Treacher Collins syndrome: present concepts of the disorder and their surgical correction.

Abstract:

:Treacher Collins Syndrome is a rare bilateral congenital deformity occurring in 1 in 10,000 births. It is also known, in the European literature, as Franceschetti Syndrome, and is additionally known as mandibulofacial dysostosis. It is a syndrome with a very wide spectrum of manifestations characterized by distortions of the orbit secondary to hypoplasia of the maxilla, mandible, and, most markedly, of the zygoma. Soft tissue deformities include lower lid colobomas, laxity and dystopia of the lateral canthus, microtia, and a paucity of the muscular aponeurosis of the midface. The syndrome is frequently accompanied by significant hearing loss, early failure to thrive, chronic respiratory insufficiency, and sleep apnea. Intelligence is usually within normal limits although learning disabilities are common in early life. These major anatomical and physiological abnormalities, as well as the psychological and social stigma associated with severe facial deformity, make this syndrome one of the most challenging reconstructive problems presented to the craniofacial surgeon.

journal_name

World J Surg

journal_title

World journal of surgery

authors

Argenta LC,Iacobucci JJ

doi

10.1007/BF01660753

subject

Has Abstract

pub_date

1989-07-01 00:00:00

pages

401-9

issue

4

eissn

0364-2313

issn

1432-2323

journal_volume

13

pub_type

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