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J Korean Soc Plast Reconstr Surg. 1997 Jan;24(1):199-204. Korean. Case Report.
Ahn KY , Jang KS , Park DH , Hwang JB .
Department of Plastic and Reconstructive Surgery, College of Medicine, Catholic University of Taegu-Hyosung, School of Medicine, Taegu, Korea.
Department of Pediatrics, College of Medicine, Catholic University of Taegu-Hyosung, School of Medicine, Taegu, Korea.
Abstract

The Beckwith-Wiedemann syndrome, originally described by Beckwith in 1963 and Wiedemann in 1964, included congenital anomalies such as macroglossia, exomphalos, postnatal somatic gigantism, severe hypoglycemia, abdominal wall defect, capillary nevus flameus and hemihypertrophy. Macroglossia is the most common manifestation of Beckwith-Wiedemann syndrome, with studies reporting between 82 and 95 percent of the cases. Macroglossia may cause the upper airway obstruction, deglutition difficulty, articulation interference and protrusion of dentoalveloar structures resulting in anterior open bite and a mandibular prognathism. We experienced a 5 month-old male with upper airway obstruction, deglutition difficulty and recurrent upper airway infection due to macroglossia associated with Beckwith-Wiedemann syndrome and significant improvement in respiration, feeding and oral competence at 14 months follow-up after rhomboid resection and primary closure of tongue.

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