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
Background: Substantial health inequities exist for Indigenous Peoples in Canada. The remote and distributed population of Canada presents unique challenges for access to and use of surgery. To date, the surgical outcome data for Indigenous Peoples in Canada have not been synthesized. Methods:We searched 4 databases to identify studies comparing surgical outcomes and utilization rates of adults of First Nations, Inuit or Métis identity with non-Indigenous people in Canada. Independ ent reviewers completed all stages in duplicate. Our primary outcome was mortality; secondary outcomes included utilization rates of surgical procedures, complications and hospital length of stay. We performed meta-analysis of the primary outcome using random effects models. We assessed risk of bias using the ROBINS-I tool.Results: Twenty-eight studies were reviewed involving 1 976 258 participants (10.2% Indigenous). No studies specifically addressed Inuit or Métis populations. Four studies, including 7 cohorts, contributed adjusted mortality data for 7135 participants (5.2% Indigenous); Indigenous Peoples had a 30% higher rate of death after surgery than non-Indigenous patients (pooled hazard ratio 1.30, 95% CI 1.09-1.54; I 2 = 81%). Complications were also higher for Indigenous Peoples, including infectious complications (adjusted OR 1.63, 95% CI 1.13-2.34) and pneumonia (OR 2.24,). Rates of various sur gical procedures were lower, including rates of renal transplant, joint replacement, cardiac surgery and cesarean delivery. Interpretation:The currently available data on postoperative outcomes and surgery utilization rates for Indigenous Peoples in Canada are limited and of poor quality. Available data suggest that Indigen ous Peoples have higher rates of death and adverse events after surgery, while also encountering barriers accessing surgical procedures. These findings suggest a need for substantial re-evaluation of surgical care for Indigenous Peoples in Canada to ensure equitable access and to improve outcomes. Protocol registration: PROSPERO-CRD42018098757 Disclaimer: Three of the authors identify as Indigenous: Jason McVicar (Métis), Nadine Caron (First Nations) and Donna May Kimmiliardjuk (Inuk).
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