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Babies born with Pierre- Robin sequence commonly present with respiratory distress and feeding difficulties due to mandibular hypoplasia and posteriorly placed tongue. The associated cleft palate leads to recurrent upper respiratory tract and ear infections. These patients will require various surgical interventions to correct the anatomical abnormalities and other related defects. This activity outlines the evaluation and management of the Pierre- Robin sequence and highlights the role of the inter-professional team in managing patients with this condition. Objectives: Outline the anesthetic management considerations for patients with Pierre- Robin sequence. Summarize the anticipated complications during the intraoperative and postoperative period among patients with Pierre-Robin sequence. Describe the common intubation methods before administering general anesthesia among patients with Pierre- Robin sequence. Explain the importance of monitoring patients with Pierre- Robin Sequence after extubation in the post-anesthesia care unit. Access free multiple choice questions on this topic.

Pierre Robin sequence (PRS) is a triad of micrognathia, posterior-inferior displacement of the tongue base (glossoptosis), and airway obstruction.[1] 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.[2][3] 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.[4][5] 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.[6] 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.[7][8] 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.[9] 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.[10][11] Patients often require palatoplasty to correct the palatal defect, fix feeding difficulties, and facilitate normal speech development.

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.[10][11] 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).

Infants born with Pierre Robin Sequence are at risk of developing airway-related complications, hypoxia, cor-pulmonale, and failure to thrive. Active multidisciplinary management of such infants is indispensable to reduce the complications of neurocognitive impairment secondary to the associated malformations. The preparation of a neonate/ infant who is scheduled to undergo surgery or anesthesia should involve the child's family, pediatrician, anesthesiologist, plastic surgeon, and other specialists caring for the child. Conveying relevant information among the individuals caring for the patient is vital to ensure a pleasant perioperative course. Pre-operatively, involving the nursing staff to provide appropriate feeding techniques to ensure adequate hydration and nutrition will reduce the incidence of regurgitations, aspiration and will improve growth and development. Shared decision-making involving the interprofessional provider team may prevent unnecessary surgeries and complications post-surgery. In addition, shared decision-making would allow them to determine the best possible management plan for the PRS patient. To establish a culture of patient safety and safe medication handling in the operation room and post-anesthesia care unit, the involvement of pharmacists is critical. Pharmacists should be involved in analyzing adequate dose adjustments in neonates, dosing frequencies, especially opioids and anti-emetics. Pharmacists can also be a part of educational activities and alert anesthesia providers about changes in labels or concentrations of anesthetic medications.[20] Close postoperative monitoring of the PRS patients in the Post Anesthesia Care Unit (PACU) by the nursing staff and anesthesia providers can lead to early recognition of post-op complications, and early interventions will prevent catastrophes. Nursing professionals also play a critical role in helping neonate's parents/ family verbalize their concerns and prepare them to care for the baby appropriately. They can provide reassurance, reduce their anxiety and promote coping. Thus, a multidisciplinary team approach can enhance patient-centered care, improve outcomes, patient safety, and improve team performance. Various aspects of teamwork to improve patient outcomes and safety include: Adopting a quality collaboration by showing mutual respect and trust.

Close postoperative monitoring of the PRS patients in the Post Anesthesia Care Unit (PACU) by the nursing staff and anesthesia providers can lead to early recognition of post-op complications, and early interventions will prevent catastrophes. Nursing professionals also play a critical role in helping neonate's parents/ family verbalize their concerns and prepare them to care for the baby appropriately. They can provide reassurance, reduce their anxiety and promote coping. Thus, a multidisciplinary team approach can enhance patient-centered care, improve outcomes, patient safety, and improve team performance. Various aspects of teamwork to improve patient outcomes and safety include: Adopting a quality collaboration by showing mutual respect and trust. Developing shared goals, perception of a situation, and understanding of tasks and roles within the team. Adaptive coordination allocates tasks for new team members, facilitates information exchange and planning in critical situations, and exhibits openness and excellent communication quality. Practicing specific communication practices such as team briefing. Leadership that encourages the participation of team members in the decision-making process and increases explicit leadership behavior in critical situations.[21] [Level 5]