Diagnosis and Management of Fetal Lung Anomalies
Congenital Pulmonary Airway Malformation (CPAM)
Congenital Pulmonary Airway Malformation (CPAM) is a developmental abnormality of the fetal lung in which cystic structures form within the lung tissue. These cysts may exert pressure on surrounding structures such as the esophagus, trachea, and cardiovascular system. This pressure can lead to complications such as polyhydramnios (excess amniotic fluid) and, in severe cases, hydrops fetalis (generalized fluid accumulation in the fetus).
CPAM occurs in approximately 1 in every 4,000 live births. It is typically detected during pregnancy through detailed prenatal ultrasound, appearing as a hyperechogenic (bright) mass in the fetal lung. When further evaluation is needed, fetal MRI is used to assess the lesion and determine the degree of risk.
Management of High-Risk CPAM Cases
Cases with a Cyst Volume Ratio (CVR) greater than 1.6 or those complicated by hydrops fetalis are considered high-risk.
Following detailed ultrasound and fetal MRI evaluation, a multidisciplinary team develops an individualized treatment plan.
In select cases, fetal interventions may be performed, such as:
- Thoracoamniotic shunt placement – to drain fluid from the cystic lesion into the amniotic cavity and relieve pressure.
- Fetoscopic laser ablation – to selectively destroy cystic tissue and restore normal lung development.
These procedures are performed only in specialized fetal surgery centers by experienced perinatology and pediatric surgery teams, and they have significantly improved survival rates in severe CPAM cases.
Postnatal Management
After birth, infants diagnosed with CPAM are evaluated and managed according to their clinical presentation:
- Asymptomatic newborns: may only require regular monitoring with imaging and follow-up.
- Symptomatic newborns (with respiratory distress): typically require surgical intervention, most often lobectomy (removal of the affected lung lobe).
Current data show that with early prenatal diagnosis and appropriate management, the survival rate exceeds 90% in CPAM cases. Outcomes depend on timely diagnosis, multidisciplinary coordination, and personalized care planning.
Our Approach
Fetal lung malformations represent one of the most delicate areas in modern perinatology and fetal medicine.
At our clinic, we combine advanced diagnostic imaging, fetal therapy expertise, and holistic prenatal care to offer the most up-to-date, evidence-based, and safe management strategies for both mother and baby.
If your pregnancy has been diagnosed with CPAM, we invite you to schedule a detailed consultation and personalized evaluation with our specialist team.
