Bütow Kurt-W, Zwahlen Roger Arthur, Morkel Jean A, Naidoo Sharan
Department of Maxillofacial and Oral Surgery, Facial Cleft Deformity Clinic, University of Pretoria, Durban, South Africa; Suite A2-Maxillofacial Surgery, The Wilgers Hospital, Pretoria, Durban, South Africa; Department of Maxillofacial Surgery, Division of Dentistry, College of Health Sciences, University of KwaZulu-Natal, Durban, South Africa.
Discipline of Oral and Maxillofacial Surgery, Faculty of Dentistry, University of Hong Kong, Hong Kong SAR, PR China.
Ann Maxillofac Surg. 2016 Jan-Jun;6(1):31-4. doi: 10.4103/2231-0746.186133.
The disorder currently accepted as Pierre Robin syndrome/anomaly/sequence (PRS) has been plagued by controversy ever since initially being described. Controversy exists not only about the appropriate terminology and etiopathogenesis of the disorder but also about its management. Therefore, clinical findings and treatment outcomes of a large database of 266 PRS cases were compared with the current state of knowledge in scientific literature related to history, clinical description, diagnostic criteria, epidemiology, theories of oligohydramnios, mandibular catch-up growth, midfacial hyperplasia, and the timing of management.
AIMS OF PART 1: Display disparities of the widely published subject of PRS that exist within the literature.
A literature search related to diagnostic criteria was compared to findings of one of the largest PRS databases worldwide.
Regarding diagnostic criteria two subdivisions, the Fairbairn-Robin triad (FRT) and the Siebold-Robin sequence (SRS) can be clearly distinguished. Both present with micrognathia and glossoptosis, the former with, the latter, however, without a palatal cleft.
According to clear diagnostic criteria, PRS has to be subdivided in the future into FRT and SRS cases, as they may require different treatment approaches.
目前被认定为皮埃尔·罗宾综合征/异常/序列（PRS）的病症自最初被描述以来就一直备受争议。争议不仅涉及该病症的恰当术语和病因发病机制，还包括其治疗方法。因此，将一个包含266例PRS病例的大型数据库的临床发现和治疗结果，与科学文献中有关病史、临床描述、诊断标准、流行病学、羊水过少理论、下颌追赶生长、面中部增生以及治疗时机等方面的现有知识状况进行了比较。
展示文献中关于PRS这一广泛发表主题存在的差异。
将与诊断标准相关的文献检索结果与全球最大的PRS数据库之一的研究结果进行比较。
关于诊断标准，可明确区分出两个细分类型，即费尔贝恩 - 罗宾三联征（FRT）和西博尔德 - 罗宾序列（SRS）。两者均表现为小下颌和舌后坠，前者伴有腭裂，而后者不伴有腭裂。
根据明确的诊断标准，未来PRS应细分为FRT和SRS病例，因为它们可能需要不同的治疗方法。
