Airway Management in Pierre Robin Sequence: The Vancouver Classification

Abstract

Background: Pierre Robin sequence (PRS) is a triad of micrognathia, glossoptosis, and respiratory distress. There is no standard clinical classification used in the management of neonatal airway in patients with PRS. The goal of our study was to review the presentation and management of patients with PRS and formulate a clinical grading system and treatment algorithm. Methods: A 10-year retrospective review of all neonates diagnosed with PRS was performed after obtaining institutional ethics approval. Patients were identified using our cleft lip and palate program database. Inclusion criteria were 2 of the following 3 clinical features-glossoptosis, retrognathia, or airway obstruction. We collected demographic data, clinical information (coexisting airway morbidity, maxillary-mandibular discrepancy, type of intervention used, complications, and outcomes (feeding, length of stay, and airway status) during the first year of life. Results: Sixty-three patients met our inclusion criteria. Of these, 55 (87%) had cleft palate and 17 (27%) were syndromic. Forty-eight (76%) patients were managed by prone positioning. Of the 15 surgically managed patients, the initial procedure was floor of mouth release in 7, mandibular distraction osteogenesis (MDO) in 4, and tongue-lip adhesion in 4. Five patients with coexisting airway morbidity needed a second surgery; 2 had MDO and 3 tracheostomies (one patient was later decannulated). Seven (47%) of the surgically managed patients required a gastrostomy tube. Conclusion: At present, there is no consensus on neonatal airway management in infants with PRS. From our review of 63 patients with PRS, we hereby propose a simple 4-point classification system and treatment algorithm, based on clinical features. Résumé Historique : Le syndrome de Pierre-Robin (SPR) désigne une triade de micrognathie, de glossoptose et de détresse respiratoire. Aucune classification clinique standard n'est utilisée pour assurer l'ouverture des voies respiratoires chez les nouveau-nés présentant un SPR. La présenteétude visait à examiner la présentation et la prise en charge des patients ayant un SPR ainsi qu'à formuler un système de classement clinique et un algorithme de traitement. Méthodologie : Après avoir obtenu l'approbation du comité d'éthique de leurétablissement, les chercheurs ont procédé à une analyse rétrospective sur dix ans de tous les nouveau-nés ayant reçu un diagnostic de SPR. Ils ont recensé les patients dans leur base de données de fentes labiales et palatines. Deux des trois caractéristiques cliniques suivantes constituaient les critères d'inclusion : glossoptose, rétrognatie ou obstruction

Epidemiology of Robin sequence with cleft palate in the East of Scotland between 2004 and 2013

“…1 However, significantly higher rates of surgical intervention (including distraction osteogenesis and tongue-lip adhesion) and tracheostomy of 19-77% have been reported in several studies which are largely from North American centers. 5,[24][25][26][27] This wide variation in practice with an apparent geographical pattern suggests that physician or treatment center preferences, rather than clinical necessity, may direct RS management.…”

Pierre Robin Sequence: Diagnostic Difficulties Faced while Differentiating Isolated and Syndromic Forms

“…As more than 50 syndromes have been associated with PRS and each of these syndromes have a vast heterogenicity in their respective presentations, it often becomes very difficult for clinicians to accurately identify the associated syndrome (3,5). We faced a similar predicament.…”

“…PRS occurs in isolation or as a part of other syndromes such as Stickler, velocardiofacial, and Treacher-Collins syndromes (3). Because the severity of symptoms, presence of long-term sequalae, requirement of surgery, and mortality rate in syndromic PRS are much higher than non-syndromic/isolated PRS, early differentiation between the two is beneficial to clinicians as it increases their preparedness and helps them take decisions regarding the management plan (1,4,5). However, the phenotypic heterogenicity of the various associated syndromes, make it very hard for clinicians to distinguish between the two (3).…”

Three Cases of Pierre Robin Sequence with Upper Airway Obstruction Relieved by Nasopharyngeal Airway Insertion

“…Syndromic PRS is observed in 27% to 70% of all infants with PRS and shows poorer outcomes than those observed in infants with isolated PRS. A study reported by Li et al 7) determined that syndromic PRS was diagnosed in 47%…”

“…Although there are no studies comparing each non-surgical intervention, the successful use of NPA insertion has been more frequently reported than other interventions when prone position failed 1,7) . NPA insertion can be tried more easily and immediately without any apparatus-or mask-related discomfort.…”