Airway Management in Patients With Isolated Pierre Robin Sequence During the First Year of Life

Abstract

Managing the airway of patients with Pierre Robin sequence is diagnostically and therapeutically challenging. Like many other pathologies, Pierre Robin sequence is best managed with a multidisciplinary team. Providing a comprehensive evaluation is important to detect patients who may have silent events during activities of early life. Children with airway obstruction should have a complete assessment to anatomically define the site of airway obstruction. Therefore, management options can be targeted to the specific deficiency. The present article outlines a treatment protocol for patients who present with isolated Pierre Robin sequence. The authors propose that initial diagnostic and therapeutic interventions remain relatively noninvasive. Using this protocol, more than 80% of patients with isolated Pierre Robin sequence who the authors have examined for airway obstruction in the neonatal period have been effectively treated with positioning or tongue-lip adhesion. While more aggressive and successful techniques have been reported, the authors have not found them necessary for the majority of patients with isolated Pierre Robin sequence. The following review details their approach to patients with Pierre Robin sequence.

Robin Sequence: From Diagnosis to Development of an Effective Management Plan

“…6,62,100 Cole et al 101 suggested a unique classification system for RS based on functional parameters: severity of airway obstruction, breathing, and feeding difficulties. Care and caution must be exercised when interpreting recommendations for the whole group, because morphologic phenotype, airway physiology, and severity of RS vary.…”

A clinical narrative review of mandibular distraction osteogenesis in neonates with Pierre Robin sequence

“…Surgical interventions for significant airway narrowing among neonates with PRS include tongue-lip adhesion [4,5,22], mandibular distraction osteogenesis [4,5,12] and tracheostomy [22]. Tracheostomy immediately relieves the upper airway obstruction and is still considered the ''gold standard'' treatment option.…”

“…Although vast majority of the children born with micrognathia or PRS are either asymptomatic or can be treated with conservative management, some patients may have significant respiratory and/or feeding issues, necessitating more aggressive interventions [3][4][5].…”

“…Conservative therapy for micrognathia associated airway and feeding problems include various positioning strategies and feeding techniques, as well as placement of nasopharyngeal airways and application of nasal continuous positive airway pressure [4][5][6][7]. As the neonate grows, most patients will demonstrate catch-up mandibular growth and attain adequate strength and coordination, such that airway obstruction no longer becomes a problem.…”

“…As the neonate grows, most patients will demonstrate catch-up mandibular growth and attain adequate strength and coordination, such that airway obstruction no longer becomes a problem. A relatively large case series of children with non-syndromic PRS demonstrated that less than 10% required invasive surgical intervention (distraction and/or tracheostomy) [4][5][6]. Yet, not all patients will respond to these non-invasive interventions.…”