Leveau-Geffroy S, Perrin J-P, Khonsari R H, Mercier J
Service de Chirurgie Maxillo-Faciale et Stomatologie, CHRU, 1, Place Alexis-Ricordeau, 44093 Nantes, France.
Rev Stomatol Chir Maxillofac. 2011 Feb;112(1):11-5. doi: 10.1016/j.stomax.2011.01.002. Epub 2011 Feb 3.
Velopharyngeal insufficiency is a frequent sign of the velocardiofacial syndrome (VCFS) but its origins are not well-documented. Our aim was to establish a correlation between this functional disorder and regional morphological anomalies.
Twenty-seven of 36 patients presenting with VCFS could be included retrospectively. We measured cavum depth and velum length on lateral orthodontic X-rays, and assessed the relationship between these two measures. We compared these measures to those of reference populations. Postoperative phonation was assessed with an aerophonoscope.
The patients presented with a short velum and a deep cavum. Cranium base and upper cervical spine were malformed in 22 of the 27 patients. Seventeen of the 23 assessed patients (66%) improved their phonation after surgery.
Our data suggests that velopharyngeal insufficiency in VCFS could be the result of a more global craniospinal growth disorder the functional consequences of which remain unclear. The frequent association of morphological anomalies with mental retardation is probably responsible for the failure to normalize phonation.
腭咽功能不全是腭心面综合征(VCFS)的常见体征,但其病因尚无充分记录。我们的目的是建立这种功能障碍与局部形态异常之间的相关性。
回顾性纳入了36例VCFS患者中的27例。我们在正畸侧位X射线上测量了咽腔深度和软腭长度,并评估了这两项测量值之间的关系。我们将这些测量值与参考人群的测量值进行了比较。术后使用气流声门镜评估发声情况。
患者表现为软腭短和咽腔深。27例患者中有22例颅底和上颈椎畸形。23例接受评估的患者中有17例(66%)术后发声改善。
我们的数据表明,VCFS中的腭咽功能不全可能是更广泛的颅脊柱生长障碍的结果,其功能后果尚不清楚。形态异常与智力迟钝的频繁关联可能是发声未能恢复正常的原因。