Non-operative interventions for Pierre-Robin sequence: A systematic review and meta-analysis

Abstract

Pierre-Robin Sequence (PRS) is a sequence of micrognathia, glossoptosis, and airway obstruction. There is no standardized consensus on the management of respiratory distress for patients with PRS, and operative interventions have associated complications. The purpose of this study is to identify all modalities of non-operative airway intervention for PRS infants. Following PRISMA 2020 guidelines, Embase, Medline, Cochrane, EMCARE, and Web of Science electronic databases were searched from 1992 to 2022 reporting on PRS infants under one year of age who were managed non-operatively. Publications with non-original research designs, an exclusive focus on surgical interventions, case reports, and non-English language articles were excluded. Analysis was performed using non-pooled and pooled proportions (PP). 3280 abstracts were screened, and 88 articles included. Retrospective methodologies were most common. Of the 60 studies where both operative and non-operative interventions were included, 2924 of 4708 PRS infants were administered a non-operative intervention (PP 65.8 % [95%CI 58.5, 72.7]). Reported definitive non-operative interventions, either alone or in combination with another non-operative intervention, included infant positioning (n = 1664), orthodontic appliances (n = 1299), nasopharyngeal tube insertion (n = 983), supplemental oxygen (n = 306), non-invasive ventilation (n = 290), oral airway (n = 46), endotracheal intubation (n = 36), and other (n = 40). The mean MINORS risk of bias score was 6.3 (range 1–12), indicating that the present review was limited by moderate methodological quality for included studies. This is the largest systematic review of non-operative interventions for PRS infants thus far. Most infants are managed non-operatively, with positioning, orthodontic appliances, and nasopharyngeal tubes being the most commonly reported modalities.