Breugem C C, Mink van der Molen A B
Division of Pediatric Plastic Surgery, Wilhelmina Children's Hospital, University Medical Center Utrecht, 3508 AB, Utrecht, The Netherlands.
J Plast Reconstr Aesthet Surg. 2009 Dec;62(12):1555-8. doi: 10.1016/j.bjps.2008.06.050. Epub 2008 Nov 1.
Different nosology has hampered our understanding of patients with Robin sequence. Defining this disorder correctly has important consequences for physicians and parents. While reviewing treatment options for Robin sequence we were surprised to see how often different definitions were used to describe this condition. This prompted us to perform a review into the current understanding when diagnosing and defining this disorder. At our Annual Dutch Cleft Palate Meeting a questionnaire was given to all those attending requesting them to summarise characteristics needed for a definition of 'Robin sequence'. Sixty-six questionnaires were returned, demonstrating 29 different descriptions. Our study demonstrates that there is widespread confusion in the Netherlands defining Robin sequence. This lack of uniformity in the definition hampers the comparison of outcome studies. The treatment of patients with Robin sequence often involves multidisciplinary involvement, making it crucial to have one common definition. We suggest using the description originally summarised by Pierre Robin consisting of micrognathia, glossoptosis and airway compromise. Once the diagnosis of Robin sequence is made other adjuncts such as an associated cleft palate, syndrome or feeding problems could be added to the description.
不同的疾病分类学阻碍了我们对罗宾序列征患者的理解。正确定义这种疾病对医生和家长都有重要意义。在回顾罗宾序列征的治疗方案时，我们惊讶地发现，描述这种病症时使用不同定义的情况是多么常见。这促使我们对目前在诊断和定义这种疾病时的理解进行一次综述。在我们的荷兰腭裂年会期间，向所有与会者发放了一份问卷，要求他们总结“罗宾序列征”定义所需的特征。共收回66份问卷，呈现出29种不同的描述。我们的研究表明，在荷兰，对于罗宾序列征的定义存在广泛的混淆。定义缺乏一致性阻碍了疗效研究的比较。罗宾序列征患者的治疗通常需要多学科参与，因此有一个共同的定义至关重要。我们建议采用皮埃尔·罗宾最初总结的描述，包括小下颌、舌后坠和气道受阻。一旦做出罗宾序列征的诊断，其他辅助特征，如伴发的腭裂、综合征或喂养问题等，可以补充到描述中。
