Pierre Robin Sequence: a perioperative review.

Abstract

The clinical triad of micrognathia (small mandible), glossoptosis (backward, downward displacement of the tongue), and airway obstruction defines the Pierre Robin sequence (PRS). Airway obstruction and respiratory distress are clinical hallmarks. Patients may present with stridor, retractions, and cyanosis. Severe obstruction results in feeding difficulty, reflux, and failure to thrive. Treatment options depend on the severity of airway obstruction and include prone positioning, nasopharyngeal airways, tongue lip adhesion, mandibular distraction osteogenesis, and tracheostomy. The neonate and infant with PRS require care from multiple specialists including anesthesiology, plastic surgery, otolaryngology, speech pathology, gastroenterology, radiology, and neonatology. The anesthesiologist involved in the care of patients with PRS will interface with a multidisciplinary team in a variety of clinical settings. This perioperative review is a collaborative effort from multiple specialties including anesthesiology, plastic surgery, otolaryngology, and speech pathology. We will discuss the background and clinical presentation of patients with PRS, as well as some of the controversies regarding their care.