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Cleft palate and Beckwith-Wiedemann syndrome.

Laroche C, Testelin S, Devauchelle B

Stomatologie and Maxillofacial Service, CHI Poissy-Saint-Germain-en-Laye Hospital, Saint Germain en Laye, France. carinelaroche@hotmail.com

OBJECTIVE: Patients with Beckwith-Wiedemann syndrome suffer numerous anomalies, which vary somewhat from case to case. Cleft palate in combination with this syndrome has rarely been reported in the literature. Through two cases, this report examines the staging of the surgical repairs and the role of macroglossia in cleft palate and the consequences of the scarred palate on mandibular development. RESULTS: Of four patients with Beckwith-Wiedemann syndrome, only two had a cleft palate. The timing of the repair in these two children was different. Speech development was satisfactory in the first case but mediocre in the second. This result seemed to be related to a poor social environment. Mandibular prognathism persisted in both cases. CONCLUSION: The treatment of patients with cleft palate and Beckwith-Wiedemann syndrome remains complex. It is preferable not to operate on a cleft palate before performing a tongue reduction plasty, but rather to combine these two surgical interventions. This would reduce the risks of anesthesia and enable the palate to heal more efficiently. Surgical treatment should be performed after the age of 6 months and before problems in speech development occur. An orthognathic surgery at adolescence could be performed if prognathism persists. While the origin of the cleft palate is still being discussed, we cannot claim that macroglossia is related to the development of cleft palate, nor that the scarred palate has an impact on the mandibular development.

Published 7 March 2005 in Cleft Palate Craniofac J, 42(2): 212-7.
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