Treatment of the Craniofacial Complications of Beckwith-Wiedemann Syndrome

Surgical treatment of macroglossia in patients with Beckwith–Wiedemann syndrome: a 20-year experience and review of the literature

“…Tongue reduction procedures are often performed in BWS patients when macroglossia causes one or more of the following problems 10,27,28 : functional deficits, such as airway obstruction, deglutition, drooling, and phonation difficulties; oro-facial alterations from excessive action of the tongue on the surrounding structures, such as the increase of the gonial angle and of the anterior facial high with anterior open-bite development, and diastema; and abnormal physical appearance of the patient, with lingual protrusion, dyslalia, and drooling, giving the false impression of mental deficiency. Numerous surgical techniques have been developed for the treatment of macroglossia 6,9,11,16,17,21,29,42 .…”

Impact of Tongue Reduction on Overall Speech Intelligibility, Articulation and Oromyofunctional Behavior in 4 Children with Beckwith-Wiedemann Syndrome

“…One can hypothesize that several factors have contributed to the improvement of speech. First, the partial glossectomy was performed in the first few years of life (between 0.5 and 3 years) as suggested by several authors [41,42] when speech development normally progresses quickly. In this way, one can assume that there is a greater chance that the child will develop age-appropriate speech patterns and that compensatory strategies (like the presence of glottal stops) or distortions (like rhotacism or sigmatism) will be prevented.…”

Long-term impact of tongue reduction on speech intelligibility, articulation and oromyofunctional behaviour in a child with Beckwith–Wiedemann syndrome

“…One can hypothesize that several factors have contributed to the improvement. First of all the partial glossectomy was performed in the first few years of life (age 2.10 years) as suggested by several authors [14,34,35] when speech development normally progresses quickly. If speech development was the only focus of concern one would advocate tongue reduction surgery before the onset of babbling.…”