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Br J Oral Maxillofac Surg. 2014 Sep;52(7):581-9. doi: 10.1016/j.bjoms.2014.02.007. Epub 2014 Apr 26.

The surgical management of Treacher Collins syndrome.

Author information

1
Southwest Cleft Service and Oral & Maxillofacial Surgery Department, North Bristol NHS Trust, Bristol, United Kingdom. Electronic address: alicobb@yahoo.com.
2
Craniofacial Service, Great Ormond Street Hospital, United Kingdom.
3
Orthodontic Department, Great Ormond Street Hospital, London, United Kingdom.
4
Maxillofacial Surgery Department, Great Ormond Street Hospital, London, United Kingdom.
5
Adult Craniofacial Surgery Department, University College London Hospitals, United Kingdom.
6
Plastic Surgery Department, Great Ormond Street Hospital, London, United Kingdom.

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.

KEYWORDS:

Franceschetti–Zwahlen–Klein syndrome; Mandibulofacial dysostosis; Treacher Collins syndrome

PMID:
24776174
DOI:
10.1016/j.bjoms.2014.02.007
[Indexed for MEDLINE]

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