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治疗患有黏膜下腭裂和重症肌无力患者的腭咽闭合不全。

Treatment of velopharyngeal inadequacy in a patient with submucous cleft palate and myasthenia gravis.

作者信息

Rikihisa Naoaki, Udagawa Akikazu, Yoshimoto Shinya, Ichinose Masaharu, Kimura Tomoe, Shimizu Sara

机构信息

Department of Plastic and Aesthetic Surgery, Chiba University Hospital, Japan.

出版信息

Cleft Palate Craniofac J. 2009 Sep;46(5):558-62. doi: 10.1597/08-049.1. Epub 2009 Feb 1.

DOI:10.1597/08-049.1
PMID:19929094
Abstract

OBJECTIVE

To describe the clinical course and management of a patient with submucous cleft palate who developed myasthenia gravis (MG) as an adult and suffered recurrent hypernasality. Few reports have described MG patients undergoing pharyngeal flap surgery for velopharyngeal incompetence, and these have described only slight speech improvement in such patients.

DESIGN

Case report.

PATIENT

The patient underwent primary pushback palatoplasty and superiorly based pharyngeal flap surgery for submucous cleft and short palate at age 7. Hypernasality showed major improvement after initial surgery. At age 19, the patient developed MG that triggered the recurrence of velopharyngeal incompetence.

INTERVENTION

After MG was treated, revision pushback palatoplasty was performed for velopharyngeal incompetence when the patient was 24 years old. Preoperatively and postoperatively, the patient was evaluated by the same speech-language-hearing therapists, each with at least 5 years of clinical experience in cleft palate speech.

RESULTS

After the second pushback palatoplasty, hypernasality and audible nasal air emission during speech decreased to mild.

CONCLUSION

Primary pushback palatoplasty and pharyngeal flap surgery were performed for the submucous cleft palate. Revision pushback palatoplasty improved velopharyngeal inadequacy induced by MG. Decreased perceived nasality positively influenced the patient's quality of life. Combined pushback palatoplasty and pharyngeal flap surgery is thus an option in surgical treatment for velopharyngeal inadequacy to close the cleft and the velopharyngeal orifice in cases of cleft palate and MG.

摘要

目的

描述一名成年后患上重症肌无力(MG)并反复出现高鼻音的黏膜下腭裂患者的临床病程及治疗情况。很少有报告描述重症肌无力患者因腭咽闭合不全接受咽瓣手术,且这些报告仅描述了此类患者的语音有轻微改善。

设计

病例报告。

患者

该患者7岁时因黏膜下腭裂和短腭接受了初次后推腭裂修复术和蒂在上的咽瓣手术。初次手术后高鼻音有显著改善。19岁时,患者患上重症肌无力,引发腭咽闭合不全复发。

干预

重症肌无力得到治疗后,患者24岁时因腭咽闭合不全接受了再次后推腭裂修复术。术前和术后,由同一位言语语言听力治疗师对患者进行评估,每位治疗师在腭裂语音方面至少有5年临床经验。

结果

第二次后推腭裂修复术后,言语时的高鼻音和可闻及的鼻腔漏气减轻至轻度。

结论

对黏膜下腭裂患者进行了初次后推腭裂修复术和咽瓣手术。再次后推腭裂修复术改善了由重症肌无力引起的腭咽闭合不全。鼻音减轻对患者的生活质量产生了积极影响。因此,联合后推腭裂修复术和咽瓣手术是治疗腭裂和重症肌无力患者腭咽闭合不全以关闭腭裂和腭咽口的一种手术选择。

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Treatment of velopharyngeal inadequacy in a patient with submucous cleft palate and myasthenia gravis.治疗患有黏膜下腭裂和重症肌无力患者的腭咽闭合不全。
Cleft Palate Craniofac J. 2009 Sep;46(5):558-62. doi: 10.1597/08-049.1. Epub 2009 Feb 1.
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