微型鼻气道正压通气系统：一种针对小下颌、罗宾序列征和舌后气道阻塞婴儿的新型分类方法。
MicroNAPS: A Novel Classification for Infants with Micrognathia, Robin Sequence, and Tongue-based Airway Obstruction.
作者信息
Resnick Cory M, Katz Eliot, Varidel Alistair
机构信息
From the Department of Oral and Maxillofacial Surgery, Harvard Medical School, Boston, Mass.
Department of Plastic and Oral Surgery, Boston Children's Hospital, Boston, Mass.
出版信息
Plast Reconstr Surg Glob Open. 2023 Sep 19;11(9):e5283. doi: 10.1097/GOX.0000000000005283. eCollection 2023 Sep.
BACKGROUND
Robin sequence (RS) describes a heterogeneous population with micrognathia, glossoptosis, and upper airway obstruction (UAO). Workup, treatment, outcomes assessment, and research inclusion are widely variable. Despite several classifications and algorithms, none is broadly endorsed. The objective of this investigation was to develop and trial a novel classification system designed to enhance clinical communication, treatment planning, prognostication, and research.
METHODS
This is a retrospective cross-sectional study. A classification system was developed with five elements: micrognathia, nutrition, airway, palate, syndrome/comorbidities (MicroNAPS). Definitions and a framework for "stage" assignment (R0-R4) were constructed. Stage "tongue-based airway obstruction" (TBAO) was defined for infants with glossoptosis and UAO without micrognathia. MicroNAPS was applied to 100 infants with at least 1-year follow-up. Clinical course, treatment, airway, and feeding characteristics were assessed. Descriptive and analytic statistics were calculated and a value less than 0.05 was considered significant.
RESULTS
Of the 100 infants, 53 were male. Mean follow-up was 5.0 ± 3.6 years. R1 demonstrated feeding-predominant mild RS for which UAO was managed nonoperatively but gastrostomy tubes were prevalent. R2 was characterized by airway-predominant moderate RS, typically managed with mandibular distraction or tongue-lip adhesion, with few gastrostomy tubes and short lengths-of-stay. R3 denoted severe RS, with similar UAO treatment to R2, but with more surgical feeding tubes and longer admissions. R4 represented a complex phenotype with 33% tracheostomies, protracted hospitalizations, and delayed palatoplasty. R0 ("at risk") and TBAO groups displayed the most variability.
CONCLUSIONS
MicroNAPS is easy to use and associated with relevant disease characteristics. We propose its adoption in clinical and research settings.
背景
罗宾序列征（RS）描述的是一组具有小颌畸形、舌后坠和上气道梗阻（UAO）的异质性人群。检查、治疗、结局评估和研究纳入标准差异很大。尽管有几种分类方法和算法，但没有一种得到广泛认可。本研究的目的是开发并试验一种新型分类系统，以加强临床沟通、治疗规划、预后评估和研究。
方法
这是一项回顾性横断面研究。开发了一个包含五个要素的分类系统：小颌畸形、营养状况、气道情况、腭部情况、综合征/合并症（MicroNAPS）。构建了“分期”（R0 - R4）的定义和框架。为无小颌畸形但有舌后坠和上气道梗阻的婴儿定义了“基于舌的气道梗阻”（TBAO）分期。将MicroNAPS应用于100例至少随访1年的婴儿。评估临床病程、治疗、气道和喂养特征。计算描述性和分析性统计数据，P值小于0.05被认为具有统计学意义。
结果
100例婴儿中，53例为男性。平均随访时间为5.0±3.6年。R1期表现为以喂养问题为主的轻度RS，上气道梗阻采用非手术治疗，但胃造瘘管使用普遍。R2期的特征是以气道问题为主的中度RS，通常采用下颌骨牵张或舌唇粘连治疗，胃造瘘管使用较少，住院时间短。R3期表示重度RS，上气道梗阻治疗与R2期相似，但手术喂养管更多，住院时间更长。R4期代表一种复杂的表型，33%的患儿需要气管切开，住院时间延长，腭裂修复延迟。R0期（“有风险”）和TBAO组表现出最大变异性。
结论
MicroNAPS易于使用且与相关疾病特征相关。我们建议在临床和研究环境中采用该分类系统。
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