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两例先天性高气道梗阻综合征(CHAOS)中喉闭锁的产前诊断。

Prenatal diagnosis of laryngeal atresia in two cases of congenital high airway obstruction syndrome (CHAOS).

作者信息

Kalache K D, Chaoui R, Tennstedt C, Bollmann R

机构信息

Department of Gynecology and Obstetrics/Unit of Prenatal Diagnosis and Therapy, Humboldt-Universität zu Berlin, Charitè School of Medicine, Germany.

出版信息

Prenat Diagn. 1997 Jun;17(6):577-81.

PMID:9203217
Abstract

Complete occlusion of the upper airways is known to cause secondary morphological changes, including bilaterally enlarged hyperechogenic lungs, dilated trachea, and hydrops. Prenatal diagnosis of upper airway obstruction has been described in several cases. In these reports, the diagnosis was primarily attributed to indirect signs, and the authors were in doubt as to whether the location of the obstructed area (larynx, glottis or trachea) could be precisely visualized by ultrasound. In this paper two cases are reported presenting the features of congenital high airway obstruction syndrome (CHAOS) diagnosed at the 22nd week of gestation. In both cases, the upper neck was perfectly visualized in a coronal plane. At the onset of fetal breathing movements, the stenotic larynx remained in a closed position. By positioning a colour signal on the fluid-filled dilated trachea, we noticed absence of flow throughout the onset of breathing activity. We conclude that the atretic area must be situated at the level of the larynx. Because of the poor prognosis of laryngeal stenosis and the presence of associated anomalies, both pregnancies were terminated in the 23rd week. This report shows that the application of colour and spectral Doppler may be helpful in the differential diagnosis of fetuses with CHAOS.

摘要

已知上呼吸道完全阻塞会导致继发性形态学改变,包括双侧肺回声增强、气管扩张和水肿。已有数例关于上呼吸道梗阻的产前诊断报道。在这些报告中,诊断主要归因于间接征象,作者对于超声能否精确显示梗阻部位(喉、声门或气管)存疑。本文报告了两例在妊娠第22周诊断为先天性高位气道梗阻综合征(CHAOS)的病例。在这两例中,胎儿上颈部在冠状面均清晰可见。在胎儿呼吸运动开始时,狭窄的喉部保持关闭状态。通过将彩色信号置于充满液体的扩张气管上,我们发现在整个呼吸活动开始时均无血流信号。我们得出结论,闭锁部位必定位于喉部水平。由于喉狭窄预后不良且伴有相关畸形,两例妊娠均在第23周终止。本报告表明,彩色和频谱多普勒的应用可能有助于CHAOS胎儿的鉴别诊断。

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