Approaches advocated for treatment of airway obstruction among neonates with Pierre Robin sequence include positioning, tongue-lip adhesion, mandibular distraction, and tracheostomy, with no established guidelines regarding which modality is appropriate for a specific patient. This report proposes an algorithm for the management of neonatal upper airway obstruction among patients with isolated Pierre Robin sequence. Data for 21 patients with isolated Pierre Robin sequence who were treated by one surgeon during a 9-year period were reviewed. Eighteen patients presented during the first 1 week of life and three patients presented late, between 12 and 33 months of age. Follow-up periods ranged from 9 to 70 months (median, 33 months). Successful airway management was achieved with positioning alone for 10 patients, with tongue-lip adhesion for seven of nine patients, with tracheostomy for two patients, and with mandibular distraction for three patients. Changes in the maxillary-mandibular discrepancy were significant with natural mandibular growth during the first 1 year of life (p < 0.0001). Oromotor studies performed 3 months or more after tongue-lip adhesion reversal (n = 9) demonstrated no appreciable deficits in tongue function, relative to other children with cleft lips/palates. A multidisciplinary team should evaluate all patients with isolated Pierre Robin sequence, to fully assess the maxillary-mandibular relationship, anatomically define the site of airway obstruction, and identify feeding difficulties. Patients should be evaluated for episodes of desaturation occurring spontaneously, during feeding, or during sleeping. Patients with desaturation should be further evaluated with double endoscopy (nasoendoscopy and bronchoscopy). If the airway obstruction is localized to the tongue base alone and cannot be controlled with positioning, then tongue-lip adhesion is the initial treatment of choice, because such patients demonstrate significant mandibular growth during the first 1 year of life. Mandibular distraction among neonates is reserved for failures of tongue-lip adhesion in which isolated tongue-base airway obstruction is documented. Neither of the patients who experienced failure of tongue-lip adhesion in this series would have been a candidate for distraction with the algorithm presented. Avoiding routine neonatal distraction serves to avoid facial scarring, nerve and tooth bud injury, and potential disturbances of intrinsic mandibular growth. Patients with persistent respiratory difficulties beyond age 9 months require reevaluation for multiple sites of airway obstruction. Mandibular distraction may be one of several modalities required to avoid tracheostomy for such patients.

Tongue-lip adhesion can be effective in relieving tongue-based airway obstruction in the neonatal period. However, longitudinal evaluation of these patients demonstrates they require additional procedures for ultimate control of their airway, feeding, and orthognathic relationship. Charts of patients with Pierre Robin sequence who underwent unsuccessful nonoperative treatment and tongue-lip adhesion procedures by the senior author between 1989 to 1999 were reviewed. In particular, secondary interventions were quantified and qualified to determine if tongue-lip adhesion was a definitive treatment of the pathology caused by this sequence. Eleven patients with ages ranging from 2 to 6 weeks underwent initial tongue-lip adhesion. Seven patients were syndromic (3 Stickler's, 1 Goldenhar's, 1 Fragile X, 1 Miller's, 1 chromosome 15 rearrangement), and four had isolated Pierre Robin sequence. Two patients had dehiscence of their initial repair within 2 weeks, for a primary surgical success rate of 82%, consistent with other published reports. A detailed follow-up of these patients revealed that 10 of 11 (91%) required additional intervention for airway or feeding issues. Of those requiring secondary intervention, an additional 1.9 secondary procedures were performed per patient to achieve control of the airway, feeding, and orthognathic problems. More than half of the patients needed gastrostomy tubes to overcome feeding difficulties. Five patients necessitated secondary surgery for recurrent airway obstruction within 4 months of the initial surgical treatment (four distractions and one repeat tongue-lip adhesion). Four others required distraction at an older age for orthognathic indications. Two patients were successfully treated with tongue-lip adhesion only. Tongue-lip adhesion has a high initial success rate for correction of neonatal airway obstruction. However, long-term follow-up indicates a high incidence of secondary intervention requirements. In retrospect, perhaps tongue-lip adhesion should be considered a temporizing procedure for most patients with Pierre Robin sequence.

Cardioprotection conferred by hyperoxia+hyperbaria is directly dependent on oxygen availability and mediated by NOS.

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.