Update Course Rewind: Congenital Lung Lesions 2022
With Dr. Chuck Snyder · StayCurrentMD
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What the experts said
Roughly 10% of congenital lung lesions diagnosed postnatally were malignant.
About half of the malignant congenital lung lesions were associated with the DICER1 mutation.
There was no malignant congenital lung lesion that had a systemic feeding vessel.
In the Midwest Consortium series of approximately 400 cystic lesions, not a single patient with antenatal diagnosis was found to have pleuropulmonary blastoma (PPB).
If a congenital lung lesion has prenatal diagnosis, the chances of being pleuropulmonary blastoma are close to zero.
Some argue that if a congenital lung lesion is diagnosed prenatally and the patient never develops symptoms, resection is not necessary.
The malignancy risk in asymptomatic prenatally diagnosed congenital lung lesions is very low but not zero.
Many prenatally suspected congenital lung lesions turn out to be nothing after birth, either not present or representing transient findings like mucus plugs.
Children aged 4-9 years with congenital lung lesions who have had recurrent pneumonias present a surgical challenge, and their chances of successful thoracoscopic resection are much lower than in the perinatal period or first year of life.
The primary concern for resecting congenital lung lesions is preventing recurrent pneumonias and bronchiectasis, not cancer risk.
The range of congenital lung anomalies varies from trivial small cysts that may not require intervention to multilobar lesions that would require pneumonectomy.
Management of congenital lung lesions must be tailored to the specific pathology of each case.
The incidence of congenital lung lesions has increased over the past decades because more prenatal screening has been occurring worldwide.
Congenital lung lesions can often lead to malignancy or recurrent infections, both of which may require surgical workup and management.
In 2021, the Midwest Pediatric Surgery Consortium reviewed 521 primary lung lesions from 11 children's hospitals to identify specific risk factors associated with malignancy.
In the Midwest Consortium series, no prenatally diagnosed lesions were malignant.
CT scan had poor sensitivity and specificity for malignancy in congenital lung lesions.
Suspicion for malignancy by CT scan and bilateral disease were predictive of malignancy in congenital lung lesions.
CPAM becomes a general bucket term for congenital lung lesions in the fetal world.
As the ability to identify specific congenital lung lesions improves with technological advances, management needs to change accordingly.
Reasons to resect congenital lung lesions include infection and risk of malignancy, with malignancy risk being a more minor reason for most practitioners.