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.
From the Departments of *Anesthesiology and †Plastic Surgery, Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania; and ‡Pediatric Otolaryngology, UH Rainbow Babies and Children’s Hospital CWRU School of Medicine, Cleveland, Ohio.
Accepted for publication March 28, 2014.
Conflicts of Interest: See Disclosures at the end of the article.
Reprints will not be available from the authors.
Address correspondence to Franklyn Cladis, MD, Department of Anesthesiology, Children’s Hospital of Pittsburgh of UPMC, 4401 Penn Ave., 5th Floor, Pittsburgh, PA 15224. Address e-mail to Cladfp@anes.upmc.edu.