Cystic Pulmonary Adenomatoid Malformation

Question 1

What is your differential diagnosis of a thoracic mass on ultrasound?

Answer 1

-BPS -CPAM -Teratoma -Congenital high airway obstruction sequence -Bronchogenic cyst -Bronchial obstruction

Question 2

Describe characteristic ultrasound features of a cystic pulmonary adenomatoid malformation.

Answer 2

-Microcystic <5mm, well defined borders -Macrocystic >5mm, poorly defined borders -Cystic and solid lung mass with arterial supply from pulmonary artery

Question 3

How do you distinguish between a CPAM and bronchiopulmonary sequestration?

Answer 3

BPS has its blood supply from the aorta and 90% are left sided with ipsilateral pleural effusion. Can be above or below the diaphragm.

Question 4

What are the different types of CPAM?

Answer 4

Type 0: acinar dysgenesis Type 1: 1 or more (2-10cm) Type 2: cysts <2cm Type 3: <0.5cm with solid appearance (assoc. w/ hydrops) Type 4: >10cm

Question 5

How do you counsel a patient if a CPAM is identified on ultrasound?

Answer 5

Postnatal: there is a increased risk for infection and malignancy Antenatal: -most will regress in utero. Regress by 26-28 weeks -If hydrops develop then can be lethal; however, betamethasone has been shown to decrease mortality (microcytic is more likely to be hydropic)

Question 6

What is the natural history of CPAM diagnosed prenatal?

Answer 6

Majority will regress. Increased risk of hydrops which is more likely with microcytic type 3 or if CVR is greater than 1.6 CVR CPAM volume ratio= (length x width x height x 0.52)

Question 7

What are potential complications related to CPAM?

Answer 7

Polyhydramnios Preterm contractions/labor Preterm delivery Hydrops from cardiac failure

Question 8

How do you follow a pregnancy if a CPAM is identified on ultrasound?

Answer 8

Hydrops >32 weeks then deliver Hydrops <32 weeks thoracoamniotic shunt Betamethasone with CVR >1.6 or hydrops or unrepressed microcystic

Question 9

What is the likelihood of associated anomalies with a CPAM?

Answer 9

3-12% includes other lung malformation, renal malformations

Question 10

What is the likelihood of an underlying genetic condition if a CPAM is identified on ultrasound?

Answer 10

No known genetic cause and no recurrence risk

Question 11

What additional work do you recommend if a CPAM is identified on ultrasound?

Answer 11

Detailed anatomy Fetal echocardiogram Fetal growth restriction +/- MRI

Question 12

How is CCAM managed?

Answer 12

Question 13

What is your next step if thoracocentesis does not work for macrocystic CCAM or steroids for microcystic CCAM?

Answer 13