Cleft palate and congenital palatopharyngeal incompetency in mandibulofacial dysostosis: frequency and problems in treatment.

In a series of 25 cases of mandibulofacial dysostosis, 7 patients had isolated clefts of the palate, 1 had a complete unilateral cleft of the lip and palate, and 8 had congenital palatopharyngeal incompetency (CPI). The CPI appeared in four forms: (a) complete agenesis of the soft palate, (b) foreshortening of the soft palate associated with a submucous defect of the hard palate, (c) submucous defect of the hard palate with adequate palatal length but inadequate elevation in speech, and (d) lack of adequate palatal elevation in the absence of a submucous defect or reduced length of the soft palate. Inadequate velopharyngeal function, whether congenital or subsequent to palatal repair, may be masked by the presence of other speech problems in this syndrome, particularly by the "muffled" voice quality which appears to be associated with an elevated and retracted tongue posture. Both prosthetic and surgical treatment of inadequate velopharyngeal function are complicated in patients with MFD by other structural anomalies, particularly inadequate oral opening and constriction of the airway.