Management of the airway is the main anesthetic concern for patients with Pierre Robin Sequence. (Image source: Anesthesia & Analgesia)

Management of the airway is the main anesthetic concern for patients with Pierre Robin Sequence. (Image source: Anesthesia & Analgesia)

One of the dreaded difficult airway disorders is Pierre Robin Sequence (PRS), also called Pierre Robin Syndrome. It is defined as a combination of cleft palate, micrognathia (small jaw), and glossoptosis (airway obstruction resulting from backward and downward displacement of the base of the tongue). The term “sequence” is often used because it is thought that a sequence of events in fetal development lead to the triad of symptoms that define the syndrome.

In this month’s issue of Anesthesia & Analgesia, Dr. Franklyn Cladis, Department of Anesthesiology, Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, PA and colleagues describe a multidisciplinary approach to the treatment of patients with PRS in their article titled “Pierre-Robin Sequence: A Perioperative Review.”

Due to the different presentations of PRS and in order to determine the appropriate treatment, patients are categorized based on the severity of airway obstruction.  Management of the airway can range from prone positioning (this may relieve obstruction in as many as 70% of patients) to procedures as complex as tongue lip adhesion (TLA), mandibular distraction osteogenesis (MDO), and even tracheostomy in patients with more severe obstruction.  Nonsyndromic infants tend to do well with conservative measures and are more likely to improve with interventions such as TLA and MDO.  Syndromic infants with PRS are more likely to need tracheostomy and/or gastrostomy tube placement.  The general consensus is that PRS patients with multiple levels of obstruction or central apnea will likely benefit from tracheostomy. The authors present an algorithm for anesthetic management in these patients.

Management of the airway is the main anesthetic concern.  PRS patients can have intraoperative airway obstruction and can be difficult to intubate.  Infants with associated syndromes such Stickler, velocardiofacial, and Treacher-Collins syndrome, might also benefit from a preoperative evaluation that includes echocardiography, particularly if they have a murmur.

Multiple techniques of securing the airway are described, including standard laryngoscopy with a paraglossal approach, placing an awake LMA, using a fiberoptic scope to direct placement of the endotracheal tube, Glidescope use, and emergent rigid bronchoscopy by the otolaryngologist.  Emergent tracheostomy and ECMO are the final and least ideal rescue techniques discussed. There is little evidence to support maintenance of anesthesia using one technique over another.  Postoperatively, chronic hypoxia from airway obstruction can increase opioid sensitivity.

PRS patients also have feeding issues. They may need long-term nasogastric or gastrostomy tube feeds if specialized feeding equipment does not provide for adequate nutrition and growth.  Swallowing and feeding assessments are usually managed by speech pathologists or occupational therapists.  These patients may come to the OR for procedures other than definitive airway repairs. The anesthesiologist may be faced with managing these patients both inside and outside the operating room for procedures such as gastrostomy tube placements and diagnostic imaging.

This article is a comprehensive review of the management of patients with Pierre Robin Sequence. Because the disorder involves airway obstruction by definition, anesthesiologists play a major role in the management of these patients.