Anesthetic Consideration in Pierre Robin Sequence

Issues of Concern

PRS poses numerous challenges to anesthesiologists. The airway is often difficult to ventilate and intubate due to craniofacial dysmorphology. Maintaining an adequate seal with a facemask can be challenging because of midface hypoplasia and retrognathia/micrognathia. Direct laryngoscopy and tracheal intubation are typically difficult during infancy but generally become more manageable with age and mandibular growth. Airway obstruction in patients with PRS can occur at the level of the oropharynx and hypopharynx, respectively, due to glossoptosis and epiglottic collapse. In patients with concurrent laryngomalacia and/or subglottic stenosis, airway obstruction may also occur at the laryngeal level.[13] 

Postoperatively, patients with PRS are at increased risk of spontaneous airway collapse. This may occur due to preexisting airway obstruction, with possible obstructive sleep apnea (OSA) exacerbated by residual anesthetic effects, airway edema from instrumentation, and increased opioid sensitivity. Patients may develop paradoxical breathing patterns, intercostal indrawing, subcostal and sternal retractions, and tracheal tug.[14]

Neonates and infants with PRS and associated OSA with chronic hypoxemia exhibit heightened opioid sensitivity due to upregulation of opioid receptors in the brainstem. Patients with severe airway obstruction have significantly reduced opioid requirements and require careful dose titration.[15] Feeding difficulties, swallowing disorders, and coexisting gastroesophageal reflux are frequently complicated by bronchial microaspiration and pulmonary infections.[16] Therefore, aspiration precautions should be implemented before elective procedures. Additionally, patients with PRS are often malnourished and have a high prevalence of failure to thrive.[17]

Clinical Significance

Preanesthetic Assessment

Neonates and infants presenting with PRS pose significant challenges to anesthesia clinicians during the intraoperative and postoperative periods. Preoperatively, anesthesiologists should obtain a detailed birth history, including a history of NICU admission for airway support, history of OSA, recent upper respiratory infections, features suggestive of reflux and aspiration, and details of prior anesthetic interventions.

A comprehensive physical examination, with an emphasis on airway evaluation and cardiopulmonary assessment, is essential for formulating an appropriate anesthetic management plan. Airway evaluation should include assessment of mouth opening, presence of cleft palate, degree of airway obstruction, severity of micrognathia, and the presence of other craniofacial skeletal abnormalities. In addition, identifying patients with features of OSA, as this helps predict intraoperative and postoperative airway complications and identify those who may not tolerate the supine position. Physical examination should include measuring oxygen saturation, heart rate, respiratory rate, and blood pressure.

Preoperative workup should include a complete blood count and other investigations guided by associated comorbidities, including serum electrolytes, renal function tests, flexible nasal endoscopy and laryngoscopy, chest radiography, and computed tomography (CT). If PRS is associated with other syndromes, a preoperative echocardiogram should be performed, particularly if a cardiac murmur is present.[18]

An emerging adjunct to preoperative planning is the use of patient-specific 3D-printed airway simulators derived from CT imaging data. A 2024 study demonstrated that these models enable anesthesiologists to visualize individual airway morphology, select appropriate intubation equipment, and rehearse intubation strategies before entering the operating room, a development of particular value in anatomically challenging PRS airways.[19]

Induction and Airway Management

Anesthesia practitioners should anticipate difficulty with ventilation, oxygenation, and intubation. Intubation may be performed with or without sedation and general anesthesia. The primary objective in managing the anticipated difficult pediatric airway is to maintain spontaneous ventilation under sedation or general anesthesia. Compared with no sedation or anesthesia, the use of anesthesia increases the success rate of first-attempt intubation and reduces both the number of attempts and the incidence of complications.[20]

Upper airway obstruction is common in syndromic children and may be alleviated with a 2-handed jaw thrust and airway adjuncts, eg, an oropharyngeal airway, nasopharyngeal airway, or laryngeal mask airway (LMA). Otorhinolaryngology support should be readily available for emergent tracheostomy.

