Overview
Congenital pulmonary airway malformation (CPAM), formerly known as congenital cystic adenomatoid malformation (CCAM), is a rare developmental abnormality of the lung in which an abnormal mass of disorganized lung tissue forms during fetal development. The malformation consists of cystic or solid lesions that arise from abnormal branching of the developing airways and replace normal lung parenchyma. CPAMs are classified into several types (Types 0 through 4) based on the size of the cysts and the tissue of origin, with Type 1 (large cysts) and Type 2 (smaller cysts) being the most common. The condition primarily affects the respiratory system, typically involving one lobe of the lung, though bilateral involvement can rarely occur. CPAM is most often detected prenatally on routine ultrasound or presents in the neonatal period with respiratory distress, including tachypnea, cyanosis, and difficulty breathing. Some cases may remain asymptomatic and be discovered incidentally later in childhood or even adulthood, sometimes presenting with recurrent pulmonary infections. In severe prenatal cases, large lesions can cause mediastinal shift, compression of the normal lung, and fetal hydrops, which carries a poor prognosis without intervention. Smaller lesions may spontaneously regress during pregnancy, though they typically persist after birth. The primary treatment for symptomatic CPAM is surgical resection, usually lobectomy, which is generally curative. For asymptomatic cases detected prenatally or in infancy, the management approach remains debated, though many centers recommend elective surgical removal due to the risk of recurrent infections and a small but recognized risk of malignant transformation (particularly pleuropulmonary blastoma in Type 4 lesions). In severe fetal cases with hydrops, prenatal interventions such as thoracoamniotic shunting or fetal surgery may be considered. Outcomes after surgical resection are generally excellent, with most children achieving normal lung function and development.
Also known as:
Clinical phenotype terms— hover any for plain English:
Sporadic
Usually appears on its own, not inherited from a parent
Neonatal
Begins at or shortly after birth (first 4 weeks)
FDA & Trial Timeline
6 eventsMurdoch Childrens Research Institute — NA
Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh — NA
Jiexiong Feng — NA
Erasmus Medical Center — NA
Amalia Magaret
Insel Gruppe AG, University Hospital Bern
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Congenital pulmonary airway malformation.
2 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Congenital pulmonary airway malformation.
Community
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Start the conversation →Latest news about Congenital pulmonary airway malformation
Disease timeline:
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A new clinical trial is recruiting patients for Congenital pulmonary airway malformation
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A new clinical trial is recruiting patients for Congenital pulmonary airway malformation
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A new clinical trial is recruiting patients for Congenital pulmonary airway malformation
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A new clinical trial is recruiting patients for Congenital pulmonary airway malformation
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A new clinical trial is recruiting patients for Congenital pulmonary airway malformation
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A new clinical trial is recruiting patients for Congenital pulmonary airway malformation
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A new clinical trial is recruiting patients for Congenital pulmonary airway malformation
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A new clinical trial is recruiting patients for Congenital pulmonary airway malformation
New recruiting trial: Oral Health Status of Cystic Fibrosis Patients. An Online Survey in Collaboration With the Vaincre la Mucoviscidose Patient Association.
A new clinical trial is recruiting patients for Congenital pulmonary airway malformation
New recruiting trial: Safety, Tolerability, and Pharmacokinetics of Multiple Dose Combinations of SION-451 and Complementary Modulators SION-2222 and SION-109 in Healthy Participants.
A new clinical trial is recruiting patients for Congenital pulmonary airway malformation
Caregiver Resources
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Common questions about Congenital pulmonary airway malformation
What is Congenital pulmonary airway malformation?
Congenital pulmonary airway malformation (CPAM), formerly known as congenital cystic adenomatoid malformation (CCAM), is a rare developmental abnormality of the lung in which an abnormal mass of disorganized lung tissue forms during fetal development. The malformation consists of cystic or solid lesions that arise from abnormal branching of the developing airways and replace normal lung parenchyma. CPAMs are classified into several types (Types 0 through 4) based on the size of the cysts and the tissue of origin, with Type 1 (large cysts) and Type 2 (smaller cysts) being the most common. The c
How is Congenital pulmonary airway malformation inherited?
Congenital pulmonary airway malformation follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Congenital pulmonary airway malformation typically begin?
Typical onset of Congenital pulmonary airway malformation is neonatal. Age of onset can vary across affected individuals.
Are there clinical trials for Congenital pulmonary airway malformation?
Yes — 2 recruiting clinical trials are currently listed for Congenital pulmonary airway malformation on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Congenital pulmonary airway malformation?
25 specialists and care centers treating Congenital pulmonary airway malformation are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.