A 1-year-old-infant known to have Robin sequence (synonym: Pierre Robin syndrome or Pierre Robin sequence) presents with chronic otitis media and significant conductive hearing loss. The pediatric otolaryngologist has booked him for bilateral myringotomy and tube insertion. An operating room time of 20 minutes has been scheduled. The patient has been evaluated in the outpatient clinic by an anesthesia colleague. Her consultation report states that the infant was hospitalized for the first 3 months of his life for severe airway obstruction and feeding difficulties. Mandibular distraction osteogenesis surgery is not performed at this center, so the airway was managed by glossopexy (tongue sutured to lip) during the first month of life. The anesthesia record for this procedure indicates that the infant was intubated awake with great difficulty by a team of pediatric anesthesia practitioners, employing an unorthodox combination of the infant-size Trachlight™ and direct straight-blade laryngoscopy. It is noted that the infant currently still sleeps on his stomach without apparent apnea or airway obstruction, but develops stridor and apnea if placed on his back.
46.2.1 What Is Robin Sequence?
Robin sequence (named for the eminent French dentist, Pierre Robin, who first described it) has become synonymous with the pediatric anesthesia practitioner's worst airway nightmare. The condition is believed to be the result of primary failure in fetal mandibular development. The resulting micrognathia leads to rostral displacement of the tongue, termed glossoptosis. In 50% of cases the displacement of the normal-sized tongue into the roof of the mouth leads to failure of fusion of the maxillary arches and a resultant cleft palate. The combination of cleft palate and malposition of the tongue leads to airway obstruction, recurrent episodes of hypoxemia and hypercapnia, pulmonary hypertension, sleep apnea, swallowing difficulties, failure to thrive, and chronic ear disease.1 Although the Robin sequence is an isolated anomaly in two-thirds of cases, similar features are found in multiple malformation patterns, including Treacher Collins syndrome, Stickler syndrome, and Velocardiofacial syndrome. Accurate diagnosis of multiple anomaly syndromes is important in formulating the airway plan because of the possible presence of associated major malformations, such as congenital heart disease.
Older texts quote a mortality of 50% for this anomaly. Good positioning (nursing in prone position), tube feeding, glossopexy (suturing the tongue to the lip to pull it forward), mandibular distraction osteogenesis surgery,2,3 prevention or treatment of aspiration pneumonia, and reduced incidence of pulmonary hypertension (less exposure to hypoxemia and hypercarbia) have combined to reduce mortality to below 5%. After birth, growth of the mandible proceeds normally, and surgical closure of the cleft palate eventually allows for the development of near-normal airway anatomy, normal swallowing, normal growth, and full physical and intellectual development.
The anesthesia practitioner respects this malformation because the trachea of these patients is notoriously difficult to intubate and BMV is often difficult, which is unfortunate because they frequently need surgery. To compound the matter, the ...