European Society of Anaesthesiology and Intensive Care and British Journal of Anesthesia 2024 Guidelines

The 2024 European Society of Anaesthesiology and Intensive Care and British Journal of Anesthesia guidelines provide the following recommendations, each directly applicable to PRS airway management:

• Videolaryngoscopy: A videolaryngoscope with an age-appropriate standard blade is recommended as the first-line intubation technique in neonates and infants, superseding direct laryngoscopy as the default approach. Videolaryngoscopy offers improved glottic visualization, enables team-based supervision of intubation attempts, and has been shown to increase first-attempt success rates in the pediatric difficult-airway population. When a hyperangulated blade is used, a preshaped stylet should be utilized to facilitate endotracheal tube passage through the acutely angled view.[20]

• Apnoeic oxygenation: The provision of supplemental oxygen during intubation attempts, via nasal cannula, transnasal humidified rapid-insufflation ventilatory exchange (THRIVE), or pharyngeal insufflation, extends safe apnea time and reduces the risk of desaturation during laryngoscopy.[20]

• Limiting intubation attempts: Each failed intubation attempt contributes to progressive airway edema and trauma, thereby increasing the difficulty of subsequent attempts. If the initial technique fails, the operator, device, or approach should be modified. After 2 to 3 unsuccessful attempts, the strategy should escalate to placement of a supraglottic airway for rescue ventilation and oxygenation, and the team should explicitly consider whether to proceed with an alternative intubation strategy or awaken the patient.[20] 

• Postextubation respiratory support: High-flow nasal oxygenation (HFNC), continuous positive airway pressure (CPAP), or nasal intermittent positive pressure ventilation should be considered for postextubation respiratory support in at-risk neonates.[20]

Airway Technique Selection

The following airway techniques may be indicated in the following settings:

• Fiberoptic intubation: Flexible fiberoptic intubation, whether performed awake under topical anesthesia or asleep while preserving spontaneous ventilation, remains the gold standard for known or anticipated severe airway difficulty. Flexible fiberoptic intubation is particularly indicated when nasal intubation is required (as in tongue–lip adhesion or mandibular distraction osteogenesis procedures) and when airway anatomy is too distorted for effective videolaryngoscopy.[21]

• Hybrid videolaryngoscopy with fiberoptic bronchoscopy: A combined approach that uses videolaryngoscopy for indirect glottic visualization while advancing a fiberoptic bronchoscope through the glottis to guide endotracheal tube placement has demonstrated efficacy in the Pediatric Difficult Intubation Registry.[22]

• Supraglottic airway as an intubation conduit: A supraglottic airway can serve as both a rescue ventilation device and a conduit for fiberoptic-guided or blind intubation. In resource-limited settings where fiberoptic bronchoscopes are unavailable, intubation with devices (eg, an i-gel or Air-Q) using an appropriately sized endotracheal tube has been reported to be an effective strategy in PRS neonates.[23][Shah PJ et al. Airway Management in a Neonate with Pierre–Robin Syndrome: Challenges for the Anesthesiologist. 2024] 

While using direct laryngoscopy for intubation, the right paraglossal approach is more effective than the standard midline technique. However, this approach can make endotracheal tube passage more challenging, and a bougie may be required to facilitate intubation.[23] Given the 63% failure rate of direct laryngoscopy in PRS neonates reported by Marston et al, it should not be considered a reliable first-line technique in this population.[21]

Anesthesia Agents

For awake fiberoptic intubation in neonates, intravenous access should ideally be secured before induction. The pharynx and oral cavity are anesthetized using topical lidocaine (2%) via atomizer and jelly. The total lidocaine dose should be limited to 4 to 5 mg/kg. A vagolytic agent (atropine or glycopyrrolate) is administered to prevent bradycardia and to act as an antisialagogue. If intubating orally, an intubating supraglottic device may be helpful. Upon visualization of the vocal cords, an intravenous induction agent may be administered. A neuromuscular blocking agent is then administered before advancing the scope through the vocal cords to reduce the risk of laryngospasm.[18]

Anesthesia may be induced with either volatile or intravenous agents (eg, propofol). Sevoflurane is the most commonly used volatile agent in pediatric practice and is recommended for the management of the difficult airway. Isoflurane may also be used as an alternative. Desflurane should be avoided in patients with bronchial hyperreactivity, as it may precipitate laryngospasm, coughing, and increased airway secretions. 

