Beckwith-Wiedemann 증후군 3개월 여아에서 시행된 설절제술
Reduction Glossectomy in a 3-Month-Old Female Infant With Beckwith-Wiedemann Syndrome
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Beckwith-Wiedemann syndrome (BWS) is a genetic overgrowth disorder presenting with vari- ous clinical manifestations, including macroglossia, visceromegaly, gigantism, neonatal hypo- glycemia, and anterior abdominal wall defects. Most patients with BWS suffer from swallow- ing and respiratory difficulty due to macroglossia, which requires surgical intervention. However, the tongue has a complex neuromuscular anatomy which is closely related to swal- lowing, phonation, and respiration. Therefore, care should be taken when performing reduction glossectomy for BWS patients to minimize complication after surgery. This case report de- scribes a successful surgical intervention for macroglossia in a 3-month-old female infant di- agnosed with BWS, who presented respiratory and swallowing difficulty. The keyhole shape glossectomy was performed and the patient was orally fed without respiratory difficulty 3 weeks following the surgery.
Abstract
Beckwith-Wiedemann syndrome (BWS) is a genetic overgrowth disorder presenting with vari- ous clinical manifestations, including macroglossia, visceromegaly, gigantism, neonatal hypo- glycemia, and anterior abdominal wall defects. Most patients with BWS suffer from swallow- ing and respiratory difficulty due to macroglossia, which requires surgical intervention. However, the tongue has a complex neuromuscular anatomy which is closely related to swal- lowing, phonation, and respiration. Therefore, care should be taken when performing reduction glossectomy for BWS patients to minimize complication after surgery. This case report de- scribes a successful surgical intervention for macroglossia in a 3-month-old female infant di- agnosed with BWS, who presented respiratory and swallowing difficulty. The keyhole shape glossectomy was performed and the patient was orally fed without respiratory difficulty 3 weeks following the surgery.