(510) Congenital pulmonary airway malformations and bronchopulmonary sequestrations: features of prenatal evaluation that predict postnatal surgery

Congenital pulmonary airway malformations (CPAM) and bronchopulmonary sequestrations (BPS) are residual masses of embryonic lung bud anomalies that can be detected on prenatal ultrasound. When indicated, postnatal resection is performed to alleviate respiratory symptoms or prevent recurrent pneumonias. Beyond increased CPAM-volume ratio (CVR), prenatal sonographic features of CPAM/BPS that herald postnatal symptoms remain elusive. This study sought to identify prenatal characteristics that predict need for postnatal surgery for fetuses with CPAM/BPS.

Study Design:

The maternal-fetal medicine database of a quaternary medical center was queried for pregnancies diagnosed with CPAM or BPS from January 2004 to November 2022. Patient and disease characteristics were collected from the paired medical records of pregnant patient and child. Initial and final prenatal ultrasounds were reviewed. Appropriate statistical methods were applied to categorical and continuous variables.

Results:

From 9,699 records at a single high-volume fetal center, we identified 122 pregnancies with a diagnosis of CPAM or BPS on prenatal ultrasound. Outcomes for 19 cases were unknown or pending and were excluded. Of 103 fetuses with known outcomes, 60 (58%) underwent postnatal surgery and 43 (42%) did not. A higher CVR predicted likelihood of surgery (p=0.05). Presence of cardiac displacement on last ultrasound before birth associated with surgery (p=0.027), while cardiac displacement on initial ultrasound did not (p=0.18). CPAM/BPS type, sonographic appearance of the mass, and indicators of non-immune hydrops were not associated with surgical management. Steroid administration prenatally trended towards greater CVR reduction for pathology-proven CPAM than BPS, but this did not reach statistical significance. Median follow up time of the cohort was 4.5 [1.8-9.2] years.

Conclusion:

Late gestation cardiac displacement may predict increased risk of surgical intervention – an association not previously described. This study adds to existing literature demonstrating a positive association between CVR and postnatal surgery.