In addition to inhalational agents, anesthesia may be maintained using total intravenous anesthesia (TIVA) with propofol and alpha-2 agonists (eg, dexmedetomidine), which can reduce opioid requirements and provide both analgesic and sedative effects. TIVA may also decrease the incidence of emergence agitation.[24][25] The key advantage of TIVA in PRS is the ability to achieve adequate depth of anesthesia while relatively preserving respiratory drive (via dexmedetomidine), thereby minimizing opioid exposure in a population with heightened opioid sensitivity.[26][27]

Dexmedetomidine is a centrally acting alpha-2 adrenergic agonist that decreases central sympathetic outflow. A notable characteristic of dexmedetomidine is preservation of respiratory drive. Dexmedetomidine is associated with a low incidence of apnea, respiratory depression, and airway obstruction, making it particularly advantageous in children prone to airway collapse. These properties, combined with a relatively short half-life, make it a useful sedative agent for ambulatory pediatric procedures, eg, CT and MRI.[26]

Ketamine causes minimal respiratory depression by maintaining a near-normal ventilatory response to carbon dioxide; therefore, this agent is well-suited for procedures performed under sedation. Ketamine also produces bronchodilation and mitigates intubation-induced bronchospasm. However, ketamine increases airway secretions; therefore, antisialagogues should be administered prophylactically before its use.[25]

Intraoperative Monitoring and Extubation

Intraoperative monitoring should include quantitative neuromuscular monitoring and temperature monitoring (rectal or nasal), in addition to standard monitoring with electrocardiography (ECG), noninvasive blood pressure (NIBP), end-tidal carbon dioxide, and pulse oximetry. Complete reversal of neuromuscular blockade with appropriate monitoring, followed by awake extubation, should be ensured. A nasopharyngeal airway may be inserted before extubation to reduce the risk of postoperative airway obstruction.

Prolonged surgical duration, pressure exerted on the base of the tongue by retractors, and airway edema may exacerbate preexisting airway obstruction, making these patients particularly susceptible to postoperative respiratory complications.[28] The risk of postoperative complications may be reduced by simple maneuvers, eg, neck extension and jaw thrust, or by the use of nasopharyngeal or oropharyngeal airways. Postoperatively, nursing the child in a lateral position with the neck extended may further help reduce complications.[14]

Following extubation, high-flow nasal oxygenation, CPAP, or nasal intermittent positive pressure ventilation should be instituted as clinically indicated.[20] Additionally, to prevent postoperative respiratory obstruction, nonopioid analgesics, regional anesthesia techniques, and local infiltration should be considered.

Enhancing Healthcare Team Outcomes

PRS is a congenital craniofacial disorder characterized by micrognathia, glossoptosis, and upper airway obstruction, frequently associated with cleft palate and syndromic conditions such as Stickler and Treacher-Collins syndromes. Infants commonly present with respiratory distress, feeding difficulties, aspiration, gastroesophageal reflux, obstructive sleep apnea, and failure to thrive. Airway compromise results from posterior displacement of the tongue and may worsen during anesthesia or sedation because of difficult mask ventilation, intubation challenges, airway edema, and heightened opioid sensitivity. Evidence-based management includes prone or lateral positioning, nasopharyngeal airway placement, videolaryngoscopy, fiberoptic intubation, opioid-sparing anesthetic strategies, and postoperative respiratory support with high-flow nasal oxygen or CPAP. Severe cases may require tongue-lip adhesion, mandibular distraction osteogenesis, or tracheostomy to relieve obstruction and support growth and development.

Interprofessional collaboration improves patient-centered outcomes by promoting coordinated airway planning, preventing complications, and enabling timely interventions. Pediatricians, anesthesiologists, surgeons, neonatologists, advanced practitioners, nurses, pharmacists, respiratory therapists, and otorhinolaryngologists each contribute to comprehensive perioperative care through shared decision-making and structured communication. Clinicians collaborate to assess airway severity, optimize nutrition, manage aspiration risk, and determine appropriate surgical or anesthetic strategies. Pharmacists assist with opioid dose adjustment and medication safety, while nursing staff provide feeding support, postoperative monitoring, parent education, and early recognition of respiratory compromise.[29] Effective teamwork, leadership, situational awareness, and standardized communication strategies reduce preventable complications, improve safety, and support long-term developmental outcomes.[30]

Acknowledgment of Assistance

The author acknowledges the assistance of Claude AI and ChatGPT (OpenAI) in reference formatting, literature review, refining the language, and improving the clarity and readability of the manuscript. All scientific content, references, and conclusions are the responsibility of the author.