{"title":"罗宾序列:新生儿管理。","authors":"Tyler Van Heest, Ethan G Muhonen, Gregory C Allen","doi":"10.1542/neo.25-12-e780","DOIUrl":null,"url":null,"abstract":"<p><p>Robin sequence (RS) is defined by the clinical triad of micrognathia, glossoptosis, and airway obstruction. The presence of glossoptosis can also obstruct fusion of the palatal shelves during development, resulting in the characteristic wide U-shaped cleft palate. RS can present in isolation (isolated RS) or in association with a congenital syndrome or other abnormalities (syndromic RS or RS plus). Diagnosis is primarily clinical but can be identified prenatally on ultrasonography or magnetic resonance imaging. Management of RS focuses on improving glossoptosis and relieving airway obstruction. Conservative management includes positioning, nasopharyngeal airway, orthodontic appliances, and respiratory support strategies. Surgical interventions include tracheostomy, tongue-lip adhesion, and mandibular distraction osteogenesis. All management strategies can be effective in the appropriately selected patient. Future areas of research include understanding the genetics of RS, improving surgical outcomes with preoperative planning and advanced biomaterials, and improving prenatal identification of children with clinically significant RS.</p>","PeriodicalId":19465,"journal":{"name":"NeoReviews","volume":"25 12","pages":"e780-e792"},"PeriodicalIF":0.0000,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Robin Sequence: Neonatal Management.\",\"authors\":\"Tyler Van Heest, Ethan G Muhonen, Gregory C Allen\",\"doi\":\"10.1542/neo.25-12-e780\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Robin sequence (RS) is defined by the clinical triad of micrognathia, glossoptosis, and airway obstruction. The presence of glossoptosis can also obstruct fusion of the palatal shelves during development, resulting in the characteristic wide U-shaped cleft palate. RS can present in isolation (isolated RS) or in association with a congenital syndrome or other abnormalities (syndromic RS or RS plus). Diagnosis is primarily clinical but can be identified prenatally on ultrasonography or magnetic resonance imaging. Management of RS focuses on improving glossoptosis and relieving airway obstruction. Conservative management includes positioning, nasopharyngeal airway, orthodontic appliances, and respiratory support strategies. Surgical interventions include tracheostomy, tongue-lip adhesion, and mandibular distraction osteogenesis. All management strategies can be effective in the appropriately selected patient. Future areas of research include understanding the genetics of RS, improving surgical outcomes with preoperative planning and advanced biomaterials, and improving prenatal identification of children with clinically significant RS.</p>\",\"PeriodicalId\":19465,\"journal\":{\"name\":\"NeoReviews\",\"volume\":\"25 12\",\"pages\":\"e780-e792\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-12-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"NeoReviews\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1542/neo.25-12-e780\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q2\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"NeoReviews","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1542/neo.25-12-e780","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"Medicine","Score":null,"Total":0}
Robin sequence (RS) is defined by the clinical triad of micrognathia, glossoptosis, and airway obstruction. The presence of glossoptosis can also obstruct fusion of the palatal shelves during development, resulting in the characteristic wide U-shaped cleft palate. RS can present in isolation (isolated RS) or in association with a congenital syndrome or other abnormalities (syndromic RS or RS plus). Diagnosis is primarily clinical but can be identified prenatally on ultrasonography or magnetic resonance imaging. Management of RS focuses on improving glossoptosis and relieving airway obstruction. Conservative management includes positioning, nasopharyngeal airway, orthodontic appliances, and respiratory support strategies. Surgical interventions include tracheostomy, tongue-lip adhesion, and mandibular distraction osteogenesis. All management strategies can be effective in the appropriately selected patient. Future areas of research include understanding the genetics of RS, improving surgical outcomes with preoperative planning and advanced biomaterials, and improving prenatal identification of children with clinically significant RS.
NeoReviewsMedicine-Pediatrics, Perinatology and Child Health
CiteScore
2.20
自引率
0.00%
发文量
110
期刊介绍:
Co-edited by Alistair G.S. Philip, MD, FAAP, and William W. Hay Jr., MD, FAAP, NeoReviews each month delivers 3 to 4 clinical reviews, case discussions, basic science insights and "on the horizon" pieces. Written and edited by experts, these concise reviews are available to NeoReviews subscribers at http://neoreviews.aappublications.org. Since January 2009, all clinical articles have been mapped to the American Board of Pediatrics (ABP) content specifications in neonatology.