As otolaryngologists, we are often requested to evaluate newborns, infants, and children with upper airway obstruction. Nasal pyriform aperture stenosis, posterior choanal stenosis or atresia, Pierre Robin sequence (PRS), craniofacial anomalies, and laryngotracheal disorders are among the etiologies of upper airway obstruction. PRS is classically known as a triad of micrognathia, glossoptosis, and cleft palate. In PRS, the mandible may be of normal size, but it is displaced posteriorly. Retrognathia, lack of support of the genioglossus muscle, and negative pharyngeal pressure during inspiration lead to of the compromise upper airway.

Positioning, oropharyngeal airway, nasopharyngeal (NP) airway, tongue–lip adhesion (TLA), glossopexy, prosthesis placement, endotracheal intubation, and tracheotomy are many nonsurgical and surgical airway interventions. Choosing the most effective, reliable, and safe airway intervention for retrognathic patients is as challenging as it is controversial.

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 2000² 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 pro-lapsed 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 83⁴ of PRS patients. In addition to retrognathia 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.

Discussion

Attempts have been made to classify PRS patients based on the severity or type of upper airway obstruction. Benjamin and Walker⁵ divided their patients into three groups based on their responses to the treatment protocol, which included neonatal intensive care unit (NICU) monitoring, pulse oximetry, and sequential use of all airway interventions (i.e., posturing, NP tube, endotracheal intubation, tracheotomy). Benjamin and Walker⁵ reported patients with mild and moderate obstruction were managed successfully with posturing and NP airway, respectively. Patients with severe obstructive symptoms requiring endotracheal intubation or tracheotomy tended to have associated congenital anomalies and severe micrognathia. Freed et al.⁶ and Bull et al.⁷ used polysomnography (PSG) as an objective measurement of upper airway obstruction, in deciding the type of airway intervention, and in evaluation of treatment efficacy. Although they did not use the same criteria, both groups believed that oxygen desaturation, carbon dioxide retention, and obstruction during sleep were critical indicators of severe airway obstruction. Bull et al.7 observed PRS infants with multiple congenital anomalies were affected with greater degrees of obstruction and oxygen desaturation, feeding complications, gastroesophageal reflux disease, and developmental impairment, hence the necessity for surgical intervention.

Sher⁸

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