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非综合征型与综合征型皮埃尔·罗宾序列征的语音预后及咽瓣需求

Speech prognosis and need of pharyngeal flap for non syndromic vs syndromic Pierre Robin Sequence.

作者信息

de Buys Roessingh Anthony S, Herzog Georges, Cherpillod Jacques, Trichet-Zbinden Chantal, Hohlfeld Judith

机构信息

Department of Pediatric Surgery, University Hospital Center of the Canton of Vaud (CHUV), Multidisciplinary Cleft Team, Lausanne 1011, Switzerland.

出版信息

J Pediatr Surg. 2008 Apr;43(4):668-74. doi: 10.1016/j.jpedsurg.2007.09.050.

Abstract

BACKGROUND

The aim of this retrospective study was to evaluate speech outcome and need of a pharyngeal flap in children born with nonsyndromic Pierre Robin Sequence (nsPRS) vs syndromic Pierre Robin Sequence (sPRS).

METHODS

Pierre Robin Sequence was diagnosed when the triad microretrognathia, glossoptosis, and cleft palate were present. Children were classified at birth in 3 categories depending on respiratory and feeding problems. The Borel-Maisonny classification was used to score the velopharyngeal insufficiency.

RESULTS

The study was based on 38 children followed from 1985 to 2006. For the 25 nsPRS, 9 (36%) pharyngeal flaps were performed with improvements of the phonatory score in the 3 categories. For the 13 sPRS, 3 (23%) pharyngeal flaps were performed with an improvement of the phonatory scores in the 3 children. There was no statistical difference between the nsPRS and sPRS groups (P = .3) even if we compared the children in the 3 categories (P = .2).

CONCLUSIONS

Children born with nsPRS did not have a better prognosis of speech outcome than children born with sPRS. Respiratory and feeding problems at birth did not seem to be correlated with speech outcome. This is important when informing parents on the prognosis of long-term therapy.

摘要

背景

这项回顾性研究的目的是评估非综合征性皮埃尔·罗宾序列(nsPRS)与综合征性皮埃尔·罗宾序列(sPRS)患儿的言语结局及咽瓣需求。

方法

当出现小下颌后缩、舌后坠和腭裂三联征时诊断为皮埃尔·罗宾序列。根据呼吸和喂养问题,患儿在出生时被分为3类。采用博雷尔 - 迈松尼分类法对腭咽闭合不全进行评分。

结果

该研究基于1985年至2006年随访的38例患儿。对于25例nsPRS患儿,9例(36%)进行了咽瓣手术,3类患儿的发声评分均有改善。对于13例sPRS患儿,3例(23%)进行了咽瓣手术,这3例患儿的发声评分有所改善。即使我们比较3类患儿,nsPRS组和sPRS组之间也没有统计学差异(P = 0.3)(P = 0.2)。

结论

nsPRS患儿的言语结局预后并不比sPRS患儿好。出生时的呼吸和喂养问题似乎与言语结局无关。这在告知家长长期治疗的预后时很重要。

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