罗宾序列:新生儿管理。

Q2 Medicine NeoReviews Pub Date : 2024-12-01 DOI:10.1542/neo.25-12-e780
Tyler Van Heest, Ethan G Muhonen, Gregory C Allen
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引用次数: 0

摘要

Robin序列(RS)是由小颌、舌下垂和气道阻塞的临床三联征定义的。舌下垂的存在也会阻碍腭架在发育过程中的融合,导致特征性的宽u型腭裂。RS可单独出现(孤立性RS)或与先天性综合征或其他异常(综合征性RS或RS +)相关。诊断主要是临床,但可以确定产前超声检查或磁共振成像。RS的治疗重点是改善光泽度和缓解气道阻塞。保守治疗包括体位、鼻咽气道、正畸器具和呼吸支持策略。手术干预包括气管切开术、舌唇粘连和下颌牵张成骨术。所有的管理策略在适当选择的患者中都是有效的。未来的研究领域包括了解RS的遗传学,通过术前计划和先进的生物材料改善手术结果,以及改善临床显著RS患儿的产前识别。
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Robin Sequence: Neonatal Management.

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.

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来源期刊
NeoReviews
NeoReviews Medicine-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.
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