Pierre Robin Syndrome Case Study
Paediatrics is the medical science related to the diagnosis and treatment of childhood illness. One of the illness such as Pierre Robin Syndrome (PRS) also called Pierre Robin Complex or Sequence was identified by Pierre Robin a French stomatologist in 1923. That is a facial difference condition with a very small lower jaw (micrognathia or retrognathia) (Figure 1), a normal size of tongue but in the setting of a very small jaw that the tongue looks large tends to fall a downward or backward-positioned toward the throat (glossoptosis)(Figure 2) can act as an obstruction and result in a U-shaped cleft palate presented at birth (birth defect) (Redett, 2008). This malformation development occurs in the womb and approximately one in 8,500 to 14,000
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live-births (Redett, 2008). PRS was diagnosed by examining the infant and not by special diagnostic tests.
During a baby or infant, they must put in a prone left or right lateral face position to prevent tongue obstruct the airway causes apnea and breathing problem.
That is a primary concern, and may lead to hypoxia, failure to thrive and cerebral impairment. So, they can’t place on his/her back in supine position. However, the study was done at Hospital USM the researchers found PRS’s children did not respond to prone position but responded to supine position with slight tilting of the head was shown by increasing of oxygen saturation to 98% under room air could be maintained at greater than 95% (Figure 3) (Wong, Suzina, Hazama, & Irfan, 2011). Therefore, head rotation not only reduced the airway obstruction, it also reduced the incidence of aspiration (Wong, et al., 2011).
The PRS’s infants also have potential complications gastro-esophageal reflux causes aspiration pneumonia and difficulties during sucking, swallowing and feeding. These complications may increase the risk for mortality in early life. Closed monitoring and follow up most important to prevent complications and ensure adequate weight gain. Speech therapy can help PRS’s children to solve their problem with speech articulation difficulties causes’ abnormal speech.
Whereas, PRS’s children sometime presented with a small and curled epiglottis on itself (omega-shaped) is called laryngomalacia (Figure 4). That is a congenital abnormality of the laryngeal cartilage which approximation of the posterior edges of the epiglottis contributes to the inspiratory obstruction (Desir,
2015).
live-births (Redett, 2008). PRS was diagnosed by examining the infant and not by special diagnostic tests.
During a baby or infant, they must put in a prone left or right lateral face position to prevent tongue obstruct the airway causes apnea and breathing problem.
That is a primary concern, and may lead to hypoxia, failure to thrive and cerebral impairment. So, they can’t place on his/her back in supine position. However, the study was done at Hospital USM the researchers found PRS’s children did not respond to prone position but responded to supine position with slight tilting of the head was shown by increasing of oxygen saturation to 98% under room air could be maintained at greater than 95% (Figure 3) (Wong, Suzina, Hazama, & Irfan, 2011). Therefore, head rotation not only reduced the airway obstruction, it also reduced the incidence of aspiration (Wong, et al., 2011).
The PRS’s infants also have potential complications gastro-esophageal reflux causes aspiration pneumonia and difficulties during sucking, swallowing and feeding. These complications may increase the risk for mortality in early life. Closed monitoring and follow up most important to prevent complications and ensure adequate weight gain. Speech therapy can help PRS’s children to solve their problem with speech articulation difficulties causes’ abnormal speech.
Whereas, PRS’s children sometime presented with a small and curled epiglottis on itself (omega-shaped) is called laryngomalacia (Figure 4). That is a congenital abnormality of the laryngeal cartilage which approximation of the posterior edges of the epiglottis contributes to the inspiratory obstruction (Desir,
2015).