Overview
Congenital pulmonary airway malformation type 1 (CPAM type 1), also known as congenital cystic adenomatoid malformation type 1 (CCAM type 1), is a rare lung condition that develops before birth. It occurs when a section of the lung does not form properly during fetal development, resulting in one or more large fluid-filled or air-filled cysts (sacs) in the lung tissue. Type 1 is the most common form of CPAM, making up about 60-70% of all cases. The cysts in type 1 are typically large, usually between 2 and 10 centimeters in diameter. This condition affects how the lungs work because the abnormal cystic tissue takes up space where normal, functioning lung tissue should be. In many cases, CPAM type 1 is detected during a routine prenatal ultrasound. After birth, some babies may have breathing difficulties, recurrent lung infections, or respiratory distress, while others may have no symptoms at all initially. The severity depends on the size and location of the cysts and whether they compress surrounding healthy lung tissue. The main treatment for CPAM type 1 is surgical removal of the affected portion of the lung, a procedure called lobectomy or segmentectomy. Surgery is generally recommended even in patients without symptoms because of the risk of recurrent infections and a very small but possible risk of malignant transformation later in life. The prognosis after surgery is generally excellent, with most children going on to live normal, healthy lives. In rare cases where the malformation is very large, it may cause complications before or shortly after birth that require urgent intervention.
Also known as:
Key symptoms:
Breathing difficulty at birth or in infancyRapid breathingBluish skin color due to low oxygenRecurrent lung infections or pneumoniaCough that keeps coming backChest tightness or discomfortFeeding difficulties in newbornsFailure to thrive or poor weight gainAbnormal findings on prenatal ultrasoundAir trapping in the affected lung areaChest asymmetry in severe casesNo symptoms at all in some cases (found incidentally)
Sporadic
Usually appears on its own, not inherited from a parent
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for Congenital pulmonary airway malformation type 1.
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Specialists
View all specialists →No specialists are currently listed for Congenital pulmonary airway malformation type 1.
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 1.
Community
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
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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.
Questions for your doctor
Bring these to your next appointment
- Q1.How large is the malformation and where exactly is it located in my child's lung?,When is the best time to have surgery, and what type of surgery do you recommend?,What are the risks of surgery versus the risks of waiting or not having surgery?,Will my child's lung function be normal after surgery?,What is the risk of the cyst coming back or developing into something more serious?,What follow-up tests and appointments will be needed after surgery?,Are there any activity restrictions my child will need to follow after recovery?
Common questions about Congenital pulmonary airway malformation type 1
What is Congenital pulmonary airway malformation type 1?
Congenital pulmonary airway malformation type 1 (CPAM type 1), also known as congenital cystic adenomatoid malformation type 1 (CCAM type 1), is a rare lung condition that develops before birth. It occurs when a section of the lung does not form properly during fetal development, resulting in one or more large fluid-filled or air-filled cysts (sacs) in the lung tissue. Type 1 is the most common form of CPAM, making up about 60-70% of all cases. The cysts in type 1 are typically large, usually between 2 and 10 centimeters in diameter. This condition affects how the lungs work because the abnor
How is Congenital pulmonary airway malformation type 1 inherited?
Congenital pulmonary airway malformation type 1 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 1 typically begin?
Typical onset of Congenital pulmonary airway malformation type 1 is neonatal. Age of onset can vary across affected individuals.