A case of Beckwith-Wiedemann syndrome treated by tongue reduction

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Beckwith-Wiedemann syndrome, also called EMG syndrome, is characterized by exomphalos, macroglossia, and gigantism. Here we report a case of Beckwith-Wiedemann syndrome accompanied with macroglossia in a 14-year-old male.The patient was referred to our hospital for macroglossia and malocclusion in March 2008. The patient was diagnosed with Beckwith-Wiedemann syndrome by the clinical features: exomphalos, inguinal hernia, cryptorchism, macroglossia, and ear creases.The patient underwent Egyedi-Obwegeser glossectomy under general anesthesia in February 2009, and was discharged from our hospital without any subjective complaints. At the time of this report, the patient had been followed up for 22 months. There has been no evidence of recurrence and the occlusion has been improving.
特定非営利活動法人日本口腔科学会の論文