title: Pierre Robin sequence: Subdivision, data, theories, and treatment - Part 2: Syndromic and nonsyndromic Pierre Robin sequence.
abstract: BACKGROUND
  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. 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, relating to history, clinical description, diagnostic criteria, epidemiology, theories of oligohydramnios, mandibular catch-up growth, midfacial hyperplasia, and the early management.
  Contribute to the sparse scientific knowledge about pathogenesis and involved genetics.
  
  
  METHODS
  An analysis of this large database was conducted focusing on genetic involvement, family history, and the incidence of additional syndromes.
  
  
  RESULTS
  Beside of differences related to clinical signs of dyspnea, feeding problems and mortality rates, various concomitant syndromes, and genetic abnormalities were found in cases of Fairbairn-Robin triad (FRT) and Siebold-Robin sequence (SRS), in addition to differences in relation to clinical signs of dyspnea, feeding problems, and mortality rates.
  
  
  CONCLUSIONS
  Multiple FRT cases presented with various concomitant syndromes and genetic abnormalities, but only one type occurred in two SRS cases. The latter presented a significantly different mortality rate when compared to the FRT subgroup.
authors: 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.
authors: Kurt-W
authors: KW
authors: Bütow
authors: Department Maxillofacial and Oral Surgery, University of the Western Cape, Cape Town, South Africa.
authors: Jean A
authors: JA
authors: Morkel
authors: Department of Maxillofacial and Oral Surgery, Facial Cleft Deformity Clinic, University of Pretoria, Durban, South Africa.
authors: Sharan
authors: S
authors: Naidoo
authors: Discipline of Oral and Maxillofacial Surgery, Faculty of Dentistry, University of Hong Kong, Hong Kong SAR, PR China.
authors: Roger Arthur
authors: RA
authors: Zwahlen
keywords: Fairbairn–Robin triad; Pierre Robin sequence; Siebold–Robin sequence; glossoptosis; micrognathia; pathogenesis