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Treacher-Collins syndrome

ORPHA:861· ICD-10 Q75.4

Definition

A rare genetic mandibulofacial dysostosis characterized by bilateral symmetrical oto-mandibular dysplasia including underdeveloped cheekbones (malar hypoplasia), a very small low jaw (micrognathia) and downward-slanting palpebral fissures, coloboma of the lower eyelids, microtia, hearing loss and without abnormalities of the extremities. Intelligence is normal.

Prevalence
1-9 / 1 000 000
Inheritance
Autosomal dominant, Autosomal recessive
Age of onset
Antenatal, Neonatal