Basart H, König A M, Bretschneider J H, Hoekstra C E L, Oomen K P Q, Pullens B, Rinkel R N P M, van Gogh C D L, van der Horst C M A M, Hennekam R C
Department of Pediatrics, Academic Medical Center/Emma Pediatric Hospital, Amsterdam, Netherlands.
Department of Plastic, Reconstructive and Hand Surgery, Academic Medical Center, University of Amsterdam, Amsterdam, Netherlands.
Clin Otolaryngol. 2016 Oct;41(5):467-71. doi: 10.1111/coa.12552. Epub 2016 Feb 9.
Robin Sequence (RS) is usually defined as the combination of micrognathia, glossoptosis and upper airway obstruction. No objective criteria to diagnose RS exist. To compare management strategy results, a single RS definition using objective criteria is needed. The most frequently used primary diagnostic tool for glossoptosis is awake Flexible Fiberoptic Laryngoscopy (aFFL).
To determine the reliability of the aFFL videos as an independent diagnostic tool itself, rather than on the complete evaluation of a patient.
DESIGN, SETTING, PARTICIPANTS: All RS individuals from an existing cohort with an available aFFL video were included retrospectively. Thirty age-matched patients without pathologic findings on aFFL were used as controls. aFFL videos were scored by six otolaryngologists as: a. Marked glossoptosis, b. Mild glossoptosis, c. Severity unknown, d. No glossoptosis, e. Insufficient video quality. Videos were anonymised and rated twice, in altered sequences, after a washout period of minimally 2 weeks.
Inter-rater and intrarater agreement.
Twenty-six videos of 16 RS patients and 30 videos of controls were included. Inter-rater agreement was fair in the whole group (κ: 0.320) and RS group (κ: 0.226), and fair to moderate in determining presence of glossoptosis (total group κ: 0.430; RS κ: 0.302; controls κ: 0.212). The intrarater agreement for the presence of glossoptosis in RS was moderate (κ: 0.541).
aFFL offers fair to moderate inter-rater agreement, with moderate intrarater agreement, in evaluating glossoptosis in RS. Using aFFL as the single tool in choosing management strategies in RS seems insufficient. There is need for a more reliable, patient friendly diagnostic tool or an internationally accepted aFFL scoring system, to diagnose glossoptosis in RS.
罗宾序列征（RS）通常被定义为小下颌、舌后坠和上气道阻塞的组合。目前尚无诊断RS的客观标准。为了比较治疗策略的结果，需要一个使用客观标准的单一RS定义。用于舌后坠的最常用的主要诊断工具是清醒状态下的纤维喉镜检查（aFFL）。
确定aFFL视频作为一种独立诊断工具本身的可靠性，而非基于对患者的全面评估。
设计、地点、参与者：对现有队列中所有有可用aFFL视频的RS个体进行回顾性纳入。30名年龄匹配且aFFL检查无病理结果的患者作为对照。6名耳鼻喉科医生对aFFL视频进行评分：a. 明显舌后坠；b. 轻度舌后坠；c. 严重程度未知；d. 无舌后坠；e. 视频质量不足。视频进行了匿名处理，并在至少2周的洗脱期后，以不同顺序进行了两次评分。
评分者间和评分者内一致性。
纳入了16例RS患者的26个视频和30个对照视频。评分者间一致性在整个组中为中等（κ：0.320），在RS组中为中等（κ：0.226），在确定舌后坠的存在方面为中等至良好（整个组κ：0.430；RS κ：0.302；对照κ：0.212）。RS患者中舌后坠存在情况的评分者内一致性为中等（κ：0.541）。
在评估RS患者的舌后坠时，aFFL的评分者间一致性为中等至良好，评分者内一致性为中等。将aFFL作为选择RS治疗策略的单一工具似乎并不充分。需要一种更可靠、对患者更友好的诊断工具或国际认可的aFFL评分系统来诊断RS患者的舌后坠。
