Pierre Robin Sequence (PRS) is a collection of physical characteristics of a newborn that includes micrognathia (small jaw), glossoptosis (tongue falls to back of throat), and airway obstruction. The incidence of PRS varies from 1:5000-1:85,000. Although most patients with PRS have a cleft palate, it is not a requirement for the diagnosis. Pierre Robin is thought to be secondary to a fixed fetal position in utero that inhibits appropriate mandibular growth. Pierre Robin infants often present with feeding dysfunction, failure to thrive, and respiratory distress. PRS may present as an isolated sequence or may be associated with a syndrome. The most common syndromes are Stickler, velocardiofacial (22q,11.2 deletion), fetal alcohol syndrome, and Treacher-Collins Syndrome. If mild airway obstruction is present, airway function can be improved with prone positioning and placement of nasopharyngeal airway with resultant forward placement or mechanical bypass of the tongue. Approximately 25-50% of PRS patients will require surgical intervention, including tongue-lip adhesion, mandibular distraction osteogenesis (MDO), or tracheostomy for airway management as well as gastrostomy tube placement for feeding management.
Airway management in an uncorrected PRS patient can be a potentially difficult undertaking that should be performed in a center that is comfortable with difficult pediatric airways. Nasal intubation is preferred for both tongue-lip adhesion and MDO. While the most conservative approach to securing the airway is an awake fiberoptic intubation, numerous publications discuss successful mask induction with asleep intubation. Marston, et al. described successful ventilation after mask induction in 100% of their 33 patients with the aid of naso- or oro-phargyneal airway. Direct laryngoscopy (DL) was successful within two attempts in 37% of the infants; subsequent flexible fiberoptic bronchoscopy was successful in the remainder of the patients. Several airway techniques have been described to assist in the difficult airway for the patient with PRS. These techniques include LMA, fiberoptic scope, shikani scope, Airtraq, and Air-Q scope. An LMA can also be placed in an awake or an asleep infant with PRS to ventilate and then to guide a fiberoptic intubation. Alternative intubation methods with a videolaryngoscope (Glidescope) may also be useful.
Pierre-Robin is characterized by microganthia, glossoptosis (downward displacement or retraction of the tongue), and cleft soft palate thought to be attributed to in utero mandibular hypoplasia (forcing the tongue posterior). Can occur both in isolation or as a component of a malformation. Obstruction is a problem in these patients and when born, neonates may require prone positioning, nasal airways, or even suturing of the tongue to the lip. Obstruction may lead to cor pulmonale. These patients may have central apnea and often have feeding abnormaliities. Airway abnormalities tend to improve with age.
ANTICIPATE A VERY DIFFICULT AIRWAY. A variety of techniques have been used, including LMA, intubating LMA, fiberoptic bronchoscope, retrograde intubation, and rigid bronchoscopy.
Post-operatively, keep in mind the high risk of apnea (both obstructive and central).
- Intubation: anticipate difficult airway
- Extubation: anticipate significant obstruction
- Other Abnormalities: central apnea, cor pulmonale, feeding abnormalities, possible syndrome
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D A Markakis, S C Sayson, M S Schreiner Insertion of the laryngeal mask airway in awake infants with the Robin sequence. Anesth. Analg.: 1992, 75(5);822-4 [PubMed:1416138]
Takashi Asai, Atsushi Nagata, Koh Shingu Awake tracheal intubation through the laryngeal mask in neonates with upper airway obstruction. Paediatr Anaesth: 2008, 18(1);77-80 [PubMed:18095971]
P A Stricker, S Budac, J E Fiadjoe, M A Rehman Awake laryngeal mask insertion followed by induction of anesthesia in infants with the Pierre Robin sequence. Acta Anaesthesiol Scand: 2008, 52(9);1307-8 [PubMed:18823480]