Pediatric Surgery International

, Volume 24, Issue 6, pp 643–657 | Cite as

Congenital cystic lung disease: contemporary antenatal and postnatal management

  • Richard G. AzizkhanEmail author
  • Timothy M. Crombleholme
Review Article


Congenital cystic lung disease comprises a broad spectrum of rare but clinically significant developmental abnormalities, including congenital pulmonary adenomatoid malformations, bronchopulmonary sequestrations, bronchogenic cysts, and congenital lobar emphysema that result from perturbations in lung and airway embryogenesis. As congenital lung lesions are now more commonly recognized antenatally, mothers require accurate prenatal counseling and appropriate perinatal management. In light of long-term complications of infection and malignancy, there is growing consensus that infants with asymptomatic lesions should undergo elective excision of congenital pulmonary adenomatoid malformation (CPAM) or bronchopulmonary sequestration (BPS). This review will focus on advancements and current practice in the diagnosis and management of CPAM and BPS, identifying aspects of the literature that are confusing or controversial. Although our knowledge and pre- and postnatal management of lung lesions will continue to evolve and improve, there is a compelling need for a unified clinical and pathological classification system that creates a common platform for discussion, clinical management, and research.


Congenital pulmonary adenomatoid malformation (CPAM) Congenital cystic adenomatoid malformation (CCAM) Bronchopulmonary sequestration Intralobar sequestration Extralobar sequestration Fetal surgery Fetal intervention EXIT procedure 


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Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • Richard G. Azizkhan
    • 1
    Email author
  • Timothy M. Crombleholme
    • 1
  1. 1.The Fetal Care Center of Cincinnati, Division of Thoracic and General Pediatric Surgery, Cincinnati Children’s Hospital Medical CenterUniversity of Cincinnati School of MedicineCincinnatiUSA

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