some cases , tyke with Pierre Robin Syndrome also tend to have pulmonary hypertension as well as pulmonic stenosis . Otitis mediais also a circumstance which a child has to deal with oftentimes as a solution of Pierre Robin Syndrome .
How Is Pierre Robin Syndrome Treated?
The main focus of treatment in children with Pierre Robin Syndrome is to treat breathing difficulty that may arise as a final result of obstructed airways . Hence , a child with Pierre Robin Syndrome will be observed for any problems with respiration . In cases of breathing job , a tracheotomy may have to be insert to assist in ventilation till the time the airline obstacle is cleared and the child is able to breathe unremarkably .
For cleft roof of the mouth , a surgery will be postulate which is normally done after the baby is a duo of years old to correct the deformity . If the fissure roof of the mouth results in speech problems for the child then the medico may recommendspeech therapyto correct the speech .
A child with Pierre Robin Syndrome may have frequent capitulum infections and hence treatment for this condition is also mandatory to prevent any complication like hearing damage to manifest as a result of Pierre Robin Syndrome .
surgical process is not require to correct the small jaw since with historic period the size of the jaw becomes closer to normal . Above all , a genetic counseling is required for fellowship member of multitude who have small fry with Pierre Robin Syndrome .
