Background

Pierre Robin, a French stomatologist, was acknowledged for his recognition of the triad with the associated respiratory and nutritional problems.1 PRS affects 1 in every 20 002 to 30,0003 live births. Randall et al.1 observed true micrognathia in only two-thirds of their PRS patients and suggested that mandibular retrognathia is a more important cause of upper airway obstruction than micrognathia. The cleft palate, when present, is usually a U-shaped palatal defect. During embryogenesis, the malpositioned mandible prevents the tongue from descending below the palatal plane and interferes with the palatal fusion in the midline. Although the tongue is of normal size, it is prolapsed into the nasopharynx and posterior oropharynx. Whether this process occurs as a result of hereditary influence, chromosomal disorder, intrauterine position, trauma, or infections remains to be determined.

PRS may occur as an isolated condition or in association with other syndromes, such as Stickler, velocardiofacial (VCF), fetal alcohol, or Treacher Collins, and other unique patterns of malformation. The incidence of associated syndromes may be observed in up to 83%4 of PRS patients. In addition to retrog-nathia and glossoptosis, many other factors may complicate the respiratory obstruction, including skull base abnormalities (Stickler, Treacher Collins), nasopharyngeal hypotonia (VCF), nasal stenosis (VCF, Treacher Collins), or neurologic compromise (VCF, fetal alcohol syndrome).

Most retrognathic infants and children manifest the obstructive respiratory symptoms at birth or days to weeks after birth. The severity of obstruction, ranges from minimal upper airway sounds to severe respiratory distress, failure to thrive, and cor pulmonale. Thus, airway management of PRS patients should be a systematic approach based on the site(s), severity of upper airway obstruction, and associated diseases involving other organs.

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