Highlights
- •More than half (56%) of the prenatally diagnosed CPAM/BPS will reduce in size during the third trimester of gestation.
- •A quarter of the lesions (29%) will become undetectable late in gestation.
- •19% diagnosed CPAM/BPS will develop symptoms after the neonatal period, and 81% will remain asymptomatic.
- •One third (31%) of the lung lesions in our institution needed surgical resection.
- •Large lesion size and mediastinal shifting correlate with a higher rate of surgical resection.
Abstract
Background
The natural history of congenital pulmonary airway malformations (CPAM) and bronchopulmonary
sequestrations (BPS) is not fully understood, and the management of the newborn with
an asymptomatic lesion is a controversial issue. We aimed to study the natural history
and outcome of CPAM/BPS at our institution with a policy of watchful waiting, and
to investigate if any prognostic factors in the pre- and/or postnatal- period may
predict the need for surgery.
Material and methods
A retrospective review study was conducted of children prenatally diagnosed with CPAM
and/or BPS during the 18-year period, from 2002 to 2020. Data from the pre and postnatal
period was collected and analysed.
Results
Sixty- six patients with prenatally observed lung lesions were entered in the study,
with an overall survival rate of 94%. Fifty-six percent of the lesions decreased in
size during gestation. Thirty-one percent had surgery and 69% could be managed conservatively
with a median follow-up of 4 years. Nineteen percent developed symptoms after the
neonatal period. Children with a presence of mediastinal shift on postnatal imaging
(p = 0.003), with a high CVR (p = 0.005) and a large lesion size during gestation (p = 0.014) were significantly more likely to require surgery.
Conclusion
Prenatal regression is common among prenatally diagnosed CPAM/BPS and the majority
of children that are asymptomatic beyond the neonatal period will remain asymptomatic
throughout their childhood. Future analysis with a longer follow-up might give new
insights in order to identify children at risk of developing symptoms.
Level of evidence
III
Keywords
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Article info
Publication history
Published online: March 26, 2022
Accepted:
March 22,
2022
Received in revised form:
March 21,
2022
Received:
August 27,
2021
Identification
Copyright
© 2022 Published by Elsevier Inc.
