Congenital high airway obstruction syndrome

Case contributed by Srinivas Salavakam


25 year old pregnant lady came for an anomaly scan.

Patient Data

Age: 20 weeks gestation
Gender: Female

Gestational age of the fetus corresponds to 20 weeks. 

Trachea is dilated and fluid-filled. Bilateral echogenic and enlarged lungs noted with everted hemidiaphragms. Heart is compressed and pushed anteriorly.

Moderate amount of ascitic fluid seen in peritoneal cavity.

In addition to the above findings, there was talipes deformity of the left foot.

Case Discussion

Congenital high airway obstruction syndrome (CHAOS) is commonly caused either by atresia or stenosis of the larynx or trachea. Other causes are laryngeal agenesis, subglottic stenosis or atresia, laryngeal webs or cysts, tracheoesophageal fistula. 

The condition is frequently lethal 4. However, EXIT (ex utero Intrapartum treatment) procedure can be offered if the location of the obstruction is compatible with establishment of an airway and CHAOS is an isolated finding 2,3.

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