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Congenital velopharyngeal incompetence

ORPHA:2291· ICD-10 J39.2

Definition

A rare otorhinolaryngologic malformation characterized by the isolated finding of a short and immobile soft palate with anatomical disproportion of the velopharyngeal structures, preventing velopharyngeal closure. Patients present with delayed speech development and hypernasal speech.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal dominant
Age of onset
Neonatal