Treatment of Velopharyngeal Insufficiency in Kabuki Syndrome: Case Report
Journal of the Korean Society of Plastic and Reconstructive Surgeons
; : 203-206, 2011.
Article
en Ko
| WPRIM
| ID: wpr-200155
Biblioteca responsable:
WPRO
ABSTRACT
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.
Palabras clave
Texto completo:
1
Índice:
WPRIM
Asunto principal:
Valores de Referencia
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Logopedia
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Anomalías Múltiples
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Insuficiencia Velofaríngea
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Enfermedades Vestibulares
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Dermatoglifia
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Cara
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Enfermedades Hematológicas
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Japón
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Discapacidad Intelectual
Límite:
Humans
País/Región como asunto:
Asia
Idioma:
Ko
Revista:
Journal of the Korean Society of Plastic and Reconstructive Surgeons
Año:
2011
Tipo del documento:
Article