Basart Hanneke, Kruisinga Frea H, Breugem Corstiaan C, Don Griot J Peter W, Hennekam Raoul C, Van der Horst Chantal M A M
Department of Plastic, Reconstructive and Hand Surgery, Academic Medical Centre, Amsterdam, The Netherlands.
Department of Pediatrics, Emma Children's Hospital, Amsterdam, The Netherlands.
J Craniomaxillofac Surg. 2015 Jan;43(1):92-6. doi: 10.1016/j.jcms.2014.10.015. Epub 2014 Nov 5.
Robin Sequence (RS) is characterized by micrognathia and upper airway obstruction (UAO), with or without cleft palate, causing respiratory and feeding problems. Management options are: positioning; nasopharyngeal airway (NPA); tongue-lip adhesion (TLA); mandibular distraction (MDO); and tracheostomy. Controversy exists in literature regarding RS definition and management. Here we describe definitions, management strategies and criteria in opting for management strategies, used by Dutch and Belgian cleft teams.
A specifically designed questionnaire was sent to members of all 16 Dutch and Belgian cleft teams.
14 cleft teams returned 35 questionnaires. All used micrognathia as definition criterion, 93.4% cleft palate, 51.5%glossoptosis and 45.7% UAO. Six different RS definitions were used; even within a single team >1 definition was used. All teams used different management strategies: all used positioning, 10 NPA, 6 TLA, 7 MDO, 8 tracheostomy, 5 refer patients with invasive treatment indication. Criteria in opting management modalities were: O2-saturation (89.3%), clinical presentation (86.2%), growth and feeding problems (69.0%), polysomnography (62.1%), and differed within teams.
The Dutch and Belgian cleft teams use variable RS definitions, different management modalities and criteria in choosing management strategies. A single, strict definition and evidence-based management guidelines should be formulated for optimal patient care.
罗宾序列征（RS）的特征为小颌畸形和上呼吸道梗阻（UAO），可伴有或不伴有腭裂，会导致呼吸和喂养问题。治疗选择包括：体位调整；鼻咽气道（NPA）；舌唇粘连术（TLA）；下颌骨牵张成骨术（MDO）；以及气管切开术。关于RS的定义和治疗，文献中存在争议。在此，我们描述荷兰和比利时腭裂治疗团队所使用的定义、治疗策略以及选择治疗策略的标准。
向所有16个荷兰和比利时腭裂治疗团队的成员发送了一份专门设计的问卷。
14个腭裂治疗团队返回了35份问卷。所有团队都将小颌畸形作为定义标准，93.4%将腭裂作为标准，51.5%将舌后坠作为标准，45.7%将上呼吸道梗阻作为标准。使用了六种不同的RS定义；甚至在单个团队中也使用了>1种定义。所有团队都采用了不同的治疗策略：所有团队都使用体位调整，10个团队使用鼻咽气道，6个团队使用舌唇粘连术，7个团队使用下颌骨牵张成骨术，8个团队使用气管切开术，5个团队将有创治疗指征的患者转诊。选择治疗方式的标准包括：血氧饱和度（89.3%）、临床表现（86.2%）、生长和喂养问题（69.0%）、多导睡眠图（62.1%），并且各团队之间存在差异。
荷兰和比利时腭裂治疗团队使用不同的RS定义、不同的治疗方式以及选择治疗策略的标准。应为实现最佳的患者护理制定单一、严格的定义和基于证据的管理指南。
