J Korean Soc Plast Reconstr Surg.  1997 Jan;24(1):199-204.

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

• Ahn KY
• Jang KS
• Park DH
• Hwang JB

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.

Keyword

Macrossia; Beckwith-Wiedemann syndrome; Tongue resection

MeSH Terms

Abdominal Wall
Airway Obstruction
Beckwith-Wiedemann Syndrome*
Capillaries
Deglutition
Follow-Up Studies
Gigantism
Hernia, Umbilical
Humans
Hypoglycemia
Infant
Macroglossia*
Male
Mental Competency
Nevus
Open Bite
Prognathism
Respiration
Tongue*