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CASE PRESENTATION

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A 6-year-old child with Pierre Robin Syndrome (PRS) presents for bilateral inguinal hernia repair. As a newborn, he underwent tongue-lip adhesion surgery for upper airway obstruction and feeding difficulties. The patient's anesthetic record reads, “Anterior larynx. Grade 4 Cormak/Lehane (C-L) direct laryngoscopic view despite laryngeal pressure, with an inability to intubate the trachea. Subsequently, a nasal flexible bronchoscopic intubation was successfully performed.” It is noted the patient has been free of any problems since then, and no other surgery has been performed.

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INTRODUCTION

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What Is Pierre Robin Syndrome?

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Named after the French physician who described its symptomatology and management, Pierre Robin Syndrome (or Sequence; PRS) is a congenital disorder characterized by a triad of craniofacial anomalies: mandibular hypoplasia, cleft secondary palate, and glossoptosis (rostral displacement of the tongue).1 Due to this combination of features, PRS is commonly viewed as a classic anticipated difficult airway scenario for pediatric anesthesia practitioners. In half of PRS cases, the positioning of the tongue during development precludes fusion of the maxillary arches, resulting in an often wide, U-shaped cleft palate; this, combined with backward displacement of the tongue, causes airway obstruction, respiratory difficulties, sleep apnea, difficulty swallowing, chronic hypoxemia, pulmonary hypertension, and failure to thrive. Accurate pre-procedural diagnosis is important to ensure that the physical features are not part of another multiple malformation disorder, such as Treacher Collins syndrome, Stickler syndrome, or Velocardiofacial syndrome, since the airway plan will be affected by the presence of associated abnormalities (e.g., congenital heart disease). Recently, a genetic link was found for PRS, increasing the potential for diagnostic accuracy in families and individuals.

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Management of breathing and feeding difficulties in infants with PRS has reduced the mortality rate to less than 5%. Enteral feeding or prone positioning during nursing can achieve satisfactory nutrition for growth,2 and respiratory difficulty can, in many cases, be managed without operative intervention.3 After birth, the mandible grows normally, and surgery to correct the cleft palate results in improved airway anatomy and swallowing ability. Tracheotomy is rarely indicated to treat respiratory problems. Occasionally, mandibular distraction surgery is used to lengthen the mandible, which brings the tongue forward and improves breathing and feeding. With effective treatment, most PRS individuals undergo normal childhood growth and development and experience a healthy, normal adult life.

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The implications of the manifestations of PRS are obvious for an anesthesiologist attempting to secure the airway: the trachea is not intubated easily, and bag-mask-ventilation (BMV) is usually difficult. Compounding these problems is that PRS individuals often require surgery, presenting the anesthesiologist with the unique situation of repeated administrations of general anesthesia on a patient with a notoriously difficult airway.

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What Are Other Congenital or Acquired Abnormalities Associated with Airway Difficulty?

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Several congenital syndromes feature head, neck, and cervical spine anomalies which may complicate the pediatric airway, as ...

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