莫尔综合征中的腭部联合异常：术前腭部肌电图是否有用？

Combined anomalies of the palate in Mohr syndrome: is preoperative electromyography of the palate useful?

作者信息

Velepic Mitja S, Sasso Antun, Velepic Marko M, Lustica Ivo, Starcevic Radan, Komljenovic Dejan

机构信息

Clinic of Pediatry, Rijeka University Medical School, Clinical Hospital Center Rijeka, Rijeka, Croatia.

出版信息

J Pediatr Surg. 2004 Feb;39(2):220-2. doi: 10.1016/j.jpedsurg.2003.10.023.

DOI:10.1016/j.jpedsurg.2003.10.023

PMID:14966745

Abstract

The authors present a girl with typical characteristics of oral-facial-digital syndrome type II (Mohr syndrome) with a cleft soft palate and pendulous tongue nodules. Because of feeding difficulties, electromyography was performed of both morphologically identical halves of the soft palate. One half showed a normal muscle action potential and in the other half electrical silence was registered. Exploratory surgery during palatoplasty showed a fatty hamartoma in the half of the palate in which no electric potentials had been registered.

摘要

作者报告了一名患有II型口面指综合征（莫尔综合征）典型特征的女孩，伴有软腭裂和悬垂性舌结节。由于喂养困难，对形态相同的软腭两半进行了肌电图检查。其中一半显示正常的肌肉动作电位，而另一半记录到电静息。腭裂修复术中的探查手术显示，在未记录到电位的软腭半侧发现了一个脂肪错构瘤。