A case of Beckwith-Wiedemann syndrome in a patient undergoing partial tongue resection for macroglossia

The Beckwith-Wiedemann syndrome, also called EMG syndrome, is characterized by umbilical cord hernia, macroglossia, and gigantism. It may be accompanied by deformity of the ear lobe, hypertrophy of the internal organs, low blood sugar, and hemihypertrophy.We report a case of Beckwith-Wiedemann syndrome in a 1-year-3-month-old girl. The diagnosis was made at the Department of Pediatrics, Kansai Medical College Hospital. She had no erupted teeth, and could not close her mouth completely because of macroglossia. There was also hemihypertrophy on her right side at presentation. The patient had occasional cramps because of low blood sugar (33mg/dl). Vshaped resection of the tongue was performed with the patient under general anesthesia after the blood sugar level had recovered to 76mg/dl. The patient made an uneventful recovery and was able to close her mouth. Eleven years after surgical treatment, she had no difficulty in mastication, swallowing, and speech. However, there was some impairment in the ability to sense acid and bitter tastes, and eruption of the permanent teeth was delayed.