Surgical Considerations in 22Q11.2 Deletion Syndrome

被引:24
作者
Kirschner, Richard E. [1 ]
Baylis, Adriane L. [1 ]
机构
[1] Ohio State Univ, Nationwide Childrens Hosp, Coll Med, Dept Plast Surg,Sect Plast & Reconstruct Surg, Columbus, OH 43205 USA
关键词
22q11.2 deletion syndrome; Velocardiofacial syndrome; DiGeorge syndrome; Velopharyngeal dysfunction; CARDIO-FACIAL SYNDROME; ABNORMAL CAROTID ARTERIES; VELOCARDIOFACIAL SYNDROME; CLEFT-PALATE; VELOPHARYNGEAL INSUFFICIENCY; CHILDREN; MANAGEMENT; DISORDERS; SPEECH; PHARYNGOPLASTY;
D O I
10.1016/j.cps.2013.12.002
中图分类号
R61 [外科手术学];
学科分类号
摘要
The 22q11.2 deletion syndrome (22q11DS) may be associated with several palatal abnormalities, including overt cleft palate, submucosal cleft palate, palatopharyngeal disproportion, and velar hypotonia. The syndrome is the genetic disorder most commonly associated with velopharyngeal dysfunction (VPD). The complex causes of VPD in affected patients combine with the complexity of associated medical disorders to render surgical management of the velopharynx particularly challenging. Optimization of surgical outcomes requires precision in diagnosis, surgical management, and multidisciplinary care. This article provides an overview of 22q11DS and provides a review of the assessment and surgical management of VPD in affected individuals.
引用
收藏
页码:271 / +
页数:13
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