A Case of Beckwith-Wiedemann Syndrome Treated Velopharyngeal Incompetence by Speech-A id

Abstract

Cases of Beckwith-Wiedemann syndrome (BWS) combined with cleft palate are rare. In this report, a case of BWS involving cleft palate and recurrent laryngeal nerve paralysis which showed velopharyngeal incompetence after palatoplasty was reported.
A 6-year and 4 month-old boy was referred to our hospital because of a speech disorder. He was diagnosed as BWS at the department of genetics in a children's Medical Center immediately after birth. He underwent palatoplasty and tongue plasty there, but his speech showed hypernasality and breathy hoarseness.
He was advised to apply for a speech appliance for velopharyngeal incompetence and was referred to our clinic. A palatal lift prosthesis was initially adapted because of his short and weak-moving soft palate and severe gag reflex, and then followed by a speech-aid 11 months later. His velopharyngeal function was assessed as in good condition a year after the treatment. We suggest that the speech appliance is effective for treatment of velopharyngeal incompetence in patients who are thought to be poor candidates for secondary surgery.