Pierre Robin Sequence (PRS)

Anticipate difficulty at every step - Mask ventilation, oxygenation, and intubation are all hard. The obstruction is multilevel: glossoptosis at the oropharynx, collapse of the epiglottis onto the tongue base at the hypopharynx, and sometimes laryngomalacia or subglottic pathology below that. Fixing one level does not open the airway.

Direct laryngoscopy is the technique that fails - The blade has nowhere to sweep the tongue, because the mandibular space to sweep it into is what is missing. Limit direct laryngoscopy attempts and move to an alternative early — flexible bronchoscope, often through a supraglottic airway, video laryngoscope, or retrograde wire. If you do use direct laryngoscopy, the right paraglossal approach beats the standard midline one, though passing the tube is then harder and usually needs a bougie.

Keep them breathing - The objective in the anticipated difficult pediatric airway is to maintain spontaneous ventilation under sedation or general anesthesia. Sevoflurane is the recommended volatile for this; ketamine preserves a near-normal ventilatory response to carbon dioxide, and dexmedetomidine is favorable in children prone to airway collapse. Two-handed jaw thrust with an oral or nasopharyngeal airway relieves most of the obstruction while you work.

Position is a treatment, not a comfort measure - Prone or lateral positioning relieves the obstruction in about 70% of these neonates by letting the mandible and tongue fall forward. Use it in holding, in transport, and in PACU. Asking the parents whether the baby has to sleep prone tells you how severe the obstruction really is.

Have ENT and a tracheostomy plan in the room - Emergency tracheostomy or bronchoscopy is the backup. For tongue-lip adhesion and mandibular distraction osteogenesis the standard is nasal intubation, which changes how you secure the tube and how you extubate.

The airway improves with growth — but only in nonsyndromic PRS - Catch-up mandibular growth reduces obstruction with age, so an older child may genuinely be easier than the infant record suggests. Do not extend that assumption to syndromic PRS, where the airway can stay difficult or worsen.

Aspiration and reflux - Poor suck-swallow-breathe coordination, cleft palate, and gastroesophageal reflux produce recurrent aspiration and failure to thrive. Ask specifically about reflux symptoms, previous aspiration, and recent upper respiratory infection on the preoperative history.

Extubate awake, with the airway already protected - Reverse neuromuscular block completely with TOF monitoring and extubate awake. Placing a nasopharyngeal airway before extubation prevents postoperative obstruction. Afterwards, nurse the child lateral with the neck extended.

Opioids are the postoperative hazard - Preexisting obstruction, OSA, and chronic hypoxia leave these children opioid-sensitive. Lean on non-opioid analgesics, local infiltration, and regional blocks. A long palatoplasty adds airway edema and tongue-base pressure from the retractor on top of the baseline obstruction, so keep them monitored in PACU longer than the case length suggests.

Preoperative studies that change the plan - Get the flexible nasolaryngoscopy report for the level of obstruction, polysomnography if OSA is suspected, and an echocardiogram if there is a murmur or an associated syndrome. Above all, review which airway technique worked last time — the previous anesthetic record is the most useful document you have.

Pierre Robin sequence is the triad of micrognathia, glossoptosis, and upper airway obstruction, with cleft palate present in most cases. It is a sequence rather than a syndrome: one defect starts the whole chain. Mandibular hypoplasia around the seventh week of gestation leaves the tongue unable to follow its normal downward path, so the tongue base sits high and posterior, blocks fusion of the palatal shelves before the tenth week, and produces a U-shaped cleft palate. That same posteriorly displaced tongue obstructs the pharynx after birth.

Incidence is roughly 1 in 8,500 to 1 in 14,000 newborns. More than half have an associated syndrome — Stickler most commonly, then velocardiofacial (22q11 deletion) and Treacher Collins. In nonsyndromic PRS the mandible catches up and obstruction improves with age; in syndromic PRS it may not.