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KMID : 0358820110380020203
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Journal of Korean Society of Plastic and Reconstructive Surgeons
2011 Volume.38 No. 2 p.203 ~ p.206
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Treatment of Velopharyngeal Insufficiency in Kabuki Syndrome: Case Report
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Lee San-Ha
Wang Jae-Kwon
Park Mi-Kyong
Baek Rong-Min
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Abstract
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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.
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KEYWORD
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Kabuki syndrome, Velopharyngeal insufficiency, Speech evaluation, Pharyngeal flap
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