Pierre-Robin

= Pierre-Robin =







60. Patients with the Pierre Robin Sequence: (a) May fail to thrive, owing to airway obstruction. (b) Rarely have clinically obvious airway obstruction. (c) Often require tracheostomy at birth. (d) Should be investigated for other anomalies. (e) Should not be given opioid analgesia postoperatively.

-multiple etiologies resulting from mandibular aplasia -characterized by association of cleft palate, glossoptosis, and micrognathia
 * Pierre-Robin Syndrome**

Glossoptosis à downward displacement/retraction of tongue

Incidence -1 per 8500 live births -male to female ratio 1:1, except in the x-linked form

Genetic Inheritance: -association with deletion of 2q32.3-q33.2 has been demonstrated -X-linked form and an association with trisomy 18

Pathophysiology -hypoplasia of mandibular area prior to 9 weeks in utero à posterior position of tongue that prevents palatal shelves from closing on the midline

Diagnosis -glossoptosis -micrognathia -cleft soft palate -mandible grows during first few months so that normal mandibular profile is common at the age of 4-6 years old



Clinical Aspects -in addition to three classical signs à cardiovascular (cor pulmonale) -neuromuscular dysfunctions (brainstem dysfunction, central apnea) -facial abnormalities à obstructive apnea, respiratory distress in the neonatal period -**prone position** à prevent tongue from falling back -**nasopharyngeal airway**, **suturing of tongue to the lip**, **tracheostomy**



-feeding difficulties secondary to facial malformation and/or neurological swallowing problems

Precautions before anesthesia: -obtain full history of apnea (central and/or obstructive), repiratory complications, hospital stays, protracted intubations, tracheotomy, feeding, growth, and development -evaluate for difficult tracheal intubation and lung ventilation -most obstructive apnea is severe, most tracheal intubation and ventilation by mask is difficult -evaluate for tracheomalacia and stenosis after intubation or tracheostomy: chest radiograph -full medical history, search for existence of apnea -evaluate other factors of respiratory distress: coexistence of central apnea, tonsil hypertrophy, cardiomyopathy -evaluate for present infection in orolaryngotracheal area and within lungs from recurrent pulmonary aspiration -evaluate for esophageal reflux

Anesthetic considerations: -high complication rate due to difficult airway management, DL and tracheal intubation extremely difficult especially in very young -difficulty decreases with age -consider IV access prior to induction of anesthesia -clearly planned difficult airway procedures à fiberoptic intubation, LMA, digitally assisted, blind nasal intubation in prone position with hyperextension of neck -well controlled, light anesthesia induction with preservation of spontaneous ventilation (halothane, sevoflurane) -possibility of manually assisted ventilation using a face mask must be verified prior to any attempt of DL -close post-op observation to detect any apnea

-prone position à can be used for pts with swallowing problems, postop pharyngeal bleeding to avoid pulmonary inhalation -muscle relaxants contraindicated until confirmation of maintenance of face-mask ventilation and/or DL and tracheal intubation can be performed à major concern is difficult or impossible ventilation by face mask -possibility of re-intubation must be considered before tracheal extubation

** 60. Patients with the Pierre Robin Sequence: ** (a) Subtle airway obstruction is a common cause of failure to thrive in this group. (b) This is part of the diagnostic criteria. (c) Rarely required, less invasive measures, particularly nasopharyngeal airway, are tried first. (d) As with any CLP patient, though incidence of other anomalies is higher with PRS. (e) Require the same analgesia as any patient, carefully titrated and with appropriate observation.
 * (a) True; (b) False; (c) False; (d) True; (e) False **