Korean J Obstet Gynecol.  2012 Feb;55(2):115-118. 10.5468/KJOG.2012.55.2.115.

A case of congenital high airway obstruction syndrome caused by complete tracheal obstruction with associated anomalies

• Cho HJ
• Kim YM
• Lee HY
• Won HS

• KMID: 2078124
• DOI: http://doi.org/10.5468/KJOG.2012.55.2.115

Abstract

Congenital high airway obstruction syndrome (CHAOS) is caused when the upper airway is obstructed or severely narrowed. The prenatal ultrasound findings of CHAOS include large echogenic lungs, inverted diaphragms, dilated airways, and fetal ascites and/or hydrops. Recently, exutero intrapartum treatment (EXIT) procedure or fetoscopic tracheostomy are being widely used for the treatment of CHAOS. However, CHAOS with early presentation of hydrops confers ominous sign even with EXIT procedure. We report a case of CHAOS with hydrops and associated anomalies that was confirmed by autopsy.

Keyword

Congenital high airway obstruction syndrome; Prenatal ultrasound; Fetal hydrops; Autopsy

MeSH Terms

Airway Obstruction
Ascites
Autopsy
Diaphragm
Edema
Hydrops Fetalis
Lung
Tracheostomy

Figure

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  Fig. 1 Ultrasound images of a fetus with congenital high airway obstruction syndrome at 20 weeks of gestation. (A) Dilated fluid filled trachea and massive ascites. (B) Transverse scan of an enlarged echogenic lung, compressed heart and fetal hydrops.

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  Fig. 2 Postmortem findings show a totally obstructed trachea (arrow).

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  Fig. 3 Microscopic examination of the obstructed portion of the opened trachea shows proliferated fibroblasts around soft tissue (arrow). In this view, a totally obstructed wall opposite the trachea was confirmed (arrowhead) (H&E, ×40).

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