Congenital high airways obstruction syndrome (CHAOS)

Case contributed by Ammar Ashraf
Diagnosis certain

Presentation

30 year old pregnant lady came for an anomaly scan.

Patient Data

Age: 20 weeks gestation
Gender: Female

Single viable intrauterine pregnancy with variable presentation. Average gestational age is 15 weeks 2 days by fetal biometry. Fetal heart rate is 167 bpm. Placenta is posterior in location and away from the internal os. Decreased amniotic fluid volume; only one pocket of amniotic fluid measuring 7 mm is seen (oligohydramnios). Both kidneys are not confidently identified. Bilateral enlarged echogenic fetal lungs and flat diaphragm. Mildly dilated fluid filled trachea and bronchi suggestive of laryngeal atresia. Mild fetal ascites. Cervix measures 30 mm in length with closed os.

Case Discussion

Bilateral enlarged echogenic lungs with dilated fluid filled trachea and bronchi; these sonographic features are suggestive of congenital high airway obstruction syndrome (CHAOS). The patient was seen by the perinatologist two days later for a detailed anomaly scan; however, the scan showed intrauterine fetal death (IUFD). The differential diagnosis of echogenic fetal lung is congenital pulmonary airway malformation (CPAM) type III, which is usually unilateral whereas CHAOS is bilateral. Dilated airway is another differentiating feature which is seen only in the CHAOS.

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