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CASE PRESENTATION

A 6-year-old child with Pierre Robin sequence (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. direct laryngoscopy Cormack/Lehane Grade 4 view despite external laryngeal manipulation, with an inability to intubate the trachea.” Subsequently, a nasal flexible bronchoscopic intubation was successfully performed. The patient has not had any other surgeries since.

INTRODUCTION

What Is Pierre Robin Sequence?

Named for the French dental surgeon who described its symptomatology and management, PRS (also known as Pierre Robin syndrome), is a congenital disorder characterized by a triad of congenital 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 a broad, U-shaped cleft palate. Changes in the shape of the palate combined with the backward displacement of the tongue cause airway obstruction, respiratory difficulties, sleep apnea, and difficulty swallowing which can lead to chronic hypoxemia, pulmonary hypertension, and failure to thrive. Accurate preprocedural diagnosis is critical to ensure that a child’s physical features are not more consistent with another congenital syndrome (e.g., 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).

Appropriate management of breathing and feeding difficulties in infants with PRS reduces their mortality to less than 5%. Enteral feeding and prone positioning during nursing can improve nutritional intake.2 Although some respiratory issues are managed without operative intervention, most require surgical intervention.3 Most commonly, surgery to correct the cleft palate improves airway anatomy and swallowing ability. Tracheotomy is rarely indicated to treat respiratory problems in children with PRS. Mandibular distraction surgery is occasionally required to lengthen the mandible to bring the tongue forward and improve breathing and feeding mechanics. With effective treatment, most PRS individuals undergo normal childhood growth and development and experience healthy, normal adult life.

The craniofacial manifestations of PRS heavily impact an anesthesia practitioner’s plans for induction and airway management as tracheal intubation and face-mask ventilation (FMV) are generally difficult. Patients with PRS often require multiple surgeries and general anesthetics. Thus, an effective airway management plan is essential to avoid compounding traumatic events.

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

Several congenital syndromes feature head, neck, and cervical spine anomalies that complicate the pediatric airway. These syndromes are summarized in Table 49.1 (see also Table 46.1).4,5 Other anomalies are instead acquired. Examples of acquired anomalies include temporomandibular joint dysfunction following trauma (e.g., forceps ...

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