Korean J Otorhinolaryngol-Head Neck Surg.  2025 Feb;68(2):74-77. 10.3342/kjorl-hns.2024.00185.

Reduction Glossectomy in a 3-Month-Old Female Infant With Beckwith-Wiedemann Syndrome

• Kim G
• Oh S
• Kim Y
• Choi N

• KMID: 2565005
• DOI: http://doi.org/10.3342/kjorl-hns.2024.00185

Abstract

Beckwith-Wiedemann syndrome (BWS) is a genetic overgrowth disorder presenting with various clinical manifestations, including macroglossia, visceromegaly, gigantism, neonatal hypoglycemia, and anterior abdominal wall defects. Most patients with BWS suffer from swallowing and respiratory difficulty due to macroglossia, which requires surgical intervention. However, the tongue has a complex neuromuscular anatomy which is closely related to swallowing, phonation, and respiration. Therefore, care should be taken when performing reduction glossectomy for BWS patients to minimize complication after surgery. This case report describes a successful surgical intervention for macroglossia in a 3-month-old female infant diagnosed 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.

Keyword

Beckwith-Wiedemann syndrome; Glossectomy; Macroglossia