Airway Management in Pierre Robin Sequence

Abstract

There is a great deal of controversy regarding the long-term management of airway obstruction in children with Pierre Robin sequence. All 23 pediatric otolaryngology fellowship programs were surveyed to determine their current practice patterns. Issues that were addressed included methods of evaluation of airway obstruction, including polysomnography, choices for home care in children in whom observation and positioning treatment failed, and complications of the various treatment modalities. The lack of unanimity among the respondents mirrors the controversy found in the literature. Though tracheotomy appears to be favored as the method of long-term management, responses from those surveyed often were based on emotions as much as on scientific data. This report details those findings and offers suggestions to clinicians involved with the management of patients with Pierre Robin sequence with airway compromise.

Robin sequence: A retrospective review of 115 patients

“…Similar results with prone positioning have been reported by other authors: Schaefer et al [14], 45%; Kirschner et al [12], 69.2%; Cruz and coworkers [32], 55%. This method is reserved for infants with minor airway obstruction in whom treatment is short-term [32,33]. Other non-operative treatment modalities, including laryngeal mask [34,35], nasopharyngeal airway [7], and prolonged intubation [9,10,31], have been reported with variable success.…”

“…Advantages are several: decreased thoracic dead-space, decreases airway resistance, permits ventilatory support, facilitates pulmonary toilet. Myer et al [33] surveyed 23 pediatric otolaryngologic fellowship programs to clarify general treatment guidelines for patients with Robin sequence. Fifty-two percent of respondents chose tracheotomy as the treatment of choice in patients who failed observation and positioning, and 91% considered tracheotomy as a safe and reliable method of long-term airway management.…”

Prognosis of airway obstruction and feeding difficulty in the Robin sequence

“…A variety of techniques exist for the management of upper airway obstruction in PRS, including prone positioning, nasopharyngeal airway placement, tongue-lip adhesion, mandibular distraction osteogenesis, and tracheostomy [2,3,[6][7][8][9][10][11]. At our institution, PRS patients with upper airway obstruction unresponsive to positional therapy or temporizing measures (such as endotracheal intubation or nasopharyngeal airway placement) are offered either tracheostomy or mandibular distraction osteogenesis.…”

Subglottische Trachealstenosen

“…Entzündungen (z. B. Tracheitis, Bronchiolitis), das Pierre-Robin-Syndrom [15], Adipositas und das obstruktive SchlafapnoeSyndrom [19] können ebenfalls mit Stenosen der Trachea assoziiert sein.…”