Pierre robin sequence ultrasound11/4/2023 The jaw may surgically need to be brought forward over a given period of time in order for the airway to be more open (mandibular distraction). The most significant and immediate consideration in PRS addressed immediately is the airway to determine the degree of breathing difficulty. A number of the clinical features of Pierre Robin sequence (PRS) can be treated through certain therapies and/or surgeries. Infants and children with Pierre Robin sequence need to be evaluated and managed by a Craniofacial Team for planning and implementation of treatment. The diagnosis of Pierre Robin sequence or syndrome is made based on the clinical findings described in the definition above. Stickler syndrome, Treacher Collins syndrome, Velocardiofacial syndrome, and fetal alcohol syndrome are a few of the conditions that sometimes exhibit the features characteristic of Pierre Robin. Pierre Robin sequence may be an isolated finding or it can sometimes be part of a genetic syndrome or chromosomal abnormality. There is no evidence that prenatal exposure or inter-utero trauma is related to the disorder. Why this sequence of events occurs is not clearly understood. Thus, the palate is not able to fuse and the child will be born with a cleft palate. The tongue, as a result, remains back in the mouth cavity and becomes forced up into the roof of the mouth. For reasons yet unclear, the head of the fetus does not rise off the chest causing forward growth of the lower jaw to be restricted. However, poor development of the lower jaw during the 6th to 11th week of fetal life seems to be a likely explanation. The causes for the disorder are not entirely understood. In 14% of cases, children with Pierre Robin have congenital heart disease. Other common difficulties affected children face include reflux (due to negative pressure created within the chest cavity from obstructed airways), ear infections (as a result of the cleft palate), frequent colds, oral defensiveness (as a result of reduced oral stimulation from extended tube feeding), speech problems, and overcrowded teeth. Their bodies expend an unusually high number of calories doing so this often leads to weight loss and a failure to thrive. Because affected children have to work overtime to get the oxygen they need, food and liquids tend to make it more difficult for the children to obtain enough oxygen. Feeding problems are usually related to these breathing difficulties. This can be very dangerous for a child if he or she is not receiving enough oxygen to the brain. Because the tongue is seated back in the mouth cavity, it tends to fall back and block the airway. Quite often, breathing difficulties are a serious problem for affected children. Pierre Robin sequence varies from child to child. Until more information is gained, however, the term syndrome cannot be entirely ruled out. Although the causes of Pierre Robin are not entirely understood, the features appear to be the results of a series of events - thus making it a sequence. A sequence, on the other hand, is a pattern of problems that are believed to have occurred from a single event. Although the two names are used interchangeably, there is a slight difference between a sequence and a syndrome that requires clarification.Ī syndrome is a condition recognized by multiple factors that are present and related to the same genetic condition. Pierre Robin sequence (or syndrome) is a congenital disorder that presents as a group of problems including a wide U-shaped cleft palate, a small, recessed lower jaw (micrognathia), and some degree of airway obstruction due to a downwardly displaced tongue (glossoptosis).ĭifferent people will refer to this particular disorder as either Pierre Robin sequence or Pierre Robin syndrome.
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