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가부키 증후군 환자의 구개인두부전증의 치료: 증례보고
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  • 가부키 증후군 환자의 구개인두부전증의 치료: 증례보고
저자명
이산하,왕재권,박미경,백롱민,Lee. San-Ha,Wang. Jae-Kwon,Park. Mi-Kyong,Baek. Rong-Min
간행물명
大韓成形外科學會誌
권/호정보
2011년|38권 2호|pp.203-206 (4 pages)
발행정보
대한성형외과학회
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이 논문은 한국과학기술정보연구원과 논문 연계를 통해 무료로 제공되는 원문입니다.
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기타언어초록

Purpose: Kabuki syndrome is a multiple malformation syndrome that was first reported in Japan. It is characterized by distinctive Kabuki-like facial features, skeletal anomalies, dermatoglyphic abnormalities, short stature, and mental retardation. We report two cases of Kabuki syndrome with the surgical intervention and speech evaluation. Methods: Both patients had velopharyngeal insufficiency and had a superior based pharyngeal flap operation. The preoperative and postoperative speech evaluations were performed by a speech language pathologist. Results: In case 1, hypernasality was reduced in spontaneous speech, and the nasalance scores in syllable repetitions were reduced to be within normal ranges. In case 2, hypernasality in spontaneous speech was reduced from severe level to moderate level and the nasalance scores in syllable repetitions were also reduced to be within normal ranges. Conclusion: The goal of this article is to raise awareness among plastic surgeons who may encounter such patients with unique facial features. This study shows that pharyngeal flap operation can successfully correct the velopharyngeal insufficiency in Kabuki syndrome and post operative speech therapy plays a role in reinforcing surgical result.