de Buys Roessingh Anthony S, Herzog Georges, Hohlfeld Judith
Department of Pediatric Surgery, University Hospital Centre of the Canton of Vaud (CHUV), Lausanne, Switzerland.
J Pediatr Surg. 2007 Sep;42(9):1495-9. doi: 10.1016/j.jpedsurg.2007.04.024.
BACKGROUND/PURPOSE: The study describes a safe and least aggressive method to resolve airway obstruction in children born with a Pierre Robin sequence (PRS).
In a retrospective study, we analyzed the assessment of airway obstruction at birth and for the following months. The definition of PRS was based on the anatomical anomaly triad cleft palate, micro/retrognathia, and glossoptosis with some degree of airway obstruction. We defined 3 categories of children depending on their difficulties of breathing or eating at birth.
From 1984 to 2004, 48 children were born in our hospital with a diagnosis of PRS. There were 32 children with nonsyndromic PRS (nsPRS) and 16 with syndromic PRS (sPRS): respectively, 40% (13) and 32% (5) had slight respiratory and/or feeding problems; 26% (8) and 56% (9), isolated feeding difficulties; 34% (11) and 12% (2), severe respiratory and feeding problems. Pharyngeal tube was used in 8 children with nsPRS and in 2 with sPRS. Neonatal surgery was not necessary. Primary palatoplasty was performed at almost the same time as for the patients with isolated cleft palate.
Children born with PRS have a good prognosis at birth provided that adequate respiratory support is given using either positive airway pressure mask or pharyngeal tube.
背景/目的：本研究描述了一种安全且侵入性最小的方法，用于解决患有皮埃尔·罗宾序列征（PRS）的儿童的气道阻塞问题。
在一项回顾性研究中，我们分析了出生时及随后几个月气道阻塞的评估情况。PRS的定义基于解剖学异常三联征腭裂、小颌/后缩颌以及舌后坠，并伴有一定程度的气道阻塞。我们根据出生时呼吸或进食困难的程度将儿童分为3类。
1984年至2004年，我院共出生48例诊断为PRS的患儿。其中32例为非综合征性PRS（nsPRS），16例为综合征性PRS（sPRS）：分别有40%（13例）和32%（5例）有轻微呼吸和/或喂养问题；26%（8例）和56%（9例）有单纯喂养困难；34%（11例）和12%（2例）有严重呼吸和喂养问题。8例nsPRS患儿和2例sPRS患儿使用了咽管。无需进行新生儿手术。一期腭裂修复术与单纯腭裂患儿几乎同时进行。
患有PRS的患儿出生时若能使用正压通气面罩或咽管给予充分的呼吸支持，则预后良好。
