Overview
Congenital pulmonary airway malformation type 3 (CPAM type 3), also known as congenital cystic adenomatoid malformation type 3 (CCAM type 3), is a rare lung condition that is present at birth. In this condition, a portion of the lung develops abnormally during pregnancy, forming a mass of solid-appearing tissue made up of very small cysts (tiny fluid or air-filled spaces) that are usually less than 0.5 centimeters in size. Unlike other types of CPAM where the cysts are larger, type 3 tends to involve an entire lobe of the lung and appears more solid on imaging. This can crowd out normal lung tissue and affect the baby's ability to breathe properly. Babies with CPAM type 3 may present with breathing difficulties at birth or shortly after, including rapid breathing, low oxygen levels, and respiratory distress. In severe cases detected before birth, the large mass can shift the heart and other structures in the chest (called mediastinal shift) and may even cause fluid buildup around the baby (fetal hydrops), which is a serious complication. Prenatal ultrasound often detects this condition during routine pregnancy scans. The main treatment for CPAM type 3 is surgical removal of the affected portion of the lung (lobectomy). In some severe prenatal cases, interventions before birth may be considered. After successful surgery, many children do well and can lead relatively normal lives, though long-term follow-up with lung specialists is important. CPAM type 3 is considered the rarest and often the most severe of the CPAM subtypes.
Also known as:
Key symptoms:
Difficulty breathing at birthRapid breathing (tachypnea)Low oxygen levelsBluish skin color (cyanosis)Chest appears larger on one sideHeart and chest organs shifted to one sideFluid buildup around the baby before birth (fetal hydrops)Feeding difficultiesFailure to thriveRecurrent lung infectionsRespiratory distress requiring oxygen supportAbnormal lung mass seen on imaging
Sporadic
Usually appears on its own, not inherited from a parent
Neonatal
Begins at or shortly after birth (first 4 weeks)
FDA & Trial Timeline
1 eventData sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Congenital pulmonary airway malformation type 3.
1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Specialists
View all specialists →No specialists are currently listed for Congenital pulmonary airway malformation type 3.
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 type 3.
Community
No community posts yet. Be the first to share your experience with Congenital pulmonary airway malformation type 3.
Start the conversation →Latest news about Congenital pulmonary airway malformation type 3
No recent news articles for Congenital pulmonary airway malformation type 3.
Follow this condition to be notified when news becomes available.
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
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.
Questions for your doctor
Bring these to your next appointment
- Q1.How large is the lung mass and how much of the lung is affected?,When is the best time to perform surgery, and what type of surgery is recommended?,What are the risks of surgery for my baby?,Will my child's remaining lung tissue be enough for normal breathing and activity?,How often will my child need follow-up imaging and lung function tests?,Is there any risk of the abnormal tissue becoming cancerous later in life?,Are there any activity restrictions my child will need to follow as they grow up?
Common questions about Congenital pulmonary airway malformation type 3
What is Congenital pulmonary airway malformation type 3?
Congenital pulmonary airway malformation type 3 (CPAM type 3), also known as congenital cystic adenomatoid malformation type 3 (CCAM type 3), is a rare lung condition that is present at birth. In this condition, a portion of the lung develops abnormally during pregnancy, forming a mass of solid-appearing tissue made up of very small cysts (tiny fluid or air-filled spaces) that are usually less than 0.5 centimeters in size. Unlike other types of CPAM where the cysts are larger, type 3 tends to involve an entire lobe of the lung and appears more solid on imaging. This can crowd out normal lung t
How is Congenital pulmonary airway malformation type 3 inherited?
Congenital pulmonary airway malformation type 3 follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Congenital pulmonary airway malformation type 3 typically begin?
Typical onset of Congenital pulmonary airway malformation type 3 is neonatal. Age of onset can vary across affected individuals.
Are there clinical trials for Congenital pulmonary airway malformation type 3?
Yes — 1 recruiting clinical trial is currently listed for Congenital pulmonary airway malformation type 3 on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.