Intubation can be performed with or without sedation and general anesthesia. Īnesthesia providers should anticipate difficulty with ventilation, oxygenation, and intubation. If PRS is associated with other syndromes, a preoperative echocardiogram should be performed, especially in the presence of a murmur. Preoperative workup should encompass complete blood counts and other tests depending on comorbidities present, including serum electrolytes, renal function tests, video laryngoscopy, chest X-ray, and computed tomography (CT). Physical examination should include oxygen saturation at room air in addition to heart rate, respiratory rate, and blood pressure. In addition, it is crucial to identify patients with features of OSA as it helps to predict intraoperative and postoperative airway complications and those who do not tolerate supine positioning. Airway evaluation should include assessing the mouth opening, presence of cleft palate, degree of airway obstruction, the severity of micrognathia, presence of other craniofacial skeletal abnormalities. Preoperatively, anesthesiologists should obtain a detailed history of the patient's symptoms, including recent upper respiratory infections, features suggestive of reflux and aspiration, and details of the previous anesthetic intervention.Ī detailed physical examination emphasizing airway evaluation and cardio-pulmonary examination is imperative to formulate an appropriate anesthetic management plan. Neonates/infants presenting with PRS present significant challenges to anesthesia providers in intraoperative and postoperative periods, especially with airway management. Anesthesiology-assisted sedation may be needed in patients undergoing magnetic resonance imaging (MRI) and computed tomography (CT). The above-listed procedures require anesthetic intervention in the form of general anesthesia. Patients often require palatoplasty to correct the palatal defect, fix feeding difficulties, and facilitate normal speech development. Also, the above techniques may need to be performed in conservatively managed PRS who fail to achieve an adequate catch-up growth. Īfter initial stabilization of the patient, procedures such as tongue lip adhesion and mandibular distraction osteogenesis can correct glossoptosis, lengthen the mandible and relieve glossoptosis. 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. Patients with mild airway obstruction managed conservatively are at risk for failure to thrive due to feeding difficulties, gastroesophageal reflux, and aspiration. 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. In addition to cleft palate, glossoptosis also gives rise to airway obstruction and obstructive sleep apnea if severe. This gives rise to varying severity of cleft palate. The abnormal mandible displaces the tongue into the nasopharynx, thus preventing the fusion of palatal shelves. 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. Ī sequence is a pattern of congenital anomalies that result from a single defect during development. 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. 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. PRS affects approximately up to 1 in 14,000 newborns a year. Pierre Robin sequence (PRS) is a triad of micrognathia, posterior-inferior displacement of the tongue base (glossoptosis), and airway obstruction.
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