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Aggressive Congenital Pulmonary Airway Malformations With Dominant Cysts
DonnaMaria E Cortezzo1,2,3,4, Erica Hammer4,5,6, Timothy M Crombleholme1,4,7,8
1University of Connecticut School of Medicine, Department of Pediatrics, Farmington, Connecticut.
Neoreviews
|January 31, 2026
Summary
Congenital pulmonary airway malformations (CPAM) can pose risks during pregnancy. Fetal interventions like thoracoamniotic shunts can manage complex cases, requiring multidisciplinary care for mother and neonate.
Area of Science:
- Medical Science
- Pediatric Surgery
- Maternal-Fetal Medicine
Background:
- Congenital pulmonary airway malformations (CPAM) are congenital lung abnormalities with variable clinical presentations.
- High-risk CPAMs present significant diagnostic and management challenges for both the pregnant individual and the fetus.
Purpose of the Study:
- To present two challenging cases of fetal CPAMs requiring intervention.
- To review the literature and guide the management of CPAMs during pregnancy and in the neonatal period.
Main Methods:
- Case report of two pregnant individuals with fetal CPAMs.
- Fetal intervention utilizing thoracoamniotic shunts.
- Comprehensive literature review on CPAM management.
Main Results:
- Early presentation (13 weeks gestation) and rapidly increasing CPAM volume ratio were observed in the cases.
- Successful fetal intervention with thoracoamniotic shunts was performed.
- Subsequent prenatal and neonatal care was complex, necessitating a multidisciplinary approach.
Conclusions:
- Fetal CPAM management requires careful consideration of lesion characteristics and gestational age.
- Multidisciplinary prenatal counseling, individualized delivery planning, and neonatal management are crucial for optimal outcomes.
- Thoracoamniotic shunts can be an effective fetal intervention for select CPAM cases.
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