Cleft Palate, Cleft Lip, and Pierre Robin Sequence

Abstract

Pierre Robin sequence (PRS) is characterized by micrognathia, glossoptosis, and airway obstruction. Often associated with cleft palate, PRS is usually an isolated finding but is associated with a syndrome one-third of the time. The micrognathia and glossoptosis lead to airway obstruction, respiratory compromise, and feeding difficulties. Severe cases and prolonged and repeated airway obstruction and respiratory distress can lead to failure to thrive, hypoxemia, pulmonary hypertension, cardiac arrest, and death. Treatment options for the management of airway obstruction in PRS depends on the degree of obstruction and any associated comorbidities and range from noninvasive respiratory support to surgical correction of the physical defect. Patients with PRS should be considered as challenging to ventilate and intubate, and the practitioner should be well prepared for the possibility of difficulty with airway management. Anesthetic management is tailored to minimize postoperative upper airway obstruction and avoid disruption of the integrity of the surgical repair.