초록
Beckwith-Wiedemann syndrome은 다양한 임상적 특징을 보이는 질환이다. 거대설은 BWS 환아에서 가장 흔히 관찰되는 특징으로 그로 인한 섭식 문제와 호흡곤란이 야기될 수 있어 치과 치료 시, 주의를 요하게 된다. 또한 거대설로 야기될 수 있는 골격적인 문제에 대한 장기적인 평가가 필요하다.
Beckwith-Wiedemann syndrome comprises multiple congenital anomalies with a risk of development of childhood tumors. Macroglossia is the predominent finding in Beckwith-Wiedemann syndrome and other findings include postnatal somatic gigantism, abdominal wall defects, nevus flammeus of the face, congenital heart defects, and cleft palate. The aim of this case presentation is to describe the dental treatment of a child with Beckwith-Wiedemann syndrome. A 3-year-old boy with Beckwith-Wiedemann syndrome visited to the Seoul National University Dental Hospital for dental evaluation and treatment. Due to the multiple caries and poor cooperation, we planned to perform the dental procedure under general anesthesia. The dental procedure was successfully performed and there was no airway complications after extubation. This case suggests that general anesthesia may be useful for the patient with uncooperative Beckwith-Wiedemann syndrome, since macroglossia and laryngomalacia may exacerbates airway compromise during dental sedation.
