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KMID : 0352819950100020061
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Kosin Medical Journal
1995 Volume.10 No. 2 p.61 ~ p.70
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THE CORRECTION OF TREACHER COLLINS SYNDROME
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Abstract
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Abstract Treacher Collins syndrome, also known as mandibulofacial dysostosis, is an inherited disorder transmitted by an autosomal dominant gene. This syndrome is a rare bilateral congenital deformity occuring in 1 in 10.000 births. It is a
syndrome
with a very wide spectrum of manifestatios characterized by distorsions of the orbit secondary to hypoplasia of the maxilla, mandible, and most markedly of the zygoma. Soft tissue deformities include lower lid colobomas and dystopia of the
lateral
canthus, microtia, and a paucity of the muscular aponeurosis of the midface. This syndrome is accompanied by significant hearing loss, early failure to thrive, chronic respiratory insufficient and sleep apnea. Recently, attention has been focused
of the
zygomatic deficiency, and several techniques have been advocated for its repair. These include autogenous rib onlay grafting, autogenous split-thickness cranial bone onlay grafting and vasculized pedicled split-thickness cranial bone flaps. The
author
experienced an 19 year old female patient of Treacher Collins syndrome who were noted an examination to have bilateral malar hypoplasia and corrected it by means of rib bone graft and depressed malar eminence corrected by rib bone onlay graft and
also
corrected eyelid
coloboma with chondrocutaneous composite graft.
We have followed the patient for 3 months and got a satisfactory result.
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KEYWORD
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