Pediatric Congenital Pulmonary Airway Malformation (CPAM)
Congenital pulmonary airway malformations (CPAM) are lesions on the lung that are present at birth. CPAM can cause physical or developmental problems.
CPAM is a rare, genetic condition (present at birth). Babies with congenital pulmonary airway malformations are born with benign (non-cancerous) lumps, lesions or cysts (fluid-filled sacs) on one of their lungs. Depending on the size and number of the lesions, it can cause physical and developmental symptoms.
Previously known as congenital cystic adenomatoid malformation (CCAM), this condition tends to affect males more than females. CPAM typically occurs in only one lung, or part of one lung.
The cause of CPAM is unknown. Research does show that sometime during the development of the fetus in the mother's womb, the lung tissue will form abnormally, and the baby will be born with a lump or cyst.
CPAM is separated into three types that are defined by the number of cysts present:
- Type I lesions – Occurs when there are one to four large cysts present in the lung(s). Outcomes are favorable for this type, and it makes up about 50 percent of the cases
- Type II lesions – Occurs when numerous cysts are present in the lung that are smaller in size. This type is associated with other problems, like cardiac, genitourinary (genital/urinary), hydrocephalus (fluid on the brain), and skeletal issues, that will affect quality of life. This type typically makes up about 40 percent of cases
- Type III lesions – Occurs when larges masses on the lung(s) cause other organs to shift (mediastinal shift), much like pulmonary sequestration. The masses may impact heart, windpipe and lung capabilities. This occurs in about 10 percent of cases.
Symptoms will vary between children and may not become apparent until they are adults. Some physical and developmental signs include the following, which can also lead to further complications:
- Accessory muscle use – heavy chest and shoulder movements, as a person struggles to breathe
- Chest pain or pressure
- Collapsed lungs
- Coughing up blood or mucus
- Cyanosis – blue/gray tint to lips or fingernails
- Failure to thrive
- Feeding difficulties
- Respiratory distress
- Respiratory infections – chronic (reoccurring) infections like bronchitis, colds or pneumonia