[size=20px]A 27 year-old woman presents for a prenatal consult. Prenatal ultrasound at 22 weeks gestation shows a single male fetus with a cystic lung lesion compatible with a congenital pulmonary airway malformation (CPAM). Her initial CPAM volume ratio (CVR) is 1.7. There are no signs of hydrops. What is the risk of the fetus developing hydrops or postnatal respiratory distress?

 

A Low risk, consider routine delivery at a community center

 

B Moderate risk, plan for delivery at tertiary care (level III) center

 

C Moderate risk, consider referral to a fetal center and/or prenatal steroids​

 

D High risk, consider referral to a fetal center and/or prenatal steroids​[/size]

C

B

C

D

D

C

correct answer

D High risk, consider referral to a fetal center and/or prenatal steroids​​

 

A multicenter study by Kunisaki et. al (Annals of Surgery 2022) evaluated outcomes in 344 children with prenatally diagnosed lung malformations. As expected, the Congenital Pulmonary Airway Malformation (CPAM)/Volume Ratio (CVR) was the single most important predictor of prenatal hydrops or postnatal respiratory symptoms.​

 

​Based on the CVR (either initial or maximum), patients can be classified as low, medium, or high risk. Any patient with either an initial CVR ≥ 1.4, maximum CVR≥ 1.7, hydrops, or other anomalies is considered high risk. These patients should receive prenatal steroids, be considered for referral to a fetal center and undergo delivery at a level III or IV center. ​​