Anesthetic Consideration in Pierre Robin Sequence

Pierre Robin sequence (PRS) is a triad of micrognathia, posterior-inferior displacement of the tongue base (glossoptosis), and airway obstruction. PRS affects approximately up to 1 in 14,000 newborns a year. PPRS can occur in isolation but is more often associated with other syndromes such as Fetal alcohol syndrome, Stickler syndrome, velocardiofacial syndrome, and Treacher-Collins syndrome. At birth, neonates mainly exhibit signs of respiratory distress (stridor, retractions, and cyanosis); some manifest with feeding difficulty, gastroesophageal reflux, aspiration, and failure to thrive. A sequence is a pattern of congenital anomalies that result from a single defect during development. In PRS, micrognathia is the single initiating event that occurs during development. It results in a cascade of secondary defects such as glossoptosis and cleft palate. The abnormal mandible displaces the tongue into the nasopharynx, thus preventing the fusion of palatal shelves. This gives rise to varying severity of cleft palate. In addition to cleft palate, glossoptosis also gives rise to airway obstruction and obstructive sleep apnea if severe. In approximately 70% of cases of PRS, placing the neonate in a prone or lateral position relieves airway obstruction, but if the neonate desaturates, then a nasopharyngeal (NP) tube can be placed to bypass upper airway obstruction. Patients with mild airway obstruction managed conservatively are at risk for failure to thrive due to feeding difficulties, gastroesophageal reflux, and aspiration. In such cases placing a gastrostomy tube until they achieve catch-up growth may help prevent the above complications. In acute severe airway obstruction, the patient must undergo an emergent tracheostomy to bypass the compromised airway. After initial stabilization of the patient, procedures such as tongue lip adhesion and mandibular distraction osteogenesis can correct glossoptosis, lengthen the mandible and relieve glossoptosis. Also, the above techniques may need to be performed in conservatively managed PRS who fail to achieve an adequate catch-up growth. Patients often require palatoplasty to correct the palatal defect, fix feeding difficulties, and facilitate normal speech development. The above-listed procedures require anesthetic intervention in the form of general anesthesia. Anesthesiology-assisted sedation may be needed in patients undergoing magnetic resonance imaging (MRI) and computed tomography (CT).