Narrative review of congenital lung lesions

Research output: Contribution to journalReview articlepeer-review


This article reviews the contemporary diagnosis and management of antenatally diagnosed congenital lung lesions. These anomalies, which include congenital pulmonary airway malformation (CPAM) (formerly congenital cystic adenomatoid malformation), bronchopulmonary sequestration (BPS), bronchogenic cyst, and congenital lobar emphysema (CLE), are relatively rare but are increasingly encountered by clinicians because of the improved resolution and enhanced sensitivity of fetal ultrasound. Serial assessment of these lesions throughout pregnancy remains the norm rather than the exception. Perinatal management strategies may differ based on initial size and growth patterns of these masses until delivery. Fetal magnetic resonance imaging and other diagnostic testing can sometimes be helpful in providing additional prognostic information. Over the last decade, maternal steroids have become standard of care in the management of larger lesions at risk for nonimmune hydrops. As a result, fetal surgical procedures, including open resection, thoracoamniotic shunting, and ex utero intrapartum treatment (EXIT), are less uncommonly performed. Decisions regarding whether delivery of these fetuses should occur in a tertiary care center with pediatric surgery coverage versus delivery at a local community hospital are now highly relevant in most prenatal counseling discussions with families. Large lung malformations may require urgent surgical removal in the early postnatal period because of respiratory distress. Other complications, such as recurrent pneumonia, pneumothorax, and cancer, are indications for postnatal lung resection on an elective basis. Many children are good candidates for minimally invasive (thoracoscopic) surgical approaches as an alternative to resection by thoracotomy. In the vast majority of cases, the overall prognosis remains excellent.

Original languageEnglish (US)
Pages (from-to)1418-1431
Number of pages14
JournalTranslational Pediatrics
Issue number5
StatePublished - May 2021


  • Bronchopulmonary sequestrations (BPS)
  • Congenital lung lesion
  • Congenital pulmonary airway malformations (CPAM)
  • Fetal surgery

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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