Congenital high airways obstruction syndrome (CHAOS)

Case contributed by Ammar Ashraf
Diagnosis certain

Presentation

20 weeks pregnant; for obstetrical ultrasound examination.

Patient Data

Age: 20 weeks gestation
Gender: Female

Single viable intrauterine pregnancy with breech presentation at the time of ultrasound examination.  Average gestational age is 18 weeks 4 days. Fetal heart rate is 144 beats per minute.  Adequate amniotic fluid volume with the largest vertical pocket measuring 5.0 cm.  Posterior placenta. Estimated fetal weight is 237 g. The cord has three vessels. Cervix measures 3.2 cm in length with closed os. A note is made of homogenously hyperechoic bilateral fetal lungs. A tubular anechoic avascular structure is seen in the mediastinum which likely represents fluid filled trachea and bronchi.

Follow up

ultrasound

Single viable intrauterine fetus with a fetal heart rate of 144 beats per minute. Posterior placenta. Relatively increased amniotic fluid volume with the largest vertical pocket of amniotic fluid measuring 8.5 cm. Estimated fetal weight is 1678 g. Re-demonstration of enlarged hyper echoic fetal lungs with fluid-filled trachea & bronchi. Interval development of mild ascites, mild bilateral hydroceles and edematous changes in the subcutaneous soft tissues of the abdomen.

Case Discussion

  • enlarged and echogenic fetal lungs with fluid-filled trachea & bronchi; these findings are suggestive of congenital high airway obstruction syndrome (CHAOS). Follow up scan showed mild ascites, mild bilateral hydroceles, edematous changes in the subcutaneous soft tissues of abdomen and relatively increased amniotic fluid volume with the largest vertical pocket of amniotic fluid measuring 8.5 cm.

  • parents refused any intervention. Two days after the 2nd scan, she delivered (spontaneous vaginal delivery) a 30 weeks preterm male baby with an average weight of 1700 grams. The baby was delivered alive with a fetal heart rate of 114 beats per minute but with no breathing. Immediate respiratory resuscitation with an Ambu bag was done. Laryngoscopy was also done which showed severe laryngeal atresia. Endotracheal intubation was attempted which was associated with significant airway trauma and was not successful. Baby expired shortly after birth.

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