{"title":"腭裂,唇裂,和皮埃尔·罗宾序列","authors":"M. Harvey, I. Ayad","doi":"10.1093/MED/9780190685157.003.0020","DOIUrl":null,"url":null,"abstract":"Pierre Robin sequence (PRS) is characterized by micrognathia, glossoptosis, and airway obstruction. Often associated with cleft palate, PRS is usually an isolated finding but is associated with a syndrome one-third of the time. The micrognathia and glossoptosis lead to airway obstruction, respiratory compromise, and feeding difficulties. Severe cases and prolonged and repeated airway obstruction and respiratory distress can lead to failure to thrive, hypoxemia, pulmonary hypertension, cardiac arrest, and death. Treatment options for the management of airway obstruction in PRS depends on the degree of obstruction and any associated comorbidities and range from noninvasive respiratory support to surgical correction of the physical defect. Patients with PRS should be considered as challenging to ventilate and intubate, and the practitioner should be well prepared for the possibility of difficulty with airway management. Anesthetic management is tailored to minimize postoperative upper airway obstruction and avoid disruption of the integrity of the surgical repair.","PeriodicalId":19711,"journal":{"name":"Oxford Medicine Online","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2018-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Cleft Palate, Cleft Lip, and Pierre Robin Sequence\",\"authors\":\"M. Harvey, I. Ayad\",\"doi\":\"10.1093/MED/9780190685157.003.0020\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Pierre Robin sequence (PRS) is characterized by micrognathia, glossoptosis, and airway obstruction. Often associated with cleft palate, PRS is usually an isolated finding but is associated with a syndrome one-third of the time. The micrognathia and glossoptosis lead to airway obstruction, respiratory compromise, and feeding difficulties. Severe cases and prolonged and repeated airway obstruction and respiratory distress can lead to failure to thrive, hypoxemia, pulmonary hypertension, cardiac arrest, and death. Treatment options for the management of airway obstruction in PRS depends on the degree of obstruction and any associated comorbidities and range from noninvasive respiratory support to surgical correction of the physical defect. Patients with PRS should be considered as challenging to ventilate and intubate, and the practitioner should be well prepared for the possibility of difficulty with airway management. Anesthetic management is tailored to minimize postoperative upper airway obstruction and avoid disruption of the integrity of the surgical repair.\",\"PeriodicalId\":19711,\"journal\":{\"name\":\"Oxford Medicine Online\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2018-10-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Oxford Medicine Online\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1093/MED/9780190685157.003.0020\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Oxford Medicine Online","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1093/MED/9780190685157.003.0020","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
摘要
Pierre Robin序列(PRS)的特征是小颌、光斑下垂和气道阻塞。PRS通常与腭裂有关,通常是孤立的发现,但三分之一的时间与综合征有关。小颌和舌下垂导致气道阻塞、呼吸衰竭和进食困难。严重的病例和长期反复的气道阻塞和呼吸窘迫可导致生长衰竭、低氧血症、肺动脉高压、心脏骤停和死亡。PRS气道阻塞的治疗选择取决于阻塞的程度和任何相关的合并症,范围从无创呼吸支持到物理缺陷的手术矫正。PRS患者应被认为是通气和插管困难的患者,医生应对可能出现的气道管理困难做好充分准备。麻醉管理是量身定制的,以尽量减少术后上气道阻塞,避免破坏手术修复的完整性。
Cleft Palate, Cleft Lip, and Pierre Robin Sequence
Pierre Robin sequence (PRS) is characterized by micrognathia, glossoptosis, and airway obstruction. Often associated with cleft palate, PRS is usually an isolated finding but is associated with a syndrome one-third of the time. The micrognathia and glossoptosis lead to airway obstruction, respiratory compromise, and feeding difficulties. Severe cases and prolonged and repeated airway obstruction and respiratory distress can lead to failure to thrive, hypoxemia, pulmonary hypertension, cardiac arrest, and death. Treatment options for the management of airway obstruction in PRS depends on the degree of obstruction and any associated comorbidities and range from noninvasive respiratory support to surgical correction of the physical defect. Patients with PRS should be considered as challenging to ventilate and intubate, and the practitioner should be well prepared for the possibility of difficulty with airway management. Anesthetic management is tailored to minimize postoperative upper airway obstruction and avoid disruption of the integrity of the surgical repair.