A CASE OF TONGUE RESECTION IN MACROGLOSSIA AS A PRESENTATION OF BECKWITH-WIEDEMANN SYNDROME.

Ahn, Ki Young; Jang, Kyoung Soo; Park, Dae Hwan; Hwang, Jin Bok

Case Report

Journal of the Korean Society of Plastic and Reconstructive Surgeons 1997;24(1):199-204.
Published online January 1, 1997.

A CASE OF TONGUE RESECTION IN MACROGLOSSIA AS A PRESENTATION OF BECKWITH-WIEDEMANN SYNDROME.

Ki Young Ahn, Kyoung Soo Jang, Dae Hwan Park, Jin Bok Hwang

¹Department of Plastic and Reconstructive Surgery, College of Medicine, Catholic University of Taegu-Hyosung, School of Medicine, Taegu, Korea.
²Department 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.

Keywords: Macrossia; Beckwith-Wiedemann syndrome; Tongue resection