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, University of Hong Kong, Hong Kong S. A. R., PR China.
Ann Maxillofac Surg. 2016 Jan-Jun;6(1):38-43. doi: 10.4103/2231-0746.186135.
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 the scientific literature related to history, clinical description, diagnostic criteria, epidemiology, theories of oligohydramnios, mandibular catch-up growth, midfacial hyperplasia, and the early management.
The aims of Part 3 debate the controversial biological theories relating to PRS.
Oligo-/poly-hydramnios, mandibular catch-up growth, and midfacial hyperplasia, the three in the literature most prevailing theories related to PRS, have been compared and discussed with the findings provided by this large database of 266 Siebold-Robin sequence (SRS) and Fairbairn-Robin triad (FRT) cases.
History and clinical findings evaluated in this database refute the first two theories. Although manifold midfacial appearances were demonstrated in FRT cases, a third of all SRS cases presented with mid-facial hyperplasia.
The three main biological theories regarding PRS could not be verified after thorough analysis of the database.
目前被认可的皮埃尔·罗宾综合征/异常/序列（PRS）自最初被描述以来就一直饱受争议。争议不仅存在于该疾病的恰当术语和病因发病机制方面，还存在于其治疗管理方面。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、面中部增生以及早期管理的现有知识状况进行了比较。
第三部分的目的是对与PRS相关的有争议的生物学理论展开辩论。
将文献中与PRS最相关的三个主流理论，即羊水过少/过多、下颌追赶生长和面中部增生，与这个包含266例西博尔德 - 罗宾序列（SRS）和费尔贝恩 - 罗宾三联征（FRT）病例的大型数据库所提供的研究结果进行了比较和讨论。
该数据库中评估的病史和临床发现反驳了前两个理论。尽管在FRT病例中表现出多种面中部外观，但所有SRS病例中有三分之一出现了面中部增生。
在对数据库进行全面分析后，关于PRS的三个主要生物学理论无法得到证实。
