Neonatal Med.  2019 Aug;26(3):179-183. 10.5385/nm.2019.26.3.179.

Three Cases of Pierre Robin Sequence with Upper Airway Obstruction Relieved by Nasopharyngeal Airway Insertion

• Oh MS
• Park YM
• Jung YH
• Choi CW
• Kim BI
• Kwon JW

• KMID: 2457187
• DOI: http://doi.org/10.5385/nm.2019.26.3.179

Abstract

Pierre Robin sequence (PRS), also called Robin sequence, is a congenital anomaly characterized by a triad of micrognathia, glossoptosis, and upper airway obstruction. Infants with PRS can present with varying degrees of respiratory difficulty secondary to upper airway obstruction. There has been no consensus for the treatment of upper airway obstruction in infants with PRS, but recent studies recommend attempting non-surgical interventions before surgical treatment. In this case report, we present 3 cases of infants diagnosed with PRS who showed persistent respiratory difficulties after birth. Before considering surgical intervention, insertion of a nasopharyngeal airway was attempted in these infants. Following this procedure, symptoms of upper airway obstruction were relieved, and all infants were discharged without surgical interventions; the nasopharyngeal airway was removed 1 to 2 months later. To date, no infant has shown signs of upper airway obstruction. Nasopharyngeal airway insertion is a highly effective and less invasive treatment option for infants with PRS. However, it is not widely known and used in Korea. Nasopharyngeal airway insertion can be preferentially considered before surgical intervention for upper airway obstruction in such infants.

Keyword

Pierre Robin syndrome; Upper airway obstruction; Nasopharyngeal airway

MeSH Terms

Airway Obstruction*
Consensus
Glossoptosis
Humans
Infant
Korea
Micrognathism
Parturition
Pierre Robin Syndrome*

Figure

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  Figure 1. Nasopharyngeal airways. The length of the tube can be selected by measuring the distance between the nares and the earlobe, which should be confirmed radiographically on a lateral neck radiograph to check if the tube is appropriately placed just above the epiglottis[11].

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  Figure 2. Cephalometry radiographs and photograph in case 1. (A) Representative case of Pierre Robin sequence in a 2- month-old infant showing micrognathia and glossoptosis with features of upper airway obstruction. (B) Image showing a nasopharyngeal airway inserted into the larynx, thereby moving the tongue forward and bypassing the tongue base. The white arrow indicating the tip of nasopharyngeal airway placed above the epiglottis. (C) Image of the same infant maintaining a nasopharyngeal airway after discharge (D) Image of the same infant at 4 months of age showing that following mandibular growth, upper airway patency is maintained without the insertion of a nasopharyngeal airway.

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