| Literature DB >> 24776174 |
Alistair R M Cobb1, Ben Green2, Daljit Gill3, Peter Ayliffe4, Timothy W Lloyd5, Neil Bulstrode6, David J Dunaway2.
Abstract
Treacher Collins syndrome (TCS), mandibulofacial dysostosis, or Franceschetti-Zwahlen-Klein syndrome, is a rare genetic disorder characterised by dysgenesis of the hard and soft tissues of the first and second branchial arches. Early operations focus on maintaining the airway, protecting the eyes, and supporting auditory neurological development. Later operations include staged reconstruction of the mouth, face, and external ear. Bimaxillary surgery can improve the maxillomandibular facial projection, but correction of malar, orbital rim, and temporal defects may be more difficult. We present a clinical review of the syndrome with a chronological approach to the operations.Entities:
Keywords: Franceschetti–Zwahlen–Klein syndrome; Mandibulofacial dysostosis; Treacher Collins syndrome
Mesh:
Year: 2014 PMID: 24776174 DOI: 10.1016/j.bjoms.2014.02.007
Source DB: PubMed Journal: Br J Oral Maxillofac Surg ISSN: 0266-4356 Impact factor: 1.651