![]() This often does not resolve with correction of airway obstruction and leads to failure to thrive without intervention. Feeding difficulty: PRS patients are unable to coordinate suck and swallow and breathing due to airway obstruction and/or concomitant cleft palate.Breathing difficulty is secondary to partial (retraction, stridor, stertor) or complete airway obstruction that may be severe enough to require endotracheal intubation.Obvious airway anomalies include a small chin and cleft palate (Figure 1). There is wide variability in the clinical presentation depending on the degree of micrognathia and airway obstruction.Less common: skeletal dysplasias, dysmorphic monogenic conditions such as Treacher-Collins syndrome, intrauterine exposures such as fetal alcohol syndrome, and maternal diabetes.Most common: Stickler syndrome, velocardiofacial (22q, 11.2 deletion, DiGeorge) syndrome.More than 40 clinical syndromes have been associated with PRS.More than half of PRS newborns have an associated syndrome, chromosomal abnormality, or other additional anomalies. Although no clear genetic abnormality has been identified, the etiology of the micrognathia may be different for syndromic and nonsyndromic PRS.Incidence of PRS ranges from 1:5000 to 1:85,000 secondary to tremendous heterogeneity in clinical presentation and lack of uniform diagnostic criteria.3 In clinical syndromes, a set of anomalies arise separately due to a common underlying pathogenesis. ![]() PRS is termed a sequence because one anomaly (micrognathia) leads to a sequential chain of events causing the other anomalies.The primary pathogenetic event that leads to PRS is unknown but generally accepted to be micrognathia causing upward and posterior displacement of the tongue, preventing closure of the palatine shelves before the 10th week gestation.Although a cleft palate is common (up to 90% of cases), it is not required for the diagnosis.glossoptosis (downward and backward displacement of the base of the tongue).micrognathia (small, symmetrically receded mandible).Pierre Robin in 1923, 1,2 PRS refers to the clinical triad of: ![]()
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